The alpha-1 antitrypsin deficiency: Signs, Symptoms and Treatment for Protect your Lungs

The alpha-1 antitrypsin deficiency: Signs, Symptoms and Treatment for Protect your Lungs


What is alpha-1 antitrypsin deficiency?


Alpha-1 antitrypsin is a protein that is made in the liver. The liver releases this protein into the bloodstream.
Alpha-1 antitrypsin protects the lungs so they can work normally. Without enough alpha-1 antitrypsin, the lungs can be damaged, and this damage may make breathing difficult.
The alpha-1 antitrypsin deficiency: Signs, Symptoms and Treatment for Protect your Lungs

Alpha-1 antitrypsin deficiency is an inherited (passed down from parents) disorder that causes low levels of, or no alpha-1 antitrypsin in the blood.

What happens if there isn't enough alpha-1 antitrypsin?


When the lungs do not have enough alpha-1 antitrypsin, neutrophil elastase is free to destroy lung tissue. As a result, the lungs lose some of their ability to expand and contract (elasticity). This leads to emphysema and sometimes makes breathing difficult. Shortness of breath may occur.
The speed at which lung tissue is destroyed varies with each person. What is known is that tobacco smoking worsens the lung damage.

How does smoking worsen lung damage caused by the disorder?


Tobacco smoke irritates and damages the lungs, prompting the body to send more white blood cells to protect them. The more white blood cells there are, the more neutrophil elastase is made, causing even more lung damage.
Also, the smoke itself changes alpha-1 antitrypsin so that it cannot do as good a job protecting the lungs from harm.

Smokers with alpha-1 antitrypsin deficiency have a faster rate of lung damage. So if you smoke, stop.

How do normal lungs work?


The alpha-1 antitrypsin deficiency: Signs, Symptoms and Treatment for Protect your Lungs

White blood cells normally found in our bodies help protect us from infection. But white blood cells also release an enzyme, called neutrophil elastase, that can damage the lungs. In normal lungs, alpha-1 antitrypsin protects the lungs from the harmful effects of neutrophil elastase.

What are the risk factors for the disorder?


Alpha-1 antitrypsin deficiency is not contagious, and you cannot "catch it" from someone. The disorder is inherited, which means that it is passed on genetically from a relative. All persons who have relatives with this disorder should consider being tested to find out whether they carry the gene for it. 

Another type is called "dysfunctional." In this case, the alpha-1 antitrypsin levels are normal but it does not work the way it should. This type of the disorder is very uncommon.

What are the signs and symptoms of the alpha-1 antitrypsin deficiency?


A person with this disorder can be short of breath during daily activities. This is because the air sacs have been destroyed, and the lungs trap air as they expand and contract during breathing.

Can this disorder be treated?


The alpha-1 antitrypsin deficiency: Signs, Symptoms and Treatment for Protect your Lungs

There are several ways you can protect your lungs from the effects of the disorder:
  • Receive immunizations for flu and pneumonia
  • Receive early treatment for lung infections by seeing your doctor at the first sign of a cold or other lung problem
  • Avoid tobacco smoke, noxious fumes, dust, and pollution
  • Stay fit by doing regular exercise
  • Increase your alpha-1 antitrypsin level. Speak with your doctor about alpha-1 antitrypsin replacement therapy
  • You can also reduce symptoms of shortness of breath by doing the following:
  • Using medications (for example, bronchodilators, or inhaled steroids) prescribed by your doctor to help open your airways
  • Using oxygen if your doctor prescribes it
  • Doing pulmonary rehabilitation (including breathing techniques). Call your local lung association to find out more.
If you have questions about alpha-1 antitrypsin deficiency, feel free to ask your nurse or doctor.