Why test for alpha-1?

Alpha-1 is the major known genetic risk factor for COPD1

Alpha-1 is a potentially fatal genetic form of COPD. It may be more common than you think, and it can be found at all ages.1-4

Prevalence of alpha-1 in the United States

AAT deficiency is a rarely diagnosed disease10

  • >100,000 people in the United States are estimated to have AAT deficiency9
  • Up to 25 million Americans are thought to have at least 1 deficient allele (S or Z), and may pass that gene on to their children2

More than 90% of those with alpha-1 are thought to be undiagnosed9


Because alpha-1 is a rarely diagnosed disease, it is essential to test now9,10


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 click here for full Prescribing Information for PROLASTIN-C.


References

  1. World Health Organization. α1-antitrypsin deficiency: memorandum from a WHO meeting. Bull World Health Organ. 1997;75(5):397-415.
  2. de Serres FJ, Blanco I, Fernández-Bustillo E. Genetic epidemiology of alpha-1 antitrypsin deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand and the United States of America. Clin Genet. 2003;64(5):382-397.
  3. National Institutes of Health. COPD: the more you know, the better for you and your loved ones. http://www.nhlbi.nih.gov/health/public/lung/copd/campaign-materials/html/copd-atrisk.htm. Accessed April 8, 2014.
  4. Data on file, Alpha-1 Genetics Laboratory.
  5. Coalition for Pulmonary Fibrosis. Epidemiology and risk factors. http://www.coalitionforpf.org/epidemiology-and-risk-factors. Accessed April 10, 2014.
  6. Sickle Cell Disease Association of America. Sickle cell disease. http://sicklecelldisorder.com/index.php/the-disease.html. Accessed April 10, 2014.
  7. Cystic Fibrosis Foundation. Frequently asked questions: what is cystic fibrosis? http://www.cff.org/AboutCF/. Accessed April 1, 2014.
  8. Huntington's Disease Society of America. What is Huntington's disease (HD)? http://www.hdsa.org/about/our-mission/what-is-hd.html. Accessed April 10, 2014.
  9. Campos MA, Wanner A, Zhang G, Sandhaus RA. Trends in the diagnosis of symptomatic patients with α1-antitrypsin deficiency between 1968 and 2003. Chest. 2005;128(3):1179- 1186.
  10. de Serres FJ. Alpha-1 antitrypsin deficiency is not a rare disease but a disease that is rarely diagnosed. Environ Health Perspect. 2003;111(16):1851-1854.