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


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


PROLASTIN®-C (alpha1-proteinase inhibitor [human]) is indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1-PI (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 the risk of severe hypersensitivity and in patients with a history of anaphylaxis or other severe systemic reactions to alpha1-PI.

Hypersensitivity reactions, including anaphylaxis, may occur. Monitor vital signs and observe the patient carefully throughout the infusion. Should hypersensitivity symptoms be observed, promptly stop infusion and begin appropriate therapy. Have epinephrine and other appropriate therapy available for the treatment of any acute anaphylactic or anaphylactoid reaction.

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.

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, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. This also applies to unknown or emerging viruses and other pathogens.

Please click here for full Prescribing Information for PROLASTIN-C.


References

  1. University of Florida College of Medicine AAT Deficiency Detection Laboratory.
  2. Wise RA. ?1-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.