Getting tested for Alpha-1 is easy. It is diagnosed with simple blood tests. Find out about a free Grifols AlphaKit you can get from your doctor.

What Is AAT Deficiency?

Alpha1-antitrypsin (AAT) deficiency (also known as Alpha-1) develops when your body has a lower than normal level of the protein AAT. It is a genetic disorder, so it is inherited.2

Because Alpha patients have low levels of the protective protein called AAT in their blood, this allows certain enzymes to attack healthy tissues in the body, primarily in the lungs. Such destruction can lead to serious lung disease (such as emphysema), liver conditions, and skin conditions.

AAT plays a critical role in protecting your lungs1

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Protecting Lungs Images Key
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Neutrophils mobilize, delivering elastase at the alveolar surface in
response to bacterial infections, environmental pollutants, or tobacco exposure.
Normal AAT levels keep neutrophil elastase in check.
AAT inhibits excess elastase; lung structure preserved.
Low levels of AAT leave lung tissue unprotected.
Excess elastase cannot be neutralized; lung elastin destroyed; lung function compromised.
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Click arrows to advance images.

  • The liver is the primary organ in your body which makes AAT.
  • Normally, AAT that is made in the liver is circulated in the bloodstream and gets distributed to lungs and other tissues.
  • In Alpha-1 patients, the abnormal AAT protein remains stuck in the liver and can't get out to the bloodstream. This results in low blood levels of AAT, which is diagnostic of the condition.
  • Abnormal accumulation of AAT protein in the liver increases the risk of liver cirrhosis and failure in individuals with Alpha-1.

next: Prevalence of Alpha-1 >

Important Safety Information

Prolastin-C, Alpha1-Proteinase Inhibitor (Human) is for adults who have emphysema caused by inherited alpha1-antitrypsin deficiency. The effect of therapy with any alpha1-proteinase inhibitor (alpha1-PI) on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials. PROLASTIN-C is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.

Prolastin-C may contain trace amounts of IgA. IgA deficient patients with antibodies against IgA should not receive Prolastin-C due to the risk of hypersensitivity.

The most common side effects during clinical trials with Prolastin-C were chills, a general feeling of being unwell, headache, rash, hot flush, and itching.

Prolastin-C is made from human plasma. Products made from human plasma may carry a risk of transmitting infectious agents, e.g., viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

Please see accompanying Prolastin-C Full Prescribing Information for complete prescribing details.

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. Köhnlein T, Welte T. Alpha-1 Antitrypsin Deficiency: Clinical Aspects and Management. Bremen, Germany: Uni-Med Verlag AG; 2007:16-39.
  2. What is Alpha-1? Alpha-1 Foundation. http://www.alphaone.org/healthcare/?c=01.what is alpha-1