What is Alpha-1
Alpha-1-antitrypsin is a protein normally produced in the liver whose primary function is to protect the lungs. When tissue in the lungs is damaged or infected, the body releases substances to help the lungs heal. An enzyme, known as neutrophil elastase, is produced to help get rid of the damaged cells or bacteria. However, if left unchecked, this enzyme can attack healthy lung tissue as well. The Alpha-1-antitrypsin protein helps to control the enzyme so it does not damage healthy lung tissue.
Some people do not have or make enough Alpha-1-antitrypsin to help protect the lungs. This is known as Alpha-1-antitrypsin deficiency (AATD) or Alpha-1. Alpha-1 is a hereditary condition. People who do not have enough Alpha-1 are more likely to get lung disease, such as emphysema.1 Once diagnosed with emphysema as a result of this condition, adults with Alpha-1 face progressive loss of lung function that can significantly impact everyday life and life expectancy.
Symptoms of Alpha-1 include shortness of breath on exertion, wheezing, and cough.
Because wheezing and shortness of breath are common symptoms, Alpha-1 is often misdiagnosed
as other chronic lung diseases, such as chronic obstructive pulmonary disease (COPD)
or asthma.2
If you think that you may have Alpha-1, click here to learn more.
Learn More About Alpha-1
The Alpha-1 Foundation is
dedicated to providing leadership and resources to increase research, improve health,
and result in the worldwide detection of and a cure for Alpha-1. This site contains
an excellent explanation of Alpha-1, provides health tips, and offers a free Alpha-1
testing kit.
The Alpha-1 Association’s goal
is to provide support, education, and advocacy for people with Alpha-1, offer information
to assist healthcare professionals, and support research on Alpha-1.
AlphaNet is a not-for-profit
health management company providing services exclusively to the Alpha community.
Its goals are to help Alphas develop greater independence and control of their health
care, promote optimal health and fund compassionate care for Alphas, and support
Alpha-1 awareness, detection, and scientific research.
Important Safety Information
Alpha1-Proteinase Inhibitor (Human), Zemaira is indicated for chronic augmentation and maintenance therapy for adults with alpha1-proteinase inhibitor (A1-PI) deficiency and emphysema. Clinical data demonstrating the long-term effects of chronic augmentation therapy with Zemaira are not available.
Zemaira may not be appropriate for the following adult individuals as they may experience severe reactions, including anaphylaxis: individuals with a known hypersensitivity and/or history of anaphylaxis or severe systemic reaction to A1-PI products or their components, and individuals with selective IgA deficiencies who have known antibodies against IgA.
In clinical studies, the following treatment-related adverse reactions were reported in 1% of subjects: asthenia (fatigue), injection-site pain, dizziness, headache, paresthesia (tingling), and pruritus (itching).
Zemaira is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.
Please see full prescribing information.
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.
- Eriksson S. Studies in α1-antitrypsin deficiency. ACTA Med Scand Suppl. 1965;432:1-85.
- Alpha-1 Foundation. What is Alpha-1? Should I be Tested? [Alpha-1 Foundation Web site], November 2003. Available at: http://www.alphaone.org/healthcare/?c=01-What-is-Alpha-1-Healthcare. Accessed January 20, 2004.