Familial Pulmonary Arterial Hypertension

WHAT YOU SHOULD KNOW:

• Familial pulmonary arterial hypertension is also called FPAH. This is a condition where the pressure in your pulmonary artery, connecting your lungs and heart, is increased. It may be caused by problems with your genes that may have been passed down to you by your parents. A gene is a little piece of information that tells the body what to do or what to make. Hypertension occurs when the small arteries in your lungs get 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 the usual, have chest pain or trouble breathing, and you may faint.
  
  
• Your caregiver will ask about medicines you have taken, and diseases or conditions 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 may include oxygen and medicines, such as blood thinners, diuretics ("water pills"), and ones to widen your arteries. 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 FPAH, but with treatment, your condition may stay the same, or improve.

INSTRUCTIONS:

Take your medicine as directed:

Call your primary healthcare provider if you think your medicine is not working as expected. Tell him if you are allergic to any medicine. Keep a current list of the medicines, vitamins, and herbs you take. Include the amounts, and when, how, and why you take them. Take the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency. Throw away old medicine lists.

• Blood thinners: Blood thinners are medicines that help prevent blood clots from forming. Clots can cause strokes, heart attacks, and death. Blood thinners make it more likely for you to bleed or bruise. If you are taking a blood thinner:
  
  
  • Watch for bleeding from your gums or nose. Watch for blood in your urine and bowel movements. Use a soft washcloth on your skin and a soft toothbrush on your teeth. This can keep your skin and gums from bleeding. If you shave, use an electric shaver. Do not play contact sports, such as football.
    
    
  • Be aware of what medicines you take. Many medicines cannot be used when taking medicine to thin your blood. Tell your dentist and other caregivers that you take blood-thinning medicine. Wear or carry medical alert information that says you are taking this medicine.
    
    
  • Take this medicine exactly as your caregiver tells you. Tell your caregiver right away if you forget to take the medicine, or if you take too much. You may need to have regular blood tests while on this medicine. Your caregiver uses these tests to decide how much medicine is right for you.
    
    
  • Talk to your caregiver about your diet. This medicine works best when you eat about the same amount of vitamin K every day. Vitamin K is found in green leafy vegetables and other foods, such as cooked peas and kiwifruit.
  
  
• Diuretics: This medicine is given to decrease edema (excess fluid) that collects in a part of your body, such as your legs. Diuretics can also remove excess fluid from around your heart or lungs and decrease your blood pressure. It is often called water pills. You may urinate more often when you take this medicine.
  
  
• Heart medicine: You may need medicine to increase the force of your heart when it pumps. This drug causes the heart to squeeze harder when it beats. When this happens, the heart may be able to move more blood with every beat.
  
  
• Vasodilators: These medicines dilate (widen) your blood vessels to help improve blood flow in your lungs. This also may decrease the strain on your heart in working hard to get blood into your lungs. You may be able to take these medicines by mouth or by inhaling them. You may need medicines that are given through an IV. You may need to be on a constant dose of IV medicine that uses an IV pump to deliver it.

Ask for information about where and when to go for follow-up visits:

For continuing care, treatments, or home services, ask for more information.

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 FPAH, 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.

Good nutrition for your heart:

Get enough calories, protein, vitamins, and minerals to help prevent poor nutrition and muscle wasting. You may be told to eat foods low in cholesterol or sodium (salt). You also may be told to limit saturated and trans fats. Do eat foods that contain healthy fats, such as walnuts, salmon, and canola and soybean oils. Eat foods that help protect the heart, including plenty of fruits and vegetables, nuts, and sources of fiber. Ask what a healthy weight is for you. Set goals to reach and stay at that weight.

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.

CONTACT A CAREGIVER IF:

• You have a fever.
  
  
• 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 © 2012. Thomson Reuters. 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.

Learn more about Familial Pulmonary Arterial Hypertension (Aftercare Instructions)