Idiopathic Pulmonary Arterial Hypertension

WHAT YOU SHOULD KNOW:

• Idiopathic pulmonary arterial hypertension is also called IPAH. This is a condition where the pressure in your pulmonary artery is increased. The cause of IPAH is not known. It occurs when the small arteries in your lungs grow smaller, making it harder for blood to flow through your lungs. While you may have no symptoms at first, over time you may get weak and more tired than usual, have chest pain or trouble breathing, and you may faint.
  
  
• Your caregiver will ask you about diseases or conditions that you have had and their treatment. He will examine you, and do blood and imaging tests. The imaging tests may include a computed tomography (CT) scan, magnetic resonance imaging (MRI), or echocardiography. Treatment for IPAH may include oxygen and medicines, such as blood thinners and diuretics. If your condition worsens even with medicines, you may need to have surgery. You may need to have a shunt (special tube) placed in your heart, or one or both of your lungs may need to be replaced with healthy lungs from a donor. You may need to have both lungs and your heart replaced. There is no cure for IPAH, but with medicines and surgery your condition may stay the same, or improve.

AFTER YOU LEAVE:

Medicines:

• Keep a written list of the medicines you take, the amounts, and when and why you take them. Bring the list of your medicines or the pill bottles when you see your caregivers. Learn why you take each medicine. Ask your caregiver for information about your medicine. Do not use any medicines, over-the-counter drugs, vitamins, herbs, or food supplements without first talking to caregivers.
  
  
• Always take your medicine as directed by caregivers. Call your caregiver if you think your medicines are not helping or if you feel you are having side effects. Do not quit taking your medicines until you discuss it with your caregiver. If you are taking medicine that makes you drowsy, do not drive or use heavy equipment.

• Blood thinners: Blood thinners are medicines that help prevent clots from forming in the blood. Clots can cause strokes, heart attacks, and death. Blood thinners may cause you to bleed or bruise more easily. Do the following if you are taking a blood thinner:
  
  
  • Watch for bleeding from your gums or nose, or in your urine or BMs.
    
    
  • Use a soft washcloth on your skin and a soft toothbrush to brush your teeth. Doing this can keep your skin and gums from bleeding.
    
    
  • Tell your dentist before dental cleanings, and other caregivers before other procedures, that you take blood thinning medicine.
    
    
  • If you shave, use an electric shaver.
    
    
  • Do not play contact sports since you may bleed or bruise easily.
    
    
  • Wear a MedicAlert bracelet or necklace that says you are taking a blood thinner medicine. You may get one from your local drugstore or contact the MedicAlert Foundation.
  
  
• Diuretics: This medicine is often called "water pills". Diuretics help your body get rid of extra fluid (edema) in your legs and ankles. This medicine may also help get rid of extra fluid in your lungs or around your heart. It may also decrease your blood pressure. You may urinate more often when taking diuretics.
  
  
• Vasodilators: These medicines widen (make bigger) the diameter of your blood vessels and help improve blood flow.

Ask your caregiver when to return for a follow-up visit. Keep all appointments. Write down any questions you may have. This way you will remember to ask these questions during your next visit.

Activity:

• If you feel dizzy or lightheaded, or you often have trouble breathing, limit your activities. Pregnancy and giving birth increases the work of your heart and lungs. If you are a female with IPAH, you and your sexual partner may need to take steps to prevent pregnancy. This may include male vasectomy, using oral contraceptives (birth control pills), or using other methods such as condoms. Talk to your caregiver about ways to prevent pregnancy.
  
  
• You may have trouble breathing when lying down. Sleeping in a position with your upper body raised may help you breathe easier. You can use foam wedges or elevate the head of your bed. Talk to your caregiver about devices that raise your upper body higher while you are lying in bed. Use a device that tilts your whole body, or places you in a sitting position. The device should not bend your body at the upper back or neck.

Diet:

• Eat a variety of healthy foods every day. Your diet should include fruits, vegetables, breads, dairy products, and protein (such as chicken, fish, and beans). Eating healthy foods may help you feel better and have more energy.
  
  
• Ask your caregiver if you should be on a special diet. You may be told to eat foods that are low in fat or cholesterol. You may also be told to limit the amount of salt you eat. Special cookbooks can make it easier to plan low fat and low salt meals.
  
  
• Weighing too much can make your heart work harder and can cause serious health problems. Talk to your caregiver about a weight loss plan if you are overweight.

Drinking liquids: You may need to decrease the amount of liquids that you drink. Ask your caregiver how much liquid you should drink each day.

For more information: Contact any of the following:

• American Lung Association
  61 Broadway, 6th floor
  New York City, NY 10006
  Phone: 1-800-586-4872
  Web Address: http://www.lungusa.org
  

• National Heart, Lung and Blood Institute
  Health Information Center
  P.O. Box 30105
  Bethesda, MD 20824-0105
  Phone: 1-301-592-8573
  Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
  

CONTACT A CAREGIVER IF:

• You have a fever (increased body temperature).
  
  
• You have new symptoms since the last time you visited your caregiver.
  
  
• Your symptoms stop you from doing your daily activities.
  
  
• You have questions or concerns about your condition, medicine, or care.

SEEK CARE IMMEDIATELY IF:

• You have chest pain or palpitations (strong, fast heartbeats).
  
  
• You have sudden swelling in your legs or abdomen (stomach).
  
  
• You have sudden trouble breathing.
  
  
• You are vomiting (throwing up) and are not able to eat or drink.
  
  
• You had a fainting spell or loss of consciousness.

Copyright © 2008 Thomson Healthcare Inc. All rights reserved. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.

The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

Link to Page

Print Page

Email Page