DRUGS & CONDITIONS

TREATING GROWTH HORMONE DEFICIENCY IN CHILDREN

If your child has been diagnosed with growth hormone deficiency, it means that tests have revealed that his pituitary gland is not producing an adequate level of growth hormone. It may be that his pituitary gland was damaged by an injury or disease, or there may be no known reason for the malfunction. Whatever the cause, if your child's hormonal system is not working properly, it needs to be regulated so he can grow at a normal rate. The standard treatment is for the child to take regular injections of human growth hormone.

Humatrope and Genotropin

There are several brand names of growth hormone, including Humatrope, Somatropin, Nutropin and Genotropin. They must be injected. Some children receive injections three or four days a week, but most doctors recommend daily injections. The good news is that the drugs come in a simple delivery system that allows the injections to be given at home. In fact, children over the age of eight often learn to administer the injections themselves. Your child's recommended dose will be based on his body weight so it may increase over time as he gets older.

There are few side effects associated with growth hormone treatment because all the injections are doing is raising hormone levels to normal.

"The side effects in kids are pretty minimal," says Dr. Anthony Karpas, an endocrinologist in Atlanta Georgia who specializes in treating growth disorders. "Some kids have joint pain, but it's not that different than normal growing pains."

A very small percentage of children on growth hormone (2 percent) experience edema -- swelling caused by excess fluid -- or carpal tunnel syndrome. Other side effects can be positive: Children with growth hormone deficiency tend to be a bit pudgy, and once they begin treatment they may lose this excess body fat and their muscle mass increases.

Parents may be concerned that their child will resist daily injections. But in most cases, Karpas says, children with growth hormone deficiency are eager to be treated. "Kids this small are aware that they're short and they're probably being teased for it. They want to get bigger so they're going to have a lot of buy-in."

Your child will most likely grow rapidly (between three and six inches) during the first year of treatment, which for most kids functions as a sort of "catch-up" period. His growth will probably level off during the second and third years of treatment to 3-4 inches per year.

Typically children with growth hormone deficiency continue treatment until they are 16 or 17, at which time they have reached full adult height. Some recommendations specify that treatment should continue until "epiphyseal closure," which means until the hip bones have fused.


References

Interview with Anthony Karpas, MD

Interview with David E. Sandberg, Professor of Pediatric Psychiatry and Psychology

Interview with David M. Cook, MD

Meta-analysis of data in Archives of Pediatrics and Adolescent Medicine: http://archpedi.ama-assn.org/cgi/content/abstract/156/3/230

Minnesota State Task force report: http://www.health.state.mn.us/htac/hgh.htm

KidsHealth article: http://kidshealth.org/teen/diseases_conditions/growth/growth_hormone_p2.html

Coutant R et al. Growth and Adult Height in GH-Treated Children with Nonacquired GH Deficiency and Idiopathic Short Stature: The Influence of Pituitary Magnetic Resonance Imaging Findings

Journal of Clinical Endocrinology and Metabolism. Vol. 86, No. 10 4649-4654. July 2001. http://jcem.endojournals.org/cgi/content/full/86/10/4649


Reviewed by Niki Saxena, M.D., a pediatrician who practices in Redwood City, CA.

Last updated December 18, 2009.

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

TREATING GROWTH HORMONE DEFICIENCY IN ADULTS

Injectable drugs

If your doctor diagnoses a growth hormone deficiency, it means that your pituitary gland is not producing enough growth hormone. You may have been growth hormone deficient as a child, or your pituitary gland may have been injured at some point during your life. Radiation treatment for cancer is one of the more common causes of damage to the pituitary gland. In order to bring your growth hormone level up to normal, your doctor may prescribe somatotropin, or Human Growth Hormone. The dosage you receive will be based on your current growth hormone (GH) level and your age. (Growth hormone levels normally decline with age.)

There are a number of brand names of growth hormone, such as Humatrope, Genotropin, Nutropin, Protropin, and Saizen. All are synthetic forms of somatotropin, and all come in a number of delivery systems. Depending on your dosage you can either use a premixed or powdered formula that is reconstituted with water. All growth hormone supplements must be injected but there are different delivery systems, including "pen" systems that press the medicine through the skin and are useful for those who fear needles. Frequency of dosage varies from a few days a week to every day.

Side effects

Unfortunately, adults are more prone to side effects from growth hormone therapy than children are. Side effects are most common when you first begin taking growth hormone, so your doctor will probably start you on a very low dose (0.1 to 0.3 mg/day) and increase it gradually, monitoring you carefully for side effects.

The most common side effects of GH therapy are swelling, headaches, and carpal tunnel syndrome, likely caused by swelling of the wrists. Aching joints are another common complaint, probably due to swelling. If you experience any of these side effects, your doctor may reduce your dosage until they go away.

Taking growth hormone can also lead to insulin resistance and diabetes, so your doctor will monitor your glucose tolerance. Luckily, the type of diabetes caused by growth hormone is reversible. Because there is also a possibility that growth hormone makes tumors grow faster, your doctor will need to monitor your overall health carefully.

In recent years, patients suffering from the wasting associated with HIV and AIDS are sometimes prescribed growth hormone to build muscle and increase their strength. These patients must take a much higher dosage to rebuild muscle and increase bone density. These doses are typically 10 to 20 times higher than those used for replacement therapy and may be as high as 4 to 8 mg a day.

The most serious side effect of taking growth hormone is called acromegaly, which is an overgrowth of tissue that gradually affects the entire body. The most common early sign of acromegaly is enlargement of the hands and feet. However this is very rare side effect and only occurs at a very high dose of growth hormone.

Finally, although experts don't have any actual evidence that growth hormone therapy causes problems during pregnancy, the FDA has not approved growth hormone use by pregnant women, and it's probably advisable not to take the risk.


References

Interview with Mary Lee Vance, MD

Interview with David M. Cook, MD Endocrinologist

American Association of Clinical Endocrinologists Medical Guidelines for Clinical practice for Growth Hormone Use in Adults and Children -- 2003 update. Endocrine Practice Vol. 9, No. 1 Jan/Feb 2003


Reviewed by Niki Saxena, M.D., a pediatrician who practices in Redwood City, CA.

Last updated February 9, 2009.

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

TREATING TURNER SYNDROME

Some girls are born with a genetic condition known as Turner Syndrome, which causes a group of related symptoms including short stature, late sexual development, and infertility. First recognized in 1938 by a doctor named Henry Turner, the condition occurs when one of the two X chromosomes is either missing or abnormal, with some genetic material missing. Turner's Syndrome is relatively common, occurring in about one of every 2,500 baby girls born. However, the severity of the syndrome varies widely and many girls are not recognized to have the condition until they reach puberty and fail to develop breasts or get their periods. Girls with Turner Syndrome may also have some noticeable physical characteristics such as a webbed neck -- in which the head is attached closely to the shoulders -- or eyelids that look droopy.

Effects of Turner Syndrome

If left untreated, girls with Turner Syndrome will remain very short -- typically 4' 8" or less -- and will not reach full sexual maturity. Their ovaries don't develop normally, resulting in infertility. Some additional complications can occur, including skeletal abnormalities, hearing loss, liver dysfunction, heart and kidney abnormalities, and thyroid dysfunction. If your doctor suspects Turner Syndrome, he will conduct a blood test to screen for the genetic anomaly that causes the condition.

Unlike some other genetic conditions, Turner Syndrome does not cause mental retardation or decreased intelligence. However some girls with Turner Syndrome have learning disabilities, especially in areas involving spatial skills, such as math. They may struggle with tasks such as puzzles and map-reading, or those involving visual organization.

There is no direct connection between Turner Syndrome and psychological difficulties, but girls with Turner Syndrome may struggle with low self-esteem and negative body image due to their differences from other girls. "The features associated with Turner Syndrome can be highly visible," says Dr. David Sandberg, a professor of psychiatry at the University of Buffalo in New York. "It can affect their ability to connect with others." Sandberg says some girls with Turner Syndrome have difficulty with intimacy and "don't develop the same depth of friendships as other girls. They recognize this, and it's hard on them." Sandberg advises parents to encourage their daughters' social development by "getting them involved in group activities where the burden isn't on them to initiate." Sandberg also advises all parents of girls with Turner Syndrome to have them evaluated for learning disabilities.

Treatment options

Although there is no complete cure for Turner Syndrome, many of the characteristics of the syndrome can be treated. If your doctor suspects Turner Syndrome, he will conduct a blood test to screen for the genetic anomaly that causes the condition. Sex hormones such as estrogen and progesterone can be administered at puberty to bring on sexual development and treatment with growth hormone is very effective in bringing girls with Turner Syndrome close to normal height. The infertility associated with Turner Syndrome occurs because the ovaries don't develop fully. While the infertility itself can't be cured, girls with Turner Syndrome can undergo in vitro fertilization and carry a baby to term. Girls with Turner Syndrome who are hypothyroid can be treated with thyroid hormone, which will also promote bone growth.

As they mature and realize that they are different from their peers, girls with Turner Syndrome may feel isolated, which can lead to depression and self esteem problems. It can help to put them in touch with other girls who have the condition through one of the Turner Syndrome organizations The Turner Syndrome Society is a good place to start: www.Turner-Syndrome-us.org or 800-365-9944.


References

http://www.turnerssyndrome.org/

David Sandberg, Professor of Pediatric Psychiatry and Psychology Children's Hospital of Buffalo (New York) (716) 878-7645

Turner Syndrome: A Guide for Families -- Pamphlet (43 pages) produced by the Turner Syndrome Society.

Nemours Foundation. Turner Syndrome. January 2005. http://www.turnerssyndrome.org/


Reviewed by Niki Saxena, M.D, a pediatrician who practices in Redwood City, California.

Last updated September 28, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

TREATING PRADER-WILLI SYNDROME

Prader-Willi Syndrome (PWS), which was named for the two doctors who first identified it, is a complex genetic disorder that causes short stature, weak muscle tone, incomplete sexual development, and obesity, as well as cognitive disabilities and behavior problems. About one in 15,000 babies inherits the genetic abnormality that causes Prader-Willi Syndrome.

DNA methylation analysis test

If a doctor suspects that a child has PWS, she will order genetic testing to confirm the diagnosis. The current test for PWS is called DNA methylation analysis and is more accurate than the tests that were used in the past. If you suspect that your child has PWS, but previous tests were negative or inconclusive, the Prader-Willi Syndrome Association advocates retesting, using the new, more accurate technology.

Physical Signs

There are many physical signs associated with PWS. These include distinctive facial features, such as almond-shaped eyes and small, downturned mouths, small hands and feet, and hair and skin that is unusually light compared with other members of the family.

Babies born with PWS often have feeding problems and poor weight gain in their first year of life, but gain weight rapidly after their first birthday. A defect in the hypothalamus, the region of the brain that regulates hunger and appetite, makes people with PWS feel constantly hungry no matter how much they eat. In addition, decreased muscle mass slows their metabolism so their bodies utilize fewer calories. (Adults with PWS need only 1000-1200 calories a day or they will gain weight.) Without intervention, many children with PWS will be obese by the time they are six.

Children with PWS grow more slowly than other children and often have delayed or incomplete sexual maturity. Boys may have undescended testes and a small penis, while girls may not get their periods.

Cognitive and behavioral problems

There are numerous cognitive and behavioral problems linked to PWS as well. Children with PWS may experience a general developmental delay of one to two years, mild to moderate mental retardation, or learning disabilities. IQ ranges from 40 to 105 with 70 being typical. They may also have problems with speech articulation due to weak mouth muscles. Children with PWS also tend to be clumsy because of weak muscle tone.

A range of behavioral problems is also associated with PWS, including temper tantrums and violent outbursts, depression, and obsessive-compulsive disorder.

But medical intervention, including treatment with growth hormone, may ease many of the problems associated with PWS. In some cases, growth hormone can help children with PWS grow taller, increase their muscle mass, and develop normal sexual characteristics. In addition, growth hormone helps increase strength and coordination. Since regular exercise is very important to keep weight gain down, this aspect of growth hormone treatment is particularly important. Serotonin reuptake inhibitors help to reduce compulsive behaviors, though they've been associated with an increase in suicidal thoughts and behavior. You should always discuss your treatment options thoroughly with your doctor.

A recent research overview at Brown University found that treatment with growth hormone helped reduce behavior problems as well as physical symptoms. Researchers speculate that the improved body image which comes with growth hormone treatment helped combat the tendency toward depression. In addition, PWS patients taking growth hormone also had more physical energy and exercised more, which also had a beneficial effect on their mood.

For more information or support, contact the Prader-Willi Syndrome Association at http://www.pwsausa.org/ or by calling the toll-free helpline, (800) 926-4797.


References

FAQ from Prader-Willi Syndrome Association (USA): http://www.pwsausa.org/faq.htm

Prader-Willi Syndrome Association website: http://www.pwsausa.org/ Fact sheet form PWSA: http://www.pwsausa.org/syndrome/basicfac.htm

Study of using growth hormone in ADULTS with Prader-Willi: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12699450&dopt=Abstract

New study starting http://www.pwsausa.org/research/genotropinstudy.htm

Wattendorf DJ et al. Prader-Willi Syndrome. American Family Physician. Volume 72, Number 5. September 1, 2005. http://www.aafp.org/afp/20050901/827.html

Mayo Clinic. Selective serotonin reuptake inhibitors. December 2006. http://www.mayoclinic.com/health/ssris/MH00066


Reviewed by Niki Saxena, M.D., a pediatrician who practices in Redwood City, CA.

Last updated September 29, 2009.

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

SUPPORTING YOUR CHILD DURING TREATMENT

If your child begins treatment with growth hormone, you will have new concerns to consider. To begin with, there is a danger of unrealistic expectations. Your child has endured endless tests and doctor's appointments, and he has undoubtedly heard lots of discussion about growth hormone and its effects. He may expect that once he begins treatment he will quickly grow as tall as his peers. Of course, with such high hopes he's likely to be disappointed if he doesn't see instant results.

Even if growth hormone treatment leads to immediate results for your child (some kids grow two to four inches during the first year, two to three times their pre-treatment rate of growth), he may be disappointed when his growth rate slows in the following years, which it is likely to do. Your child may feel a sense of failure or even guilt because his body isn't achieving the result he hoped for or that his parents and doctor seem to expect.

Keeping expectations realistic

You can help by talking to your child about what to expect and making sure that his expectations -- and your own -- are realistic. You probably have your own hopes about the treatment, but avoid talking about them too much. After all, there's a very good chance that despite growth hormone treatment, your child will remain at the lower end of the height spectrum once he's an adult. You'll need to do all you can to combat the perception that this is negative, or something your child should be ashamed of. "For some kids, growth hormone treatment can actually be detrimental because there's a very real danger of `medicalizing' the child," says David Sandberg, MD, of Baltimore, Maryland. "Without meaning to, we can make the child feel that he doesn't measure up."

For most children, especially those with Idiopathic Short Stature (ISS) who do not have a growth hormone deficiency, the results of growth hormone therapy will be "very modest -- the child might end up two and a half inches taller than he would have otherwise," Sandberg says. So it's important not to let the relative success or failure of growth hormone therapy become a central focus of your child's life.

Sandberg also cautions parents to think seriously about undertaking growth hormone therapy for a child with ISS. "We make the mistake of thinking that a single factor, such as being short, can have an inordinate effect on a child's life when actually all sorts of other things could be more important. There is no evidence that children who go on growth hormone function better in school or are happier than short children who don't."

Boosting self-esteem

Since short kids may already feel a bit insecure about their physical size and strength, it's important to bolster their self-esteem in other areas. Help your child find activities that he excels in and enjoys, such as academic pursuits, sports, and arts and crafts. If he's musical, encourage him to take up an instrument or join the school band. If he's a crack science student, encourage him to enter your local science fair.

It turns out that some children for whom growth hormone treatment is successful may actually feel sad or experience feelings of loss. Change can be hard -- even positive change -- and a child may miss the attention he received when he was smaller than everyone else, or he may fear losing the role of baby in the family. And, like all kids, as your child reaches puberty he is likely to feel awkward in his growing body as it becomes less familiar to him.

Finally, keep in mind that any type of longterm medical treatment is difficult. Your child's treatment is adding stress to his life. Make sure he has plenty of opportunities to be a kid: to run and play and get dirty, to visit with friends and pursue his creative interests. After all, your child has many unique characteristics, and his height is probably the least important of all.


References

Eric Ralph Frankenfeld, MD Endocrinologist Bellingham, Washington (also associated with St. Joseph's hospital in Bellingham) (360) 647-7800

David E. Sandberg, Professor of Pediatric Psychiatry and Psychology Children's Hospital of Buffalo (New York) (716) 878-7645


Reviewed by Niki Saxena, M.D., a pediatrician who practices in Redwood City, CA.

Last updated October 23, 2009.

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

FOUNDATIONS AND ASSOCIATIONS

Five families who all found themselves coping with growth disorder issues founded the Human Growth Foundation in 1965. Today the organization has over 1000 members and 30 chapters nationwide. The organization provides information and support, as well as grants for research, and opportunities for connecting through its website's chatroom and kids' section.

Toll Free Phone Number: 800-451-6434

 

This organization is another excellent source of both information and support. The Magic Foundation was established in 1989, and now has more than 25,000 members, and provides information on 100 different types of growth disorders. The foundation offers educational materials, a newsletter, extensive support services, and an annual convention.

Phone Number: 708-383-0808 or 1-800-3MAGIC3

 

This Texas-based nonprofit provides a variety of resources for girls with Turner Syndrome and their families. Families can find information on learning disabilities and diet, on the latest research and what to expect as your child approaches puberty. Perhaps most important, the organization has chapters around the country and even around the globe to allow girls with Turner Syndrome and their families to connect with others.

Toll Free Phone Number: 800-365-9944

 

The Prader-Willi Syndrome Association is based in Florida, and has a national network of chapters to provide support to individuals with Prader-Willi Syndrome and their families. The organization also provides information to health care professionals, and sponsors research on the condition. The Association's annual convention also provides opportunities for families to educate themselves and stay connected.

Toll Free Phone Number: 800-926-4797


Last updated October 23, 2009.

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

LEAP FORCE® GROWTH HORMONE CLUB

The CVS Caremark Leap Force® Growth Hormone Club helps members reach their treatment goals.

Growing is not always as simple as it seems. CVS Caremark knows how important it is to have the right attitude in order to produce the best results. Leap Force helps members be successful with treatment. Members can earn exciting prizes by collecting stickers for taking shots as prescribed by their doctor and for ordering their growth hormone treatment one week before they run out.

To learn more about Leap Force, please call CaremarkConnect toll-free at 1-800-237-2767.

DRUGS & CONDITIONS

WHAT IS A GROWTH DISORDER?

A growth disorder is an umbrella term for a range of problems that might be preventing your baby or child from growing as fast as would normally be expected.

These problems may include:

  • Intrauterine Growth Retardation -- This means that the baby grew more slowly than normal in the womb and was born very small for full-term.
  • Failure to Thrive -- slower than normal growth after birth, usually caused by a feeding problem, poor nutrition, or emotional disturbance. May also be a symptom of an infection, illness, or abnormality of an organ or body system.
  • Constitutional Growth Delay -- The classic "late bloomer": children who are small for their age, but growing at a normal rate. X-rays reveal delayed "bone age," which means the skeleton is at a younger stage of development than the child's growth in years. Sexual development will likely be delayed, although there are no symptoms or underlying problem affecting growth. The growth spurt that accompanies puberty comes late for these kids, but they continue to grow longer and catch up.

In some cases, a growth disorder is a symptom of a disease, such as cancer, heart conditions, and problems with the kidneys, lungs, or gastrointestinal tract, but if this is the case, your child will probably exhibit other symptoms as well. Once in a while, however, a growth problem is the clue that leads doctors to diagnose a more serious illness.

Conditions involving the endocrine system are also likely to lead to growth disorders. If the pituitary gland, a small gland at the base of the brain that secretes a number of hormones, is damaged or malfunctioning, it may not produce enough growth hormone. This condition is known as Growth Hormone Deficiency.

Hypothyroidism, in which the thyroid does not produce enough thyroid hormone, may also affect bone growth. According to Dr. Anthony Karpas, an endocrinologist in Atlanta, Georgia, many children referred to growth specialists turn out to have problems with malabsorption, which means they are not getting the full nutritional benefit from the food they eat. "It's not uncommon for kids to come in who turn out to have milk or gluten sensitivity," says Karpas. "Their growth picks up once they're put on a wheat-free and dairy-free diet."

To pinpoint the cause of your child's slow growth, you may be referred to an endocrinologist, a doctor who specializes in the endocrine system, the body's system of regulating hormones. Your pediatrician or endocrinologist may order a number of tests, including X-rays, blood tests, and urine tests. An MRI of your child's pituitary gland may help determine if it is functioning normally. A bone age X-ray of your child's hands and wrists allows the physician to compare them the hands of other children his age.

In rare cases, children's growth lags as a result of other illnesses or genetic factors. A blood test for chromosomal anomalies can test whether your child has Turner Syndrome, a genetic defect that affects only girls. Other conditions that can cause cause a decline in growth hormone production include chronic renal insufficiency, which occurs when the kidneys cease to function, and thalassemia, an inherited condition which causes severe anemia.

Because growth hormone is produced in spurts, the level of growth hormone in your child's blood is not consistent. This means that in order to check whether your child's pituitary gland is producing adequate growth hormone, your doctor has to perform a series of tests over time. Your child will be asked to fast through the night and then will be given a medication, most commonly arginine, clonidine, glucagons, L-dopa, or insulin, to force the pituitary gland to secrete growth hormone. Your child's blood will then be tested over a period of several days to determine the level of growth hormone. Since most growth hormone is produced while your child is asleep, another way to test the quantity of growth hormone is to hospitalize your child overnight and then take blood samples during a 24-hour period.

If no cause is found for your child's small size, this is known as "idiopathic" growth disorder.


References

Interview with Anthony Karpas, MD Endocrinologist in private practice, Atlanta, Georgia

Interview with David E. Sandberg, Professor of Pediatric Psychiatry and Psychology


Reviewed by Niki Saxena, M.D., a pediatrician practicing in the San Francisco Bay Area.

Last updated October 29, 2009.

Copyright © 2003 Consumer Health Interactive