Alpha-1 Antitrypsin Deficiency and Emphysema: Care Instructions

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Lungs in chest, showing airways of a lung with detail of healthy alveoli and damaged alveoli


Alpha-1 antitrypsin (AAT) is a protein normally found in your lungs and blood. It helps protect the lungs from damage that leads to the lung disease emphysema (say "em-fuh-ZEE-muh").

Some people do not make enough AAT in their bodies. This is called AAT deficiency. It is also called inherited emphysema, because it is passed down by genes that you inherit from your family. If you have AAT deficiency, you may get emphysema at a young age. People with AAT deficiency may get emphysema when they are 30 or 40 years old, especially if they smoke. If you have AAT deficiency but do not smoke, you may not get emphysema.

Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments, and call your doctor if you are having problems. It's also a good idea to know your test results and keep a list of the medicines you take.

How can you care for yourself at home?

To stay healthy

  • Do not smoke. This is the most important step you can take to prevent damage to your lungs. If you already smoke, it is never too late to stop. If you need help quitting, talk to your doctor about stop-smoking programs and medicines. These can increase your chances of quitting for good.
  • Avoid secondhand smoke and air pollution. Try to stay inside with your windows closed when air pollution is bad.
  • Talk to your doctor before you fly or travel to places that are at high altitudes.
  • Do not use aerosol products such as aerosol hair spray, spray paint, or aerosol cleaning products.
  • Avoid infections such as COVID-19, colds, and the flu.
    • Wash your hands often.
    • Stay up to date on your COVID-19 vaccines.
    • Get a flu vaccine each year, as soon as it is available. Ask those you live or work with to do the same so they will not get the flu and infect you.
    • Get the pneumococcal vaccine. It may keep you from getting pneumonia. And if you do get pneumonia, you probably will not be as sick.
    • Make sure you are current on the whooping cough (pertussis) vaccine to help prevent whooping cough.


  • Take your medicines exactly as prescribed. Call your doctor if you think you are having a problem with your medicine. You will get more details on the specific medicines your doctor prescribes.
  • If you use inhaled medicines, a spacer may help you get more medicine into your lungs. Ask your doctor or pharmacist if a spacer is right for you. If so, ask them how to use it properly.
  • You may take medicines such as:
    • Alpha-1 antitrypsin (AAT) replacement (such as Prolastin). This may slow down lung disease in people with AAT deficiency. You get these medicines through a tube in your vein, called an I.V.
    • Bronchodilators (such as albuterol or metaproterenol). These help open your airways and make breathing easier. Bronchodilators are either short-acting (they work for 4 to 9 hours) or long-acting (they work 12 to 24 hours). You inhale most bronchodilators, so they start to act quickly. Always carry your short-acting inhaler with you in case you need it.
    • Corticosteroid medicines (such as budesonide or prednisone). These reduce airway swelling. They come in inhaled or pill form.
  • Do not take any vitamins, over-the-counter drugs, or herbal products without talking to your doctor first.
  • If your doctor prescribes antibiotics for an infection, take them as directed. Do not stop taking them just because you feel better. You need to take the full course of antibiotics.


  • Get regular exercise. Walking is an easy way to get exercise. Start out slowly, and walk a little more each day.
  • Pay attention to your breathing. You are exercising too hard if you cannot talk while you are exercising.

Mental health

  • Having AAT deficiency can bring up many emotions. It is common to feel frightened, angry, hopeless, or helpless. It may help to talk with your family, friends, or a therapist about your feelings. Talking openly about your feelings can help you cope. If you think you are depressed, ask your doctor for help. Treatment may help you feel better.

When should you call for help?

Call 911 anytime you think you may need emergency care. For example, call if:

  • You have severe trouble breathing.

Call your doctor now or seek immediate medical care if:

  • You have new or worse shortness of breath.
  • You are coughing more deeply or more often, especially if you notice more mucus or a change in the color of your mucus.
  • You cough up blood.
  • You have new or increased swelling in your legs or belly.
  • You have a fever.

Watch closely for changes in your health, and be sure to contact your doctor if you have any problems.

Where can you learn more?

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The Health Encyclopedia contains general health information. Not all treatments or services described are covered benefits for Kaiser Permanente members or offered as services by Kaiser Permanente. For a list of covered benefits, please refer to your Evidence of Coverage or Summary Plan Description. For recommended treatments, please consult with your health care provider.