DRUGS & CONDITIONS

ABOUT PULMONARY ARTERIAL HYPERTENSION

Pulmonary hypertension means having high blood pressure in the lungs from various causes. Pulmonary arterial hypertension, or PAH, is increased pressure in the blood vessels that carry blood from your heart to your lungs (pulmonary arteries) to pick up oxygen. PAH is a rare, lifelong health condition.

People with PAH may feel short of breath, dizzy and have trouble staying active. People with severe PAH may have a hard time breathing, even when they are resting. The right treatment and a healthy lifestyle can help improve symptoms and quality of life.

How PAH affects the body

PAH increases pressure in the pulmonary arteries. Pulmonary arteries are the blood vessels that carry blood from the right side of the heart into the lungs so it can pick up oxygen. The average pulmonary artery pressure in someone without PAH is about 14 millimeters of mercury (mmHg) when they’re resting. In people with PAH, the average pulmonary artery pressure is greater than 25 mmHg at rest.

PAH develops because the openings of the blood vessels get smaller. This can be caused by the walls of the blood vessels:

  • Tightening
  • Becoming stiff and narrow from an overgrowth of tissue

 

These changes make it hard for the heart to pump blood through blood vessels, which in turn, increases blood pressure. Over time, the heart has to work harder to pump blood through the smaller blood vessels. This can weaken the right side of the heart. The heart can become too weak to pump enough blood to the lungs. This is called right-sided heart failure. Right-sided heart failure is the leading cause of death in people with PAH.

 


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

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DRUGS & CONDITIONS

SIGNS AND SYMPTOMS

Signs and symptoms of pulmonary arterial hypertension (PAH) may be similar to other health conditions like asthma, heart failure or chronic obstructive pulmonary disease (COPD). Common PAH symptoms may include:

  • Shortness of breath
  • Fatigue (feeling tired all the time)
  • Dizziness, especially when climbing stairs or first standing up
  • Syncope (fainting)
  • Edema (swelling) in the legs and ankles
  • Chest pain, especially during physical activity

 

People with severe PAH may experience an irregular heartbeat, racing pulse, passing out or trouble breathing at rest.

 


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

CAUSES AND RISK FACTORS

PAH affects about 15 in 1 million people. Anyone can be affected. But it’s detected more in women between 20 to 50 years of age. During childhood, PAH affects both girls and boys equally. But after puberty, women are more likely to have it than men. 

The World Health Organization (WHO) divides pulmonary hypertension into five groups, based on the cause, symptoms and how they are treated. Group 1 is PAH. The other groups are different types of pulmonary hypertension often caused by certain heart and/or lung conditions.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

WHO GROUP 1

Pulmonary arterial hypertension (PAH) can be inherited; linked to certain health conditions or drugs and toxins; or of an unknown cause. The World Health Organization (WHO) Group 1, or PAH, is then divided into four sub-categories depending on the cause:

  • Idiopathic pulmonary arterial hypertension (IPAH)
  • Heritable PAH
  • Drug and toxin-induced PAH
  • Associated with other diseases PAH (APAH)
     

PAH sub-categories

IPAH can develop at random and without a clear known cause. Heritable PAH is when the health condition runs or is passed down in a family.

PAH can be related to other causes like exposure to toxins or specific drugs. Drug and toxin-induced PAH can develop from:

  • Using diet pills (appetite suppressants)
  • Using cocaine, methamphetamine or other street drugs
  • Being exposed to toxins in contaminated foods or the environment
  • Taking selective serotonin reuptake inhibitors or SSRIs (a group of medicines often used to treat depression)
     

APAH can develop in people with conditions like:

  • Connective tissue diseases that affect joints and solid organs of the body. Examples include systemic lupus erythematosis (also known as lupus), scleroderma and mixed connective tissue disease.
  • Human immunodeficiency virus (HIV) infection that weakens the immune system, increasing the risk of infections.
  • Portal hypertension caused by liver diseases that increase blood pressure in the veins (blood vessels) that enter the liver.
  • Congenital heart disease caused by a heart defect present at birth. Defects can be simple holes in the heart or much more complex.
  • Schistosomiasis which is an infection caused by a type of Schistosoma parasite.
  • Chronic hemolytic anemia which means having a low number of red blood cell in the blood because they are destroyed. Many factors and conditions like sickle cell disease can cause it.

 


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

SUMMARY OF WHO GROUPS

See the table below for a summary of the five groups of pulmonary hypertension.

The World Health Organization (WHO) Group Summary

Groups of Pulmonary Hypertension

Group 1: Pulmonary Arterial Hypertension (PAH)

  • Idiopathic PAH
  • Heritable
    -    BMPR2
    -    ALK-1, ENG, SMAD9, CAV1, KCNK3 
    -    Unknown
  • Drug- and toxin-induced
  • Associated with
    -    Connective tissue diseases
    -    HIV infection
    -    Portal hypertension
    -    Congenital heart diseases
    -    Schistosomiasis

Group 1*: Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

Group 1**: Persistent pulmonary hypertension of the newborn (PPHN)

Group 2: Pulmonary hypertension due to left heart disease

Group 3: Pulmonary hypertension due to lung diseases and/or hypoxia

Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH)

Group 5: Pulmonary hypertension with unclear multifactorial mechanisms

Adapted from Simonneau G, Gatzoulis M, Adatia I, et al. Updated Clinical Classification of Pulmonary Hypertension. J Am Coll Cardiol. Vol. 62, No.25,Suppl D, 2013, December 24, 2013:D34-41. 5th World Symposium on Pulmonary Hypertension 2013, Nice, France. World Health Organization.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

DIAGNOSIS

Doctors diagnose pulmonary arterial hypertension (PAH) by doing a careful, full medical exam. They will:

  • Gather information about medical and family history
  • Do a physical exam to look and feel for symptoms
  • Order tests, labs and other procedures
     

Tests and procedures

Early symptoms like shortness of breath or fatigue are similar to other conditions. So, doctors use many tests and procedures to diagnose PAH, including:

  • Chest x-ray to see if the blood vessels that carry blood from the heart to the lungs (pulmonary arteries) appear to be enlarged.
  • Echocardiogram is also known as an "echo." This test takes pictures of the heart muscle, heart valves and estimates pulmonary artery pressure. It’s the most useful screening test for PAH and other types of pulmonary hypertension.
  • Electrocardiogram (ECG) measures electrical activity in the heart. This test looks for heart damage and/or abnormal heart beats or rhythms.
  • Pulmonary function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. These tests help detect if a lung disease is causing the pulmonary hypertension.
  • Right heart catheterization allows doctors to exactly measure blood pressure in the right side of the heart and the pulmonary artery. It’s the only test that directly measures the pulmonary blood pressure and is considered the most accurate. The test can also measure the amount of blood pumped out of the right side of the heart with each heart beat. Cardiac catheterization is only performed in a hospital, by specialists.
  • The six-minute walk test measures the distance a person can walk in six minutes. It’s used to monitor the severity of pulmonary hypertension, progress of the disease and response to treatment.
     

Doctors may order other tests to rule out other conditions.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

TREATMENT

Pulmonary arterial hypertension (PAH) has no cure. But treatment may help:

  • Relieve symptoms
  • Improve ability to exercise
  • Slow the progress of the disease
     

PAH is treated with medicines, procedures and other therapies. The exact treatment depends on both:

  • The type of pulmonary hypertension (based on the WHO group system)
  • Its severity (based on the WHO functional class [WHO-FC] system)
     
DRUGS & CONDITIONS

WHO FUNCTIONAL CLASS

The severity of pulmonary arterial hypertension (PAH) is based on the World Health Organization functional class (WHO-FC) system. There are four classes of WHO-FC, ranging from class I (no symptoms) to class IV (symptoms even at rest):

The World Health Organization Functional Class (WHO-FC)

Functional Class of Pulmonary Hypertension

Class I

No limitations of activity; no pulmonary hypertension symptoms* from usual activity

Class II

Mild limitation of physical activity; comfortable at rest but usual physical activity causes pulmonary hypertension symptoms

Class III

Marked limitation of physical activity; comfortable at rest but less than usual activity causes pulmonary hypertension symptoms

Class IV

Unable to perform any physical activity without symptoms; may have signs of right heart failure;  have shortness of breath and/or fatigue even at rest; pulmonary hypertension symptoms increase with any physical activity

*Pulmonary hypertension symptoms include feeling short of breath, fatigue, chest pain or feeling faint or dizzy Adapted from McGoon M, Gutterman D, Steen V, et al. ACCP Evidence-Based Clinical Practice Guidelines: Screening, Early Detection, and Diagnosis of Pulmonary Arterial Hypertension. Chest. 2004;126:14S–34S. Rubin, LJ. ACCP Evidence-Based Clinical Practice Guidelines: Diagnosis and Management of Pulmonary Arterial Hypertension: Introduction. Chest. 2004;126:7S–10S.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

MEDICINES

Many long-term medicines are available and approved by the U.S. Food and Drug Administration (FDA) for the treatment of PAH. Each group differs in how they help to reduce the blood pressure and/or workload on the heart. Depending on the medicine, they can be infused, injected, inhaled or taken by mouth (orally). See the table below for selected medicines FDA-approved to treat PAH.

Selected FDA-approved PAH Medicines

Group Name

How It Works

Selected Medicine Name

How It's Given

Common Side Effects

Endothelin-receptor antagonist (ERA) Relaxes the blood vessel walls and decreases the pulmonary blood pressure to the heart Bosentan
Ambrisentan
Macitentan
Oral Headache, nausea, increased liver enzyme levels, anemia, edema (swelling)
Phosphodiesterase-5 (PDE5) inhibitor Relaxes and open up blood vessel walls in the lungs and decreases the pulmonary blood pressure to the heart Sildenafil
Tadalafil
Oral Headache, low blood pressure, prolong erection in men, muscle ache, indigestion
Soluble guanylate cyclase (sGC) stimulator Relaxes blood vessels and increase the blood supply to the lungs Riociguat Oral Headache, upset stomach, dizziness, low blood pressure, anemia, heartburn, constipation
Prostacyclin Opens ups the arteries in the lungs and prevent blood clots from forming Treprostinil Oral, inhaled, IV or SC Headache, nausea, diarrhea, flushing, low blood pressure, jaw pain, low platelets,. For SC only: rash, site pain, redness or infections
Iloprost Inhaled Sore throat, cough, chest pain
Epoprostenol IV IV line infections, blood clots, skin reactions to the tape used to secure the IV line

IV=intravenous; SC=subcutaneous


Doctors may prescribe other medicines like calcium channel blockers, diuretics (water pills), blood thinners and digoxin to help with certain PAH symptoms. Work with your doctor to decide which treatment option is best.

 


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

SIDE EFFECTS

PAH medicines may cause various side effects depending on:

  • Which medicine is used
  • What the dose is
  • How the treatment is given
     

People who take PAH medicine and have bothersome side effects should talk to their doctor. Their doctor can give advice on how to manage side effects so that they can keep taking them. For example, to relieve nausea and vomiting, the doctor may suggest:

  • Eating smaller meals
  • Avoiding carbonated drinks
  • Avoiding greasy and fatty foods
  • Drinking cold liquids like ice water or ice tea often


PAH medicine can only help if it’s taken exactly has instructed. That’s why it’s best to keep taking PAH medicine even if bothersome side effects don’t go away fully. Never stop or change how you take your medicine without talking to your doctor first.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

SURGERY

Some people with PAH may need one or more surgeries to help relieve symptoms and improve the WHO-FC. Surgical procedures used to treat PAH include:

  • Lung transplant—Surgery that replaces the damaged lung with a healthy, donated lung.
  • Heart-lung transplant—Surgery that replaces both the heart and lung with healthy, donated organs.
  • Atrial septostomy—Procedure that relieves the pressure in the right side of the heart and increases blood flow. Atrial septostomy is rarely done in the United States.
     

This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

HEALTH AND WELLNESS

Most people with pulmonary arterial hypertension (PAH) can live full, active lives by:

  • Creating a healthy lifestyle
  • Sticking to their treatment plan
     

If you have PAH, work with your doctor and other health care providers to make lifestyle changes, learn healthy habits and create a PAH treatment plan.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

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DRUGS & CONDITIONS

LIFESTYLE CHANGES

Eat healthy

Take extra care to eat a healthy, balanced diet to keep your heart as healthy as possible. Avoid food and drinks high in fat, cholesterol, sugar, salt and alcohol. Focus on:

  • Fresh fruits and vegetables
  • Whole grains
  • ean protein and dairy
  • Plenty of water
     

Ask your doctor about ways to eat better or if you should follow a special diet.
 

Watch your weight

Get to and stay at a healthy weight. Extra weight makes your heart work harder and increases your risk for other health conditions. Your doctor can help determine your goal weight. If you need to lose weight, work with your doctor to create a healthy weight-loss plan.
 

Stay active

Most people with PAH can benefit from low-impact exercise like walking or swimming. Your exercise plan will depend on your PAH symptoms and what you enjoy. Ask your doctor to create an exercise plan that works for you.

In general, all people with PAH should follow these exercise tips:

  • Stay away from lifting more than 10 to 15 pounds. It can add too much stress on the heart.
  • Pace yourself and don’t overdo it. Exercising too much or hard can bring on the symptoms of PAH like shortness of breath or dizziness.
  •  And some people with PAH should not do certain exercises. Check with your doctor before starting a new activity.
     

Get rest

Proper rest can help you fight fatigue, a common symptom of PAH. This includes getting the right amount and quality of sleep each night. Talk to a doctor if falling or staying asleep is a problem. Follow these restful sleep tips:

  • Go to bed at the same time each night
  • Get up at the same time each morning
  • Don’t stay up too late
  • Avoid drinking caffeine or alcohol late in the day
  • Don’t eat big meals or snacks before going to bed
  • Create a quiet, peaceful place to sleep
  • Avoid naps during the day (children three years old and younger need daytime naps)
     

Reduce stress

Living with and managing PAH can be stressful. Fatigue and shortness of breath may get in the way of living your daily life. And emotional stress can decrease your quality of life. It can also affect your overall blood pressure. A healthy diet, exercise and sleep can help you cope. Other ways to help lower stress include:

  • Make a plan for the day. Focus on the key tasks and spread them out throughout the day.
  • Take time to recharge. Relax, take deep breaths, stretch, take a bath or spend time with nature.
  • Pamper yourself. Try massage therapy, meditation, yoga or new hobbies.
  • Connect with friends, family or support groups. Don’t be afraid to get help from a counselor or therapist if you feel overwhelmed.
  • Work on the things that you can change or improve. Let go of things that you can’t control.
     

This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

HEALTHY HABITS

Get vaccinated

Cold, flu and lung infections can be serious in people with PAH. Protect yourself by staying up-to-date with your vaccinations. Ask your doctor about getting your pneumonia vaccine and yearly flu shot. Talk to your family and friends about staying up-to-date too.
 

Quit smoking

Smoking causes your blood vessels to narrow, which in turn causes your blood pressure to increase. Nicotine also makes it harder for your blood cells to carry oxygen. If you smoke, talk to your doctor about ways to quit.
 

Avoid becoming pregnant

Pregnancy can put an extra workload on the heart and lungs in women with PAH. Some of the medicines used to treat PAH can also be harmful to the fetus. Doctors advise women of childbearing age with PAH to avoid becoming pregnant.

That said, contraceptives like birth control pills, patches and vaginal rings may increase the risk of blood clots which can worsen PAH symptoms. Talk to your doctor about your birth control options.
 

Plan ahead before traveling

People who use oxygen tanks should check with their doctor before traveling. If you’re flying on a plane, you may need a oxygen tank even if you don’t use it on a regular basis. That’s because the pressurized cabins of planes in the air contains less oxygen than air on the ground. Ask your doctor if you need to travel with an oxygen tank. Your doctor will provide the airline with information about your medical condition and the need for an oxygen tank.
 

See your health care team regularly

Make and keep appointments with your regular doctor, specialists and other providers. You may notice changes in your health. Talk to your doctor if you notice any changes in your symptoms, activity level or weight. Your doctor may need to monitor or change your treatment plan.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

CATHETER CARE

Some PAH medicines are given by continuous intravenous (IV) infusion into the body. Special catheters (soft hollow tubes) are needed to deliver the medicine directly into the vein. There are various types of catheters. Your doctor will choose the one that’s best for you.

People with catheters are at risk of getting catheter-related infections. Germs can enter the body through where the catheter is placed and cause catheter site infection. They can also enter through the end of the catheter line and cause infections in the blood. These infections can be serious. It may take weeks of antibiotics in a hospital to help treat them. These infections may even make PAH symptoms worse.

Know the signs of catheter-related infections and get treatment fast. Call your doctor right away if you notice any of these signs:

  • The catheter site has fluids around it, feels warm or has a smell
  • The catheter site and under the skin is red or feels tender
  • You have a fever of 100.5° F (38° C) or higher
  • You have flu-like symptoms like chills
  • You feel more tired than usual
     

The good news is that you can help prevent catheter-related infections by following these catheter care tips:

  • Wash your hands with soap and water before caring for your catheter or preparing to give your medicine.
  • Keep the end of the catheter protected and closed.
  • Keep the catheter site clean and dry by using special cleaning solutions and dressings.
  • Follow your doctor’s instructions exactly on mixing, administering and storing the medicine.
  • Call 911 and/or get medical help right away if the catheter falls out, leaks, cracks or becomes loose.
     

If you have any questions about how to care for your catheter, call your doctor or CVS Specialty™ PAH CareTeam. They can answer any catheter care questions. They can also arrange for nurses to come to your home until you feel comfortable caring for your catheter.


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

EMERGENCY PLAN

PAH is a rare health condition that needs special medical care. And many local hospitals and health care providers may not know you have PAH or be fully prepared to care for you. Know when to get medical help, and help providers by having a plan in case of an emergency.

Ask your doctor when to call 911 or get medical help. Some examples may include:

  • Having trouble with a catheter or pump
  • High fever or a fever that doesn’t go away, especially if you have an IV catheter in place
  • Fainting or feeling like you might faint
  • Coughing up blood or colored mucus
  • Chest pain
  • Fast or irregular heartbeats
  • Feeling more short of breath than usual
  • Swelling more than usual


Create a plan with your doctor on what to do in an emergency. Then follow these tips to be prepared:

  • Keep an updated list of all of your medicines. Include doses, concentrations, rates for infusion and your oxygen therapy, if you use it.
  • Have a three-day supply of all medicines and supplies to avoid a break in your therapy.
  • Keep a list of emergency contacts.
  • Teach and work with your local hospital(s) so they have your PAH medical and treatment information.
  • Keep a doctor’s note. Have your doctor fill out a note that has information about PAH, your treatment plan and doctor’s contact number.
  • Give a copy to your local hospital and all your health care providers. Have one with you at all times in case of an emergency.
  • Have tools like a thermometer and blood pressure cuffs handy to check for symptoms.
  • Talk to your doctor about getting a backup infusion pump or oxygen tank.


Always carry an emergency wallet card that describes your PAH. It should include:

  • Your name
  • Your PAH diagnosis and treatment
  • Emergency contacts’ names and numbers
  • Your doctor’s emergency contact information

 


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

STAY ON TRACK

It can be hard to remember when and how to take your PAH medicine, however, medicines work the best to manage PAH if they are taken exactly as prescribed. Never change or stop your medicine without talking to the doctor who prescribed them. Follow these tips to help stay on track for your best outcomes.

  • Take medicines as part of a daily routine
    • It’s best to take medicines the same time(s) each day. For example, when eating breakfast or setting an alarm before bed
       
  • Use medicine reminders
    • Set a standing alarm on the cell phone. Use the CVS Specialty mobile app―anyone can download and use it free from Google Play or Apple® iTunes® stores
    • Post a sticky note where it can be seen easily. For example, on the bathroom mirror or refrigerator
    • Schedule tasks or appointments on the computer
    • Use a pill box or make a check-off chart
  • Make and keep doctor and blood work appointments
    • Doctors may order blood work or other tests to check for response to treatment. Write down and keep all your appointments. Skipping could affect treatment goals
  • Join a support group
    • Join an in-person or online support group. Talking to others living with PAH may help people cope with treatment and everyday challenges
       
  • Work with the entire health care team
    • Work closely with doctors, pharmacists, nurses, laboratory and other providers. Stay in touch and don’t be afraid to ask questions. Doctors and CVS Specialty PAH CareTeam clinicians can give advice on:
      • relieving side effects
      • what to do if you miss a dose
      • creating an easy-to-follow medicine schedule and more
         
  • Go online
    • Create a secure, online specialty prescription profile to manage your PAH treatment anywhere, anytime. Just visit our homepage and register in a few easy steps.
       

    This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

    CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

    Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

    ©2016 CVS Specialty. All rights reserved. 75-36601F 122215

DRUGS & CONDITIONS

SUPPORT

Ready to Get Started with Us?

Please call CVS Specialty toll-free at 1-800-237-2767 to start filling your pulmonary arterial hypertension (PAH) prescriptions with us. We can help you get the best possible outcomes for a better quality of life. And help is always available―365 days a year.
 

Already Enjoying Our Services?

Contact your CVS Specialty PAH CareTeam toll-free at 1-800-237-2767 if you have questions or concerns about your health or treatment. A pharmacist, nurse or counselor is ready to help.
 

Resources

Learning about PAH gives people more tools to manage their health and live better. Visit these websites to learn about PAH treatment, tips for managing daily life, help for caregivers and links to other PAH news.

Pulmonary Hypertension Association (PHA)
http://www.phassociation.org

American Lung Association
http://www.lung.org

American Heart Association
http://www.heart.org/HEARTORG

National Heart, Lung, and Blood Institute
http://www.nhlbi.nih.gov
 


This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about the information you find. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

©2016 CVS Specialty. All rights reserved. 75-36601F 122215