Alpha-1 Antitrypsin Deficiency: Inherited Emphysema



Treatment

 

Medication Therapy

Specific therapy has been available for inherited emphysema since 1987: a class of medicine called augmentation therapy. This medicine replaces the alpha-1 antitrypsin protein in the blood with normal alpha-1 antitrypsin from healthy plasma donors, and it is given in a vein. The dose is adjusted based on body weight, and this treatment is often given once a week. There are three brands of augmentation therapy. They include:

  • Prolastin®
  • AralastTM
  • ZemairaTM

 

Surgery

Lung transplants or lung reduction surgery may be an option for people severely affected by Alpha-1.

 

Behavior Modification

In addition to medicines, the management of inherited emphysema includes:

  • Avoidance of infection
  • Techniques to bring up mucus
  • Oxygen therapy
  • Pulmonary rehabilitation
  • Exercise and a healthy lifestyle
  • Giving up smoking and avoiding secondhand smoke. This is particularly important to managing inherited emphysema. Smoking can accelerate or speed the development of the Alpha-1 related emphysema and shorten the lifespan.

 

BACK: Diagnosis

This information has been approved by Robert Sandhaus, MD, and Janis Berend RN, MSN, C-ANP (January 2007).

Appointments

1-800-222-5864

Alpha-1 Program

Doctors at National Jewish Health follow one of the largest groups of Alpha-1 patients in the country.  

Learn more.