Emphysema and Alpha-1

Emphysema is a lung disease in which the tiny air sacs (alveoli) in your lungs are overinflated. They overinflate because the walls of your air sacs are damaged. Since your air sacs take in oxygen and release carbon dioxide, this over-inflation makes your lungs less efficient and often results in breathlessness.

Neutrophil elastase and AAT

One cause of damage to your air sacs is the enzyme neutrophil elastase. Normally, neutrophil elastase eliminates bacteria that cause lung infections. Once the infection is resolved, your body uses AAT to stop neutrophil elastase.

If there’s not enough AAT, neutrophil elastase keeps working and can destroy healthy lung tissue. Over time, enough air sacs can be destroyed to make your lungs rigid, resulting in emphysema. If you have Alpha-1, you have less AAT and are more likely to develop emphysema.

Is it emphysema due to Alpha-1?

Alpha-1 is often misdiagnosed as emphysema. Emphysema is more likely to be Alpha-1 if there is:1

  • Early‐onset emphysema (age 45 years or younger)
  • Emphysema without a known risk factor (smoking, occupational dust exposure, etc.)
  • Lung function decline more than expected for a nonsmoker or for a smoker who quit
  • X-rays or images of your lungs that show damage in the lower parts of your lungs, which is unusual with emphysema

Testing for Alpha-1

The average Alpha patient experiences symptoms for more than 8 years and sees 3 doctors before being correctly diagnosed with Alpha-1.2 This delay is too long, especially considering the destruction of lung tissue prior to diagnosis. Alpha-1 is easily diagnosed with simple blood tests.

Ask your doctor about a free Talecris AlphaKit that can tell if you have Alpha-1.

next: Asthma and 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 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. 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.
  2. 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.