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Learn About Alpha 1 Deficiency

What It Is

Alpha-1 antitrypsin is a protein that is naturally present in your bloodstream and monitors the function of the white blood cells. The white blood cells play a role in the immune system and work to destroy foreign substances and infections in the body. Alpha-1 antitrypsin usually protects the normal tissues in your body from being destroyed by the white blood cells.

Deficiency in alpha-1 antitrypsin can cause emphysema, which is a type of chronic obstructive pulmonary disorder (COPD).

Some people have a genetic condition that causes them to have lower levels of alpha-1 antitrypsin than normal. If you have a deficiency of the protein, the white blood cells will be less well controlled and can attack the tissue in your lungs to cause damage.

With time, most individuals that have very low levels of alpha-1 antitrypsin will develop lung tissue damage and symptoms of emphysema. Some people with alpha-1 antitrypsin deficiency also experience problems in other organs of the body, such as the liver.

Symptoms of It

The symptoms of emphysema associated with alpha-1 antitrypsin deficiency are the same for someone with emphysema from another cause, such as smoking.

Shortness of breath is the most common symptom, which usually gets worse during physical activity. It can sometimes inhibit people from taking part in everyday tasks and activities, or even at rest. Other symptoms may include cough, wheezing and frequent chest infections.

How to Test for It

People with emphysema caused by alpha-1 antitrypsin deficiency tend to be diagnosed at an earlier age than those that are associated with a history of smoking.

There are several diagnostic tests that can help to make a diagnosis of emphysema, including:

Imaging tests such as X-ray and computerized tomography (CT) scans to view the structure of the lung tissue
Blood tests to measure the oxygen and carbon dioxide concentration in the blood and the level of alpha-1 antitrypsin.
Lung function tests such as spirometry to assess the breathing and function of the lungs

A blood test can also confirm a deficiency of alpha-1 antitrypsin as the cause of the condition.

Here at Central Arkansas Lung, we can perform all these diagnostic tests.

Treatment

There is no cure for emphysema and the aim of treatment is to prevent the condition from getting worse and improve symptoms. Smoking can hasten the progression of the condition and worsen symptoms, so if you are a smoker that has emphysema, it is a good idea to quit.

Bronchodilator and steroid medications are available in an inhaler device to deliver the medication directly to the airways to improve symptoms. It is important that the inhalers are used correctly for it to have the greatest benefit. We can demonstrate how to use an inhaler for you in the clinic if you would like help with this.

Some people may also need antibiotics to fight off a bacterial infection or theophylline tablets to relax the airways and help you to breathe. Oxygen can also be used to aid breathing when the symptoms of emphysema are severe.

What else can you do?

If you have emphysema, there are a few things that you can do to help reduce the severity of the condition such as:

  • Quit smoking (if you smoke)
  • Avoid irritants such as chemical fumes and air pollution
  • Exercise regularly
  • Eat a nutritious diet

If you make an effort to do these things, you can help to prevent further damage to your lungs so that you can get the oxygen you need to stay healthy partake in the activities of your day-to-day life.

Want to know more?

If you think that you may have symptoms of emphysema, it is important that you talk to medical professional as soon as possible to begin managing the condition. At Central Arkansas Lung, we will be happy to give you personalized and professional advice about treatment options and how you can live well with emphysema.

Simply call us if you’d like more information or to organize an appointment to discuss your symptoms.

Further Reading

http://patient.info/health/alpha-1-antitrypsin-deficiency-leaflet

http://emedicine.medscape.com/article/295686-overview

https://ghr.nlm.nih.gov/condition/alpha-1-antitrypsin-deficiency

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