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Familial Pulmonary Arterial Hypertension
WHAT YOU SHOULD KNOW:
Familial pulmonary arterial hypertension (FPAH) is a condition in which the pressure in your pulmonary artery is increased. The pulmonary artery is the large blood vessel that brings blood from your heart to your lungs.
- Blood thinners: These prevent blood clots. They may make you bruise or bleed more easily. Use a soft toothbrush and an electric shaver to prevent bleeding.
- Diuretics: These help your body get rid of extra fluid and protect your heart from more damage. You may urinate more often while you are taking diuretics.
- Vasodilators: These improve blood flow by making the vessels in your heart and lungs wider. These may be given as a pill, an inhaler, or in your IV.
- Take your medicine as directed. Call your healthcare provider if you think your medicine is not helping or if you have side effects. Tell him if you are allergic to any medicine. Keep a list of the medicines, vitamins, and herbs you take. Include the amounts, and when and why you take them. Bring the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency.
Follow up with your healthcare provider as directed:
Write down your questions so you remember to ask them during your visits.
Manage your FPAH:
- Eat less salt: You may need to limit the amount of sodium you eat. Check labels to find low-sodium or no-salt-added foods. Some low-sodium foods use potassium salts for flavor. Too much potassium can also cause health problems. Ask your primary healthcare provider how much sodium and potassium salt you should eat each day.
- Limit liquids: You may need to drink less fluids to help balance your fluid level. Ask how much liquid you should drink each day.
- Get regular exercise: Exercise may help decrease your symptoms and improve your heart function. Exercise also helps with weight control. Never start an exercise program before you talk with your primary healthcare provider.
- Special positions while sleeping: 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. There are many devices that you can buy to help raise your upper body while in bed. Use a device that will tilt your whole body, or bend your body at the waist. The device should not bend your body at the upper back or neck.
Contact your primary healthcare provider if:
- You have a fever.
- Your symptoms keep you from doing your daily activities.
- Your joints are painful and swollen.
- Your fingers or toes are clubbed (the ends are round and thick).
- You have questions or concerns about your condition or care.
Return to the emergency department if:
- You have shortness of breath at rest, especially when you lie down.
- Your legs or ankles are swollen.
- You are vomiting and cannot eat or drink.
- You are confused or feel like you are going to faint.
- You have chest pain or heart palpitations (strong, fast heartbeats).
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.