Alpha-1

The information provided is not intended to be a substitute for professional medical advice. A licensed healthcare professional should be consulted for diagnosis and treatment of any and all medical conditions.

About Alpha-1 antitrypsin (AAT) deficiency

Alpha-1 antitrypsin (AAT) deficiency - Alpha1 - is an inherited condition in which there are low levels or no levels of AAT in the blood.

Alpha-1 antitrypsin (AAT), also known as alpha1-proteinase inhibitor (A1-PI), is an enzyme produced by the liver and released into the bloodstream. One of the primary roles of AAT is to protect the lungs from neutrophil elastase (NE), an enzyme released by white blood cells. Neutrophil elastase can attack healthy lung tissue if not controlled by AAT.

Individuals with AAT deficiency are at high risk for developing emphysema. In addition, approximately 10% of infants and 15% of adults with AAT deficiency experience liver damage.1

The American Lung Association estimates that there are about 100,000 people with AAT deficiency in the United States.2

Therapy Options

People with lung disease related to AAT deficiency have a number of therapy options available. Talk to your healthcare provider about the options that are best for you.

Protect Your Lungs

It is very important for Alphas to do everything possible to slow or prevent lung damage. Some protective measures your doctor may recommend include:

  • Stop smoking - the first and most essential protective step
  • Avoid all forms of tobacco smoke, including second-hand smoke from other smokers
  • Avoid occupational and environmental pollutants, including dust and pollen
  • Avoid wood-burning stoves
  • Avoid fumes from household cleaning products
  • Avoid paint and/or other toxic agents

  • Top

Stay as Healthy as Possible

It is also important for Alphas to maintain overall good health. Your doctor may recommend that you:

  • Participate in a pulmonary rehabilitation plan
  • Follow a good nutrition and exercise program
  • Stay current with immunizations for flu and pneumonia
  • Reduce alcohol consumption
  • Keep regular physician appointments, take prescribed medications, and follow professional advice
  • Avoid exposure to people who are sick
  • Develop a stress-management program

“Pulmonary rehabilitation” combines many of the lifestyle and lung-protection recommendations into a structured program. Ask your doctor whether a pulmonary rehabilitation program could help you control symptoms and achieve your highest level of activity.

Consider Medications

For Treatment of Respiratory Symptoms
Some medications are available to help people who have breathing problems associated with asthma, emphysema, and COPD:

  • Bronchodilators (to reduce airway constriction)
  • Corticosteroids (to reduce inflammation)
  • Supplemental oxygen

For Treatment of Infections
To help prevent further lung damage, your doctor may also want to treat any upper respiratory infections with antibiotics as soon as possible. Be sure to contact your doctor at the first sign of an infection.

For Treatment of Mental Health Symptoms
Sometimes people with breathing difficulties experience depression, panic, or anxiety. There are therapies and/or medications that your doctor can prescribe to treat these conditions. There are also medications and devices available to help manage sleep disorders.

Augmentation Therapy

Augmentation therapy with an alpha1-proteinase inhibitor (A1-PI) such as ARALAST NP may be appropriate for you if you have alpha-1 antitrypsin (AAT) deficiency with clinically evident emphysema. Augmentation therapy can help increase the low levels of AAT in the blood and in the lungs. A1-PI augmentation therapy is not a cure for AAT deficiency. It cannot repair damage that has already occurred in your lungs. With augmentation therapy, blood levels of A1-PI rise to levels exceeding the "protective threshold" as recommended by the American Thoraic Society and European Respiratory Society.3

Augmentation therapy is administered intravenously (in a vein) once a week. You can receive this therapy in your home or workplace.

Surgical Options

Some Alpha-1 patients with advanced lung disease may be candidates for lung transplantation. This option may be considered if there is advanced lung disease that has not responded to more conservative therapy.

References

1. Alpha-1 antitrypsin deficiency. Genetics Home Reference:A Service of the National Library of Medicine. Available at http://ghr.nlm.nih.gov/condition=alpha1antitrypsindeficiency (Accessed May 6, 2009).

2. American Lung Association. Alpha-1 Antitrypsin Deficiency Emphysema. Available at http://www.lungusa.org/site/apps/nlnet/content3.aspx?c=dvLUK9O0E&b=4294229&ct=3004003 (Accessed September 18, 2008).

3. Stoller JK, Rouhani F, Brantly M, et al. Biochemical efficacy and safety of a new pooled human plasma alpha (1)-antitrypsin, Respitin. Chest. 2002; 122:66-74.

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