How Prevalent Is Alpha-1?

Alpha-1 may be more common than you think but it is rarely diagnosed.1 Up to 25 million Americans are estimated to have an abnormal gene for the production and release of AAT.2 Out of that 25 million, up to 100,000 may have Alpha-1, and 95,000 of them remain undiagnosed.3 You have Alpha-1 if you inherited an abnormal gene from both your mother and your father. Alpha-1 raises your risk of getting lung disease.2

Alpha-1 can lead to serious lung, liver, and skin diseases

If you have emphysema, asthma, chronic obstructive pulmonary disease (COPD), or chronic bronchitis, it may have been caused by Alpha-1.

Carriers are at increased risk, too

You are an Alpha-1 carrier if you inherited 1 abnormal gene from one of your parents. If you are an Alpha-1 carrier and only inherited 1 abnormal gene, compared with people with normal AAT genes, you have:

A higher-than-normal risk of lung disease2,4

ALPHA-1 DIAGNOSIS IS OFTEN MISSED

Most people with Alpha-1 suffer with symptoms diagnosed as something else. Which of these symptoms look familiar?5

  • Shortness of breath
  • Wheezing
  • Chronic cough and phlegm (chronic bronchitis)
  • Recurring chest colds or pneumonia.

Check your symptoms

 



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. de Serres FJ. Alpha-1 antitrypsin deficiency is not a rare disease but a disease that is rarely diagnosed. Environ Health Perspect. 2003;111:1851-1854.
  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. 2003:64:382-397.
  3. Campos MA, Wanner A, Zhang G, Sandhaus RA. Trends in the diagnosis of symptomatic patients with alpha 1-antitrypsin deficiency between 1968 and 2003. Chest. 2005;128(3):1179-1186.
  4. Yang P, Sun Z, Krowka MJ, et al. Alpha1-antitrypsin deficiency carriers, tobacco smoke, chronic obstructive pulmonary disease, and lung cancer risk. Arch Intern Med. 2008;168:1097-1103.
  5. Alpha-1 Antitrypsin Deficiency: A Guide For The Recently Diagnosed Individual. Alpha-1 Foundation. Version 1.6, December 2006.