Health Management

Respiratory symptoms of being an Alpha-1 carrier

If you’re an Alpha-1 carrier, you may or may not have these symptoms:5

  • Shortness of breath
  • Wheezing
  • Chronic cough and sputum (phlegm) production (chronic bronchitis)
  • Recurring chest colds
  • Decreased exercise tolerance
  • Nonresponsive asthma or year-round allergies
  • Bronchiectasis

A carrier is at increased risk for lung disease

The risk for emphysema may be greater for MZ carriers, especially if the carrier is a smoker or exposed to high levels of air pollution.1,2

The increased risk of lung disease varies by the phenotype of the carrier.1,2

Range of serum AAT levels by Phenotype

Levels ≥ 11 µM AAT level are estimated to be protective

Options if you’re an Alpha-1 carrier

If you’ve been identified as an Alpha-1 carrier you do not necessarily need treatment. However, it is critical that other family members also get tested, since there is a chance your family members have severe Alpha-1 and do need treatment.

Also, you should have your levels of AAT checked periodically to ensure that you have a level sufficient to protect your lungs and liver. Talk to your doctor about the latest thinking on treatment options for Alpha-1 carriers.

Vaccinations

Vaccinations are recommended for Alpha-1 carriers. You might not need the same treatment as someone with severe Alpha-1 but your lungs are vulnerable to pollutants and infections, and vaccines can help you protect them. Have a yearly flu vaccine and a Pneumovax® shot, which protects against certain infections, every 5 to 6 years. Ask your doctor if you need a vaccine for hepatitis A and B. These are especially important if you have established liver disease:5

  • Annual flu vaccine
  • Pneumovax vaccine (every 5 to 6 years)
  • Hepatitis A vaccine
  • Hepatitis B vaccine

What Does It Mean To Be an Alpha-1 Carrier?

AlphaNet Patient Guides

next: Patient Support If You’re an Alpha-1 Carrier >

Important Safety Information

PROLASTIN®-C (alpha1-proteinase inhibitor [human]) is indicated for chronic augmentation and maintenance therapy in adults with clinically evident emphysema due to severe deficiency of alpha1-proteinase inhibitor (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 risk of hypersensitivity.

Hypersensitivity reactions may occur. Should evidence of hypersensitivity reaction be observed, promptly stop infusion and begin appropriate therapy.

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. PROLASTIN-C is contraindicated in patients with antibodies against IgA.

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 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 11-800-FDA-1088

References
  1. University of Florida College of Medicine AAT Deficiency Detection Laboratory.
  2. Wise RA. α1-Antitrypsin deficiency. The Merck Manuals Online Medical Library. http://www.merck.com/mmpe/sec05/ch049/ch049b.html#CIHFAGBJ. Accessed July 6, 2008.
  3. Yang P, Sun Z, Krowka MJ, Aubry MC, Bamlet WR, Wampfler JA, et al. Alpha-1-antitrypsin deficiency carriers, tobacco smoke, chronic obstructive pulmonary disease, and lung cancer risk. Arch Intern Med. 2008;168(10):1097-1103.
  4. How Is Alpha-1 Diagnosed? Alpha1 Association. http://www.alpha1.org/whatisalpha1/howdiagnosed.php. Accessed on September 30, 2009.
  5. Alpha-1 Antitrypsin Deficiency: A Guide For The Recently Diagnosed Individual. Alpha-1 Foundation. Version 1.6, December 2006.