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Alpha-1 Antitrypsin Deficiency
WHAT YOU NEED TO KNOW:
Alpha-1 antitrypsin deficiency (AATD) is a condition that increases your risk for lung and liver damage. Alpha-1 antitrypsin (AAT) is made by your liver and protects your lungs and liver from infections and inflammation. Your body may not be able to make enough healthy AAT if you were born with abnormal genes that make AAT. If the AAT your liver makes is faulty, it can cause liver inflammation, damage, and may lead to liver failure. You may also develop AATD if tobacco smoke or chemical fumes decrease your AAT levels.
WHILE YOU ARE HERE:
is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.
is a small tube placed in your vein that is used to give you medicine or liquids.
You may need extra oxygen
if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your healthcare provider before you take off the mask or oxygen tubing.
Caregivers will check your blood pressure, heart rate, breathing rate, and temperature. They will also ask about your pain. These vital signs give caregivers information about your current health.
- AAT replacement: This medicine can increase your AAT to normal levels. You may get AAT replacement medicine through an IV, or you may inhale it. This medicine may help maintain your lung function and prevent further damage.
- Antibiotics: This medicine is given to help treat or prevent an infection caused by bacteria.
- Bronchodilators: You may need bronchodilators to help open the air passages in your lungs, and help you breathe more easily.
- Steroids: This medicine may be given to decrease inflammation.
- Organ transplant: You may need a lung or liver transplant if AAT causes too much organ damage. Talk to your healthcare provider if you have questions or concerns about an organ transplant.
- Blood tests: You may need blood taken for tests. These tests show how much AAT you have in your blood. Blood can be taken from a blood vessel in your hand, arm, or the bend in your elbow. It can give your healthcare providers 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. This will help your healthcare provider look for swelling or damage in your lung tissue.
- Computerized tomography scan: This test is also called a CT scan. It is a special x-ray machine with a computer that takes pictures of your lungs and liver. You may be given dye by mouth or through an IV before the pictures are taken. The dye helps your healthcare providers see the pictures better. People who have allergies to shellfish (lobster, crab, or shrimp) may be allergic to some dyes. Tell your healthcare provider if you are allergic to shellfish or have any allergies or medical conditions.
- Liver biopsy: Your healthcare provider may take a sample of your liver using a long thin needle. This sample will be sent to a lab for tests. The tests will help your healthcare provider learn how much damage your liver has.
- Liver ultrasound: This test looks inside of your body to check the size and condition of your liver. Sound waves are used to look at pictures of your liver on a TV-like screen.
- Pulmonary function tests: Pulmonary function tests, also called PFTs, help healthcare providers learn how well your lungs work. During the tests, you will breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time. This helps healthcare providers to see how well your lungs are moving and working. Your healthcare provider may also use radioactive dye that is put into your vein (blood vessel). Pictures are then taken to see how well the blood flows in your lungs. You may also be asked to breathe in a special gas. Pictures will then be taken to see how well your lungs take in oxygen.
- Bronchodilators may increase your heartbeat and make you dizzy. You may gain weight if you take steroid medicines. During an organ transplant, you could get an infection or bleed too much. Your signs and symptoms may take a long time to improve.
- AATD that is not treated can make the damage to your liver and lungs worse. The amount of oxygen your lungs can take in will decrease, and you may have trouble breathing. More painful lumps may be felt under your skin. Tumors and scars may appear on your liver and cause it to stop working. Other organs such as your kidneys may be damaged. You may die if too many organs are damaged.
CARE AGREEMENT:You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.
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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.