DRUGS & CONDITIONS

WHAT IS CYSTIC FIBROSIS?

Topics:

CF (cystic fibrosis) is one of the most common diseases that is passed from one generation to the next. Like color blindness being passed from a mother to a son, people are born with CF. Sometimes parents worry that they did something before or during pregnancy that made their child get CF. This is never the case.

Babies, children, and young adults all can have CF. It is a life-long disease that gets worse over time.

The Main Problem: Thick, Sticky Mucus

The exocrine glands which make sweat, mucus, tears, saliva, and digestive juices change because of CF. Mucus is normally thin, watery, and slippery. It is released through small tubes or ducts. Mucus keeps the inside linings of some organs moist. This helps protect the organs from infection. But CF does not allow the body to make normal mucus. Instead, CF causes the endocrine glands to make thick, sticky mucus. This thick, sticky mucus is the main problem in CF. It clogs the exocrine tubes, which causes many other problems.

How CF Affects the Body

Thick, sticky mucus clogs the bronchial tubes in the lungs. This makes it harder to breathe. The mucus also traps bacteria in the lungs, which can lead to infection that damages the lungs.

Thick, sticky mucus also blocks digestive enzymes from reaching the food in the intestines. Without enzymes, food cannot be digested well. The lack of enzymes makes it very hard for people with CF to get the right chemicals from food needed for their body to work. As a result, people with CF may be poorly nourished, have trouble gaining weight, and may not grow as they should.

People with CF may have problems with inflamed sinuses (sinusitis), nasal polyps, gallstones, liver disease, brittle bone disease, or a special form of diabetes. CF also affects the chances of men and women becoming parents. CF even affects the sweat glands. People with CF make sweat that has too much salt.

References

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Cystic Fibrosis Foundation Web site. www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. www.cff.org/AboutCF/ Accessed May 13, 2008.

National Heart Lung and Blood Institute Web site. www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008.

National Center for Biotechnical Information Web site. www.ncbi.nlm.nih.gov/books/bv.fcgi?highlight=&rid=gnd.section.242 Accessed May 13, 2008.

National Library of Medicine Web site.www.nlm.nih.gov/changingthefaceofmedicine/physicians/biography_8.html Accessed May 14, 2008.

Cystic Fibrosis Foundation Web site. www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHO GETS CYSTIC FIBROSIS?

 

CF (cystic fibrosis) is a disease that is passed from one generation to another. It is found in all races and ethnic groups. It affects about the same number of males and females. Around the world, white people (Caucasians) with ancestors from northern Europe are most likely to be born with the genes that cause CF.

 

In the U.S., about 1,000 people are diagnosed with CF each year. Right now, about 30,000 Americans have CF. CF is found in all parts of the world, but it occurs more often in some countries than others. In the United States, about one in 3,700 babies is born with CF. The number of babies born with CF varies widely among our country's ethnic groups.

Ethnic Group Born with CF
Non-Hispanic white Americans: About 1 in 3,200
Hispanic Americans: About 1 in 7,000
African Americans: About 1 in 17,000
Asian Americans: About 1 in 31,000

References

National Heart Lung and Blood Institute Web site. www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55.

Cystic Fibrosis Foundation Web site. www.cff.org/AboutCF/ Accessed May 13, 2008.

European Lung Foundation Web site. www.european-lung-foundation.org/index.php?id=86 Accessed May 15, 2008

Cystic Fibrosis Worldwide Web site. www.cfww.org Accessed May 15, 2008.

U.S. Department of Health and Human Services, Centers for Disease Control and Prevention. Newborn Screening for Cystic Fibrosis: Evaluation of Benefits and Risks and Recommendations for State Newborn Screening Programs. Atlanta, GA: 2004. 53(No. RR-13).

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Genetics Home Reference Web site, U.S. National Library of Medicine, National Institutes of Health. www.ghr.nlm.nih.gov/condition=cysticfibrosis Reviewed January, 2008. Published May 9, 2008. Accessed May 15, 2008.

Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med. 2008 Feb 28;358(9): 973-4.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT CAUSES CYSTIC FIBROSIS?

Topics:

CF (cystic fibrosis) is disease that is passed from parent to child in the genes. Genes contain the directions for making us look the way we do, such as eye color and how tall we will grow. The genes that cause CF are mutated or changed. They do not work the way they are supposed to.

Gene Changes

CF is caused by a change or mutation in the gene that tells your body how to make a protein called CFTR (cystic fibrosis transmembrane conductance regulator). Everyone has two copies of this gene. About 1 out of 31 Americans has one changed CFTR gene. This does not mean that they have CF. It means that they are "carriers" of the mutation. Because CF gene mutations are so common, there is a good chance that two people with mutations will marry. About one in 400 American marriages involves both a husband and a wife who have a mutated CF gene.

There are more than 1,500 mutations that can occur in the CF gene. The most common mutation is Delta F508. It causes more than 7 out of 10 cases of CF. Because there are so many possible mutations, not all people who have them can be found with screening tests. Most tests only look for the most common mutations.

Likelihood of a Baby Being Born with CF

For every trait, you have two genes: one from your mother and one from your father. If you have CF, you received two mutated CF genes: one from each parent. If you inherit a mutated gene from one parent and a normal gene from the other parent you will not have CF.

Everyone with one mutated CF gene is a "carrier" of CF. This means they can pass the mutated CF gene to their children. Carriers do not have CF and they have no CF symptoms. All parents of CF children either have CF themselves or are carriers. Not all children of carriers get CF. Some CF children have brothers or sisters who do not have the disease.

The pictures below show how CF is passed from parents to children.

The capital C stands for the normal CF gene.

The lower case c stands for the mutated CF gene.

A person who does not have CF has either two normal genes (CC), or is a carrier with one normal gene and one mutated gene (Cc).

A person with CF has two mutated genes (cc).

Both Parents Are Carriers

In this example, both parents are carriers (Cc and Cc). Each can pass either a normal gene or a mutated gene to their child. Two carriers have a one-in-four chance of having a child with CF. They have a two-out-of-four chance of having a carrier.

Parents Carriers

Parent with CF and Parent with Normal Genes

In this example, one parent has CF (cc). The other parent has two normal genes (CC). The CF parent can only pass on a mutated gene. The other parent can only pass on a normal gene. This means that all children will receive at least one normal gene, so none will have CF. All children will also receive at least one CF gene, so all will be carriers.

Parent Normal

Parent with CF and Carrier Parent

In the example below, one parent has CF (cc). The other parent is a carrier (Cc). The parent with CF can only pass on a mutated gene. The carrier parent can either pass on a normal gene or a mutated gene.2 There is a 50% chance their child will have CF. Those who do not have CF will be carriers.

Parent CF

Other Possibilities

If both parents have CF (cc), each could only pass a mutated gene on to their children. All of their children (100%) would have CF.

If both parents have only normal genes (CC), none of their children could receive a mutated CF gene. None of their children would have CF and none would be carriers.

The Odds

There is no way to control which genes are passed from parent to child. Giving birth to a child with CF does not change the chances for having another child with CF.

References

Cunningham JC, Taussig LM. An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Cystic Fibrosis Foundation Web site. www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.

Genetics Home Reference Web site, U.S. National Library of Medicine, National Institutes of Health. www.ghr.nlm.nih.gov/condition=cysticfibrosis Reviewed January, 2008. Published May 9, 2008. Accessed May 15, 2008.

National Organization for Rare Disorders, Inc. Web site. www.rarediseases.org/search/rdbdetail_abstract.html?disname=Cystic%20Fibrosis Accessed May 15, 2008.

National Heart Lung and Blood Institute Web site. www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Strausbaugh SD, Davis PB. Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs. Accessed May 13, 2008.

Staton Jr. GW. Chronic Obstructive Diseases of the Lung. In: Holztman MJ, ed. Respiratory Medicine. New York, NY: WebMD Inc.; 2004.

Fallon LF. Cystic Fibrosis. In: Krapp K, ed. Gale Encyclopedia of Nursing and Allied Health. Detroit, MI: Gale; 2002:626-633.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.

Chicago Center for Jewish Genetic Disorders Web site. http://www.jewishgeneticscenter.org/ Accessed May 15, 2008


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT ARE THE SYMPTOMS?

Topics:

CF (Cystic Fibrosis) causes the body's exocrine glands to make thick, sticky mucus. Most CF symptoms are caused by this mucus. Symptoms cause trouble with four main body functions: breathing, digestion, reproduction, and sweating.

Here are the most common symptoms of CF:

Breathing

The CF symptoms related to breathing are:

  • Frequent coughing that sometimes brings up thick mucus
  • Frequent lung infections such as bronchitis or pneumonia
  • Wheezing or shortness of breath
  • Nasal polyps (small growths of flesh in the nose)

The purpose of mucus in the lungs is to capture dust and germs and clear them away. This is how normal mucus helps to keep the lungs healthy. The thick, sticky mucus of CF cannot help keep the lungs healthy. It clogs the bronchial tubes in the lungs. This makes it harder to breathe. The mucus also traps bacteria in the lungs, which can lead to infection. The mucus even allows some types of bacteria to grow that don't usually affect healthy people. When infections occur over and over, in time, the lungs can be damaged. Lung infections are the most serious CF problem.

Digestion

CF affects nearly the whole digestive tract. The most common symptoms are:

  • "Failure to thrive" - poor weight gain and growth, even with a huge appetite
  • Ongoing diarrhea
  • Bulky, greasy, foul-smelling stools
  • Stomach pain due to gas in the intestines
  • Blocked first bowel movement in babies

The pancreas is an organ behind the stomach. It makes enzymes. Enzymes are proteins that the body uses to break down the food we eat. Without enzymes, food cannot be digested well. In CF, the thick, sticky mucus blocks enzymes from reaching the food in the intestines. This makes it very hard for the body to absorb nutrients. As a result, people with CF may be poorly nourished, have trouble gaining weight, and may not grow normally. About 93 out of 100 people with CF have this problem. Far fewer people have mucus that stops bile from leaving the liver. Others have mucus that keeps stool from moving normally.

Meconium is a baby's first bowel movement. It is thick, greenish-black, and sticky. Healthy infants pass this stool within the first 24-48 hours after birth. About 15 out of 100 newborns with CF cannot not pass this stool. This is known as meconium ileus. Ileus means that the baby's intestine is blocked by meconium that is more thick and sticky than normal. Meconium ileus is caused by a lack of pancreatic enzymes. Meconium ileus is usually a sign that the baby has CF. Meconium ileus is relieved with an enema or, in some cases, with surgery.

Reproduction

CF affects the ability of men and women to become parents:

  • Most men are sterile (unable to father a child).
  • Women have very thick, sticky cervical mucus that makes it harder to get pregnant.

Men with CF make normal sperm. But the sperm cannot mix with semen and leave the body because the tube it travels through is blocked by the thick mucus caused by CF. In other words, the semen of most men with CF contains no sperm. This means most men with CF are not able to father children. It is not safe to assume that all men with CF are sterile. In a few men with CF, the sperm is not blocked.

Women with CF can get pregnant. However, it can be harder for a woman with CF to conceive. The problem is that the exocrine glands in the vagina make cervical mucus that is more thick and sticky than normal. It is hard for sperm to get through this mucus barrier to reach the egg.

Another problem is that some women with CF do not have regular periods. They may also ovulate (release eggs from the ovaries) less often than normal. While it can be more difficult for women with CF to get pregnant, it is not impossible – about 100 women with CF get pregnant every year.

Sweat

When parents kiss their child, they may notice a classic sign of CF:

  • Salty-tasting skin

Sweat glands are the only exocrine glands that do not make thick, sticky mucus. Instead, they make sweat that has too much salt. People with CF have much more salt in their sweat than other people do. This is not usually a problem, since most people get plenty of salt from food. It only becomes a problem if too much salt is lost through sweat. The balance of minerals in the blood can change. This can cause:

  • Tiredness
  • Weakness
  • Muscle cramps
  • Vomiting
  • Dehydration (losing too much water from the body)
  • Heat stroke

This is most likely to happen at times when people sweat the most: in hot weather, when exercising, or with a fever. Replacing lost salt is easy: just eat more salty foods and drink extra water.

References

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

National Heart Lung and Blood Institute Web site. www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008.

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.

National Center for Biotechnical Information Web site. http://www.ncbi.nlm.nih.gov/books/bv.fcgi?highlight=&rid=gnd.section.242 Accessed May 13, 2008

Fact Sheet: Respiratory--What You Should Know About Germs. Cystic Fibrosis Foundation Web site. http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/WhatYouShouldKnow/WhatYouShouldKnowAboutGerms%2Epdf Accessed May 13, 2008

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/ Accessed May 13, 2008.

Robertson MB, Choe KA, Joseph PM; Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 2006;26(3):679-690.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

MedLine Plus Medical Encyclopedia [Internet]. Bethesda, MD: National Library of Medicine (US); Meconium. [updated 2008 Apr 28; Cited 2008 May 19]; [about 2p.] Available from http://www.nlm.nih.gov/medlineplus/ency/imagepages/9616.htm

American Academy of Family Physicians Web site. http://www.aafp.org/afp/991101ap/2043.html . Accessed May 19, 2008.

MedicineNet Web site. http://www.medterms.com/script/main/art.asp?articlekey=6372 Accessed May 19, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HOW IS CYSTIC FIBROSIS DIAGNOSED?

Topics:

Most people with CF (cystic fibrosis) are diagnosed in the first three years of life. About 7 out of 10 are diagnosed by their first birthday. Adults may also be diagnosed with CF. They have often had a healthy childhood, and only began to have CF symptoms after they were grown.

Diagnosing CF

It is not always easy to diagnose CF. Other health problems can cause symptoms like those of CF. Symptoms are different from person to and can be mild to severe. For these reasons, CF can sometimes go unnoticed for years.

Diagnosis starts with a review of a person's medical history. Since CF is inherited, the health care team will also review the person's family history. The next step is a physical exam. If the doctor suspects CF, tests are used to confirm it.

Sweat Test

The best diagnostic test for CF is the "sweat test." The sweat test is also known as the quantitative pilocarpine iontophoresis test. It is a simple, painless, and accurate test. It measures the amount of salt in the sweat. It is a very good test for CF because people with CF have much more salt in their sweat than other people do. This test is considered the best test for CF diagnosis.

There are two ways to do a sweat test. Both ways use a chemical called pilocarpine (pi-lo-KAR-pen). A small amount of it is put on the skin of the forearm or the thigh. An electrode is attached to this place. A weak electric current is used to cause sweating. One test method collects sweat on filter paper. The other method collects sweat in a tube. Either way, the test takes about 30 minutes.

If the test shows a high amount of salt in the sweat it is very likely that the person has CF. The test is done twice to double check the results. A few people with CF have normal or borderline sweat test results. Other tests are then used to find out if they have CF. The sweat test may not be the best test to use with newborns. They may not be able to make enough sweat. It is best to give this test when a baby is about two months old.

Other conditions besides CF can cause a positive sweat test result. In addition, sweat samples must be handled carefully to avoid problems. These are good reasons to have a sweat test at a center that has experience with the test. A Cystic Fibrosis Foundation-accredited center is the best place to have this test.

Gene Mutation Test

A gene mutation test is sometimes done if sweat test results were not clear.

Nine times out of 10, a gene mutation test can diagnose CF. This test looks for the 23 most common gene changes that cause CF. Everyone with CF has two mutated CF genes. Those who inherit one mutated gene and one normal gene are carriers. The gene mutation test is the only test that can identify CF carriers, and it is widely used for this purpose.

To do this test, a sample of blood or cheek cells is needed. In infants, blood is taken from the heel. In others, blood is drawn from a vein. To get cheek cells, the inside of the cheek is brushed. This test reveals which mutations have caused a person's CF. Other tests can find more rare mutations, but a person can still have CF even if no mutations are found by a test.

When the test is positive – two gene mutations found – the person has CF. A positive test result cannot predict if CF will be mild or severe. People with the exact same mutations can have different outcomes. When no mutations are found, but a person has CF symptoms, other tests are done.

Nasal Potential Difference (NPD) Test

The NPD test is done by placing an electrode on the lining of the nose. The lining is then covered in a series of liquids that contain different salts. These liquids are always given in the same order during a test. The NPD test is difficult to perform. Only a few places in the U.S. do this test. It must be used along with other tests to diagnose CF.

Newborn Screening

Every state screens newborns for certain disorders. Within 48 hours of birth, a sample of a baby's blood is taken with a "heel stick." This blood sample is tested for treatable diseases, such as sickle cell disease. The CDC (Centers for Disease Control and Prevention) recommends that states include a test to screen for CF. However, not all states include a test for CF yet. The March of Dimes would like all states to do 29 tests, including one for CF. Some states now do as few as four tests. Your doctor can tell you which tests are done in your state.

Most states that screen for CF do so with a test for IRT – immunoreactive trypsin. IRT is a pancreatic enzyme. If a baby has CF there will be a lot of IRT in the blood. The IRT test is always followed by other tests. A test for IRT alone cannot confirm CF.

In some states, if IRT is high in two tests, then a sweat test is done. In other states, if IRT is high, the baby is also tested for gene mutations. If one or two CF mutations are found, then a sweat test is done. In any state, a test for IRT can be done if a newborn has meconium ileus. (To learn more about meconium ileus read the Accordant Web article, What are the Symptoms of Cystic Fibrosis?)

References

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Nick JA, Rodman DM. Manifestations of cystic fibrosis in adulthood. Curr Opin Pulm Med. 2007;11:513-518.

National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/diag_testsweat.html Accessed May 19, 2008.

Lab Tests Online Web site. http://www.labtestsonline.org/understanding/analytes/sweat_chloride/glance.html Accessed May 19, 2008.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Lab Tests Online Web site. http://www.labtestsonline.org/understanding/analytes/cf_gene/glance.html Accessed May 19, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/diag_testnasal.html Accessed May 19, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/diag_testnewborn.html Accessed May 19, 2008.

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55. March of Dimes Web site.

http://www.marchofdimes.com/professionals/14332_1200.asp. Accessed May 20, 2008. Lab Tests Online Web site.

http://www.labtestsonline.org/understanding/analytes/trypsinogen/glance.html. Accessed May 19, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT IS THE TREATMENT FOR CYSTIC FIBROSIS?

Topics:

There is no cure for CF (cystic fibrosis), so treatments focus on the symptoms of the disease. The goals of the treatments are to:

  • Keep your lungs from getting infected, or control infection if it occurs
  • Clear as much mucus from your lungs as possible
  • Improve your breathing
  • Prevent blockages in your intestines
  • Make sure you are getting enough calories and nutrients

Today, there are more treatments for CF than ever before. Everyone with CF does not need the same treatments, so each person has their own treatment plan.

Treatments for Lung Problems

When mucus builds up in the lungs it blocks the airways and makes it hard to breathe. The extra mucus also makes it easy for bacteria to grow and cause a lung infection.

There are five main treatments for CF lung problems:

1. Airway Clearance Techniques

Airway clearance techniques (ACTs) loosen mucus and clear it from the lungs. ACTs are very important daily treatments. They help reduce lung infections and make it easier to breathe. There are many ACTs and most of them are easy to do. Some ACTs include coughing, deep breathing, or vibrating the chest by hand. Other ACTs involve wearing a special vibrating vest or blowing into a special device. A family member, friend, nurse, or therapist can perform airway clearance for small children. Older children and adults may not need any help.

2. Antibiotics

Most people with CF have mild, ongoing lung infections. Sometimes a lung infection can become serious. Antibiotics are medicines that fight the bacteria that cause lung infections. Bacteria can become resistant to some antibiotics. To avoid this, your doctor may sometimes change your antibiotic.

For mild infections, antibiotic pills, capsules, or liquids are taken by mouth. For serious infections, IV (put into a vein) antibiotics are given. IV antibiotics are often given if antibiotics taken by mouth do not work. Antibiotics can also be given in an aerosol mist that you inhale. One aerosol antibiotic, TOBI® (tobramycin) is used to fight the bacteria that cause most ongoing lung infections. Aerosol antibiotics are sometimes given along with antibiotics taken by mouth.

Some people need to take antibiotics every day. Others only need them from time to time. Your doctor decides what kind of antibiotic you need, and how often you need it, based on your sputum culture results. A sputum culture is a test that checks for infection-causing germs in your mucus.

3. Other Aerosol Medicines

There are two other kinds of inhaled treatments for CF besides aerosol antibiotics:

  • Bronchodilators. Bronchodilators are used by a lot of people with CF. They open up the airways. When inhaled just before an ACT, a bronchodilator helps move mucus. Some bronchodilators are albuterol and terbutaline.
  • Mucus-thinners. These treatments, called mucolytics, make mucus thin and easier to cough out. One medicine, Pulmozyme® (dornase alfa), is often used for those age 6 or older with mild, moderate, or severe lung disease. It helps the lungs work better.

Hypertonic saline is a different mucus thinner. It is a mist of very salty water that helps the lungs work better. It is used after a bronchodialator, to help move mucus out of the lungs.

4. Anti-Inflammatory Medicines

Anti-inflammatory medicines reduce the inflammation (swelling) of the lung tissues. Reducing lung inflammation helps prevent damage to the lungs. Ibuprophen, a non-steroid anti-inflammatory (NSAID) medicine, is often used. It may also slow the progress of CF in young children with mild symptoms. Those who take ibuprophen for CF must be closely monitored by a doctor. Corticosteroids are anti-inflammatory medicines that are used to treat other diseases, but experts are not sure yet they should be used to treat CF.

5. Exercise

Most everyone with CF should exercise. Exercise forces you to breathe deeply and may even help expand your airways. A physical therapist can design an exercise program for you based on the severity of your CF, your fitness level, and the activities you enjoy. A good exercise program includes two types of activities:

  • Endurance exercise such as swimming, running, cycling, or aerobics classes
  • Strength-building activity such as weight training

Treatments for Digestive Problems

In CF, mucus blocks the tube that takes enzymes from the pancreas to the small intestine. Without enzymes, food cannot be digested well. More than 9 out of 10 people with CF have trouble digesting fats and proteins. This makes it hard to gain weight and has a serious impact on health. The good news is that the digestive problems of CF can be treated.

Three main treatments are used for most people:

1. Nutrition

Good nutrition is needed for good health. If you have CF, you need more calories than other people. This is because your body has trouble getting nutrients from food, and also because it takes more energy for you to breathe. There are no restrictions on diet for adults or children with CF. The best diet for anyone with CF is a high fat, high protein, high calorie diet. Both adults and children with CF should eat high-calorie foods and high-calorie snacks such as nuts and milkshakes.

2. Pancreatic Enzymes

Most people with CF need to take pancreatic enzyme capsules whenever they eat. These enzymes replace the natural ones that are blocked by mucus in the pancreas. Enzymes must be taken with all meals and snacks. Capsules are taken at the beginning and also at the end of each meal. Babies with CF need enzymes to help them digest breast milk or formula. For babies, the capsules are opened and the enzymes are sprinkled into a soft acidic food like applesauce. Older children and adults just swallow them with liquid.

Taking enzymes helps you digest fats, proteins, and carbohydrates, which are the body's main sources of calories. People who take enzymes have an easier time gaining weight.

3. Vitamins

Vitamins A, D, E, and K are the fat-soluble vitamins. This means they dissolve in fat, not in water as the other vitamins do. Vitamins A, D, E, and K are absorbed in your small intestines along with the fat you eat. If you are like most people with CF, your body does not digest fat well and you do not get enough fat-soluble vitamins. Your doctor can prescribe water-soluble forms of these vitamins for you to take. Most people take them twice a day. Taking enzymes will help you absorb more vitamins. Those who take a lot of antibiotics may need extra vitamin K.

References

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008.

Mayo Clinic Web site. http://www.mayoclinic.com/health/cystic-fibrosis/DS00287 Accessed August 30, 2007.

Cystic Fibrosis Foundation Web site. http://www.cff.org:80/treatments/Therapies/#Clearing_the_Airways Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Therapies/Respiratory/AirwayClearance/ Accessed September 1, 2007.

Cystic Fibrosis Foundation Web site. http://www.cff.org/UploadedFiles/treatments/Therapies/Respiratory/PosturalDrainage/An%20Introduction%20to%20Postural%20Drainage%20and%20Percussion%201-2006.pdf Accessed September 1, 2007.

Lannefors L, Button BM, Mcllwaine M. Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments. J R Soc Med. 2004;97(44):8-25.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest. 2006;129(1):250S-259S.

Marks JH. Airway clearance devices in cystic fibrosis. Paediatr Respir Rev. 2007;8(1):17-23.

Pulmozyme [package insert]. South San Francisco, CA: Genentech, Inc.; April 2005.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Therapies/Respiratory/HypertonicSaline/ Accessed May 13, 2008.

Elkins MR, Robinson M, Rose BR, et al; for the National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group. A controlled trial of long-term inhaled hypertonic saline. N Engl J Med. 2006;354(3):229-240.

Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176(10):957-969.

Gumery L, Dodd M, Parker A, Prasad A, Pryor J, Kennedy N, eds. Clinical Guidelines for the Physiotherapy Management of Cystic Fibrosis. Bromley, United Kingdom: Cystic Fibrosis Trust; 2002.

Milla CE. Nutrition and lung disease in cystic fibrosis. Clin Chest Med. 2007;28(2):319-330.

Costa M, Potvin S, Berthiaume Y, et al. Diabetes: a major comorbidity of cystic fibrosis. Diabetes Metab. 2005;31(3):221-232.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT CAN I EXPECT?

Researchers are working hard to find a cure for CF (cystic fibrosis). There has been a lot of progress in recent years. Thanks to new medicines and better treatments, people with CF now live longer and enjoy a better quality of life than ever before.

Children and teens with CF can be just as active in sports and school as their friends. Except for scuba diving, there are no sports that are off limits for people with CF. Nine out of 10 CF teens graduate from high school. Many graduate from college too. As they enter their adult years they have the same job opportunities as others. About one third people with CF get married, and some go on to become parents.

In 1955, most children with CF did not live long enough to enter the first grade. Today, medical advances allow people with CF to live longer than ever before – about 37 years. Babies born today with CF are expected to live into their 40s, 50's, or beyond. Promising new treatments, and perhaps a cure, may not be too far off. At this time, however, CF is still a life-shortening disease. Adults with CF have to cope with progressive lung damage. For some, lung transplants are a life-extending option but not a cure for CF.

References

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Testing/NewbornScreening/ Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/ Accessed May 13, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/ Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Pipeline/ Accessed May 13, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

LUNG PROBLEMS

Topics:

At birth, babies with CF have nearly normal lungs. But as time goes on, almost all people with CF will have lung problems. Lung problems are the most serious part of the illness. For some people, lung problems become very bad. Others have milder sickness and less problems. Lung health is often the main factor that decides how well someone does with CF and how long they live. This is why taking medicines and doing daily airway clearance are so important for everyone with CF.

How Normal Lungs Work

If you could look inside your body, you might think your lungs and airways look like an upside down tree. When you breathe in, air enters a tube in your throat called the trachea. This is also called the windpipe. The windpipe has two branches called bronchial tubes. One goes into each lung. From there, the tubes get smaller and smaller. The bronchial tubes branch into smaller tubes called bronchioles. The bronchioles end in millions of tiny air sacs. The tiny air sacs are like little balloons that fill up with air when you take a breath. They flatten when you breathe out. In the tiny air sacs, a trade of gases takes place. Oxygen enters the body when you breathe in, and CO2 is removed when you breathe out.

Your right lung has three parts called lobes: upper, middle, and lower. The left lung has two lobes: upper and lower. All the lobes have bronchioles and tiny air sacs in them. In healthy lungs, the bronchioles and tiny air sacs are lined with thin mucus. The mucus gives moisture. It also protects health by trapping germs, dust, and pollen that are breathed in. After it traps them, the mucus is swept toward the throat by tiny hair-like cleaners called cilia. When the mucus gets to the throat, it is coughed out or swallowed.

How CF Affects the Lungs

In CF, the mucus in the lungs is not thin and watery. Instead, it is thick and sticky. It is so thick and sticky that the cilia cannot move it well. So, instead of keeping the lungs healthy, the mucus builds up and clogs the airways. This causes two problems. One problem is that the mucus blocks the gas trade process in the tiny air sacs. This makes it harder to breathe. The other problem is that germs cannot leave the body because they are trapped in the mucus. Unfortunately, mucus is a good food for germs. The germs feast on the mucus. They grow in number until they cause a lung infection.

The Infection-Swelling Cycle

It is easy to see why lung infections are the most common problem that happens with CF. But infection is just the beginning of an even bigger problem. The body's immune system defends us from invaders like germs. When the immune system knows that there are germs in the lungs, it sends out an army of white blood cells to attack them. The white blood cells let loose enzymes to kill the germs. The enzymes also hurt the lung tissue. This causes the lung tissue to become inflamed. The lung tissue swells. This causes more mucus to be made. The new mucus gives even more food for the germs. The body sends out more white blood cells to attack the germs, and a harmful cycle has begun.

This cycle of infection, swelling, and more mucus hurts and scars the lungs. As a result, they do not work as well. The cycle can be slowed with drugs that kill germs or slow their growth, mucus thinning medicines, and airway clearance treatments. But the lung damage caused by the cycle stays.

Bronchiectasis

Most of the lung harm from the infection-swelling cycle happens in the bronchioles. The bronchioles are small airways that are already likely to be clogged with mucus. With ongoing swelling, the bronchioles get stretched out of shape. This is a lasting change called bronchiectasis (brahn-ke-ek-ta-ses). The stretched out places fill up with pus and infection. This affects breathing and causes a mucus-making cough.

Lung Function Tests

There are many treatments for the lung problems of CF. To decide which treatments are needed, doctors must first know how well the lungs are working. Four kinds of tests may be used:

  • Chest x-rays and CT scans give an "inside" look at the lungs.
  • Pulmonary function tests measure how fast air gets in and out of the lungs.
  • Sputum (mucus) cultures show the type of germs in the lungs. (Mucus is placed in a special dish where germs can grow.)
  • Blood tests check for infection or side effects from medicines.

Other Lung Problems that May Happen

Some people with CF have these bad side effects because of their lung disease:

Pneumothorax (new-mo-THOR-aks) means "air in the chest." For people with CF it means that there is air in the space between the lungs and the chest. A pneumothorax happens when tiny air sacs in the lung burst. Air leaks out of the lung. If the leak is small, it may get better on its own. If the leak is big, it will put pressure on the lung, which may cause the lung to flatten. If this happens, the person will feel a sharp, sudden pain in the chest and/or shoulder and become short of breath. A person with these signs should go to a hospital right away. This is a very serious problem that needs urgent medical help. Up to 25 out of 100 people with CF will have a pneumothorax. For half of these people, it will happen more than one time.

Hemoptysis (he-MOP-tis-is) means coughing up blood from the lungs. It happens because infection and swelling change the blood vessels in the lungs. Hemoptysis can be the result of bronchiectasis. Blood vessels in the stretched out parts on the bronchioles may rupture during an infection.

Hemoptysis can be minor or major:

  • Minor hemoptysis is very common – about 75 out of 100 adults with CF go through it. The amount of blood can be a few streaks in the mucus or as much as a few teaspoonfuls in a day's time.
  • Major hemoptysis does not happen often. It means coughing up as much blood as a soda can would hold. If this happens, urgent medical care is needed.

References

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html Accessed May 13, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/science_lung.html Accessed June 3, 2008.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/prevent_infec.aspx Accessed June 6, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_lung.html Accessed June 3, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Therapies/Respiratory/AirwayClearance/ Accessed May 13, 2008.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/howlungswork.aspx. Accessed June 6, 2008.

Fact Sheet: Respiratory--What You Should Know About Germs. Cystic Fibrosis Foundation Web site. http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/WhatYouShouldKnowAboutGerms.pdf Accessed May 13, 2008.

Cystic Fibrosis Medicine Web site. http://www.cysticfibrosismedicine.com/htmldocs/CFText/eradpseu.htm Accessed May 14, 2008.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/pneumothorax-hemop.aspx Accessed June 6, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

AIRWAY CLEARANCE TECHNIQUES

Airway clearance techniques (ACTs) loosen thick, sticky mucus so it can be cleared from the lungs. ACTs help the lungs to work better. If you have CF, ACTs can help you stay healthy and breathe easier. ACTs should begin when CF is diagnosed and should go on for the rest of life.

Most ACTs are easy to do. Here are the daily ACTs most often used by those with CF:

Coughing and Huffing

The most basic ACT is a natural one--coughing. Coughing is a reflex that moves mucus from the lungs in a forceful way. People with CF cough much more than other people. Most healthy people cough less than 16 times a day. People with CF cough an average of 643 times a day. Some cough as often as 1,569 times a day. Coughing so much can be tiring!

After an ACT, a person must cough to clear their airway. Many people use a huff cough after an ACT. Huffing is a gentle, less tiring way to cough. Huffing is done by tightening the stomach muscles, taking a deep breath, and then pushing air out fast, with the mouth open. Coughing this way has been compared to what we do when we "huff" on a mirror to fog it up.

Chest Physical Therapy (CPT)

Chest physical therapy (CPT) is the standard way to clear the airways. CPT is also called chest PT or PD & P (postural drainage and percussion). All people with CF can benefit from CPT. CPT most often calls for the help of some other person. Some adults can do it alone with the help of a percussion tool.

CPT clears the airways by mixing five techniques:

  • Postural drainage uses gravity to pull mucus from smaller airways to larger ones where it can be coughed out. During CPT, a person lies or sits in different positions that let gravity drain the mucus as tapping and vibration break it loose. Up to 12 different positions may be used.
  • Percussion is done by some other person. He or she uses a cupped hand to pound the front, back, and sides of the chest in a steady way. This does not hurt because the cupped hand keeps a cushion of air under it. Percussion loosens mucus.
  • Vibration is done with a flat hand. The caregiver uses a vibrating motion on the chest. This shakes mucus from the walls of the lungs.
  • Deep breathing comes next. The person breathes deep enough to move the belly in and out. This deep breathing moves the loose mucus. It may cause coughing.
  • Coughing is the final step. It removes the mucus and clears the airway. This is called a "productive" cough.

If a drug to open airways has been prescribed, it should be used before CPT. If an aerosol antibiotic has been prescribed, it should be used after CPT to treat the opened airways.

CPT takes about 20 to 40 minutes. It should be done before meals or an hour and a half to two hours after a meal. Many people find that the best times to do this ACT are in the early morning and again before bed. During illness, CPT may need to be done more than twice a day.

Devices

Some ACTs use special devices. These ACTs can be done as well as, or instead of, CPT. They work as well as CPT and do not call for the help of some other person.

  • PEP (positive expiratory pressure). PEP gets air into the lungs and behind the mucus. A person breathes into a mask or mouthpiece that is hooked up to a resistor. The resistor creates pressure that holds the airways open. This lets air get behind the mucus. The person breathes in normally and breathes out harder against the resistance. After doing this many times, the person coughs or huffs. A PEP tool does not cost very much and it is portable.
  • Oscillating PEP (oscillating positive expiratory pressure). This ACT uses a hand-held tool that vibrates the small and large airways. The person blows all the way out through the device. He or she then coughs or huffs, and repeats the cycle many times. Some oscillating PEP devices are: Flutter®, Comet™, Acapella,™ and IPV (Intrapulmonary Percussive Ventilation). These devices are portable and do not cost much.
  • High-frequency Chest Compression (HFCC). In HFCC, a person wears an inflatable vest that is attached to a machine. The machine sends air pulses through the vest to make it vibrate. This loosens and thins the mucus in the airways. This ACT takes about 15-20 minutes. Every five minutes, the person stops the machine and coughs or huffs. Before doing HFCC, people often breath in medicine to open the airways. Most people do HFCC twice a day. It can be done while watching TV or using a computer. A vest costs more than other ACTs and it is not portable. Some HFCCs are: The Vest™, inCourage™, and MedPulse®.

Breathing Techniques

Two types of breathing techniques are used to clear the airways:

  • Active Cycle of Breathing Therapy (ACBT) ACBT moves mucus by getting air behind it. The breathing can be done in ways that meet each person's needs. There are three parts:
    • Controlled breathing. (Breathing gently with the lower chest while the upper chest is relaxed.)
    • Thoracic expansion exercises. (Deep breathing to open up the chest. This can include self-percussion on the chest. Some other person could also help with this.)
    • Forced expiration. (Huffing with breathing control.)
  • Autogenic Drainage (AD) Autogenic means self-drainage. AD is a way to control airflow so that the air moves mucus. With AD, a person breathes three different ways. This works to move mucus from small to large airways. The three parts of are AD are:
    • Light breathing to loosen mucus
    • Medium breathing to collect mucus
    • Heavy breathing to clear mucus

People must be trained to do AD. It can be hard to learn. It is best for those over age 8 who focus well. If you want to try AD, your doctor may suggest using PEP or oscillating PEP along with it.

Words to Know
Word How to Say it What it Means
Expiratory ek-SPI-ra-tor-e Related to breathing out air from the lungs
Oscillating AH-sil-ate-ing Moving back and forth; vibrating
Percussion per-KUH-shun Tapping on a body part. In airway clearance, the technique of pounding on the chest with a cupped hand
Portable POR-ta-bul Can be carried or moved
Postural PAHS-chur The position of the body
Technique tek-NEK A method for doing something

References

Cystic Fibrosis Foundation Web site. Accessed September 1, 2007.

Cystic Fibrosis Foundation Web site. Accessed May 13, 2008.

Consumer Fact Sheet: An Introduction to Postural Drainage and Percussion. Cystic Fibrosis Foundation Web site Accessed May 13, 2008.

Lannefors L, Button BM, Mcllwaine M. Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments. J R Soc Med. 2004;97(44):8-25.

Accordant Drug and Technology Guideline Cystic Fibrosis: High Frequency Chest Compression (HFCC) ("The Vest"). Updated March 18, 2008.

Cystic Fibrosis Foundation Web site. Accessed May 13, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Accordant Drug and Technology Guideline Cystic Fibrosis: Chest Physiotherapy (CPT). Updated March 18, 2008.

Cystic Fibrosis Education Web site. Accessed June 6, 2008.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Gumery L, Dodd M, Parker A, Prasad A, Pryor J, Kennedy N, eds. Clinical Guidelines for the Physiotherapy Management of Cystic Fibrosis. Bromley, United Kingdom: Cystic Fibrosis Trust; 2002.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

LUNG INFECTIONS IN CYSTIC FIBROSIS

Topics:

Lung infections are the most common problem for people with CF. The thick, sticky mucus in their lungs traps germs. This leads to infection.

If you have an ongoing infection that rarely goes away, this is called a chronic infection. Long-term infections can have mild signs. If your long-term infection all of the sudden gets much worse, it has become an acute pulmonary exacerbation.

Pulmonary Exacerbation

Some common signs of an exacerbation are:

  • More mucus
  • More coughing
  • Harder to exercise
  • Less hunger
  • Weight loss
  • Change in breathing
  • Coughing up blood (hemoptysis)
  • Low fever
  • Feeling tired

These signs result from the increasing infection and the swelling it causes. The main treatments for an exacerbation are antibiotics and doing extra airway clearance therapy. Some people are treated at a hospital and given IV (into a vein) antibiotics. Many people can now get IV antibiotics at home. People have more pulmonary exacerbations as they get older.

Breathing during an exacerbation takes much more energy than normal. This is why eating well is very important during an exacerbation.

If you notice any signs of an exacerbation, get in touch with your doctor right away. Getting early treatment can keep the infection from getting worse.

Germs

While causes such as air pollution can play a role, pulmonary exacerbations are most often caused by germs. Most often, bacteria are the germs to blame. If an infection does not get better with antibiotic treatment, some other kind of germ, such as a virus or mold, may be involved. A sample of your mucus will be taken to a lab for testing. The test results will tell your doctor what kind of germs you have and what kind of treatment you need.

Research has shown that the type of bacteria found in the lungs often follows a pattern. The most common bacteria change with age.

Age Group Common Bacteria
Babies Haemophilus influenzae.
Young children Staphylococcus aureus
Late teens and older Pseudomonas aeruginosa
Adults
  • Burkholderia cepacia (B. cepacia)
  • Stenotrophomonas maltophilia
  • Achromobacter xylosoxidans

Hard-to-treat Bacteria

Some bacteria are very hard to treat. This is because bacteria can change if they are exposed to antibiotics over and over. If bacteria change, some antibiotics can no longer kill or weaken them. When this happens, the bacteria are called antibiotic resistant. If bacteria become resistant to more than one main type of antibiotic, they are called multi-drug resistant. Three kinds of bacteria that can be hard to treat due to antibiotic resistance are: Pseudomonas aeruginosa, Staphylococcus aureus, and Burkholderia cepacia (B. cepacia).

One way to help keep bacteria from getting antibiotic resistant is to take antibiotic medicines the right way. This means taking them just the way your doctor orders, and finishing all of your medicine, even if you start feeling better.

If you get antibiotic resistant bacteria in your lungs it may not be possible to fully get rid of them. You can catch antibiotic resistant bacteria from others with CF. If you already have antibiotic resistant bacteria in your lungs, you can spread your infection to others.

Preventing the Spread of Germs

Germs are spread in three ways:

  • Contact. Most germs are spread this way. Contact can be direct or not direct. Direct contact is when you touch a person in some way – a handshake or a hug, for example. Not direct means touching an object such as a doorknob, or sharing an item such a drinking glass. Cold viruses, Pseudomonas, and B. cepacia are spread by contact.
  • Droplets. When a person laughs, coughs, sings, sneezes, or speaks, tiny droplets of liquid spread through the air. The droplets are too small to see, but they can fall on your eyes, nose, or mouth. If the droplets contain germs, you can get sick. The flu is spread this way.
  • Airborne. Germs can float in the air or land on dust particles. These can stay in the air for a long time. Air currents can carry them a long way. You may breathe them into your lungs, which can make you sick. The aspergillus fungus and the chickenpox virus are spread this way.

Germs are all around. You cannot fully avoid germs, but you can make it harder for infections to start. It is important to do everything you can to keep from catching or spreading germs. Everyone with CF can take these steps:

  • Get vaccinated. Chickenpox, the flu, measles, polio, whooping cough, and many other diseases can be avoided with vaccines. Ask your doctor which shots you and your family members need.
  • Keep your hands clean. You can do this by washing your hands with soap and water, or by using an alcohol-based hand sanitizer. If you see dirt on your hands, wash with soap and water. Other times, use a hand sanitizer if you can. The Cystic Fibrosis Foundation says you should clean your hands:
    • After coughing, sneezing, or blowing your nose
    • Before eating
    • After using the bathroom
    • Before and after breathing treatments or airway clearance
    • Before you take medicine
    • After shaking hands with someone
    • Any time your hands look dirty (use soap and water)
    • After being at a gym or in group play areas
    • After visiting a doctor's office, waiting room, or lab
    • After using common items: pens, handrails, elevator buttons, grocery carts, ATMs (automated teller machines)

    Do not touch your eyes, nose, or mouth. This keeps germs on your hands from getting into your body.

  • Keep your nebulizers and airway clearance tools clean. Everything you use to take inhaled medicines or to do airway clearance must be kept clean. It is also very important to get rid of germs on these items. Follow the manufacturer's advice.
  • Stay at least three feet away from those who are sick or have CF. Do not ride in a car or get into any other small space with those who have CF. If you live with other people who have CF do not share your respiratory or airway clearance tools with them. Do airway clearance in different rooms. In schools, children with CF should not share the same classroom.

Words to Know:

Word How to Say it What it Means
Anorexic An-a-REK-sik Lacking appetite
Exacerbation ig-ZAS-er-ba-shen When symptoms get more severe
Pulmonary PUL-ma-ner-e Related to the lungs

References

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/prevent_infec.aspx. Accessed June 6, 2008.

Ferkol T, Rosenfeld M, Milla CE. Cystic fibrosis pulmonary exacerbations. J Pediatr. 2006;148(2):259-264.

Goss CH, Burns JL. Exacerbations in cystic fibrosis 1: epidemiology and pathogenesis. Thorax. 2007;62(4):360-367.

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Bacteria and Antibiotic Resistance in People with Cystic Fibrosis. University of Southern California. Available at: http://www.cff.org/UploadedFiles/treatments/Therapies/Respiratory/Antibi... Accessed May 13, 2008.

Fact Sheet: Respiratory--What You Should Know About Germs. Cystic Fibrosis Foundation Web site. http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/WhatY.... Accessed May 13, 2008.

Fact Sheet: Respiratory--Stopping the Spread of Germs. Cystic Fibrosis Foundation Web site. http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/Stopp.... Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/StayingHealthy/Germs/MRSA/. Accessed May 13, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

LUNG TRANSPLANTS AND CYSTIC FIBROSIS


Cystic fibrosis is the third most common reason for lung transplants. Every year, between 120 and 150 people with CF have lung transplants. Since 1991, nearly 1,600 people with CF have received transplanted lungs.

Transplants Do Not Cure CF

Most often, people with CF have bilateral (both sides) lung transplants. Since both lungs are damaged by CF, both lungs are replaced. Successful lung transplants let people with CF to be more active, lively, and independent. Their new lungs are free of infection and damage. Having healthy new lungs also makes it easier to gain weight. It is important to keep in mind, however, that lung transplants do not cure CF. Even though the new lungs do not have the faulty CF gene, the rest of the body is still affected by CF. The pancreas, sweat glands, and other organs are not helped by a lung transplant. This means you still have to take enzymes and vitamins after getting new lungs.

Types of Transplants

There are two sources of lungs for transplant – cadavers and living donors. In a cadaveric transplant, the lungs come from a person who has died (was "brain dead"). A living donor transplant involves two donors who are alive. Human lungs do not regrow, so a donor can only give up one lobe of their lungs. One living donor gives a lower right lobe. The other living donor gives a lower left lobe. These two lobes are then transplanted into the person with CF. Living donor transplant operations are more complex than cadaveric transplants. This is because three people have major surgery at the same time.

A person has to volunteer to be a living donor. He or she must be in good physical condition and in good health. A living donor cannot have high blood pressure, heart or kidney disease, cancer, or high blood sugar. Most living donors are between the ages of 18 and 60. They can be men or women and of any race. Living donors must have a blood type that will work with the blood type of the person who will get their lung lobe.

Living donor and cadaveric transplants have about the same success rate. Cadaveric transplants are much more common than living donor transplants. It is important to find out which kind of transplants a center has done, and what their success rate is.

Who Can Have a Lung Transplant?

  • There are many people waiting for lung transplants, but there is a shortage of donor lungs. This is why people are chosen carefully for transplant. If you have very bad lung disease, a lung transplant may be a choice for you. If your CF care team thinks a transplant may be a good idea, you will be referred to a transplant center. Tests will be done to:
    • See how well your heart, lungs, and kidneys are working
    • Find out how much of your decreased lung function is due to clogged airways and how much is due to lung damage that cannot be changed
    • Find out what kind of bacteria are in your lungs

The transplant center will want to know about your social support system – friends, family, and health workers. They will also think about your mental and emotional health, and whether you can follow a complex care plan after surgery.

Some reasons always rule out a lung transplant. These are called absolute contraindications (kon-tra-IN-da-ka-shuns). Other reasons may rule it out for some people. These are called relative contraindications.

Absolute contraindications include reasons such as:

  • Current or recent abuse of tobacco, narcotics, or alcohol
  • Recent cancer
  • Non-lung infections that cannot be cured, such as HIV or hepatitis C
  • Sepsis (a serious blood stream infection)
  • Not following prescribed treatments in the past

Relative contraindications may, but do not always, rule out a lung transplant. Reasons that may be allowed if treated before or after transplant, or if well controlled, include:

  • High blood sugar
  • Brittle bones
  • Sinus disease
  • GERD (gastroesophageal reflux disease)
  • Antibiotic-resistant bacteria in the lungs

The Lung Sharing System

If you are over age 12 and approved for a transplant, you will get a lung allocation score. This score is used to help decide who should get lungs when they become ready. The score is a number from 0 to 100. The score comes from health information such as lab test results and disease diagnosis. When lungs become ready, a person with a higher score has priority over a person with a lower score.

Other reasons are also used in deciding who should get lungs. The age and size of a donor help doctors know if the lungs will "fit" the recipient. For example, lungs from a child or teenage donor are offered to a young person before they are offered to an adult. The donor and the recipient must also have blood types that will work together. One more factor is the distance between the recipient and the hospital where the donor is. This is important because lungs can only be kept outside the body for 2-4 hours.

Since 1991, nearly 1,600 people with CF have received transplanted lungs.

Children under age 12 do not get a lung allocation score. They are offered lungs based on how long they have been waiting for a transplant, their blood type, and how far they live from the donor's hospital.

After the Transplant

The immune system protects the body from invaders like germs. It attacks anything that does not belong in the body. This is why the immune system reacts against transplanted lungs. After lung transplant surgery, a person must take medicines that hold back the immune system. High doses of these medicines are needed so that the body does not reject the new lungs.

These medicines can raise the chance of getting some problems, such as infection. One reason for removing both lungs is that if only one sick lung were removed, the other sick lung could infect the new lung with bacteria. Lung infections may still happen right after surgery when taking immune suppressing medicines. If there are still Pseudomonas or B. cepacia germs in the nose or sinuses, they may move into the lungs. The immune suppressing medicines make it harder for the body to fight these infections. These medicines also raise the chance of getting cancer. Other serious side effects can include high blood sugar, kidney problems, and brittle bones.

Overall, people with CF do as well or even better with lung transplants than those with other diseases. One year after a lung transplant, about 90 out of 100 people are still living. Five years after a transplant 59 out of 100 are still alive. After ten years, 38 out of 100 are still living.

References

Orens J, Estenne M, Arcasoy, S, et al. International Guidelines for the Selection of Lung Transplant Candidates: 2006 Update – A Consensus Report From the Pulmonary Scienti?c Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006;25:745–55.

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/LungTransplantation/. Accessed May 13, 2008.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/lungtrans.aspx?print=1. Accessed June 6, 2008.

Fallon LF. Cystic Fibrosis. In: Krapp K, ed. Gale Encyclopedia of Nursing and Allied Health. Detroit, MI: Gale; 2002:626-633.

Lannefors L, Button BM, Mcllwaine M. Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments. J R Soc Med. 2004;97(44):8-25.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Sueblinvong V, Whittaker LA. Fertility and pregnancy: common concerns of the aging cystic fibrosis population. Clin Chest Med. 2007;28:433-443.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

NUTRITION AND CYSTIC FIBROSIS


Nutrients are in the food we eat that we use to live and grow. Most of the nutrients in food get taken into the body through the small intestine. Nutrients can only be taken into the body if enzymes from the pancreas are present. In about 93 out of 100 people with CF, mucus blocks the ducts of the pancreas. This stops the enzymes from getting to the food in the small intestine. Without enzymes, food cannot be broken down for use by the body. Food passes out of the body without being digested. This is called malabsorption.

Malnutrition

Malabsorption of nutrients can lead to malnutrition. Malnutrition means that the body is not getting enough nutrients to work the right way. Because nutrients are not being absorbed, people with CF can be malnourished even if they are hungry and eat a lot of food. They may be shorter and weigh less than a healthy person who is their same age and gender.

Taking enzymes helps delay malnutrition. Most people with CF need to take enzyme pills with all their meals and large snacks. Babies need enzymes with their formula or breast milk. These enzymes replace the natural ones that cannot get through the blocked pancreas ducts.

Nutrition and Lung Health

Enzymes alone do not always stop malnutrition. Because of lung disease, people with CF need many more calories than other people do. With CF, it takes more energy to breathe. At rest, a person with CF is still spending more energy than a person who does not have CF. Even mild lung disease calls for many calories to fight swelling and infection.

After age two, everyone with CF needs 110% to 200% more calories than others do. For some teens and adults, this may mean eating as many as 5,000 calories a day. Compare that to the average adult who should only eat about 2,000 calories each day. Children may need up to 2,800 calories a day. But, when people do not feel well, they may not feel hungry. Many also have CFRD (cystic fibrosis-related diabetes) or other CF problems. These factors also lead to malnutrition.

The better your diet is, the better your lung function is likely to be. When your diet is poor, lung disease is much more likely to develop. The good news is that forceful nutrition can slow down or put off lung problems. Great nutrition may even help people with CF live longer. One reason for this is that good nutrition and a good supply of calories help the body fight lung infections. Less lung infections means stronger lungs, which often allows for a longer, healthier life. Most of the time, a higher body weight means better lung function.

The Importance of Early Care

Eating right early on is one of the best ways to avoid malnutrition. Thanks to newborn testing for CF, many parents can now give their child high quality nutrition right from birth. When a person is diagnosed later, CF care providers pay special attention to nutrition in the first 12 months after the diagnosis.

Research shows that a child's nutrition rank at age three can predict how well their lungs will work at age six. Since children with CF are at high risk for malnutrition, it is best to form good eating habits when children are young.

This is not always easy. One study of the eating habits of CF children under age 12 showed that most of them:

  • Skipped at least one meal or snack every few days.
  • Got enough calories only one day out of four.
  • Ate low fat rather than high fat meals about half the time.
  • Forgot to take their enzymes about 15% of the time.

There are many ways to improve nutrition. Research shows that the methods all work well, but the most important thing is to start using them early. When good nutrition is started before problems begin, there is a better chance of success. Good early nutrition can have a good effect on health for the rest of a child's life.

Nutritional Therapies

Because of the important role that nutrition plays in CF, preventing malnutrition is a main goal. Nutritional treatment for CF includes three basic parts:

  • Diet. People with CF need a healthy, balanced diet that is very high in calories. It should also be high in fat, protein, and salt. People with CF should try to get about 2-4 servings of protein each day. One serving can be found in two tablespoons of peanut butter or a piece of meat or fish that's the size of a deck of playing cards. Thirty-five to forty percent of calories should come from fats. High fat foods include butter, cheese, and nuts. Extra salt and fluids are needed, especially in hot weather or when exercising. There are no rules on diet for adults or children with CF.
  • Enzymes. People with CF must take enzymes to help them break down food. Enzyme pills are taken by mouth. Inside the pill there are small beads of enzymes. The beads have a coating that keeps them from dissolving until they get to the small intestine. For babies, capsules are opened and the beads are sprinkled on an acidic food such as applesauce. The amount of enzymes a person needs is based on their age, the size of their body, and the type of food they are eating.
  • Vitamins. Vitamins A, D, E, and K are fat-soluble vitamins. They dissolve in the fat you eat. Since digesting fats is a problem for most people with CF, water-soluble forms of these vitamins are taken.

If malnutrition, slow growth, or slow weight gain are problems, more forceful steps can be taken to help nutrition. Some choices are:

  • Behavior Treatment. When children ages 1-12 have slow growth, behavioral treatment may help. It may also help if a child has mealtime behaviors that are causing problems. Behavior treatment is used along with nutrition advice and other treatments to help children gain weight.
  • Tube Feeding. If a child is not growing well, or if an adult is having trouble gaining weight, tube feeding may be the answer. Tube feedings are an easy way to give extra calories. Feedings are often done at night while a person is sleeping. Tube feedings can be done in four ways:
    • OG (orogastric): by mouth
    • NG (nasogastric): through the nose
    • GT (gastrostomy): through the stomach
    • JT (jejunostomy): through the intestine
  • Hunger Stimulants. Two medicines, megestrol acetate and cyproheptadine, can be used to try to increase hunger. But experts do not agree if these help much.
  • Growth Hormones. Some studies have shown that recombinant human growth hormone (rhGH) improves both height and weight for children. In one study, the improvements lasted even after the rhGH treatments were stopped. A different study found that rhGH improved growth, but not weight. The people who did this study think that younger people with good lung function will benefit the most from growth hormone treatments.

Checking Nutrition Level

Both children and adults need to have their nutrition level reviewed often. Everyone with CF should have a checkup at least four times a year.

Checking BMI

The best way to tell if a person's nutrition is good is to see if their weight is right for their height. For children, growth in height is also important. Both of these are measured by body mass index (BMI). BMI is a number that stands for a person's height and weight. A higher BMI most often means better lung function.

Three different BMI scales are used: one for babies and children under age two, one for children and teens ages 2-20, and one for adults 20 and older.

  • For adults, the goal is a BMI of 23 for men and 22 for women. An adult with a BMI of less than 19 has malnutrition. As age increases, so does malnutrition. It is thought that about 38 out of 100 adults with CF are in the malnutrition grouping. These adults need forceful nutrition treatment.
  • For children and teens (ages 2-20), the BMI scale is based on percentiles. The goal for this age group is to keep up a BMI at or above the 50th percentile. Children and teens with a percentile of less than 25 are "at risk." When the score is at or under the 10th percentile, the child or teen has malnutrition and needs forceful nutritional treatment.
  • For children younger than two, the goal is to reach the 50th percentile on the "weight for length" scale by their second birthday. Young children are considered "at risk" when their value falls below the 25th percentile. Values in the 10th percentile or below means the child is not getting enough nutrients. Steps must be taken to correct the problem.

More than half of adults with CF have a BMI that is lower than the recommended level. This is also true for children. A low BMI is a worry for both adults and children because of the link between good nutrition and living longer. A dietician is a healthcare expert who specializes in helping people get the right amount of nutrients. You or your child should see a dietician who has experience in managing people with CF.

A low BMI is a concern for both adults and children because of the link between good nutrition and living longer.

There are easy-to-use BMI calculators for children and adults on the Center for Disease Control and Prevention Web site. Go to www.cdc.gov and type "BMI calculator" in the search box.

Checking Malabsorption

  • To make sure of the best possible nutrition, it is important to check both children and adults for any signs that food is not being digested. The signs are:
  • Poor weight gain even with a very good appetite.
  • Many loose and/or large bowel movements.
  • Foul smelling stools.
  • Mucus or oil in stools.
  • A lot of gas or stomach pain.
  • Bloating.

If these signs are present, the type of enzymes or how much you take may need to be changed. Never raise or lower the dose of enzymes without talking to your doctor or dietitian. Taking too many enzymes can harm the large bowel. Taking too few enzymes may cause DIOS (distal obstructive syndrome), a block in the bowel.

Other Signs of Poor Nutrition

There are two other signs that suggest nutrition is poor. These are:

  • Delayed puberty. For girls this means no breast growth by age 13 or not starting periods by age 16. For boys this means no genital changes or enlargement of the testicles by age 14. Delayed puberty is also a worry for social and emotional reasons.
  • Weight plateau. This means there is no increase in weight for a period of time. For children under age five, a weight plateau has been reached if there is no weight gain for three months or more. For children over age five, a weight plateau is defined as no increase in weight for six months or more.

Ten Tips for Adding Extra Calories

  • Drink whole milk at every meal. Better yet, make "super milk" by mixing the same amounts of whole milk and half and half.
  • Use cream on your cereal.
  • Make smoothies by mixing ice cream and fruit with supplement drinks like Ensure®, Boost®, or Carnation® Instant Breakfast.
  • Make "nut dust" by grinding up nuts and sprinkling it on any food you like.
  • Butter your veggies, hot soups, potatoes, and pastas.
  • Add eggs to hamburger patties before you cook them.
  • Add extra cheese to your pizza.
  • Add powdered milk to casseroles, meatloaf, and puddings.
  • Put peanut butter and syrup on pancakes and waffles.
  • Add butter or mayonnaise to sandwiches.

Remember: fats – found in foods such as butter, oil, nuts, cheese, and cream – have 9 calories per gram. That's more than twice as many calories as you get from a gram of protein or carbohydrates!

References

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/pdf/Enzymes_Children.pdf. Accessed July 5, 2008.

Pitts J, Flack J, Goodfellow J. Improving Nutrition in the cystic fibrosis patient. J Pediatr Health Care. 2008. 28:137-140.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.

Milla CE. Nutrition and lung disease in cystic fibrosis. Clin Chest Med. 2007;28(2):319-330.

Stallings VA, Stark L, et al; for the Practice Guidelines on Growth and Nutrition Subcommittee; Ad Hoc Working Group. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108:832-839.

Cystic Fibrosis Foundation. Nutrition: For Teens With Cystic Fibrosis. Bethesda, MD: Cystic Fibrosis Foundation; 2002.

Cystic Fibrosis Foundation. Nutrition: For Your Child With Cystic Fibrosis (Four to Seven years) . Bethesda, MD: Cystic Fibrosis Foundation; 2002.

Cystic Fibrosis Foundation. Nutrition: Bone Health and Cystic Fibrosis. Bethesda, MD: Cystic Fibrosis Foundation; 2006.

Gordon CM, Anderson EJ, Herlyn K, Hubbaard JL, Pizzo A, Gelbard R, et al. Nutrient status of adults with cystic fibrosis. J Am Diet Assoc. 2007;107:2114-2119.

Cystic Fibrosis Foundation. Nutrition: Cystic Fibrosis-Changes Through Life. Bethesda, MD: Cystic Fibrosis Foundation; 2002.

Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patient with cystic fibrosis [medical position papers]. J Pediatr Gastroenterol Nutr. 2002;35(3):246-59.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/kids/living/keeping_nutri_protein.html. Accessed July 5, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/kids/living/keeping_nutri_fatfiber.html. Accessed July 5, 2008.

Cystic Fibrosis Foundation. Nutrition: For Your Infant With Cystic Fibrosis (Birth to One Year). Bethesda, MD: Cystic Fibrosis Foundation; 2002.

National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html. Accessed May 13, 2008.

Hardin DS, Adams-Huet B, Brown D, et al. Growth hormone treatment improves growth and clinical status in prepubertal children with cystic fibrosis: results of a multicenter randomized controlled trial. J Clin Endocrinol Metab. 2006:91(12):4925-4929.

Schnabel D, Grasemann C, Stabb D et al. for the German Cystic Fibrosis Growth Hormone Study Group. A multicenter, randomized, double-blind, pacebo-controlled trial to evaluate the metabolic and respiratory effects of growth hormone in children with cystic fibrosis. Pediatrics. 2007;119(6):e1230-8.

Cystic Fibrosis Foundation, Patient Registry 2006 Annual Report, Bethesda, Maryland.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/kids/living/keeping_nutri_calories.html. Accessed July 5, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

EMOTIONAL AND SOCIAL ASPECTS OF GROWING UP WITH CYSTIC FIBROSIS

Topics:

In many ways, children growing up with CF are no different than other children. They go to school, play sports, enjoy hobbies, and have fun with friends. But children with CF have a daily routine that includes doing airway clearance and taking enzymes and medicines.

Some studies have found no emotional or social differences between children who have CF and children who do not. Other studies have shown self-esteem and behavior problems due to CF. Experts say that children with CF do best when their parents raise them with the same rules and discipline as any other child. To avoid jealous feelings from brothers and sisters, children with CF should be treated as close as possible to how their brothers and sisters are treated.

Anxiety

A study compared children with CF to healthy children. All the children were between 7 and 14 years old. The study found that the two groups were the same in self-concept, amount of sadness, and behavior. The results of other studies also agree with this. The first study did find, however, that the 7 to 10-year-olds with CF had more anxiety than the healthy group. The older children with CF, ages 11-14, were not more anxious than the healthy group.

The researchers think they may know why the younger children were more anxious than the older ones. All of the children who were age 10 and older had been to a "CF school" that was made by a hospital. There, they learned all about CF. They had a chance to ask hard questions they may have been afraid to ask their parents. The people who did this study think that knowing a lot about CF took away the anxiety of the older children.

Knowing the reasons for airway clearance, eating right, and taking enzymes helps get children ready to one day do their own CF care.

Teaching your child about CF may help lower his or her anxiety. Knowing the reasons for airway clearance, eating right, and taking enzymes also helps get children ready to one day do their own CF care. As your child grows, share information about CF that is right for the child's age.

Studies show that having good information also helps lower the harmful effects of CF on the family. Books can help you, your child, and your relatives better understand CF. To see a list of books about CF for adults and children, click on Resource Center under the Accordant.com logo on your Accordant home page. Choose the Shops and Stores link to Amazon.com.

Friends

Even when children know a lot about CF, it may not be easy for them to answer questions from others. A friend at school may ask, "Why do you take so many pills?" A teammate may ask, "Why do you cough so much?" Parents can help their children learn how to handle these questions. A simple, matter-of-fact approach is best. For example, the child could tell the school friend, "I take these enzymes to help my body break down food." He could tell the teammate, "Coughing helps my lungs." The parent and child can decide together on the best answers to give. Then the parent can pretend to be a friend who is asking questions. This lets the child practice answering. This is called role playing. CF care providers can also suggest good ways for a child to answer questions about CF.

Children want to fit in with their peers. They may not want their friends at school to know they have CF. They may not want others to see them take enzymes before they eat lunch. Some children may take their enzymes in private before they go to lunch. Others, to avoid feeling shame or questions, may "forget" to take them or throw them away.

Teachers can help avoid embarrassment for their students with CF. When a teacher treats coughing, taking enzymes, and frequent bathroom visits as normal, class members are likely to follow his or her lead. An easy way to educate teachers about CF is to give them a copy of the Cystic Fibrosis Foundation booklet, A Teacher's Guide to Cystic Fibrosis. You can find this on the Foundation's Web site (www.cff.org).

Freedom

Children with CF should not be overprotected. To grow up well adjusted, they need to have the normal freedoms of childhood. Independence should be encouraged. Children with CF should also be encouraged to help other people, read about other cultures, and care about the needs of others. These things will help them learn not to focus only on self.

By the time they enter school, most five and six-year-olds can take their enzymes and medicines on their own. By age 12, some are ready to direct their own CF care. As pre-teens grow, it is normal to want more freedom. This can include rebelling against doing airway clearance, taking enzymes, and sticking to schedules. Parents need to be patient and keep in mind that this is also part of growing up.

By age 14, teens should be able to take charge of their own care. At clinic visits, they should see their doctor one-on-one first, and then with a parent. By the end of high school, teens should be ready to transition from a pediatric care team to an adult care team. This can be postponed for those waiting for a lung transplant, or those who are not doing well medically. The move to an adult care team is one more step toward independence.

The Teen Years

Teens with CF face a double challenge. They must deal with all the normal changes of teen years while coping with CF too. Teens with CF face some special problems:

Physical Changes: The teen years are normally a time of rapid physical growth. Teens with CF may have nutritional issues that slow their growth. They may be shorter and weigh less than others their age. Girls may even lose body fat. They are at greater risk for nutritional problems than boys are. On the plus side, research has found that being underweight did not cause anxiety for the 10 to 14-year-olds in their study. The researchers think this may be because being slim is more socially acceptable than being too heavy.

Of greater worry to teens, perhaps, is the fact that poor eating habits can delay puberty. For girls this means no breast growth by age 13 or not starting periods by age 16. For boys, delayed puberty means no genital changes or enlargement of the testicles by age 14. Those who have been well-nourished during childhood are more likely to grow and develop normally as teenagers.

Many teens find that tube feedings are a good way to add extra calories. Feedings can be done at night, privately, to add to daytime meals and snacks.

Social Life: When peers are maturing physically, but teens with CF are not, they may be affected emotionally and socially. For some, their lack of growth lowers self-esteem or creates a poor feelings about your body. They may find it harder to form close friendships. They may start dating later than their friends, or they may date less often. Some may become socially cut off.

Teens with CF may be self-conscious about their cough. They may get tired more easily than their friends do. Some may feel embarrassed about having to take a lot of pills or needing to go to the bathroom often. The best approach, many teens say, is to just be open with friends about having CF. They say this does not change their friendships and it can help avoid awkward moments. Recent research shows that, overall, teens with CF lead active lives and have the same social hobbies as others their age.

Unsafe Behaviors: Researchers say that teens with CF take part in fewer unsafe behaviors than other teens. They think this may be at least partly due to the delay in their physical maturity. One study compared teens with CF with teens who did not have CF. Less of the teens with CF had ever used alcohol or smoked tobacco or pot. They were also less likely to have had sex. They did less things that could put them at risk, such as drinking and driving, fighting, or carrying a weapon. Of the teens who had used alcohol or marijuana, those with CF were older than other teens when they tried these things for the first time. Boys with CF had sex for the first time later than their peers. But, girls with CF had sex for the first time sooner than their friends. In the study, 21% of the teens with CF had smoked and 28% reported having had sex.

While the rates of unsafe behaviors are lower for teens with CF, these behaviors are often more dangerous for them. For example, an unplanned pregnancy is more unsafe for a girl with CF than for a healthy girl. Another example is smoking, which can cutback lung function for those with CF.

Planning for the Future: Teens need to think about what they want to do after high school. Will they go to college? Do they want to learn a trade? Like other teens, those with CF should choose their career based on their skills, interests, and goals. Many universities now offer distance learning for many majors. This can be a good choice for some teens with CF. Those going away to school should ask about the CF care they can get in that city.

Some teens with CF may be fearful about the future. Some may have low expectations for themselves. They may need extra support as they make choices about the future. While it is important to make realistic plans, it is also good to keep in mind that people with CF can have almost any career that others can have. Certain jobs should be considered carefully. For example, health care jobs or work that involves children may put those with CF at greater risk for infection. One's current or likely future physical limits should also be considered. Self-employment offers the most flexibility. Jobs that can be done from home are also good choices for many people.

References

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Bregnballe V. Thastum M, Schiøtz PO. Psychosocial problems in children with cystic fibrosis. Acta Paediatr. 2007 Jan;96(1):58-61.

Harrop M. Psychosocial impact of cystic fibrosis in adolescence. Paediatr Nurs. 2007. 19(10):41-45.

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/AtSchool/TeachersGuide/. Accessed July 9, 2008.

Fact Sheet: Nutrition: Pancreatic Enzyme Replacement in People with Cystic Fibrosis. Cystic Fibrosis Foundation Website.http://www.cff.org. Accessed May 13, 2008.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Britto MT, Garrett JM, Malcom A, Dugliss J, Daeschner CW, Johnson CA, et al. Risky behavior in teens with cystic fibrosis or sickle cell disease: a multicenter study. Pediatrics. 1998;101(2):250-256.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.

Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patient with cystic fibrosis [medical position papers]. J Pediatr Gastroenterol Nutr. 2002;35(3):246-59.

Cystic Fibrosis Foundation. Nutrition: For Teens With Cystic Fibrosis. Bethesda, MD: Cystic Fibrosis Foundation; 2002.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HOPE FOR THE FUTURE: RESEARCHING NEW CYSTIC FIBROSIS TREATMENTS

 

The first research money was given to CF (cystic fibrosis) researchers in 1955. Since then, much has been learned about CF. Learning more about CF has made it possible to make many medicines and treatments. Because of past research, people with CF now live longer than ever before in history.

 

Until a cure is found, CF research will carry on. More new and exciting treatments are being explored than in years past. Some of these are:

Medicines That Reduce Swelling in the Lungs

Six medicines that can lower swelling in the lungs are being studied. Some of them are now being used to treat other diseases. One of the studies is focused on infant formula that has a fatty acid called DHA. People with CF do not seem to have enough DHA. This study may show whether the formula will have an effect on the babies' CF.

Medicines That Fight Infection

Eight medicines that fight lung infections are also being studied. Some of the medicines are spray mists, some are taken by mouth, and some are vaccines. One is a powder that is designed to be breathed in. Inhaling a powder may be a faster, easier way to fight infections than current treatments.

Mucus Treatments

Two treatments are being studied to see how well they work to thin mucus and clear it from the airways.

Protein Assist or Repair Treatment

Work is moving ahead on five treatments that could correct the way the faulty CFTR protein works. The goal is to help or fix this protein so it will let salt move normally in and out of the cells in the lungs and other organs. One of these treatment uses curcumin, which comes from the Asian spice, tumeric.

Restore Salt Transport

Researchers are working on four treatments to get more water into the mucus in the lungs. These treatments would correct the amount of salt on the surface of lung cells.

Lung Transplant Medicine

One medicine is being studied to see if it will help improve the results of lung transplants in people with CF.

Nutritional Supplements

Two nutritional supplements are being studied. Both may be able to help the body deal with the effects of CF. One is a vitamin that was made just for people with CF. The other would take the place of an enzyme normally made in the pancreas.

Gene Therapy

In 1989 scientists found the most common gene mutation that causes CF. Since that time, many people have dreamed of the day when CF would finally be cured through gene therapy.

The idea behind gene therapy is that healthy genes can be used as a kind of "medicine." Healthy genes are delivered to body cells, often by a specially made virus, known as a vector. When a healthy gene gets inside a cell, it takes over the gene that is not working right. Taking over the faulty gene that causes CF, many believe, would cure the illness.

Unfortunately, a good way to get healthy genes into the cells has not yet been found. Many delivery systems have been tried, but so far none of them has worked well. Gene therapy research goes on, however, in hopes that the dream of curing CF will come true. Researchers are now trying gene therapy with a delivery system that is not a virus.

References

Cystic Fibrosis Foundation Web site. http://www.cff.org/research/ResearchMilestones/index.cfm?dspPrintReady=Y. Accessed May 31, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/. Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site http://www.cff.org/research/ClinicalResearch/index.cfm?dspPrintReady=Y. Accessed May 31, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Pipeline/. Accessed May 13, 2008.

MedLine Plus [Internet].Bethesda, MD: National Library of Medicine (US); [updated 2008 May 1] Tumeric (Curcuma longa Linn.) and Curcumin; [reviewed 2008 Mar 1; cited 2008 May 31]; [about 7 p]. Available from: http://www.nlm.nih.gov/medlineplus/druginfo/natural/patient-turmeric.html.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHEN SHOULD I CALL THE DOCTOR?

 

CF can be a difficult disease to manage. Many problems, however, can be solved or at least made better with the help of a doctor or nurse. It is a good idea to talk to your doctor about anything that concerns you. You can also call your AccordantCare nurse with questions. Many times, a small change can solve a problem. Other times, prompt health care is needed.

 

You should call your doctor...

...if you have a lung infection that all of the sudden gets worse. This is called a pulmonary exacerbation. The most common signs are:

  • More mucus
  • More coughing
  • Harder to exercise
  • Less hunger
  • Weight loss
  • Change in breathing
  • Coughing up blood (hemoptysis)
  • Low fever
  • Feeling tired

...if you have very bad stomach pain and/or swelling, nausea, and throwing up. These signs could mean there is a problem with your pancreas or your bowels. Tell your doctor if you also have a fever, a rapid heartbeat, or are not having regular bowel movements.

...if you have a sudden sharp pain in your chest/and or shoulder, and shortness of breath. This may be air in the space between your lungs and your chest. If this happens, your lung could flatten. A flattened lung is a serious problem, but do not panic. Go to a hospital ER if you cannot reach your doctor by phone.

...if you are thinking about getting pregnant, or you think you may be pregnant. If you are thinking about having a baby, it is best to talk to your doctor before you get pregnant. CF pregnancies are considered "high risk." With pre-pregnancy planning, your doctor can help you get as healthy as possible before you become pregnant. You and your doctor can plan your medicines, and talk about eating habits. You can also talk about genetic counseling. If you have any other health issues, these can also be talked about early.

If you are already pregnant, see your doctor as soon as you can. Your doctor should make sure the medicines you take are safe for your baby. You will need care from a team of doctors who understand CF.

...if you are coughing up as much blood as a soda can holds. Many people with CF cough up small amounts of blood. This is called hemoptysis. Coughing up a few teaspoonfuls of blood in a day's time is not a problem. Coughing up very large amounts of blood can be serious. Stay calm. Go to a hospital ER if you cannot reach your doctor by phone.

...if you have unexplained weight loss or you are having a hard time gaining weight. This could be a sign of CFRD (cystic fibrosis-related diabetes). For people with CF, the more common signs of high blood sugar may be missed because they can also be caused by CF. Tell your CF care team about your weight issue and ask them to do a test to check for high blood sugar.

References

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/prevent_infec.aspx. Accessed June 6, 2008.

Ferkol T, Rosenfeld M, Milla CE. Cystic fibrosis pulmonary exacerbations. J Pediatr. 2006;148(2):259-264.

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_lung.html. Accessed June 3, 2008.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/pneumothorax-hemop.aspx. Accessed June 6, 2008.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Aronson BS, Marquis M. Care of the adult patient with cystic fibrosis. Medsurg Nurs. 2004;13(3):143-155.

Gumery L, Dodd M, Parker A, Prasad A, Pryor J, Kennedy N, eds. Clinical Guidelines for the Physiotherapy Management of Cystic Fibrosis. Bromley, United Kingdom: Cystic Fibrosis Trust; 2002.

Grossman S, Grossman LC. Pathophysiology of cystic fibrosis: implications for critical care nurses. Crit Care Nurse. 2005;25(4):46-51.

Hardin DS, Brunzell C, Schissel K, Schindler T, Moran A. Managing Cystic Fibrosis-Related Diabetes (CFRD). Bethesda, MD: Cystic Fibrosis Foundation; 2002.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

ADULTS WITH CYSTIC FIBROSIS

Topics:

CF is no longer a disease that only affects children. According to the Cystic Fibrosis Foundation Patient Registry, in 2006 45 out of 100 people with CF were age 18 or older. The number of adults with CF is likely to keep on growing. There are two reasons for this:

  • Better Treatments: Over the past 30 years, the medicines and treatments for CF have gotten better. As a result, people with CF are now living well into their adult years. In the 1950s, children with CF rarely lived long enough to enter the first grade. In the 1970s, children with CF lived to about age 16. By 2006, at least half of those with CF were living to age 37. Babies born with CF today are likely to live into their 50s or 60s. Earlier diagnosis and better treatments continue to offer longer lives to those with CF.
  • Late Diagnosis: While seven out of ten people with CF are diagnosed before their first birthday, more people are now diagnosed much later, as adults. Ten out of 100 people with CF (10%) are now diagnosed in their adult years. Many of these adults were mostly healthy during childhood. Most of them have a milder form of CF. They often have mild lung problems, less infection, and normal enzymes.

These facts mean that CF is now, and will continue to be, a disease that affects adults as well as children.

Adult Life with CF

Adults with CF must deal with many more emotional and health-related issues than children with CF do. Most adults learn to cope with these challenges. Many adults with CF lead relatively normal, happy lives that include a career, family, and friends.

Health Issues for Adults

CF is a progressive disease. This means that it gets worse as time goes by. For this reason, adults with CF often have more health problems than younger people. Adults with CF also have some problems that do not happen very often in children with the disease.

Besides the normal CF problems, adults may face these extra health issues as they grow older:

  • Lung Problems: Overall, adults have worse lung disease than children do. Pulmonary exacerbations are common for adults. (A pulmonary exacerbation is a lung infection that all of the sudden gets much worse.) After age 18, about 6 out of 10 people have pulmonary exacerbations. By contrast, only about 2 out of 10 children under age 6 have them. In adults, lung infections are most often caused by the Pseudomonas aeruginosa bacteria. These bacteria in the end change into a form that is hard to treat and may lead to worsening health.

    Compared to children with CF, adults are at greater risk for lung problems such as hemoptysis (coughing up blood from the lungs) and pneumothorax (flattened lung).

  • Cystic Fibrosis Related Diabetes (CFRD): Diabetes is a disease that affects how the body uses the energy it gets from food. CFRD is a type of diabetes that only affects people with CF. It is different from the more common types of diabetes that affect those who do not have CF. CFRD happens because of damage to the pancreas, so it is more likely to occur in people who have blockage of the pancreas.

    CFRD is an age-linked problem of CF. Before age 10, less than 1 out of 10 children with CF have CFRD. After age 30, more than 4 out of 10 people with CF have it. As the number of adults with CF continues to grow, doctors expect more people with CF to get CFRD.

    CFRD often happens along with worsening lung health or nutritional rank. Like the other types of high blood sugar, CFRD can affect the eyes, kidneys, and the nerves in the hands, arms, legs, and feet. Adults with CFRD need regular exams to check for these problems.

  • CF-Related Bone Disease: CF-related bone disease starts in childhood but it may not be found until the adult years. Bone disease includes osteoporosis (ahs-te-o-pa-ro-sis) and osteopenia (ahs-te-o-pe-ne-a). Osteoporosis means brittle bones. It is a disease that causes bones to become weak and likely to break. It can affect any bone in the body. Many children and adults with CF have osteopenia (weaker bones). This is caused by lung or diet problems, or by taking steroid medicines. But the more advanced disease of brittle bones is found more often in adults than in children. Those who have had lung transplants are also more likely to have brittle bones. Overall, about 9 out of 100 people with CF have bone disease.
  • Joint Disease: There are two kinds of joint disease that are more common in adults with CF than in children. One is a type of arthritis that comes and goes. The other is HPOA (hypertrophic pulmonary osteoarthropathy).

    When the arthritis occurs it may affect any of the joints. The joints swell and become hot, red, tender, and very painful. A flare of arthritis can last as long as 10 days. It is treated with anti-inflammatory medicines. About 12 out of 100 people with CF have this kind of arthritis.

    HPOA causes swelling of the membranes around the long bones. It often happens when lung infections get worse. It causes pain in both the bones and the joints. It is treated with anti-inflammatory medicines. About 8 in 100 people with CF have HPOA.

    To learn more about CF-related bone and joint disease read the Accordant Web article, Bone and Joint Health.

  • Nutrition: Adults with CF need special nutrition. Nutrition has a big impact on overall health. Many adults with CF find that it is hard to keep up the weight that is right for them. The best way to tell if your weight is right for you is by your BMI (body mass index). BMI is a number that stands for a person's height and weight. For adults with CF the goal is a BMI of 23 for men and 22 for women. About 22 out of 100 young adults with CF (aged 18-30) have a BMI that less than 18.5. There is an easy to use BMI calculator on the Center for Disease Control and Prevention Web site. Go to www.cdc.gov and type "BMI calculator" in the search box.

    It is important to pay attention to BMI because there is a clear link between good nutrition and living longer. Nutrition also plays a big role in lung and bone health.

  • Raised Risk of Cancer: Most of the time, having CF does not raise the risk for cancer. There is a raised risk for cancers of the digestive tract, however. This risk gets larger with age. The raised risk applies to the esophagus, stomach, small and large bowels, liver, and pancreas. You can help guard against these cancers by eating a healthy diet and not smoking. You should also be sure to have the colon cancer screenings that are recommended for everyone.

Keys to Coping

Two keys to coping well with these health issues are good ongoing health care and good self-care.

Teamwork Leads to Better Health Care

The Cystic Fibrosis Foundation has stated, "A strong partnership between patients and health care providers working as a team is critical to achieve the best possible health for people with CF." If you are an adult with CF, make it your goal to be a full partner with your CF care team. You have many demands on your time, financial resources, and personal life. Your health care team can partner with you to find the CF care measures that best match your needs and lifestyle.

Emotional Issues for Adults

Relationships, finances, employment, and many other concerns impact adults in both practical and emotional ways. For those dealing with CF, there may be added emotional issues such as:

Depression: Almost 1 in 5 adults with CF has signs of depression. Depression is a common problem among those with long term illnesses. There are many good treatments for depression, so do not wait to seek help for this common problem. Talk to your doctor if you have five or more of these signs nearly every day for 2 weeks:

  • Feeling sad or empty
  • Less interest or pleasure in hobbies
  • Appetite change with weight loss or gain
  • Sleeping much more or less than normal
  • Fatigue or loss of energy
  • Feeling worthless or guilty
  • Being either restless or slowed down
  • Hard to think or focus
  • Repeated thoughts of death or taking one's own life

Emotional Impact of a Late Diagnosis

When CF is diagnosed after age 18, people most often have mild signs or, in some cases, no signs at all. This may be because they have inherited a less common gene mutation that causes mild CF. For many adults, the diagnosis of CF comes as a total shock. Some people feel fear, denial, anger, or guilt. Some feel overwhelmed. Others, who have suspected that something was wrong, may feel relief.

10% of people with CF are now diagnosed in their adult years.

Women are more likely than men to be diagnosed late. Men are more likely to have a wider range of health problems that led to their diagnosis. Those receiving a late diagnosis are more likely to be college graduates, married, and working full time.

A late diagnosis of CF usually happens in one of these ways:

  • A person with no signs is diagnosed as a result of having a child with CF.
  • A man with no CF signs learns that his infertility is caused by CF.
  • A person sees a doctor for a problem such as an airway infection, recurring pancreatitis, or ongoing sinus infections. The problem is recognized as a CF sign, leading to a diagnosis of CF.
  • People who have suspected they had CF, but were told they were "too old" to have it, finally find out that they were right.

It takes time to adjust to the diagnosis of CF. It can also be hard to go from a lifestyle with no CF treatments to a lifestyle that must now include daily airway clearance or taking enzymes with every meal. With so many changes, it may be comforting to know that a late diagnosis most often means a much better outlook. Your CF health care team and your AccordantCare nurse can help you with the changes you need to improve your health.

References

Bilton D. Managing adults with cystic fibrosis. The Practitioner. 2007; 251(1700):46-52.

Cystic Fibrosis Foundation, Patient Registry 2006 Annual Report, Bethesda, Maryland.

Wilschanski M, Durie PE. Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut. 2007;56:1153-1163.

World Health Organization. Services for adults with cystic fibrosis: report of a joint WHO/ICF(M)IACFA meeting. The Hague, The Netherlands, 7-8 June 1999.

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Widerman E. Communicating a diagnosis or cystic fibrosis to an adult: what physicians need to know. Behav Med. 2002;28 (2)45-52.

Nick JA, Rodman DM. Manifestations of cystic fibrosis in adulthood. Curr Opin Pulm Med. 2007;11:513-518.

Aronson BS, Marquis M. Care of the adult patient with cystic fibrosis. Medsurg Nurs. 2004;13(3):143-155.

Fallon LF. Cystic Fibrosis. In: Krapp K, ed. Gale Encyclopedia of Nursing and Allied Health. Detroit, MI: Gale; 2002:626-633.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/prevent_infec.aspx. Accessed June 6, 2008.

Goss CH, Burns JL. Exacerbations in cystic fibrosis 1: epidemiology and pathogenesis. Thorax. 2007;62(4):360-367.

Ferkol T, Rosenfeld M, Milla CE. Cystic fibrosis pulmonary exacerbations. J Pediatr. 2006;148(2):259-264.

Elston C, Geddes D. Inflammation in cystic fibrosis---when and why? friend or foe? Semin Respir Crit Med. 2007;28:286-294.

Hardin DS, Brunzell C, Schissel K, Schindler T, Moran A. Managing Cystic Fibrosis-Related Diabetes (CFRD). Bethesda, MD: Cystic Fibrosis Foundation; 2002.

Costa M, Potvin S, Berthiaume Y, et al. Diabetes: a major comorbidity of cystic fibrosis. Diabetes Metab. 2005;31(3):221-232.

Boyle MP. Update on maintaining bone health in cystic fibrosis. Curr Opin Pulm Med. 2006;12(6):453-458.

Roche Laboratories Inc. If you're over 50, its time to learn about osteoporosis. Roche Laboratories Inc; 2007.

Mayo Clinic Web site. http://www.mayoclinic.com/health/osteoporosis/DS00128/DSECTION=2. Accessed February 21, 2008.

Gordon CM, Anderson EJ, Herlyn K, Hubbaard JL, Pizzo A, Gelbard R, et al. Nutrient status of adults with cystic fibrosis. J Am Diet Assoc. 2007;107:2114-2119.

Stallings VA, Stark L, et al; for the Practice Guidelines on Growth and Nutrition Subcommittee; Ad Hoc Working Group. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108:832-839.

Journal of the American Medical Association Patient Page. http://jama.ama-assn.org/cgi/content/full/299/20/2466. Accessed June 22, 2006.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/en/latediagnosis.aspx. Accessed June 6, 2008.

Widerman E. Health status and sociodemographic characteristics of adults receiving a cystic fibrosis diagnosis after age 18 years. 2000; Chest;118(2):427-33.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/nccdphp/dnpa/bmi/adult_BMI/about_adult_BMI.htm. Accessed July 10, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

FAMILY PLANNING

Topics:

People with CF (cystic fibrosis) are now living longer than ever before. This means that those with CF can now choose to have children. However, because CF is a life-shortening disease that may be passed on to one's children, family planning decisions must be made carefully.

Men's Fertility

About 98 out of 100 men with CF are unable to father a child, because their semen does not contain any sperm. This is because the tube that carries sperm from the testicles is usually missing or damaged by CF.

Men who were diagnosed with CF as adults are more likely to be able to father children. This is because they may have a milder form of CF. Men with CF should not assume that they are infertile. The only way to be sure is to have their semen tested.

If the test shows that there are no sperm, it is still possible to become a father. Doctors now have several ways to collect sperm. The sperm can be used right away to fertilize a woman's egg, or they can be frozen for use later. One method is ICSI (intracytoplasmic sperm injection). In ICSI, sperm is drawn out through a needle and put inside an egg in a lab dish. If an embryo grows, it is placed into the woman's uterus. ICSI works 50 out of 100 times (a 50% success rate).

Women's Fertility

In 1960 the first baby was born to a woman with CF. Since then, many others have had successful pregnancies. In 1986, 45 pregnancies were reported. According to the Cystic Fibrosis Foundation Patient Registry, 209 women with CF were pregnant in 2006.

Half of the women with CF can get pregnant. When the lungs and pancreas work normally, fertility can be nearly as good as for women who do not have CF. But CF may prevent pregnancy in several ways:

  • Women with CF may not ovulate normally. (Ovulation is the release of an egg from the ovary.) Women with severe lung disease or poor nutrition are less likely to ovulate normally.
  • Like the mucus in the airways, cervical mucus is thicker and contains less water than normal. Normal cervical mucus gets thin around the time of ovulation. Thinner mucus helps the sperm get through to reach the egg. In women with CF, there is no thinning at ovulation.
  • Most women with CF have a normal reproductive system. Some have defects that make it harder to get pregnant such as cysts on the ovaries or fewer places on the ovaries that release eggs. Some women with CF are born without a uterus or vagina. It is thought that perhaps the CF gene plays a role in causing this before birth.

CF pregnancies are considered high risk. However, women with CF can do well if they are healthy before they conceive and get good medical care while pregnant. Experts say that careful planning is the best way to help ensure a successful CF pregnancy. By planning ahead, lung health and nutrition can be improved as much as possible before pregnancy.

Careful planning is the best way to help ensure a successful CF pregnancy.

Women with CF are as sexually active as women who do not have the disease. Since many women with CF are fertile, it is important to use birth control to avoid an unplanned pregnancy.

Decisions

In a survey of men with CF, there was a very high interest in having children. Most of the men (84%) said they wanted to be fathers. Some men already had children, and many others (78%) said they wanted to have children in the future even if their CF was severe. A study with women who had CF found that at least 7 out of 10 thought that having children was important now or would be important in the future.

There are many things to think about before deciding if having children is right for you. You should think about the possible risks and benefits for each family member. Here are some key things to think about:

CF is Inherited

A person with CF should get genetic advice when making decisions about family planning. Their partner can be tested to see if he or she carries the CF gene. These steps are important because of these facts:

  • A parent with CF will pass a mutated gene to all of their children. If the other parent has only normal CF genes, all of this couple's children will be carriers, but none will have CF.
  • If one parent has CF and the other parent is a carrier, there is a one in two (50%) chance they will have a child with CF. They also have a one in two (50%) chance of having a child who is a carrier.

For more information about how CF is inherited, read the Accordant Web article, What Causes CF?

Normal Physical Changes of Pregnancy May Cause Problems for Women with CF

Most CF pregnancies go well and have a good outcome. This may be partly because women with CF who get pregnant usually have better lung function and better nutrition than those who do not get pregnant. However, the normal changes of pregnancy may cause problems for some women with CF. Some examples:

  • In a normal pregnancy, the body changes the way it handles insulin. Women with CFRD (cystic fibrosis-related diabetes) are more likely to need treatment during pregnancy. Others may develop pregnancy-related diabetes. Controlling diabetes during pregnancy helps the mother gain weight. It also reduces the risk of premature birth and low birth weight.
  • The hormone changes of pregnancy can cause more mucus to be made. The sinuses may be more congested and it may be harder to breathe. There may be a need to do extra airway clearance.
  • During pregnancy, the amount of pumping blood increases and the heart works harder. These changes may cause problems for women with severe lung disease. Problems may be very serious for women with high blood pressure in the arteries of the lungs.
  • The growing baby may create pressure that makes GERD (gastroesophageal reflux disease) worse. This is where food and stomach acid rises up through the esophagus. This can make lung function worse.
  • Women with severe lung disease should be aware that pregnancy may increase their reaction to PRA (panel reactive antibodies). PRA measures how sensitive a person is to the antibodies of donors. A high PRA makes it harder to find a lung transplant donor.

Other Facts About Pregnancy and CF

  • Pregnancy does not put at risk the mother's long-term survival. In fact, the 10-year survival rate for women who have had a baby is higher than for those who were never pregnant.
  • Health care during pregnancy requires more time, work, and doctor visits than are usually needed for CF.
  • Women with CF have more pregnancy complications than women who do not have CF.
  • Women are advised to wait at least two years after a lung transplant before becoming pregnant.
  • Pregnancy may change the way the body absorbs and uses medicines.

Babies Born to CF Mothers are:

  • More likely to be born premature, especially if the mother does not gain enough weight during pregnancy.
  • More likely to have a low birth weight.

Children with a CF Mother or Father are:

  • Always carriers of the CF gene.
  • Likely to suffer the loss of their CF parent before they are grown.

If you are making family planning decisions, your CF health care team can assist you by providing up-to-date information and support. Your AccordantCare nurse can also answer questions and make sure you have everything you need to make informed decisions.

References

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Testing/NewbornScreening/. Accessed May 13, 2008.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Johns Hopkins Cystic Fibrosis Web site. http://www.hopkinscf.org/main/whatiscf/effects_repro.html. Accessed June 3, 2008.

Bilton D. Managing adults with cystic fibrosis. The Practitioner. 2007; 251(1700):46-52.

Sawyer SM, Farrant B, Cerritelli B, Wilson J. A survey of sexual and reproductive health in men with cystic fibrosis: new challenges for adolescent and adult services. Thorax. 2005;60:326-330.

Sueblinvong F, Whittaker LA. Fertility and pregnancy: common concerns of the aging cystic fibrosis population. Clin Chest Med. 2007;433-443.

Johns Hopkins Cystic Fibrosis Web site. http://www.hopkinscf.org/main/whatiscf/treat_repro.html. Accessed June 3, 2008.

Cystic Fibrosis Foundation, Patient Registry 2006 Annual Report, Bethesda, Maryland.

Johannesson M. Effects of pregnancy on health: certain aspects of importance for women with cystic fibrosis. Journal of Cystic Fibrosis. 2002;9-12.

Aronson BS, Marquis M. Care of the adult patient with cystic fibrosis. Medsurg Nurs. 2004;13(3):143-155.

Goss CH, Rubenfeld GD, Otto K, Aiken ML. The effect of pregnancy on survival in women with cystic fibrosis. Chest. 2003;1460-1468.

Tonelli MR, Aitken ML. Pregnancy in cystic fibrosis. Curr Opin Pulm Med. 2007;13:537-540.

McMullen AH, Pasta DJ, Frederick PD, et al; for the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Impact of pregnancy on women with cystic fibrosis. Chest. 2006;129(3):706-711.

Scientific Registry of Transplant Recipients Web site. http://www.ustransplant.org/glossary.aspx?term=Panel%20Reactive%20Antibody%20(PRA) Accessed June 25, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

CYSTIC FIBROSIS AND THE DIGESTIVE SYSTEM

Esophagus

What does the esophagus do?

The esophagus is the tube that takes food from your mouth to your stomach when you swallow.

How can CF affect the esophagus?

Chronic coughing and doing chest physical therapy can lead to GERD – gastroesophageal reflux disease. This is also called "heartburn" or acid reflux).

What does the gall bladder do?

The gall bladder stores and releases bile, a fluid made by the liver that helps digest fats.

How can CF affect the gall bladder?

CF causes the liver to make bile that is not normal. Gallstones may form in the gall bladder as a result. Gallstones can be painful, but surgery to remove the gall bladder is not often needed.

Pancreas

What does the pancreas do?

The pancreas makes enzymes that are needed to digest food. It also makes insulin, a hormone that controls the amount of sugar in the blood.

How can CF affect the pancreas?

Mucus blocks the release of enzymes, causing pancreatic insufficiency. This means that the enzymes do not reach the small intestine where they are needed for digestion.

Pancreatitis is swelling of the pancreas. Anyone with CF can get this at any age, but it almost always occurs in those who were diagnosed with CF as adults. People who get pancreatitis usually have enzymes that do reach the small intestine. It often occurs in those with few lung problems or no symptoms of CF.

CFRD (cystic fibrosis-related diabetes), is a type of diabetes that only people with CF get.

Liver

What does the liver do?

The liver filters the blood and removes toxins from it. It also makes bile.

How can CF affect the liver?

CF causes the liver to make bile that is not normal. This bile has too much acid and too little moisture.

Thick mucus clogs the bile ducts. This blocks the flow of bile. The plugs of mucus and abnormal bile cause swelling and scarring in the ducts. These changes are called cirrhosis.

Hepatic steatosis ("fatty liver") occurs when mucus clogs the bile ducts. This allows fat to build up in the liver. This causes liver damage.

Portal hypertension is an increase in blood pressure in the main vessel of the liver. This can be caused by the scarring from cirrhosis.

Small Intestine

What does the small intestine do?

The small intestine absorbs most of the nutrients from the food you eat.

How can CF affect the small intestine?

With CF, enzymes cannot get into the small intestine because they are blocked by thick mucus in the pancreas. Without enzymes, the small intestine cannot absorb most of the proteins, fats, and carbohydrates in food. This is called malabsorption. Malabsorption causes pain, gas, and large, greasy, foul-smelling stools. Malabsorption also makes it very hard to gain weight. These problems can be greatly improved by taking enzymes with meals and snacks.

DIOS (distal intestinal obstruction syndrome) is a blockage in the small intestine. It is similar to meconium ileus in newborns. DIOS occurs when food and mucus block the bowel. Nearly all people who get DIOS have pancreatic insufficiency. DIOS occurs only in people with CF.

The abnormal bile from the liver has too much acid. This can damage the lining of the small intestine.

Large Intestine

What does the large intestine do?

The large intestine soaks up water and makes feces (stool or waste). Constipation (having too few bowel movements) is a side effect of malabsorption.

How can CF affect the large intestine?

Rectal prolapse is when the inner lining of the rectum comes out through the anus. A doctor can gently put it back in place.

Taking too high a dose of enzymes can damage the large intestine. It becomes swollen and gets narrow. This is called fibrosing colonopathy. Fibrosing colonopathy only occurs in people with CF. It can be avoided by taking the correct dose of enzymes. No one should take extra enzymes without talking to their doctor first.

Pain

When you have pain, your doctor needs to know exactly where your pain is. Pain can have many causes. Knowing where you hurt helps your doctor find out what is causing your pain.

  • Pain in the area over your stomach may be caused by pancreatitis, GERD, or gallstones.
  • Pain in the area around your navel (belly button) may be caused by DIOS and appendicitis.
  • Pain in the lowest part of your abdomen can be caused by malabsorption, DIOS, or fibrosing colonopathy.

References

Robertson MB, Choe KA, Joseph PM; Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 2006;26(3):679-690.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_pangi.html. Accessed June 3, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_liver.html. Accessed June 3, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55.

Nick JA, Rodman DM. Manifestations of cystic fibrosis in adulthood. Curr Opin Pulm Med. 2007;11:513-518.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Hardin DS, Brunzell C, Schissel K, Schindler T, Moran A. Managing Cystic Fibrosis-Related Diabetes (CFRD). Bethesda, MD: Cystic Fibrosis Foundation; 2002.

Cleveland Clinic Web site. http://my.clevelandclinic.org/disorders/Portal_Hypertension/hic_Portal_H.... Accessed June 16, 2008.

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Fallon LF. Cystic Fibrosis. In: Krapp K, ed. Gale Encyclopedia of Nursing and Allied Health. Detroit, MI: Gale; 2002:626-633.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/pdf/DIOS_Children.pdf. Accessed June 16, 2008.

American Academy of Family Physicians Webs site. http://www.aafp.org/afp/991101ap/2043.html. Accessed May 19, 2008.

MedLine Plus Medical Encyclopedia [Internet]. Bethesda, MD: National Library of Medicine (US); Meconium. [updated 2008 Apr 28; Cited 2008 May 19]; [about 2p.] Available from http://www.nlm.nih.gov/medlineplus/ency/imagepages/9616.htm

MedicineNet Web site. http://www.medterms.com/script/main/art.asp?articlekey=6372. Accessed May 19, 2008.

The Geneva Foundation for Medical Eduation Web site. http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=193. Accessed June 16, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/treat_pangi.html. Accessed Jun 3, 2008.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

BONE AND JOINT HEALTH IN

Many people with CF have cystic fibrosis-related bone disease. Some studies show that up to three out of four adults with CF have it. Recent research shows that poor bone health in adults starts during childhood and the teen years. Weak bones can cause serious problems such as fractures, bone pain, and changes in bone shape. Those with severe bone disease cannot have lung transplants. These are good reasons why everyone with CF should learn how to keep their bones healthy.

Bone Remodeling

Bones are living tissue that is always changing. The cycle of changes is called bone remodeling. In remodeling, old bone is broken down and new bone is made. When new bone is made faster than old bone breaks down, your bones get stronger. But when old bone breaks down faster than new bone is made, bones get weak.

Bone Weakness

Under a microscope you can see that normal, healthy bone tissue has holes in it. It looks something like a honeycomb. When the holes are a normal size, they help make bones strong, just as the holes in bricks make them strong. But when the holes get too big, bones get thin and weak. The honeycomb structure gets brittle and it can break easily.

Mild bone thinning is called osteopenia (ahs-te-o-pe-ne-a). Osteopenia affects more than half of all adults and teens with CF. Children with CF can have osteopenia too.

More serious bone thinning is called osteoporosis (ahs-te-o-pa-ro-sis). Osteoporosis means porous bones. It is a disease that causes bones to become weak and likely to break. It can affect any bone in the body. Osteoporosis causes 1.5 million fractures each year in the U.S. Because it is painless and has no symptoms, many people don't know they have it until they break a bone. Up to 77 out of 100 adults with CF will develop osteoporosis.

How CF Affects the Bones

In early childhood, children with CF have bone strength similar to other children. During the teenage years, CF begins to cause problems with bones. Normally, about 85-90% of a person's bone strength has been built by age 18 in girls and by age 20 in boys. During puberty, hormones play a role in helping to grow and strengthen the bones. When strong bones are built during childhood and adolescence, it may prevent osteoporosis later in life when bone loss normally speeds up.

CF can interfere with the bone building timetable by delaying puberty until late in the teenage years. This means that during the early teen years, less bone is built than normal. Teens with CF can enter adulthood with less bone in their "bone bank." This can set the stage for osteopenia or osteoporosis in the adult years.

Reasons for decreased bone density in those with CF include:

  • Poor nutrition. Most people with CF have trouble digesting food, especially fats. This makes it harder to get enough of the vitamins, calcium, and other minerals that make bones strong. Two of the most important vitamins for bone health, D and K, are fat soluble vitamins. Many people with CF have low levels of vitamin D. Without enough vitamin D, the body cannot use calcium well. Taking enzymes and special water-soluble vitamins helps improve overall nutrition, including bone health.
  • Not enough weight bearing exercise. Bones get stronger and denser when they are made to bear weight. Weight bearing exercise includes activities such as walking, lifting weights, climbing stairs, dancing, and playing soccer. If you have not been active in a while, start with just a few minutes a day. Slowly increase the time until you are exercising for 30 minutes a day.
  • Chronic lung disease. Lung infections can make bones weaker. When the immune system responds to infection, proteins called cytokines are released. Cytokines may draw out calcium from the bones. They may also slow new bone growth.
  • Steroid medicines. Nearly everyone with lung disease that is serious enough to consider transplant has some degree of bone disease. After a lung transplant about 7 out of 10 people develop osteoporosis. This is partly caused by corticosteroid medicines that are taken to keep the body from rejecting the transplant.

Risk of Fracture

CF-related bone disease is a concern because it raises the risk of breaking a bone. The risk is the same as for a woman after change of life with severe osteoporosis and a history of broken bones. Compared to healthy people of the same age, people with CF-related bone disease are 100 times more likely to break a bone in the spine. They are 10 times more likely to break a rib.

Between 10 and 40 out of 100 people with CF-related bone disease have an outward curve in the upper back that makes the back looked rounded or humped. It occurs more often in those with severe lung disease. It often gets worse with age, and is often more severe in women than in men.

Monitoring Bone Density

A bone mineral density test (BMD) is a quick, painless x-ray that shows the thickness of the bones in your spine, upper leg, and other places. It can predict your chances for fracture. The test used most often is a DEXA (dual-energy x-ray absorptionmetry) scan. Everyone with CF should have a DEXA scan by age 18. DEXA results are reported as "Z-scores" for those under age 18 and "T-scores" for those 18 and older. This test is repeated every 1-5 years. If your score shows that your bones are weak you will have scans more often.

Treatments

CF-related bone disease is treated with:

  • Good nutrition. A key treatment for CF-related bone disease is maintaining a healthy weight during the teen years, especially around the time of puberty. To do this, you to eat need plenty of calories. It is also very important to get plenty of the nutrients your bones need most—calcium, magnesium, phosphorus, fluoride, zinc, protein, and vitamins D, A, and K.
  • Medicines. Medicines called bisphosphonates make bones stronger by helping with bone remodeling. They can reverse osteoporosis or at least keep it from getting worse. These medicines are now the usual treatment for those with CF-related osteoporosis. However, they are usually not used with children younger than seven.
  • Growth hormone. Children and teenagers treated with human growth hormone gain bone mass. They also grow in height and weight. Growth hormone is not used with adults.

Self Care

Here are some things you can do to help keep your bones healthy:

  • Exercise regularly. To strengthen your bones, do at least 30 minutes of weight bearing exercise each day. Pick activities that you really enjoy. You will be more likely to continue to exercise if you are having fun. If you have osteoporosis, avoid high-impact activities and those that cause you to bend, flex, or lift something heavy. Ask your doctor which activities are safe for you.
  • Get help with depression. Depression is a major risk factor for osteoporosis. Almost 1 in 5 adults with CF has symptoms of depression. There are many good treatments for depression, so do not wait to seek help for this common problem.

To strengthen your bones, do at least 30 minutes of weight bearing exercise each day.

  • Take care of yourself each day. If you smoke, quit. Smoking makes it harder for your body to absorb calcium. Be careful about what you drink too. Caffeine and some soda ingredients may affect your bone health. Do not drink more than three cups of coffee a day and go easy on sodas that contain caffeine. Drinking a lot of alcohol makes it harder for your body to absorb calcium. If you think you may be drinking too much alcohol, tell your doctor or another health care professional. Alcoholism is a big risk factor for osteoporosis for men.
  • Get some sunshine! Without vitamin D, your body cannot absorb calcium well. The best source of vitamin D is not food. It is the sun. Your skin makes vitamin D when it is exposed to sunshine. Spending about 10 minutes in the sun two or three times a week is enough to raise your vitamin D level. In the winter, depending on where you live, you may not be able to get enough sun exposure. Your doctor may suggest taking extra vitamin D or using an ultraviolet lamp or tanning bed to make your skin produce vitamin D. If you are taking antibiotics, check with your doctor before you spend time in the sun. Some antibiotics make you sun-sensitive.

Joint Disease: CFA and HPOA

There are two kinds of joint disease that are problems for people with CF. One is a type of arthritis that comes and goes. The other is HPOA (hypertrophic pulmonary osteoarthropathy).

The arthritis is sometimes called CFA (cystic fibrosis-related arthritis) or CFAA (cystic fibrosis-associated arthritis). It usually starts after age 10. CFA may affect any joints. Most often it affects large joints such as the knees, elbows, shoulders, or wrists. The joints swell and become hot, red, tender, and very painful. A bout of CFA usually lasts less than a week, but can last as long as 10 days. Then it goes away for a while.

CFA often occurs when lung infections get worse. It starts suddenly and may include "flu" type symptoms, fever, and skin rash. CFA is treated with non-steroidal anti-inflammatory (NSAID) medicines, such as ibuprophen. If NSAIDs do not work well, steroid medicines such as prednisolone are also used. Sometimes medicines such as methotrexate or azathioprine are used. About 12 out of 100 people with CF have bouts of CFA. It usually causes no lasting damage to the joints.

HPOA is more common in adults than in children, and usually starts after age 20. It causes inflammation of the membranes around the long bones. It can cause the joints and the ends of the long bones to swell. It usually shows up in large joints and follows a certain pattern. For example, if the left wrist or ankle is affected, the right one is too. HPOA often occurs when lung infections get worse. It may get worse during cold weather. It causes pain in both the bones and the joints. Like CFAA, it is treated with non-steroid or steroid anti-inflammatory medicines. Resting and applying heat can also help reduce the pain, stiffness, and swelling. HPOA is more common in the late stages of lung disease. It may go away if CF is treated aggressively. About 8 in 100 people with CF have HPOA.

How Much Calcium Do You Need?

Age 9 and up: 1300 – 1500 milligrams (mg) a day
Under age 9: ask your doctor

Good food sources of calcium include:

Food Serving Size Calcium Content
Whole milk 1 cup 290 mg
Plain, non- fat yogurt 1 cup 490 mg
Ricotta cheese (part skim) 1 cup 670 mg
American cheese 1 ounce 130 mg
Cheese pizza 1 slice 220 mg
Calcium fortified orange juice 1 cup 300 mg
Spinach 1 cup 240 mg
Almonds ½ cup 190 mg

If your doctor also suggests taking calcium pills, take the calcium carbonate form.

References

Aris RM, Merkel PA, Bachrach LK, et al. Guide to bone heatlh and disease in cystic fibrosis: consensus statement. J Clin Endocrinol Metab. 2005. 90(3): 1888-1896.

Boyle MP. Update on maintaining bone health in cystic fibrosis. Curr Opin Pulm Med. 2006;12(6):453-458.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/pdf/Bone_Health.pdf. Accessed June 3, 2008.

Mayo Clinic Web site. http://www.mayoclinic.com/health/osteoporosis/DS00128/DSECTION=2. Accessed February 21, 2008.

Cystic Fibrosis Foundation. Nutrition: Bone Health and Cystic Fibrosis. Bethesda, MD: Cystic Fibrosis Foundation; 2006.

Hardin DS, Adams-Huet B, Brown D, et al. Growth hormone treatment improves growth and clinical status in prepubertal children with cystic fibrosis: results of a multicenter randomized controlled trial. J Clin Endocrinol Metab. 2006:91(12):4925-4929.

Roche Laboratories Inc. If you're over 50, its time to learn about osteoporosis. Roche Laboratories Inc; 2007.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/main/whatiscf/effects_muscleb.html. Accessed June 3, 1008.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Centers for Disease Control and Prevention Web http://www.cdc.gov/nccdphp/dnpa/physical/everyone/get_active/index.htm. Reviewed March 24, 2008. Updated March 24, 2008. Accessed April 28, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/LungTransplantation/. Accessed May 13, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/teens/living/keeping_bone.html. Accessed June 3, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/main/whatiscf/treat_muscle.html. Accessed June 3, 1008.

Depression and osteoporosis. (Press release.) Harvard Health Publications, June 1, 2007. Available at: http://www.health.harvard.edu/press_releases/depression-osteoporosis.htm. Accessed April 15, 2008.

Cystic Fibrosis Foundation, Patient Registry 2006 Annual Report, Bethesda, Maryland.

Journal of the American Medical Association Patient Page. http://jama.ama-assn.org/cgi/content/full/299/20/2466. Accessed June 22, 2006.

Cystic Fibrosis Education Web site. http://www.cfeducation.ca/pdf/Vitamins.pdf. Accessed June 3, 2008.

Rovner AJ, Stallings VA, Schall JI, et al. Vitamin D insufficiency in children, adolescents, and young adults with cystic fibrosis despite routine oral supplementation. 2007. Am J Cin Nutr. 86:1694-1699.

Thornton J, Rangaraj S. Pharmacological agents (anti-inflammatory and analgesic) for managing symptoms in people with cystic fibrosis-related arthritis [abstract taken from Cochrane Database Syst Rev. 2008 Jan 23;(1):CD006838.]. Available at http://www.ncbi.nlm.nih.gov/pubmed/18254117?ordinalpos=1&itool=EntrezSys.... Accessed July 3, 2008.

Huston G. Joint pain and arthritis in cystic fibrosis. Department of Rheumatology, Seacroft University Hospital, UK. 2002. Available at http://www.cysticfibrosismedicine.com. Accessed July 3, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

OTHER EFFECTS OF

Many people know that CF causes problems with the lungs and the digestive system. But CF can also affect some other parts of the body too.

Muscles

CF can make the leg and arm muscles weak. It can also sometimes cause the diaphragm muscle to be weak. The diaphragm is a thin, parachute-shaped muscle that separates the heart and lungs from the other organs. It is the most important muscle that you use when you breathe. It moves down when we breathe in, and up when we breathe out. Problems with the diaphragm can worsen breathing. CF affects the arm and leg muscles more often than it affects the diaphragm.

Muscle weakness is common among those with CF. It can be caused by many things. Some of the possible causes are:

  • Poor nutrition
  • Not using the muscles very much
  • Weight loss
  • Not enough calories from proteins
  • Inflammation
  • Corticosteroid medicines (the amount of weakness depends on the dose)

Good nutrition and regular exercise can help keep muscles working well.

Bladder

People with CF cough a lot – an average of 643 times a day! Coughing so much puts ongoing stress on the bladder muscles. This can cause a loss of control over urine flow when coughing, sneezing, laughing, or lifting something. It happens when the pelvic floor muscles are weak. The pelvic floor muscles are like a sling that runs from your pubic bone to your tail bone. Urine leakage occurs because the pressure inside the bladder is greater than the pressure from these muscles that control the release of urine.

Loss of control over urine flow affects both men and women, but it is more common in women. About 65 out of 100 women with CF have this problem. Women with poor lung function are most likely to have it.

If you have urine leakage tell your doctor. There are good ways to solve this problem. You can learn how to strengthen your pelvic floor muscles with exercises. The exercises are done by pretending to stop the flow of urine without using any muscles in your abdomen or legs. The pelvic floor muscles are tensed for about 10 seconds, then relaxed. If the exercises do not work for you, there are other treatment options too.

Kidney Stones

About 6 out of 100 people (6%) with CF get kidney stones. Of these people, about 40% get kidney stones more than once. People who do not make much urine are more likely to get kidney stones. Those who often have diarrhea may be more likely to get calcium-based stones. Calcium-based stones are the type that occur most often in those with CF.

Kidney stones cause pain, fever, nausea, and vomiting. If kidney stones are not treated, infection can occur. The main treatments are drinking a lot of water and taking pain relievers. Most kidney stones pass out of the body within in 48 hours. If they do not pass out, other treatments can be done.

Upper Airway

The upper airway is made up of the nose, the sinuses, and the throat. The main job of the upper airway is to filter and warm the air you breathe before it goes to the lungs.

CF can cause two problems in the upper respiratory tract:

Sinusitis Sinuses are air spaces in the bones of your head. There are four pairs of sinuses. They are lined with mucus membranes. Sinusitis is swelling of the mucus membranes in the sinuses. In CF this inflammation can be caused by thick mucus that blocks the sinuses. The blockage may be hard to clear up, and this can lead to a sinus infection.

Adults with chronic (long-lasting) sinusitis may have bacteria in their sinuses that is like the bacteria often found in the lungs. Symptoms of a sinus infection can include a low fever, nasal drainage, tiredness, and nasal polyps. A sinus infection is treated with medicines such as antibiotics, antihistamines, and decongestants. Sometimes the sinuses are drained with surgery. It is very important to treat chronic sinusitis before lung transplant surgery. If surgery is done before sinusitis is treated, the new lungs may get infected.

Nasal Polyps Nasal polyps are small growths of flesh on the lining of the inside of the nose. Nasal polyps are less common than sinusitis. They are often found in children with CF. Polyps do not usually cause problems, but sometimes they can make it harder to breathe. If they block breathing, polyps can be surgically removed or treated with medicine. The cause of nasal polyps is not known.

References

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/main/whatiscf/treat_muscle.html. Accessed June 3, 1008.

A.D.A.M. Medical Encyclopedia [Internet]. Atlanta, GA: A.D.A.M., Inc.; ©2005. Diaphragm; [updated 2007 July 26; cited 2008 July 10]; [1p.]. Available from: http://www.nlm.nih.gov/medlineplus/ency/imagepages/19072.htm.

Med Terms Web site. http://www.medterms.com/script/main/art.asp?articlekey=2983. Accessed July 10, 2008.

Barry SC, Gallagher CG. Corticosteroids and skeletal muscle function in cystic fibrosis. J Appl Physiol. 2003:95(4):1379-84.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_bladder.html. Accessed June 3, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/main/whatiscf/effects_muscleb.html. Accessed June 3, 2008.

Med Terms Web site. http://www.medterms.com/script/main/art.asp?articlekey=6650. Accessed July 11, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_sinus.html. Accessed June 3, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Med Terms Web site. http://www.medterms.com/script/main/art.asp?articlekey=22702. Accessed July 10, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHY DO I NEED SO MANY DOCTORS?

If you have CF (cystic fibrosis) you may see three or four doctors—or maybe even more. You probably also see other types of health care providers. Have you wondered, "Are all of these really necessary? Seeing so many different health care providers can take a lot of time and be tiring. Sometimes you might wonder what all the different people do and if it is even worth it.

Today, there are more than 145 medical specialties. There are so many specialties because no one doctor can know everything about every illness. This is especially true when it comes to a complex long-term illness like CF. This is why many doctors choose to become specialists. Specialists are experts in just one area of care. They work with other doctors to care for people with chronic conditions like CF.

The team approach is the best way to make sure that you get the very best CF care. Your CF team watches your growth, provides advice, and plans your care to help you stay as healthy as possible.

A CF health care team often includes these members:

  • CF Doctor. This doctor leads the CF care team. Often this doctor has internal medicine training, as well as training and experience with CF. He or she teaches other doctors and health care providers about CF. He or she may also do CF research. The CF doctor works with your pediatrician or family doctor to ensure the best care.
  • Nurses. Nurses can have different roles on the CF team. Nurse practitioners or nurse specialists have more training than other nurses. They work closely with the CF doctor and help the other nurses coordinate patient care and education. A CF nurse has extra training in CF care. He or she helps organize the team and teaches you and your family about CF.
  • Dietitian. A registered dietitian gives advice about nutrition. A dietitian advises those who are underweight or malnourished, or at risk for these problems. He or she advises children and parents about enzymes, vitamins, and high-calorie diets. A dietitian can also help with eating behavior problems.
  • Social Worker. The social worker's role is to help ensure a good exchange of ideas and concerns between the family and the care team. He or she also teaches the family how to deal with the stress of an ongoing illness. The social worker helps get teens ready to be on their own. The social worker also helps answer questions about the cost of CF care.
  • Psychologist or Counselor. Psychologists and counselors are mental health workers. They can help children, teens, parents, and families cope with the stress of CF. They can support those who feel grief or fear. They can also teach ways to manage or change children's behaviors. Some counselors can help with spiritual issues too.
  • Pulmonologist. This doctor is an expert in diseases of the lungs and airways. He or she tests lung function and orders medicines or devices that help patients breathe better.
  • Gastroenterologist. This doctor is an expert in problems of the digestive tract. He or she is an expert on the esophagus, stomach, small and large intestines, pancreas, and liver. Gastroenterologists do tests when there are symptoms like stomach pain.
  • Endocrinologist. This doctor works with those who have illnesses that involve hormones. Endocrinologists treat conditions like diabetes, thyroid problems, osteoporosis, or not having enough growth hormone.
  • Worship Leader. This person can offer spiritual support and a listening ear. He or she can help children and parents adjust to life with a chronic illness. A chaplain can provide emotional support and a pastoral point of view when decisions must be made.
  • Respiratory and Physical Therapists. A respiratory therapist gives breathing tests, and is responsible for the care and use of equipment such as nebulizers. A physical therapist teaches people with CF about the benefits of exercise. He or she designs exercise programs based on a person's disease severity, fitness level, and preferences. When someone with CF is in the hospital, either type of therapist can provide airway clearance and other treatments. They also teach people how to do their own care at home.
  • Child Life Specialist. The child life specialist teaches skills for dealing with the stress of CF. The child life specialist also designs activities for visits to the clinic or the hospital. This includes play therapy that helps children of different ages understand their treatments.
  • Your Accordant Care Nurse is another source of information and support for you. Your nurse is always happy to answer your questions and talk about any concerns you have. He or she will work with you one-on-one to help you follow your CF care plan.

References

American Board of Medical Specialties Web site. Accessed April 24, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patient with cystic fibrosis [medical position papers]. J Pediatr Gastroenterol Nutr. 2002;35(3):246-59.

Counselors. Occupational Outlook Handbook, 2008-09 Edition. Washington. D.C.: Bureau of Labor Statistics, U.S. Department of Labor. Updated December 18, 2007. http://www.bls.gov/oco/ocos067.htm . Accessed April 24, 2008.

American Association of Christian Counselors Website. Accessed April 24, 2008.

American College of Physicians Web site. Accessed July 23, 2008.

American College of Physicians Web site. Accessed July 23, 2008.

American College of Physicians Web site. Accessed July 23, 2008.

Children's Hospitals and Clinics of Minnesota Web site. Accessed July 23, 2008.

Gumery L, Dodd M, Parker A, Prasad A, Pryor J, Kennedy N, eds. Clinical Guidelines for the Physiotherapy Management of Cystic Fibrosis. Bromley, United Kingdom: Cystic Fibrosis Trust; 2002.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

YOUR HEALTH CARE TEAM

 

Are you a team player or do you go it alone? Being part of a team when managing a chronic condition makes treatment and daily living easier for you and your medical team. Communication and trust are an important part of making sure you are getting the best possible care.

 

Today, good healthcare requires a team effort. Your healthcare team includes not only your doctors, but also nurses, physician assistants, pharmacists and all the other professionals who participate in your care. The members of your healthcare team are experts in their medical specialties. But the only expert on you... is you! You are the most important member of your own healthcare team.

Studies show that people with chronic conditions tend to view their role on the healthcare team in one of four ways. Which of the following statements best describes your point of view?

  • My doctors really know what they're doing. I let them make all the decisions about my healthcare for me.
  • Nothing I can do will make any difference in my health, so why even try?
  • I'm proactive about managing my healthcare. I know how to get what I need. I'm the patient, so it's up to me to call the shots.
  • I trust the expertise of my doctors. I consider their advice and discuss options with them before making the healthcare decisions that are right for me.

Here's what the statement you chose might indicate about your attitude toward healthcare:

If you chose A, you have probably assumed a passive role in your healthcare. You defer to the decisions of healthcare professionals and usually don't voice your opinion.

If you selected B, you probably feel powerless over your health and believe that "whatever will be, will be." You are withdrawn from your healthcare team and interact with them only as much as is absolutely necessary.

Did you choose C? You probably take a consumerism approach to healthcare. You are well informed about your condition and about the healthcare system. You may feel weighed down by the responsibilities of managing your condition.

If your choice was D you have probably established a cooperative alliance with your healthcare team. You balance the advice of healthcare experts along with your own experience and knowledge of yourself. As a result, you make confident, informed choices about healthcare.

In case you haven't already guessed, the preferred answer is D! This answer represents a person who has embraced the patient's unique and vital role on the healthcare team. If D wasn't your answer, here are some tips that may help you forge a better partnership with your healthcare team.

Become knowledgeable about your condition.

The more you know about your condition, the better equipped you are to work with your healthcare team. As the patient member of the team, you have a responsibility to educate yourself about your condition. Your knowledge will enable you to ask better questions and to make better decisions about your care. If you don't know where to start, ask a librarian to help you do a computer search of some medical libraries.

Study your medical records.

Request a copy of your medical records. (You may be charged for this, so if your records are very large, you may want to only request summaries.) Studying your records will help educate you about your condition. Start a file at home for your records. You can use this file to store notes from your doctor visits, magazine articles about the disease, and other information. This is a positive step toward becoming more active in managing your condition.

Ask Questions.

There are at least two good reasons for asking questions. (1) The answers you receive will build up your knowledge about your medical condition. (2) Asking questions demonstrates that you want to actively participate in your care and that you respect professionals' opinions. This strengthens the patient-team partnership.

Share your concerns.

Are you worried about the costs of your treatments or medicines? Tell your doctor and pharmacist. There may be other alternatives.

Do you need more information? Ask about brochures, videos, books or Web sites. Talk with a nurse or health educator. You may also want to schedule an appointment just to talk with your doctor.

Are the treatments or medicines your doctor is recommending unacceptable or impractical for you? If there's a chance you won't comply with the regimen your doctor is suggesting, be honest about it. Work together to find a solution that is acceptable to both of you.

The point is, if you have concerns of any kind you must voice them. Your feelings and your point of view are critical to the mission you and your healthcare team want to accomplish. After all, you're the most important member of the team!

References

Quackwatch. Doctor-Patient Communication Tips. http://www.quackwatch.org/02ConsumerProtection/commtips.html

United Health Foundation. Take charge of your care. http://www.unitedhealthfoundation.org/charge.html


Last Modified Date: September 10, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

MEDICINES FOR CYSTIC FIBROSIS

Topics:

Here is a look at the medicines most often used to treat CF.

Antibiotics

Antibiotics are medicines that fight germs. They are the main medicines used to keep lung infections from getting worse. For a mild infection, you may be given antibiotic tablets, capsules, or liquids to take by mouth. For a serious infection, or if antibiotics taken by mouth are not working well, your doctor may use IV (into a vein) antibiotics. Some antibiotics are given in an aerosol mist that you breath in. Sometimes using two or more antibiotics at the same time works best.

The antibiotic you need depends on the kind of infection you have:

The Pseudomonas aeruginosa (P. aeurginosa) bacteria is the most widely found cause of ongoing lung infections. The antibiotics most often used to treat P. aeurginosa are:

TOBI® is a form of the antibiotic tobramycin that is breathed in. It was the first aerosol antibiotic made just for people with CF. The FDA approved it in 1997. Right now, 15,000 people around the world are using TOBI®. This medicine helps the lungs work better and lowers the need for other antibiotics that fight this type of germ. TOBI® can be used by those over the age of six.

Zithromax® is one more name for the antibiotic, azithromycin. It is used to treat many problems, such as sore throats and ear infections. It is taken by mouth. In one study, Zithromax® was shown to help lung function and weight gain. The people who took this medicine spent less days in the hospital because of lung infections. It may work well for those with CF because it also lowers swelling in the airways. Zithromax® can be used by those over age six.

Other antibiotics used to treat P. aeruginosa include:

  • Ciprofloxacin
  • Colistin
  • Colimycin
  • Meropenem or Imipenem
  • Ceftazidime or Cefepime
  • Aminoglycosides (tobramycin, gentamicin, amikacin)
  • Chlorampenicol

A Staphylococcus aureus (S. aureus) infection can most often be successfully treated. Most of the bacteria can be killed in two to four weeks. The antibiotics most often used to treat S. aureus infections are:

  • Dicloxacillin
  • Cephalexin
  • Clindamycin
  • Amoxicillin-clavulanate

A Burkholderia cepacia (B. cepacia) infection can be very hard to treat. These bacteria are resistant to many antibiotics. Often, two or more antibiotics are used at the same time. The antibiotics most often used to treat B. cepacia infections are:

  • Meropenem
  • Ceftazidime
  • Aminoglycosides
  • Chloramphenicol
  • Tetracycline
  • Cotrimoxazole

Bronchodilators

Bronchodilators are a common treatment for CF. They are medicines that are breathed in to open up the airways. Some bronchodilators are:

  • Albuterol
  • Terbutaline
  • Salmeterol
  • Formoterol

These medicines also help the lungs work better. When a bronchodilator is breathed in before doing an airway clearance technique (ACT), it helps move mucus. These medicines can be used by those age six and older. (See Accordant web article Airway Clearance Techniques to learn more.)

Mucolytics

Mucolytics are medicines that are breathed in to thin the mucus in the lungs. Thinner mucus is easier to cough out. Two mucolytics are often used:

Pulmozyme® is one more name for the medicine, dornase alfa. It is also known as DNase. This medicine was made just for people with CF. It breaks up mucus and makes it less sticky. This medicine helps the lungs work better. It can help keep CF from getting worse by reducing the chance of getting lung infections. Right now, more than 18,000 people in the U.S. are using Pulmozyme®. It is a daily CF treatment for people over the age of five.

Hypertonic Saline is extra-salty sterile water. (Sterile means it has no germs.)

It thins mucus and makes it easier to clear from the airways. Hypertonic saline helps the lungs work better, mainly if it is used after a bronchodilator. It is a safe, low cost, and useful treatment that helps both children and adults with CF.

Antifungal Medicine

The aspergillus fungus often lives in the airways of people with CF. ABPA (allergic bronchopulmonary aspergillosis) is an allergic response to the fungus. If you get ABPA your doctor may give you Vfend® or Sporanox®. These are antifungal medicines that lower the amount of aspergillus fungus in the lungs. They are taken by mouth once or twice a day until signs go away.

Steroids

A corticosteroid medicine is used along with antifungal medicines to treat ABPA. Steroids fight the lung swelling that happens with the allergic reaction to the fungus. Corticosteroid pills or liquids are given until the signs of ABPA go away.

Some steroids are:

  • Pediapred®
  • Prelone®
  • Medrol®
  • Inhaled Steroids (Flovent®, Advair, Symbicort®, Pulmicort)

Even though people with CF have a lot of lung swelling, experts do not think steroids make it better. This is why they are most often only given to those who also have asthma or ABPA.

Ibuprophen

Ibuprophen is a non-steroidal anti-inflammatory (NSAID) medicine. Non-steroidal means that it has no steroid medicine. Anti-inflammatory means that it shrinks the swelling of the lung tissues. Reducing lung swelling helps stop damage to the lungs. Ibuprophen slows the rate of lung damage. In one four-year study, those who took it had better growth and spent less time in the hospital. The good results of this medicine only show up after taking it every day for a few years. Those age six and older can take this medicine.

References

Cystic Fibrosis Foundation Web site. http://www.cff.org:80/treatments/Therapies/#Clearing_the_Airways. Accessed May 13, 2008.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.

National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html. Accessed May 13, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Driscoll JA, Brody SL, Kollef MH. The epidemiology, pathogenesis and treatment of Pseudomonas aeruginosa infections. Drugs. 2007;67(3):351-368.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176(10):957-969.

Cystic Fibrosis Foundation Web site. http://www.cff.org/research/ResearchMilestones/index.cfm?dspPrintReady=Y. Accessed May 31, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Pipeline/. Accessed May 13, 2008.

Accordant Drug and Technology Guideline Cystic Fibrosis: Tobramycin Inhalation Solution (TOBI®) Last revised March18, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Therapies/Respiratory/Azithromycin/. Accessed May 13, 2008.

Cystic Fibrosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PN: Saunders; 2004:1437-1450.

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/StayingHealthy/Germs/Bcepacia/. Accessed May 13, 2008.

Elborn JS. Practical management of cystic fibrosis. Chronic Respiratory Disease. 2006;3(3):161-165.

Gardner J. What you need to know about cystic fibrosis. Nursing (Lond). 2007;37(7):52-55.

Accessed June 3, 2008.

Accordant Drug and Technology Guideline Cystic Fibrosis: dornase alfa (Pulmozyme®) Last revised March 18, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Therapies/Respiratory/HypertonicSaline/. Accessed May 13, 2008.

Elkins MR, Robinson M, Rose BR, et al; for the National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group. A controlled trial of long-term inhaled hypertonic saline. N Engl J Med. 2006;354(3):229-240.

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/StayingHealthy/Germs/ABPA/. Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/treatments/Therapies/Respiratory/Ibuprofen/. Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/. Accessed May 13, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

BLOOD PRESSURE AND CYSTIC FIBROSIS

Topics:

Q. What is blood pressure?

A. Every beat of your heart forces oxygen-rich blood into the aorta, the largest artery in your body. When the heart contracts, the blood in the aorta is at its maximum pressure. Between heartbeats, blood is at its minimum pressure. A blood pressure reading records these two pressures, which are displayed as a fraction. The top number is the maximum pressure (systolic); the bottom number is the minimum pressure (diastolic).

Blood pressure can vary from one moment to the next. Emotions, posture, physical activity, medications, sleep and other things can cause blood pressure to change.

Q. How can I find out what my blood pressure is?

A. Blood pressure is measured with a sphygmomanometer. This medical instrument has an inflatable cuff that is placed around the upper arm. The upper arm is the best place to take a blood pressure reading because it is approximately the same elevation as the aorta. Blood pressure is expressed as "millimeters of mercury" (mm Hg). This is a measurement of how high mercury rises in a tube under the force exerted by the blood pressure.

Your doctor's office probably checks your blood pressure at each visit. Between visits you can use blood pressure machines available for customers at many pharmacies. You can also purchase a sphygmomanometer for home use.

Q. What do the blood pressure numbers mean?

A. Both of the numbers in a blood pressure reading are important indicators of heart and blood vessel health. Blood pressure of less than 120 over 80 is considered a normal reading for adults. If you have diabetes, a normal blood pressure goal is 130/80. Values of 120-139 over 80-89 are considered "prehypertensive." These people are at higher risk for developing hypertension and need to monitor their blood pressure more closely. A blood pressure equal to or greater than 140 over 90 is considered high. Blood pressure that is lower than your normal value can result from dehydration, internal bleeding, blood loss, or shock from heart failure.

High blood pressure makes the heart work harder and weakens it over time. It also increases the chances of heart attack, stroke, kidney failure or congestive heart failure. Elevated blood pressure usually has no symptoms. Readings in the "high" range (140/90 or greater) could signal current or potential problems. Monitor your blood pressure and tell your doctor if your blood pressure readings are 140/90 or higher.

Q. Can stress affect my blood pressure?

A. Stress is a factor for high blood pressure. Our minds and bodies respond to stress in much the same way that we respond to physical dangers. Stress stimulates the release of the same powerful hormones that enable a person to either flee to safety or defend himself in a crisis. This is the classic "fight or flight" response. A chronic illness like cystic fibrosis can create not only physical stress, but also financial, social, emotional and occupational stresses. Any of these or any combination of these stresses can indeed affect blood pressure.

You may not be able to eliminate all of the stress that accompanies CF. What can change, however, is the way you think about stressful events and circumstances. This is the basic idea behind many of the complementary and alternative treatments categorized as "body/mind medicine." (See the "Pain, Stress and Mood" and the "Complementary and Alternative Medicine" sections of the Accordant Library for information on managing stress.)

Q. Why is it important for patients with cystic fibrosis (CF) to monitor blood pressure?

A. Blood pressure that is either too low or too high gives your doctor important information about your health. Patients, particularly those with cystic fibrosis related diabetes, should pay attention to their blood pressure.

Your doctor may ask you to monitor your blood pressure and to report readings that exceed a specific range. Always follow your doctor's advice concerning blood pressure.

References

Medline Plus Medical Encyclopedia. Blood Pressure. http://www.nlm.nih.gov/medlineplus/ency/article/003398.htm. Accessed October 13, 2009.

American Heart Association. Blood Pressure. Available at: http://www.americanheart.org/presenter.jhtml?identifier=4473. Accessed October 13, 2009.

American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=4623. Accessed October 13, 2009.

American Diabetes Association. http://forecast.diabetes.org/magazine/diabetes-101/tension-mounts-high-blood-pressure Accessed October 13, 2009.

American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=3034848. Accessed October 13, 2009.


Last Modified Date: October 13, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

EXERCISE AND CYSTIC FIBROSIS

Topics:

If you have CF, exercise is very good for you. It makes you breathe deeply, which helps move the mucus in your lungs. Exercise makes your breathing muscles stronger. Most everyone with CF should exercise. Even if your CF is severe, you should talk to your doctor about exercise.

Brisk Exercise

Children with CF are as active as other children. But they often do less brisk types of exercise than other children. An exercise is brisk if it makes you sweat and breathe hard. This is true even if their lung function and nutrition are good. Some children may not take part in brisk exercise because they know their parents, teachers, or coaches are concerned about them. This may cause children to doubt their own ability to do strong exercise. Parents, teachers, and health care team members should allow both boys and girls to be as active as possible. They should support brisk exercise because it could actually help children live longer. Girls often have less lung function than boys do, so they may gain even more from brisk exercise.

One study found that teenagers with mild to moderate lung disease could safely raise the time they spent doing brisk exercise. In this study, each teen had an exercise plan that was made just for them. A physical therapist can design a personal exercise program for anyone with CF. The therapist will decide what to include based on the severity of the person's CF, their fitness level, and the kinds of exercise they like to do.

Words to Know

Dehydrated: when too much water has been lost from the body. People with CF need two kinds of brisk exercise. They need endurance exercise, such as swimming, running, cycling, or taking aerobics classes. They also need strength training exercise, such as lifting weights, to benefit the upper and lower body.

Many people do not want to start an exercise program. This is very natural and not special to CF. But if exercise can be made fun more people will do it. What interests the person with CF? Make sure that person is involved in deciding what is done and how it is done. Build up slowly.

Replacing Salt, Fluids, and Calories

During brisk exercise, it is easy for people with CF to get dehydrated. Because of CF, you lose more salt than other people when you sweat. To make up for this, replace both salt and fluids when you are active. Drink between 6 and 12 ounces of fluid every 20 to 30 minutes while exercising. Avoid drinks that contain caffeine. They increase fluid loss. Water and sports drinks are better choices. Sports drinks contain both salt and carbohydrates. The extra carbohydrates help keep blood sugar up. There is not enough salt in sports drinks to replace the all the salt you lose during exercise. Salty snacks are needed too. Potato chips and pretzels are good choices. Keep in mind that exercise also burns calories. Do not forget to replace the calories you lose through exercise. An extra snack or two can help.

Exercise and ACTs

No matter what type of ACT (airway clearance technique) you use, exercise can make it more effective. By itself, exercise is not a method of airway clearance. But when exercise is added to another ACT, more mucus is removed from the airways. It also slows down the process that makes lung function get worse over time. This is why exercise is now seen as a way to help manage CF. Exercise is a CF treatment that is also a normal part of many healthy people's lives. Exercising helps adults and children with CF feel that they are just like their friends.

Other Benefits of Exercise

People with CF also get:

  • Stronger bones: Bones get stronger and denser when they are made to hold, or bear, weight. Weight bearing exercise includes activities such as walking, lifting weights, climbing stairs, dancing, and playing soccer. Building bone though brisk exercise is important because people with CF are more likely to get brittle bones. If you already have brittle bones, do not do high-impact exercises or those that make you bend, flex, or lift something heavy. Ask your doctor which activities are safe for you.
  • Better blood sugar control: If you have CFRD (cystic fibrosis-related diabetes) exercise may reduce the amount of insulin you need to take. Exercise may also improve your overall blood sugar control.
  • A better body image: Body image may improve as a person with CF builds muscles through exercise.

Regular, brisk exercise also:

  • Helps lower blood pressure.
  • Strengthens your heart.
  • Increases good cholesterol and lowers bad cholesterol.
  • Helps reduce your risk for:
    • Heart disease
    • Colon cancer
    • Breast cancer
    • Stroke
  • Makes the muscles around your joints stronger. This reduces strain on the joints and helps protect them.
  • Helps you relax. Reduces tension, irritability, and stress.
  • Reduces fatigue.
  • Eases depression and anxiety. Exercise releases endorphins, the body's natural "feel good" chemicals.
  • Helps you sleep better at night.

When Not to Exercise

When you have certain problems, you should not exercise until your doctor says it's OK. But after the problem gets better you should start to exercise again. Times when it is good to put off exercise are:

  • When a lung infection is getting worse (acute pulmonary exacerbation)
  • During a bout of painful joints
  • When you feel that you cannot get enough air (breathlessness)
  • After having a baby by C-section
  • If you have a blockage in your bowel
  • If you have pneumothorax (air in the chest or flattened lung)
  • If you are coughing up a lot of blood
  • After you have just had surgery
  • When you have a fever

References

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/AtSchool/TeachersGuide/. Accessed July 9, 2008.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

Gumery L, Dodd M, Parker A, Prasad A, Pryor J, Kennedy N, eds. Clinical Guidelines for the Physiotherapy Management of Cystic Fibrosis. Bromley, United Kingdom: Cystic Fibrosis Trust; 2002.

Nixon, PA, Orenstein DM, Kelsey SF. Habitual physical activity in children and adolescents with cystic fibrosis. Med Sci Sports Exerc. 2001 Jan;33(1):30-5.

Baker CF, Wideman L. Attitudes toward physical activity in adolescents with cystic fibrosis: sex differences after training: a pilot study. J Pediatr Nurs. 2006 Jun;21(3):197-210.

Nutrition: School, Enzymes, and Sports for the Child with Cystic Fibrosis. Bethesda, MD: Cystic Fibrosis Foundation; 2002.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/teens/living/keeping_nutri_exer.html. Accessed July 23, 2008.

McIlwaine M. Chest physical therapy, breathing techniques and exercise in children with CF. Paediatr Respir Rev. 2007 Mar;8(1):8-16.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

Mayo Clinic Web site. Link from http://www.nlm.nih.gov/medlineplus/exerciseandphysicalfitness.html. Accessed April 30, 2008.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/nccdphp/dnpa/physical/everyone/index.htm. Reviewed March 24, 2008. Updated April 10, 2008. Accessed April 30, 2008.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/nccdphp/dnpa/physical/everyone/health/index.htm. Reviewed March 24, 2008. Updated March 26, 2008. Accessed April 28, 2008.

Lorig K, Fries JF. Exercise for fitness and better living. In: The Arthritis Helpbook. 6th ed. Cambridge, MA: Da Capo Press; 2006:133-191.

Mayo Clinic Web site. http://www.mayoclinic.com/health/depression-and-exercise/MH00043. Accessed April 30, 2008.

Centers for Disease Control and Prevention Web http://www.cdc.gov/nccdphp/dnpa/physical/everyone/get_active/index.htm. Reviewed March 24, 2008. Updated March 24, 2008. Accessed April 28, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

IMMUNIZATIONS

Cystic Fibrosis and Flu Shots

Flu shots are recommended for people with cystic fibrosis. People with chronic lung conditions who get the flu can get much sicker and have a harder time recovering. In people with cystic fibrosis, the flu could lead to severe pneumonia. If you develop the flu, you will most likely feel worse than your usual pulmonary exacerbation. The flu shot does not contain live viruses. The nasal spray flu vaccine contains weak live viruses and should not be used by a person with an ongoing illness. The nasal spray vaccine is only approved for healthy people between the ages of 2 and 49 years. Serious problems from flu shots are very rare. Talk to your doctor about getting a flu shot.

The Flu

Influenza, (the flu) is a virus that spreads from person to person. It is caused by influenza viruses. Each year in the US, 36,000 people die from problems related to the flu. Many more must go to the hospital. People with certain health problems are more likely to have serious problems related to the flu.

Flu Symptoms

Signs that you could have the flu include:

  • fever*
  • sore throat
  • cough
  • runny or stuffy nose
  • headache
  • chills
  • feeling very tired or week
  • body ache
  • vomiting and diarrhea (more common in children than adults)

* Not everyone who has the flu will have a fever

The Flu Shot

The best protection against getting the flu is to get the flu shot. This will help protect your body if you later come in contact with flu viruses during the flu season. The flu shot is made from killed or dead viruses. It was shown to be more effective than the nasal spray flu vaccine in healthy adults during the 2007-2008 flu season. The nasal-spray flu vaccine contains weak, live viruses. It is only approved for healthy people between the ages of 2 and 49 years, who are not pregnant.

The shot typically protects against 3 types of viruses that scientists expect to cause the flu during the flu season. The expected viruses change from year to year, so the vaccine changes, as well. There is not a guarantee that you will not get the flu after you have the flu shot. The ability of the flu shot to protect you depends on how closely the shot matches the strain of flu virus that causes illness each year. If you do get sick, your symptoms may be much less severe.

The best time to receive the flu shot is in September or as soon as it is available. Protection begins about 2 weeks later. If you have not received a flu shot early in the season, you should still think about getting one later.

Getting the flu shot will not cause you to get the flu. Serious problems from getting the flu shot do not usually happen. You may have some soreness, redness, or swelling where the shot was given. Some people may experience fever, body aches, and scratchy throat or cough after getting the flu shot. This usually begins after the flu shot and may last 1-2 days.

Who Should Get the Flu Shot?

Anyone who wants to reduce their chances of getting the flu or spreading it to others should get a flu shot. The CDC also recommends the flu shot for children who are 6 months through 18 years of age.

The CDC (Centers for Disease Control & Prevention) says it's extra important for these groups of people to get a flu shot:

  • All children who are 6 months through 18 years of age.
  • All adults who are 50 years of age or older.
  • People who have long-term health problems that affect the lungs, heart, kidney, liver, blood, brain or nervous system, metabolism, and those who are diabetic.
  • People who have a weak immune system caused by medicine or autoimmune disease.
  • People who have any condition that raises the chances of fluid getting into the lungs.
  • Those who live in a nursing home or long-term care clinic.
  • Women who may be pregnant during flu season.
  • Anyone who can transmit the flu to those at high risk, including health care workers and daycare providers.
  • People who live with or care for children younger than 5 years of age or adults older than 50 years of age. It is particularly important for household contacts of children who are 6 months of age or younger to get a flu shot.

You should not get a flu shot if you:

  • Are allergic to eggs.
  • Are sick right now and have a fever. You may get the flu shot as soon as you are well again.
  • Are a child less than 6 months of age.
  • Had a bad reaction to a flu shot in the past.
  • Have developed Guillain-Barré syndrome (GBS) within 6 weeks of getting a vaccine in the past.

Women who may be pregnant should not receive the nasal spray flu vaccine, because it contains live virus.

The H1N1 Flu Shot?

The flu season this year could be much worse, because of a different type of H1N1 flu virus (also called swine flu) that is spreading worldwide. This virus and the regular flu virus could cause a more severe flu season.

The CDC has identified the virus that will be used to make the H1N1 flu shot. The CDC has recommended that the following groups of people receive the H1N1 flu shot in addition to the seasonal flu shot:

  • Women who may be pregnant during the flu season.
  • People who live with or care for children younger than 6 months of age.
  • People between 6 months and 24 years of age.
  • People between 25 and 64 years of age with chronic illness or weak immune system caused by medicine or autoimmune disease.
  • People who work in healthcare or emergency medical services.

At this time, the FDA has approved two doses for children 9 years of age and younger. Receiving the regular flu shot will not protect you from the H1N1 flu. It is important to receive both.

People who may have had a swine flu shot during the 1976 outbreak will still need to get the new H1N1 shot to be protected. The H1N1 flu for this flu season is different.

Prevent the Flu

Most adults can spread the flu to other people up to 1 day before they get sick and up to 5 days after symptoms appear. That means you could spread the flu to people before you know that you are sick. It is very important to help stop the spread of the flu.

Here are some things you can do every day to prevent getting and spreading the flu:

  • Wash your hands often with soap and water, especially after you cough or sneeze. Use an alcohol-based hand cleaner if you do not have soap and water handy.
  • Cover your mouth and nose with a tissue when you cough or sneeze. Throw the tissue away.
  • Try not to touch your eyes, nose, or mouth. This is how germs enter the body.
  • Stay away from people who are sick.
  • Stay home from work or school if you are sick with flu-like symptoms.

What to Do if You Get the Flu

If you think that you have the flu, call your doctor. There are medicines called antivirals that can help with symptoms from the flu. These medicines should be taken within 2 days of getting sick. The drugs can help you feel better and may shorten the number of days you are sick. Your doctor will decide whether you should take antiviral medicines. Some people do not need to take antiviral medicines. Antiviral medicines do not take the place of the flu shot.

For more information about getting a flu shot, visit the Centers for Disease Control and Prevention website at www.cdc.gov/flu

The Pneumonia Vaccine

Pneumonia is a disease of the lungs. Pneumonia is caused by pneumococcal germs that infect the respiratory tract. Pneumonia is a common problem related to the flu and it can lead to death. The flu can also lead to pneumonia.

Getting the pneumonia vaccine is the only way to keep from getting sick with pneumonia. The pneumonia vaccine can prevent serious illness or even death. The pneumonia vaccine is very important, especially if you have long-term illness. Very young children and adults over 65 years of age are more likely to get sick from these bacteria. Those with long-term health problems or a weakened immune system are also more likely to get sick. The vaccine protects against many common types of pneumococcal bacteria, including those most likely to cause disease. This vaccine is safe and effective. This vaccine has very mild side effects in less than 1% of people who get the shot.

There are 2 types of pneumococcal vaccines — one for adults and one for very young children. The polysaccharide vaccine is recommended for adults. This vaccine for adults is a vaccine that contains many different strains of the bacteria. This vaccine is recommended for the following groups of people:

  • Adults aged 65 or older;
  • Anyone aged 2 to 64 years of age with a long-term health problem (e.g., lung disease, cardiovascular disease, or other chronic conditions);
  • Adults aged 19 to 64 years of age
    • who smoke, or
    • have asthma;
  • People who are 2 to 64 years of age who have a weak immune system because of certain medicines (e.g., steroids or certain cancer drugs).

The conjugate vaccine is used in children. This vaccine for children is a vaccine joined to a harmless carrier to provide greater protection against sickness. Conjugate vaccines are used in children who are:

  • under 2 years of age.
  • between 2-5 years of age who have not already gotten the vaccine AND have sickle cell disease, a weak immune system due to illness or medicine, or heart or lung disease.
  • other children under 5 years who attend group day care or are of Alaskan, Native American or African American descent.

The pneumonia vaccine can prevent serious disease in children, such as meningitis and blood infections. It may also prevent some types of ear infections.

You should talk to your doctor about vaccination if you are pregnant. Pneumococcal vaccination of pregnant women is not currently recommended. Some women who have conditions that increase their risk of getting pneumonia may need to be vaccinated before becoming pregnant.

How many doses of pneumococcal vaccine are needed?

If you are 65 years of age or older, a second dose of pneumonia vaccine is advised if:

  • it has been 5 years or longer since your first shot, and
  • you were 64 years of age or younger when you received the first shot.

A second dose is also specifically advised for those people 2 through 64 years of age who:

  • have sickle cell disease
  • are taking medications that weaken the immune system, such as chemotherapy or long-term steroids
  • have kidney failure or nephrotic syndrome
  • have spleen damage or no spleen
  • have HIV infection or AIDS
  • have had an organ or bone marrow transplant
  • have cancer, leukemia, lymphoma, or multiple myeloma

Dosing Schedule for Children Less Than 15 Months of Age

The routine schedule for pneumococcal vaccine is 4 doses: one dose each at 2, 4, 6, and 12-15 months of age. Children who did not receive shots at these ages can still be vaccinated. You should talk to your doctor about the number of doses needed.

Children who are vaccinated as infants will have greater protection against serious disease. Children younger than 1 year of age are more likely to be really sick if they get pneumococcal disease. It is very important to get the pneumonia vaccine on a regular schedule beginning at 2 months of age.

Additional Help is Available

You may still have questions about vaccination. There are many sources of information. You can:

  • ask your doctor or your AccordantCareTM nurse for more information.
  • call the Centers for Disease Control Information line at 1-800-232-4636
  • visit your health plan's web site.
  • visit the Centers for Disease Control web site (http://www.cdc.gov/vaccines).
  • visit the Immunization Action Coalition web site (http://www.immunize.org).

References

Cystic Fibrosis Foundation Flu Fact Sheet. Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/StayingHealthy/Germs/Influenza/ Accessed September 09. 2009.

Centers for Disease Control and Prevention. http://www.cdc.gov/flu/protect/keyfacts.htm. Accessed August 31, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/vaccines/pubs/vis/downloads/vis-flu.pdf Accessed September 19, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/flu/takingcare.htm Accessed August 31, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/Features/Flu/ Accessed September 21, 2009/

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/flu/about/season/current-season.htm Accessed September 19, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/flu/professionals/acip/flu_vax_adults0910.htm Accessed August 31, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/flu/protect/preventing.htm Accessed September 21, 2009.

Centers For Disease Control and Prevention Web site. http://www.cdc.gov/h1n1flu/vaccination/public/vaccination_qa_pub.htm Accessed September 25, 2009.

Center for Disease Control and Prevention Web site. http://www.cdc.gov/flu/symptoms.htm Accessed September 19, 2009.

US Food and Drug Administration Website. http://www.fda.gov/Drugs/DrugSafety/InformationbyDrugClass/ucm100228.htm Accessed September 21, 2009.

National Institute of Allergy and Infectious Diseases. Pneumococcal Pneumonia. http://www3.niaid.nih.gov/topics/pneumonia/ Accessed August 31, 2009.

National Institutes of Allergy and Infectious Diseases Web site. http://www3.niaid.nih.gov/topics/pneumonia/prevention.htm. Accessed September 21, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/vaccines/pubs/vis/downloads/vis-ppv.pdf. Accessed August 31, 2009.

National Foundation for Infectious Diseases Web site. http://www.nfid.org/pdf/factsheets/pneuadult.pdf Accessed September 22, 2009.

Centers for Disease Control and Prevention Web site. http://www.cdc.gov/vaccines/pubs/vis/downloads/vis-pneumoconjugate.pdf Accessed August 31, 2009.

Monto, A. S., Ohmit, S. E., and Petrie, J. G. Comparative efficacy of inactivated and live attenuated influenza vaccines. N Engl J Med. 2009;361(13):1260-1267.


Last Modified Date: October 1, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

QUESTIONS FOR YOUR DOCTORS

Questions...most people with a lifelong illness have them. If you are the parent of a child with CF, very likely, you have questions too. Your questions are important, and your doctor can probably answer most of them. But some people's questions about CF do not get answered – but only because they are never asked!

Why Ask Questions?

People who ask questions usually get answers. Those who ask questions are likely to know more about their disease. They understand more about how their treatments are supposed to help them. People who ask questions often feel like more of a partner with their doctors too.

So, what keeps people from asking their questions?

Some people feel nervous in medical settings. They freeze and forget what they wanted to ask about. Others worry that their questions might seem "stupid" to the doctor. Some people have such high opinions of doctors that they believe they should not "question the doctor."

If you are one of these people, it is time to think again! Asking questions about CF is not only your right, but it is the right thing to do. This is especially true if you are caring for a child with CF. Any question is a "good" question if the answer will tell you what you need to know, or just want to know.

Sample Questions

It is normal for CF patients and parents to have all types of questions. Here are some examples. As you read them, think about what you would like to ask your doctor.

Asking questions about CF is not only your right, but it is the right thing to do.

Medical questions

  • Do I need to do airway clearance therapy even when I am feeling fine?
  • Why do I need to take enzymes if I am just having a snack?
  • Does a lung transplant cure CF?

Questions about how CF will affect your life

  • Can I get pregnant?
  • Is it safe for a person with CF to play sports?
  • Are there any types of work a person with CF should not do?

Questions about sensitive issues

(These questions may be the hardest to ask. Sometimes the best way to start is to say, "I need to ask you a question about something personal.")

  • How can I tell my parents that they are overprotecting me?
  • Can you help me with depression?
  • I am the smallest person in my class at school. Is there anything that can help me grow faster?

Questions about education and support for CF

  • Can you recommend some books or Web sites about CF?
  • Do you know of a local CF support group?
  • Who should I call when I have a question?

Make Three Lists

To get the most out of your time with the doctor, plan ahead. Make three lists:

List #1: Symptoms. If you are seeing the doctor because of a problem, write a list of your or your child's symptoms and answer these questions:

  • When did the symptoms start?
  • How long do they last?
  • What seems to bring them on or make them better?
  • Do they relate to food, medicine, or lifestyle changes?

List #2: Questions. Put all your questions in writing.

  • Put your most important questions first on the list. Ask these questions at the beginning of the appointment. This way, even in a brief meeting your biggest concerns will be addressed.
  • Some people fax or mail their list to the doctor ahead of time.

List #3: Medicines. Make this list if you are seeing a doctor for the first time.

  • Include all prescription and over-the-counter products such as vitamins, herbal products, and antacids. Record the dose and how often you or your child takes it. If you or your child has ever had a bad reaction to a medicine, tell the doctor.
  • You may fill out the chart below, photocopy it, and give it to your doctor.
  • Keep a copy for yourself so you can use it again later.
Medication Why I Take It Dose Times Per Day
       
       
       

Note: Some doctors prefer that you put all your medicines in a bag and bring them to the appointment. Before you make your list, find out if this is what your doctor prefers.

References

National Institute on Aging Web site. http://nihseniorhealth.gov/talkingwithyourdoctor/printerFriendly.html?allTopics=entireTopic&selectedTopics=selectTopic&print=Confirm+print+selection. Accessed March 3, 2008.

National Eye Institute web site. http://www.nei.nih.gov/health/talktodoc.asp. Accessed March 1, 2008.

MedLine Plus [Internet]. Bethesda, MD: National Library of Medicine (US); [updated 2008 March 3] talking with your doctor; [reviewed 2007 Sept 21; cited 2008 March 3]; [1 p]. Available from: http://www.nlm.nih.gov/medlineplus/talkingwithyourdoctor.html.

Lorig K, Fries JF. Working with your doctor: a joint venture. In: The Arthritis Helpbook. 6th ed. Cambridge, MA: Da Capo Press;2006:303-312.

American Academy of Family Physicians Web site. http://familydoctor.org/online/famdocen/home/pat-advocacy/healthcare/837.html. Accessed March 1, 2008.

Agency for Healthcare Research and Quality Web site. http://www.ahrq.gov/consumer/quicktips/doctalk.htm. Accessed March 1, 2008.

Barrett, Stephen. Doctor-patient communication. Quackwatch Web site. Available at: http://www.quackwatch.org/02ConsumerProtection/commtips.html. Revised October 9, 2006.Accessed April 17, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHY QUIT SMOKING?

Topics:

If you have CF (cystic fibrosis) it is not safe for you to smoke. Smoking is not good for anyone, but it is especially bad for people with CF. Smoking makes it harder for your lungs to work right. It irritates the lining of the lungs and increases coughing. It also makes the lungs produce more mucus. These factors make it easier for a lung infection to start.

Smoking may make airway clearance treatments less effective. It may take away the benefits the lungs get from exercise. Smoking is also bad for people with CF because it can reduce hunger. This can result in weight loss. Losing weight due to smoking would be bad for anyone who is already not growing normally.

Secondhand Smoke

Secondhand smoke is any smoke that comes from cigarettes, cigars, or pipes, or that is breathed out by a person who is smoking. If you are the parent of a child with CF, you should not smoke because secondhand smoke is harmful to your child. For those with a certain CF gene type, secondhand smoke can even shorten their lives.

Teach your child to avoid secondhand smoke. Do not allow other people to smoke around your child. The only way to fully protect yourself and your child from secondhand smoke is to make sure you stay in non-smoking settings. Make your home and car smoke-free. Ensure that your child's day care center and school are also smoke-free.

We Know It's Not Easy

You probably hear it from all of your doctors. You must quit smoking! You might even want to quit now. It's very hard to quit smoking. Maybe you have already tried once. Maybe you have tried to quit a few hundred times. The most important thing is that you try again.

It's very hard to quit smoking. Most people do not succeed the first time they try. This is normal and it's nothing to be ashamed about. There is not one right way to quit. What works for you might not work for someone else. There are many tools and aids to help you quit when you decide that you are ready.

The most important part of the process is deciding that you want to quit. Your doctor and your AccordantCare™ nurse can help you quit. Each person who decides that it's time for them does so for a different reason. For some people that reason is health. For other people it's to save money. Still others want to live to see a special life event, like a wedding or graduation. There is no right or wrong reason to quit.

Once you decide that you want to quit there are several things you can do to make it easier:

  • Make the decision to quit smoking. Choose a date to quit that is two to four weeks away. It should be a normal day, not a special day. Special days, like birthdays and holidays, can be stressful enough already. Keep your stress low on your quit day.
  • Get ready to quit. Talk to your doctor about how he or she can help if you decide to use some type of nicotine replacement therapy.
  • Make a list of the reasons why it is important to quit. Keep this list handy and refer to it often. Your list will not be the same as someone else's list. That is great! Your list should be different.
  • Keep track of where, when and why you smoke. If you always smoke when you do certain things and you know that, find a new, healthier habit to replace your cigarettes. To try:
    • Chew sugarless gum or suck on hard candy
    • Go for a walk right after meals
    • Take a different route to and from work
    • Keep your hands busy with a hobby like woodworking or knitting
  • Throw away all tobacco, ash trays, lighters, and anything else that you use when you smoke.
  • Stay away from the people and places that tempt you to smoke. You can return to them after you've been smoke free for awhile.
  • Put the plan into action. Stop smoking! Do things other than smoke. Talk to friends. Read a book. Do things that you like to do.
  • Reward yourself for every day you do not smoke. You could put money in a jar for each day you do not smoke. At the end of a set period of time, buy yourself something special. Whatever your reward system, make it meaningful for you.

The Good News

If you quit smoking, the benefits start right away. You will benefit even if you already have a smoking-related disease. Consider these facts:

  • Just 20 minutes after you stop smoking, your heart rate and blood pressure drop.
  • 12 hours after you stop smoking the carbon monoxide in your blood drops to a normal level.
  • Women who quit smoking before pregnancy, or by their fourth month, cut their risk of having a low birth weight baby. Their risk is the same as for women who have never even smoked.
  • A year after you quit, your risk for heart disease will be half as much as a smoker's risk.

The Best Reason to Quit

The very best reason to quit smoking is that it will help you and your family live longer. About half of all smokers who choose not to quit die from an illness related to smoking. Smoking takes about 13 years of life from the average male smoker. It takes about 14½ years from the average female smoker. About 440,000 Americans die every year because of smoking. The Surgeon General has said that to quit smoking is "the single most important step that smokers can take to enhance the length and quality of their lives."

Are You Ready to Quit?

If you have ever tried to quit smoking, you know it's not easy. Many people try to quit several times before they succeed. Check with your health plan. Many offer special programs to help people quit smoking. If you are thinking about quitting, ask your AccordantCare nurse for help, too.

References

Cystic Fibrosis Foundation Web site. http://www.cff.org/LivingWithCF/StayingHealthy/LungHealth/Smoking/. Accessed May 13, 2008.

Verma A, Clough D, McKenna D, Dodd M, Webb AK. Smoking and cystic fibrosis. J R Soc Med. 2001;94(Suppl.40):29-34.

Tyc VL, Throckmorton-Belzer L. Smoking rates and the state of smoking interventions for children and adolescents with chronic illness. Pediatrics.2006;118(2);471-478.

American Lung Association Web site. http://www.lungusa.org/site/c.dvLUK9O0E/b.4061173/apps/s/content.asp?ct=.... Accessed July 22, 2008.

Cystic Fibrosis Foundation, Patient Registry 2006 Annual Report, Bethesda, Maryland.

Johns Hopkins Medical Institutions. Secondhand smoke exposure worsens cystic fibrosis. Available at: http://www.sciencedaily.com/releases/2008/01/080129160734.htm. Accessed May 15, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/PED/content/PED_10_13X_Guide_for_Quitting_... Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/PED/content/PED_10_2x_Tobacco-Related_Canc.... Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/NWS/content/NWS_1_1x_More_Cancers_Linked_t.... Accessed April 28, 2008.

The Osteoporosis Report: Special Issue. A Quarterly Newsletter for the National Osteoporosis Foundation. Summer 2007. Volume 22, Number 2. http://www.nof.org/osteoporosis/newsletter_PDFs/summer_07.pdf. Accessed April 28, 2008.

International Osteoporosis Foundation Web site. http://www.iofbonehealth.org/news/news-detail.html?newsID=193. Accessed April 28, 2008.

Mayo Clinic Web site. http://www.mayoclinic.com/health/osteoporosis/DS00128/DSECTION=2. Accessed February 21, 2008.

National Osteoporosis Foundation Web site. http://www.nof.org/prevention/risk.htm. Accessed April 28, 2008.

Fact Sheet: Health Effects of Cigarette Smoking. Centers for Disease Control and Prevention Web site. http://www.cdc.gov/tobacco/data_statistics/Factsheets/health_effects.htm. Updated January 2008. Accessed March 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/PED/content/PED_10_2X_Women_and_Smoking.as.... Accessed April 28, 2008.

Chantix Web site. http://www.chantixhome.com/skin_disorders.html. Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/NWS/content/NWS_1_1x_Smoking_Linked_to_Ski.... Accessed April 28, 2008.

Mayo Clinic Web site. http://www.mayoclinic.com/health/smoking/AN00644. Accessed April 28, 2008.

Chantix Web site. http://www.chantixhome.com/smoking_and_cataract.html. Accessed April 28, 2008.

American Dental Association Web site. http://www.ada.org/public/topics/smoking_tobacco_faq.asp. Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/CRI/content/CRI_2_2_2X_What_causes_bladder.... Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/CRI/content/CRI_2_2_2X_What_causes_kidney_.... Accessed April 28, 2008.

Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(1)(suppl 1):1S-39S.

American Cancer Society Web site. http://www.cancer.org/docroot/CRI/content/CRI_2_2_2X_What_causes_stomach.... Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/CRI/content/CRI_2_2_2X_What_causes_pancrea.... Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/CRI/content/CRI_2_2_2X_What_causes_colorec.... Accessed April 28, 2008.

American Heart Association Web site. http://www.americanheart.org/presenter.jhtml?identifier=4545. Accessed April 28, 2008.

American Cancer Society Web site. http://www.cancer.org/docroot/CRI/content/CRI_2_2_1X_What_is_adult_acute.... Accessed April 28, 2008.

Chantix Web site.http://www.chantixhome.com/head_to_toe.html. Accessed April 28, 2008.

Fact Sheet: Women and Tobacco. Centers for Disease Control and Prevention Web site. http://www.cdc.gov/tobacco/data_statistics/factsheets/women_tobacco.htm. Updated November 2006. Accessed April 28, 2008.

March of Dimes Web site. http://www.marchofdimes.com/professionals/19695_1171.asp. Accessed April 28, 2008.

Smokefree Families National Partnership Web site. http://www.helppregnantsmokersquit.org/vp/channels/facts.aspx?. Accessed April 28, 2008.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

CYSTIC FIBROSIS FOUNDATIONS

There are many organizations and Web sites that provide CF patients and their families with helpful information and support. Here are some you may wish to contact.

The Cystic Fibrosis Foundation

The Cystic Fibrosis Foundation
6931 Arlington Road
Bethesda, Maryland 20814
Phone: 301-951-4422 or 800-FIGHT CF (344-4823)
Fax: 301-951-6378
E-Mail: info@cff.org
Web: http://www.cff.org/

CF Foundation Care Centers

CF Foundation Care Centers The CF Foundation supports a network of over 100 care centers across the United States. Each center's care team provides specialized treatment for CF. Teams include a social worker who can provide information about financial assistance, state and federal programs and other valuable information.

A list of all the care centers supported and accredited by the Cystic Fibrosis Foundation can be found at: http://www.cff.org/chapters_and_care_centers/ Or, to find the CF Foundation care center nearest you call 800-FIGHT CF (344-4823).

The Boomer Esiason Foundation

Boomer Esiason Foundation - HEADQUARTERS
52 Vanderbilt Avenue, 15th Floor
New York, NY 10017
Phone: 646-292-7930
Fax: 646-292-7945

Boomer Esiason Foundation - Long Island
200 B Armstrong Road
Garden City Park, NY 11040
Phone: 516-746-0077
Fax: 516-746-4437

E-Mail: info@esiason.org
Web: http://www.esiason.org/

Cystic Fibrosis Research, Inc.

Cystic Fibrosis Research, Inc.
Bayside Business Plaza
2672 Bayshore Parkway Suite 520
Mountain View, CA 94043
Phone: 650-404-9975
Fax: 650-404-9981
Web: http://www.cfri.org/

CFRI provides funds for CF research. It offers a free newsletter and has support groups for teens and adults with CF. There are also support groups for parents and grandparents of children and adults with CF. CFRI sponsors an annual CF Teen and Adult Day Retreat and fund raising events. It also offers ways to volunteer in the fight against CF. The Web site includes a searchable database of CF topics. Under the Web Resources tab you will find a very extensive list of online resources.


Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS Health company. All rights reserved. This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team. This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.