Patient management

The management of alpha-1-antitrypsin (A1AT) deficiency (also known as alpha-1) and associated obstructive pulmonary disease is based on current recommendations from the American Thoracic Society/European Respiratory Society1 and the Global Initiative for Chronic Obstructive Lung Disease.2 The approach is multimodal and includes both nonpharmacologic and pharmacologic components.

Nonpharmacologic Recommendations

  • Avoidance of smoking1
  • Avoidance of exposure to chemicals that can cause respiratory irritation1
  • Use of supplemental oxygen when indicated (eg, during air travel)1
  • Regular physical activity2
  • Nutritional support or supplementation, if needed2
  • Pulmonary rehabilitation in patients with functional impairment1
  • Consider lung transplantation in carefully selected patients with severe functional impairment and airflow obstruction1

Pharmacologic recommendations

  • Appropriate vaccination for influenza and pneumococcus1
  • Use of inhaled bronchodilators1
  • Appropriate management of acute exacerbations, including use of systemic corticosteroids, antibiotics, and noninvasive ventilator support if needed1
  • Intravenous administration of A1AT (or augmentation therapy), in patients with established airflow obstruction attributable to severe A1AT deficiency1
 
Important safety information

PROLASTIN®-C (alpha1-proteinase inhibitor [human]) is indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1-PI (alpha1-antitrypsin deficiency).

The effect of augmentation therapy with any alpha1-proteinase inhibitor (alpha1-PI), including PROLASTIN-C, on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with PROLASTIN-C are not available.

PROLASTIN-C is not indicated as therapy for lung disease in patients in whom severe alpha1-PI deficiency has not been established.

PROLASTIN-C is contraindicated in IgA-deficient patients with antibodies against IgA due to the risk of severe hypersensitivity and in patients with a history of anaphylaxis or other severe systemic reactions to alpha1-PI.

Hypersensitivity reactions, including anaphylaxis, may occur. Monitor vital signs and observe the patient carefully throughout the infusion. Should hypersensitivity symptoms be observed, promptly stop infusion and begin appropriate therapy. Have epinephrine and other appropriate therapy available for the treatment of any acute anaphylactic or anaphylactoid reaction.

PROLASTIN-C may contain trace amounts of IgA. Patients with known antibodies to IgA, which can be present in patients with selective or severe IgA deficiency, have a greater risk of developing potentially severe hypersensitivity and anaphylactic reactions.

The most common drug-related adverse reaction observed at a rate of >5% in subjects receiving PROLASTIN-C was upper respiratory tract infection. The most serious adverse reaction observed during clinical trials with PROLASTIN-C was an abdominal and extremity rash in 1 subject.

Because PROLASTIN-C is made from human plasma, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. This also applies to unknown or emerging viruses and other pathogens.

Please see full Prescribing Information for PROLASTIN-C.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.


References

  1. 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. Global Initiative for Chronic Obstructive Lung Disease. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. 2016. http://goldcopd.org/global-strategy-diagnosis-management-prevention-copd-2016/. Accessed July 14, 2016.

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