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Treatment Options

Preventing and slowing the progression of lung disease is the primary goal of treatment for Alpha-1 deficiency. Leading guidelines recommend the following treatment options for patients with Alpha-1 deficiency.1

SYMPTOMATIC TREATMENTS ALPHA-1 AUGMENTATION THERAPY 1,2 SURGERY2
  • Bronchodilators1
  • Useful in patients with Alpha-1 deficiency and COPD
  • Overuse iscommon
  • The only treatment specifically for Alpha-1 deficiency
  • Augmentation therapy is an IV infusion of purified, human alpha-1 antitrypsin
  • Indicated for patients with Alpha-1 deficiency and emphysema
  • Not appropriate for emphysema patients without evidence of Alpha-1 deficiency
  • For patients with end-stage lung or liver disease who do not respond to more conservative therapy
  •  Surgical options include organ transplantation (lung or liver) and lung volume reduction surgery
  • Corticosteroids1,2
  • Useful in patients with Alpha-1 deficiency and bronchial hyperactivity to help during exacerbations
  • Antibiotics1
  • For patients with bronchitis or upper respiratory infections
  • Supplemental oxygen1,2
  • For patients with low blood oxygen levels, respiratory infections, or progressive destruction of lung tissue

 

 
  • Pulmonary rehabilitation
  • To maximize airflow

 

 
  • Nutritional counseling
  • To help patients develop appropriate dietary habits

 

 

References

1. American Thoracic Society/European Respiratory Society Task Force. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818-900.

2. Coan MH, Brockway WJ, Eguizabal H, Krieg T, Fournel M. Preparation and properties of alpha1- proteinase inhibitor concentrate from human plasma. Vox Sang. 1985;48:333-342.
 
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