Family Testing for Alpha-1

Alpha-1 is a genetic condition. Other members of your family may have the same gene pattern as you. The risk of having Alpha-1 or being a carrier for Alpha-1 is 3 out of 4 if both parents carry the Pi Z gene. If they are carriers for Alpha-1, they could pass it on without even knowing it.


Alpha-1 occurs when a person inherits one abnormal gene from each of his or her parents.

If tests indicate you are an Alpha-1 or a carrier: there are steps you can take to manage your disease, which may reduce your risk of lung disease. Early behavioral management may prevent deterioration of your lung function and reduce your risk of serious lung disease.

Healthcare providers and patients should discuss the advantages and disadvantages of testing before making a final decision.

  1. If one of the parents has two abnormal genes (i.e., is an Alpha or Pi ZZ type), then the child has a 100% chance of being a carrier.
  2. If each parent has only one abnormal gene (i.e., is an Alpha carrier), then the child has a 50% chance of being a carrier, a 25% chance of being an Alpha, and a 25% chance of having two normal alleles.

FREE TEST KIT

Up to 25 million Americans are estimated to carry an abnormal Alpha-1 gene1

Many of the estimated 25 million carriers for Alpha-1 have an increased risk of lung disease.1,2 Request a free AlphaKit at www.MyAlphaTest.com/pro-c



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. de Serres FJ, Blanco I, Fernández-Bustillo E. Genetic epidemiology of alpha-1 antitrypsin deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand and the United States of America. Clin Genet. 2003:64:382-397.
  2. Yang P, Sun Z, Krowka MJ, Aubry MC, Bamlet WR, Wampfler JA, et al. Alpha1-antitrypsin deficiency carriers, tobacco smoke, chronic obstructive pulmonary disease, and lung cancer risk. Arch Intern Med. 2008;168:1097-1103.