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.

CARE AGREEMENT:

You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

RISKS:

Risks of IPAH treatment include allergic reactions to the medicines used for treatment. You may lose too much blood during surgery, or get an infection. Transplant surgery may lead to organ rejection, which is when the transplanted body organ does not work well or at all. If you do not get treated for IPAH, your symptoms may get worse and damage other organs. Over time, your heart may fail, and you may even die. Talk to your caregiver if you are worried or have concerns about IPAH, or your medicine or care.

WHILE YOU ARE HERE:

Informed consent: You have the right to understand your health condition in words that you know. You should be told what tests, treatments, or procedures may be done to treat your condition. Your doctor should also tell you about the risks and benefits of each treatment. You may be asked to sign a consent form that gives caregivers permission to do certain tests, treatments, or procedures. If you are unable to give your consent, someone who has permission can sign this form for you. A consent form is a legal piece of paper that tells exactly what will be done to you. Before giving your consent, make sure all your questions have been answered so that you understand what may happen.

Activity: At first you may need to rest in bed. It may be easier for you to breathe if you rest with the head of your bed raised. You can also breathe easier if you rest your head on three or four pillows. Resting in a reclining chair may also help you breathe better. Another way to breathe easier is by saving your energy and resting more. If you have trouble breathing, call your caregiver right away. You may get out of bed when your breathing has improved.

IV: An IV is a tube placed in your vein for giving medicine or liquids. This tube is capped or connected to tubing and liquid.

Medicines: Your caregiver may give you the following kinds of medicines:

• Blood thinners: This medicine helps stop clots from forming in the blood. Clots can cause strokes, heart attacks, and death. Blood thinners may make it easier to bleed or bruise. While taking this medicine, use a soft toothbrush to prevent bleeding gums. If you shave, use an electric shaver.
  
  
• 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 your blood vessels and help improve blood flow.

Oxygen: You may need extra oxygen to help you breathe easier. It may be given through a plastic mask over your mouth and nose. It may be given through a nasal cannula, or prongs, instead of a mask. A nasal cannula is a pair of short, thin tubes that rest just inside your nose. Tell your caregiver if your nose gets dry or if the mask or prongs bother you. Ask your caregiver before taking off your oxygen. Never smoke or let anyone else smoke in the same room while your oxygen is on. Doing so may cause a fire.

Pulse oximeter: A pulse oximeter is a machine that tells how much oxygen is in your blood. A cord with a clip or sticky strip is placed on your ear, finger, or toe. The other end of the cord is hooked to a machine. Caregivers use this machine to see if you need more oxygen.

Tests: You may also need to have any of the following:

• Blood gases: This test is also called an arterial blood gas or ABG test. Blood is taken from an artery (blood vessel) in your wrist, arm, or groin. The groin is the area where your abdomen meets your upper leg. Your blood is tested for the amount of "gases" in it, such as oxygen, acids, and carbon dioxide.
  
  
• Blood tests: You may need blood taken for tests. The blood can be taken from a blood vessel in your hand, arm, or the bend in your elbow. It is tested to see how your body is doing. It can give your caregivers more information about your health condition. You may need to have blood drawn more than once.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
  
  
• 12-lead ECG: This test, also called an EKG, helps caregivers look for damage or problems in different areas of the heart. Caregivers may need to prepare your skin by shaving off some hair, or cleaning it with a gritty lotion. Sticky pads are placed on your chest, arms, and legs. Each sticky pad has a wire that is hooked to a machine or TV-type screen. A short period of electrical activity in your heart muscle is recorded. Caregivers will look closely for certain problems or changes in how your heart is working. This test takes about 5 to 10 minutes. It is important that you lie as still as possible during the test. You may need this test more than once.
  
  
• Imaging tests: Dye may be used in certain tests to make pictures show up better. Tell your caregiver if you are allergic to iodine or shellfish (lobster, crab, or shrimp), as you may also be allergic to this dye.
  
  
  • Computed tomography scan: This is also called a CT scan. A special x-ray machine uses a computer to take pictures of your heart and lungs. It may also be used to look at your blood vessels.
    
    
  • Magnetic resonance imaging: This test is also called MRI. During the MRI, pictures are taken of your chest. An MRI may be used to look at organs, such as the heart and lungs, and tissues, such as blood vessels. You will need to lie still during an MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal objects. This may cause serious injury. Tell your caregiver if you have any metal implants in your body.

Treatment options:

• Shunts: This is surgery to help blood flow from one part of the heart to another using a shunt (special tube). The tube is placed in the left ventricle (chamber) of your heart. This decreases the work of the right ventricle, and allows more blood flow to the rest of your body. When there is greater blood flow, the delivery of oxygen to your body also increases. This surgery may also be done to prepare you for a lung, or heart and lung transplant.
  
  
• Transplants: This is surgery done to remove and replace a damaged organ with a healthy organ from a donor. You may have one or both lungs transplanted, or both lungs and your heart may be replaced. Transplants may be done for people whose conditions worsen even after treatment.

Vital signs: This includes taking your temperature, blood pressure, pulse (counting your heartbeat), and respirations (counting your breaths). To take your blood pressure, a cuff is put on your arm and tightened. The cuff is attached to a machine which gives your blood pressure reading. Caregivers may listen to your heart and lungs by using a stethoscope. Your vital signs are taken so caregivers can see how you are doing.

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