What does it mean to be a carrier?

What is an Alpha-1 carrier?

Alpha-1 is a hereditary condition passed from parents to children through genes. If you have 1 abnormal gene instead of 2, you are a carrier for Alpha-1.

For the most part, there are 2 genes for each trait a person has. One gene comes from the mother, 1 from the father. People with Alpha-1 received 2 abnormal genes for making AAT, that is, the body's instructions omitted the steps to produce and distribute AAT.4

WHAT DO ALPHA-1 LUNGS LOOK LIKE?

CT scan of an alpha-1 deficient patient



TESTING FOR ALPHA-1 IS IMPORTANT

Getting tested for Alpha-1 is easy. It is diagnosed with simple blood tests. Find out about a free Grifols AlphaKit you can get from your doctor.



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. What is Alpha-1? Alpha-1 Foundation. http://www.alphaone.org/healthcare/?c=01-What-is-Alpha-1-Healthcare. Accessed October 6, 2009.
  2. Köhnlein T, Welte T. Introduction. Alpha-1 Antitrypsin Deficiency: Clinical Aspects and Management. Bremen, Germany: Uni-Med Verlag AG; 2007:16-39.