CSL Behring is committed to helping people with Alpha-1 Antitrypsin Deficiency live full, healthy lives.
Alpha-1 Antitrypsin Deficiency is commonly called Alpha-1, AAT deficiency or
genetic COPD. It is a hereditary disorder that may increase a person’s chances of developing lung disease such as chronic obstructive pulmonary disease (COPD) or emphysema.
The disorder is caused by a lack of the protein alpha-1 antitrypsin. This protein is made in the liver and protects the lungs from damage caused by infection and inhaled irritants such as smoke.1
People with Alpha-1 have very low levels of alpha-1 antitrypsin, meaning their lungs don't have the same protection as people without the disorder. This leaves them at risk for life-threatening lung and/or liver diseases. 3,4 The most serious liver diseases are cirrhosis and liver cancer.1
Alpha-1 proteinase inhibitors treat this chronic disorder by replacing the missing alpha-1 antitrypsin protein.
Zemaira®, CSL Behring’s Alpha-1 proteinase inhibitor, is the first Alpha-1 augmentation therapy approved by the US Food and Drug Administration as highly purified. In the US, Zemaira is indicated for chronic augmentation and maintenance therapy in adults with alpha-1 deficiency and clinical evidence of emphysema.
Early diagnosis and treatment are crucial to help those with Alpha-1 have the fullest life possible.
Diagnosis of Alpha-1 Antitrypsin Deficiency
Alpha-1 is often misdiagnosed as COPD, asthma or emphysema because the
symptoms are similar: shortness of breath, chronic cough, difficulty breathing, and wheezing.1 On average, people with Alpha-1 will see 3 doctors over 7 years before they are accurately diagnosed.2 During that time, lung damage progresses.
Prevalence of Alpha-1 Antitrypsin Deficiency
Alpha-1 Antitrypsin Deficiency is one of the most common serious hereditary disorders.4
- Worldwide, 117 million people may have Alpha-1 Antitrypsin Deficiency-associated phenotypes 4,5
- 3.4 million people are at risk of expressing severe Alpha-14,5
- Of the 100,000 people in the US estimated to have Alpha-1 Antitrypsin Deficiency,3,4,6 approximately 95% don’t even know they have it4,6
- About 3% of all people with COPD may have undetected Alpha-11
Testing for Alpha-1 Antitrypsin Deficiency
Alpha-1 is a laboratory diagnosis, not a clinical diagnosis. Therefore it can only be definitively diagnosed by a simple blood test. 7
Ask your healthcare professional whether you should be tested for Alpha-1 or visit
www.Alpha1Answers.com for additional information about the condition.
1. Alpha-1 Foundation -- What is Alpha1? Alpha1 Fast Facts. Available at: http://www.alpha-1foundation.org/alphas/?c=01-What-is-Alpha-1-Alphas. Accessed on January 19, 2011
2. COPD International Network-You Are Not Alone. Available at http://www.copdinternational.com/Library/alpha-1.htm. Accessed on January 19, 2011.
3. Ranes J, Stoller JK. A review of Alpha1 Antitrypsin deficiency. Semin Respir Crit Care Med. 2005; 26:154-166.
4. American Thoracic 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; 168:818-900
5. de Serres FJ. Worldwide racial and ethnic distribution of alpha 1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys. Chest. 2002; 122(5):1818-1829
6. 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.
7. Alpha-1 Foundation – Testing for Alpha-1. Available at http://www.alpha-1foundation.org/healthcare/?c=02-Testing-for-Alpha-1.
Accessed on January 19, 2011