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If you have alpha-1 antitrypsin deficiency (AAT deficiency), there's treatment that can help you can feel better, live longer, and breathe easier.

Augmentation Therapy

There's only one specific treatment that actually targets COPD caused by AAT deficiency -- augmentation therapy. It's also called replacement therapy. It’s been around for 25 years, but it's now attracting more attention. 

It's used alongside the regular COPD treatments.   " Augmentation therapy for [AAT deficiency] seems to be very effective," says Robert A. Sandhaus, MD, PhD, clinical director of the Alpha-1 Foundation and professor of medicine at National Jewish Health in Denver. "I think it could change the prognosis for the condition."

If you have AAT deficiency, you don't have enough of the AAT protein that normally protects the lungs from damage. Augmentation therapy raises your levels of that protein.

You get an infusion through an IV tube into a vein in your arm. Augmentation therapy won't improve your breathing on the spot. But the extra AAT moves through your blood to your lungs and may slow down COPD damage. 

Side effects are usually mild. You may have a headache, muscle aches, or flu-like symptoms that last about a day.

The infusion takes about an hour. You need to get it every week, probably for the rest of your life. Usually, you would get it at a hospital or medical center, or you may be able to learn how to do it at home on your own.

 

How Well Does Augmentation Therapy Work?

There's debate about how well augmentation therapy works. Some studies show it can reduce further lung damage and help people with AAT deficiency live longer. Other studies have had less clear results.

Even so, the bulk of the evidence seems to show it helps. "We don't have the most rigorous evidence yet, but it seems that augmentation therapy can slow down the disease," says Norman Edelman, MD, chief medical officer of the American Lung Association.

Sandhaus is more positive. "I'm very confident it works," he says. Sandhaus is one of the authors of a new study of augmentation therapy. The results of the study will be presented at the American Thoracic Society International Conference in 2013. It was funded by CSL Behring.

is the cause of your copd genetic

Is the Cause of Your COPD Genetic?

Knowing if you have alpha-1 is the first step to getting help from your doctor. An alpha-1 blood test is the only way for you and your doctor to know whether you have alpha-1. And if you do, your doctor can discuss potential treatment options and testing for other family members.

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Important Safety Information

PROLASTIN®-C (alpha1-proteinase inhibitor [human]) is indicated for chronic augmentation and maintenance therapy in adults with clinically evident emphysema due to severe deficiency of alpha1-proteinase inhibitor (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 risk of hypersensitivity.

Hypersensitivity reactions may occur. Should evidence of hypersensitivity reaction be observed, promptly stop infusion and begin appropriate therapy.

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. PROLASTIN-C is contraindicated in patients with antibodies against IgA.

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 and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

Please see PROLASTIN-C full Prescribing Information for complete prescribing details.