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®
• Aralast^TM
• Zemaira^TM

 

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).