Symptoms and Diseases Caused by Alpha-1

One reason Alpha-1 is so seldom diagnosed is that the symptoms of Alpha-1-induced lung disease are identical to those of other common lung diseases such as emphysema, asthma, and COPD.

In people with Alpha-1, lung symptoms appear between the ages of 30 and 60. If you're a smoker with Alpha-1, you could develop symptoms 10 years earlier than a nonsmoker.1

Common signs and symptoms of Alpha-1 include:2

  • Shortness of breath
  • Wheezing
  • Chronic cough and phlegm production (bronchitis)
  • Recurring chest colds or pneumonia
  • Low tolerance for exercise
  • Non-responsive asthma or year round allergies
  • Chronic widening of the bronchial tubes (bronchiectasis)
  • Family history of lung and/or liver disease

Alpha-1 and lung disease

If you are an Alpha, you have too much neutrophil elastase (an enzyme that can damage your lungs) and not enough AAT (an enzyme that can protect your lungs from excess neutrophil elastase).

Over time, untreated Alpha-1 can cause lung disease, including emphysema.

Learn more about Alpha-1 lung disease at the website of the Alpha-1 Foundation, dedicated to finding a cure for Alpha-1.

Other clinical manifestations: liver diseases

Liver disease is the second most-frequently diagnosed medical problem caused by Alpha-1. Alpha-1 can reduce the amount of AAT released from the liver into the bloodstream, which can cause liver disease at all ages.

Learn more about Alpha-1 liver disease from the Alpha-1 Foundation.

Other clinical manifestations: skin disease

Panniculitis is a rare skin disease caused by Alpha-1, and the least known of its complications. Unmediated proteases can damage the layer of fatty, fibrous tissue under the outer layer of skin.

Learn more about Alpha-1 skin disease from the Alpha-1 Foundation.

Please talk with your doctor for additional information on Alpha-1.

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. Anthonisen N. Chronic obstructive pulmonary disease. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd edition. Philadelphia, PA: Saunders Elsevier. 2008:619-626.
  2. Alpha-1 Antitrypsin Deficiency: A Guide For The Recently Diagnosed Individual. Alpha-1 Foundation. Version 1.6, December 2006.