Why test for alpha-1?

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

Testing all COPD patients for alpha-1 can help detect those at increased risk for lung disease.

Test all adults with symptomatic emphysema, regardless of smoking history11.

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 LIQUID is an alpha1-proteinase inhibitor (human) (alpha1-PI) 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).

Limitations of Use


  • The effect of augmentation therapy with any alpha1-PI, including PROLASTIN-C LIQUID, on pulmonary exacerbations and on the progression of emphysema in alpha1-PI deficiency has not been conclusively demonstrated in randomized, controlled clinical trial
  • Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with PROLASTIN-C LIQUID are not available
  • PROLASTIN-C LIQUID is not indicated as therapy for lung disease in patients in whom severe alpha1-PI deficiency has not been established

PROLASTIN-C LIQUID is contraindicated in immunoglobulin A (IgA)-deficient patients with antibodies against IgA or patients with a history of anaphylaxis or other severe systemic reaction to alpha1-PI products.

Hypersensitivity reactions, including anaphylaxis, may occur. Monitor vital signs and observe the patient carefully throughout the infusion. If hypersensitivity symptoms occur, promptly stop PROLASTIN-C LIQUID infusion and begin appropriate therapy.

Because PROLASTIN-C LIQUID 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.

The most common adverse reactions during PROLASTIN-C LIQUID clinical trials in >5% of subjects were diarrhea and fatigue, each of which occurred in 2 subjects (6%).

Please see full Prescribing Information for PROLASTIN-C LIQUID.


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.
  11. American Thoracic Society/European Respiratory Society. 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(7):818-900.