The Alpha-1 Patient Profile

Test ALL COPD patients regardless of age, smoking history, or FEV1 decline1

Age, smoking, FEV1

Age, smoking history, and FEV1 levels alone cannot predict who has alpha-12

Alpha-1 can be found at all ages3,4

While the symptoms of alpha-1 usually appear between the ages of 20 and 50 years of age, data obtained over a 3-year period (2004–2007) from the renowned Alpha-1 Genetics Laboratory in Florida showed that the majority of newly diagnosed patients were 50 years of age or older.3,4

Given the disparity in patient diagnoses, ALL COPD patients should be tested for alpha-1, regardless of age.1

Smokers and ex-smokers make up a large part of the diagnosed alpha-1 population
Smoking accelerates lung function decline in patients with alpha-1. In a national registry study of 1,129 patients with alpha-1, 80% were either current smokers (8%) or ex-smokers (72%).2,5
Given the disparity in patient diagnoses, ALL COPD patients should be tested for alpha-1, regardless of smoking history.1

FEV1 levels alone should not determine who to test
These readings can be misleading, as some nonsmokers have low FEV1 levels and some smokers have high FEV1 levels.2

Given the disparity in patient diagnoses, ALL COPD patients should be tested for alpha-1, regardless of FEV1 levels.1

FEV1=forced expiratory volume in 1 second.

An alpha-1 family tree

When you identify an alpha-1 patient, you identify an entire family
at risk1

Any deficient allele should prompt an immediate discussion of testing the whole family.6
Alpha-1 patients with at least one deficient allele have a genetic susceptibility to lung disease. Early testing is essential so that family members—if tested positive for alpha-1—may utilize disease-management programs to potentially limit the progression of lung disease.1,6

Family testing may help reduce the risk of alpha-1–attributable lung disease for future generations6,7


Next: Test for Alpha-1 >


IMPORTANT SAFETY INFORMATION

PROLASTIN®-C LIQUID is an alpha1-proteinase inhibitor (human) (alpha1-PI) 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).

Limitations of Use


  • The effect of augmentation therapy with any alpha1-PI, including PROLASTIN-C LIQUID, on pulmonary exacerbations and on the progression of emphysema in alpha1-PI deficiency has not been conclusively demonstrated in randomized, controlled clinical trial
  • Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with PROLASTIN-C LIQUID are not available
  • PROLASTIN-C LIQUID is not indicated as therapy for lung disease in patients in whom severe alpha1-PI deficiency has not been established

PROLASTIN-C LIQUID is contraindicated in immunoglobulin A (IgA)-deficient patients with antibodies against IgA or patients with a history of anaphylaxis or other severe systemic reaction to alpha1-PI products.

Hypersensitivity reactions, including anaphylaxis, may occur. Monitor vital signs and observe the patient carefully throughout the infusion. If hypersensitivity symptoms occur, promptly stop PROLASTIN-C LIQUID infusion and begin appropriate therapy.

Because PROLASTIN-C LIQUID 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.

The most common adverse reactions during PROLASTIN-C LIQUID clinical trials in >5% of subjects were diarrhea and fatigue, each of which occurred in 2 subjects (6%).

Please see full Prescribing Information for PROLASTIN-C LIQUID.


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. DeMeo DL, Sandhaus RA, Barker AF, et al. Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency. Thorax. 2007;62(9):806-813.
  3. National Institutes of Health. Alpha-1 antitrypsin deficiency. Genetics home reference. http://ghr.nlm.nih.gov/condition/alpha-1-antitrypsin-deficiency. Accessed April 17, 2014.
  4. Data on file, Alpha-1 Genetics Laboratory.
  5. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin. Am J Respir Crit Care Med. 1998;158(1):49-59.
  6. Campos MA, Wanner A, Zhang G, Sandhaus RA. Trends in the diagnosis of symptomatic patients with α1-antitrypsin deficiency between 1968 and 2003. Chest. 2005;128(3):1179-1186.
  7. Vidal R, Blanco I, Casas F, Jardí R, Miravitlles M; Committee on the National Registry of Individuals with Alpha-1 Antitrypsin Deficiency. Guidelines for the diagnosis and management of α1-antitrypsin deficiency. Arch Bronconeumol. 2006;42(12):645-659.