Emphysema and Alpha-1

Emphysema is a lung disease in which the tiny air sacs (alveoli) in your lungs are overinflated. They overinflate because the walls of your air sacs are damaged. Since your air sacs take in oxygen and release carbon dioxide, this over-inflation makes your lungs less efficient and often results in breathlessness.

Neutrophil elastase and AAT

One cause of damage to your air sacs is the enzyme neutrophil elastase. Normally, neutrophil elastase eliminates bacteria that cause lung infections. Once the infection is resolved, your body uses AAT to stop neutrophil elastase.

If there's not enough AAT, neutrophil elastase keeps working and can destroy healthy lung tissue. Over time, enough air sacs can be destroyed to make your lungs rigid, resulting in emphysema. If you have Alpha-1, you have less AAT and are more likely to develop emphysema.

Is it emphysema due to Alpha-1?

Alpha-1 is often not diagnosed in patients with emphysema because the symptoms are similar. Emphysema is more likely to be related to alpha-1 if there is:1

  • Early-onset emphysema (age 45 years or younger)
  • Emphysema without a known risk factor (smoking, occupational dust exposure, etc.)
  • Lung function decline more than expected for a nonsmoker or for a smoker who quit
  • X-rays or images of your lungs that show damage in the lower parts of your lungs, which is unusual with emphysema

Testing for Alpha-1

The average Alpha patient experiences symptoms for more than 8 years and sees 3 doctors before being correctly diagnosed with Alpha-1.2 This delay is too long, especially considering the destruction of lung tissue prior to diagnosis. Alpha-1 is easily diagnosed with simple blood tests.

Ask your doctor about a free Grifols AlphaKit that can tell if you have 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. Campos MA, Wanner A, Zhang G, Sandhaus RA. Trends in the diagnosis of symptomatic patients with alpha-1 antitrypsin deficiency between 1968 and 2003. Chest. 2005;128(3):1179-1186.