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Alpha-1 Antitrypsin Deficiency and Emphysema: Care Instructions

Lungs showing a closeup of a normal lung and a lung with emphysema

Your Care Instructions

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 or nurse call line 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 second-hand smoke, air pollution, very cold or very hot air, and high altitudes. Stay at home 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 colds and flu.
    • Get the pneumococcal vaccine. It may not keep you from getting pneumonia. But if you do get pneumonia, you probably will not be as sick.
    • 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.
    • Try to stay away from people with colds or the flu.
    • Wash your hands often.


  • Take your medicines exactly as prescribed. Call your doctor or nurse call line 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 him or her how to use it properly.
  • You may take medicines such as:
    • AAT enzyme replacement (Prolastin). These AAT proteins may slow down lung disease in people with AAT deficiency. You get these medicines through a tube in your vein, called an IV.
    • Bronchodilators (such as salbutamol). These help open your airways and make breathing easier. Bronchodilators are either short-acting (they work for 2 to 6 hours) or long-acting (they work for 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 while you are away from home.
    • Corticosteroids medicines (such as budesonide or prednisone). These reduce airway swelling. They come in pill or inhaled form. You must take these medicines every day for them to work well.
  • 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, helpless, and even guilty. 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 can 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 or nurse call line 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 colour 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 or nurse call line if you have any problems.

Where can you learn more?

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Care instructions adapted under license by your healthcare professional. If you have questions about a medical condition or this instruction, always ask your healthcare professional. Healthwise, Incorporated disclaims any warranty or liability for your use of this information.