AAT Augmentation

There are several therapy options that you may want to consider for your patients with AAT deficiency.

AAT Augmentation

Although there is no cure for AAT deficiency, AAT augmentation can be used to augment the concentration of the alpha1-proteinase Inhibitor (A1-PI) in the blood and lungs of A1-PI deficient patients with clinically evident emphysema.1 Not all individuals with AATD will develop symptomatic emphysema.

Symptomatic Treatment

Management of obstructive lung disease in individuals with AAT deficiency is similar to management of other obstructive lung diseases, including use of:

  • Inhaled bronchodilators
  • Supplemental oxygen, when needed
  • Antibiotic therapy for respiratory infections
  • Brief courses of systemic corticosteroids during acute exacerbations
  • Pulmonary rehabilitation for individuals with functional impairment

Therapies

Healthy Lifestyle

A healthy lifestyle is important for patients with AAT deficiency. Health practices that can slow or prevent further lung damage are particularly important.

It is critical that you advise AAT deficient patients to stop smoking. Encouraging them to reduce their exposure to occupational and environmental pollutants, including second-hand tobacco smoke and all types of dust, is also important.

Patients should be strongly advised to stay current with immunizations, especially for influenza and pneumonia.

You also should encourage your patients to:

  • Participate in a pulmonary rehabilitation plan
  • Adhere to a good nutrition and exercise program
  • Reduce alcohol consumption
  • Develop a stress-management program
  • Avoid exposure to people who are sick
  • Maintain regular physician appointments and comply with prescribed medications and professional advice

Surgery for Advanced Disease

Lung transplantation can be considered for patients who do not respond to more conservative therapy or for those who have extensive lung damage.

GLASSIA [Alpha1-Proteinase Inhibitor (Human)]

GLASSIA is indicated for chronic augmentation and maintenance therapy in individuals with emphysema due to congenital deficiency of alpha1-proteinase inhibitor (Alpha1-PI), also known as alpha1-antitrypsin (AAT) deficiency.

  • The effect of augmentation therapy with GLASSIA or any Alpha1-PI product on pulmonary exacerbations and on the progression of emphysema in Alpha1-PI deficiency has not been demonstrated in randomized, controlled clinical trials.
  • Clinical data demonstrating the long term effects of chronic augmentation and maintenance therapy of individuals with GLASSIA are not available.
  • GLASSIA is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.

Detailed Important Risk Information for GLASSIA

  • GLASSIA is contraindicated in IgA deficient patients with antibodies against IgA. GLASSIA is contraindicated in individuals with a history of severe immediate hypersensitivity reactions, including anaphylaxis, to Alpha1-PI products.
  • GLASSIA is made from human plasma. It may carry a risk of transmitting infectious agents, such as viruses, and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
  • Administer GLASSIA at room temperature at a rate not greater than 0.04 mL/kg body weight per minute. If anaphylactic or severe anaphylactoid reactions occur, discontinue the infusion immediately.
  • Administer GLASSIA within 3 hours of entering the vials.
  • Safety and effectiveness in patients over 65 years of age have not been established.
  • Two serious adverse reactions observed on two separate occasions during clinical studies with GLASSIA were cholangitis and exacerbation of chronic obstructive pulmonary disease (COPD).
  • The most common product-related adverse reactions in clinical studies were headache and dizziness.

Please see GLASSIA Full Prescribing Information for full prescribing details.

ARALAST NP [Alpha1-Proteinase Inhibitor (Human)]

ARALAST NP is indicated for chronic augmentation therapy in patients having congenital deficiency of A1-PI with clinically evident emphysema.

  • The effect of augmentation therapy with ARALAST NP on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials.
  • Clinical data demonstrating the long-term effects of chronic augmentation or replacement therapy of individuals with ARALAST NP or ARALAST are not available.
  • ARALAST NP is not indicated as therapy for lung disease patients in whom congenital A1-PI deficiency has not been established.

Detailed Important Risk Information for ARALAST NP

  • ARALAST NP is contraindicated in IgA deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity.
  • ARALAST NP is derived from pooled human plasma. It may carry a risk of transmitting infectious agents, e.g., viruses and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
  • The recommended rate of administration (≤0.08 mL/kg/min) should be closely followed and vital signs monitored continuously. If anaphylactic or severe anaphylactoid reactions occur, the infusion should be discontinued immediately.
  • Safety and effectiveness in patients over age 65 years of age have not been established.
  • ARALAST NP should be administered at room temperature within three (3) hours after reconstitution and should be administered alone, without mixing with other agents or diluting solutions.
  • The safety of ARALAST NP was evaluated with ARALAST in a crossover clinical PK comparability study. The most common adverse events deemed related to ARALAST NP included headache and musculoskeletal discomfort. No serious adverse reactions or deaths were reported in the study. In the ARALAST pivotal study, the most common adverse events were headache and somnolence.

Please see ARALAST NP Full Prescribing Information for full prescribing details.

References

  1. ARALAST NP [Alpha1-Proteinase Inhibitor(Human)] Prescribing Information. Baxter Healthcare, Westlake Village, CA; April 2010.