Who/Why/How To Test for Alpha-1?

Who should be tested for Alpha-1?

Several medical organizations recommend similar Alpha-1 testing guidelines. Here's a brief summary of those guidelines from the American Thoracic Society (physicians who focus on illness of the chest).

American Thoracic Society (ATS) Guidelines

ATS guidelines recommend testing a broad range of patients with lung conditions:1

  • All adults with symptomatic emphysema regardless of smoking history
  • All adults with symptomatic COPD regardless of smoking history
  • All adults with symptomatic asthma whose airflow obstruction is incompletely reversible after bronchodilator therapy
  • Asymptomatic patients with persistent obstruction on pulmonary function tests with identifiable risk factors (smoking, occupational exposure, etc.)
  • Consider testing of asymptomatic individuals with persistent airflow obstruction without risk factors (no smoking or no known occupational exposure, etc.)

ATS guidelines also provide guidance on testing family members of Alphas:

  • Test all siblings of Alphas
  • Consider testing of offspring, parents or distant relatives of Alphas

The National Institutes of Health (NIH) advises testing similar patient types.2:

Read the NIH Statement on the Diagnosis and Management of Individuals with Alpha1-Antitrypsin Deficiency on the NIH website.

Why test for Alpha-1?

Testing for Alpha-1 increases the knowledge of your personal risk of lung disease and other complications. You also gain a broader base of knowledge on which to base future healthcare decisions.

Testing allows you to do things and take steps that may slow the progression of your Alpha-1. Knowing whether you have severe Alpha-1, or are simply a carrier of the Alpha-1 gene, helps you and your family make decisions about work, lifestyle, and having children.

Discuss the advantages and disadvantages of testing with your healthcare provider.

How do you test for Alpha-1?

The AlphaKit is a free blood sample collection kit. The test must be performed by your doctor. You can order a FREE AlphaKit to take to your next doctor's visit at www.MyAlphaTest.com/pro-c.

Modern testing methods are simple, quick, and accurate. They enable diagnosis of Alpha-1 from a few drops of blood dried onto a piece of filter paper.

If you and your doctor agree that testing is right for you, the test will:

  • Measure the level of AAT in your blood
  • Test your DNA to see if you carry 1 or 2 abnormal genes for Alpha-1

SHOULD YOUR FAMILY BE TESTED?

The American Thoracic Society recommends testing for siblings of people with Alpha-1 or carriers of AAT genes. NIH advises testing for family members who may be at risk. Find out who in your family may be at risk. Learn about family testing for Alpha-1.



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. 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. National Institutes of Health. Who is at risk for alpha-1 antitrypsin deficiency? www.nhlbi.nih.gov/health/dci/Diseases/aat/aat_risks.html. Accessed May 15, 2009.