PROLASTIN-C, Alpha-1 - Proteinase Inhibitor (Human)

Medical treatment for Alpha-1

While there is no cure for Alpha-1, there is a lot you can do to treat and manage it. Drug therapy, preventative treatment, and rehabilitation programs are all important components in
managing Alpha-1.

Augmentation therapy

Augmentation therapy “augments” or adds to the low level of AAT produced by your body. Augmentation therapy is an intravenous (IV) infusion of AAT derived from human plasma. It increases the concentration of AAT in your blood and lungs. Infusions are typically given weekly.^1,3

Augmentation is the only pharmaceutical therapy specifically approved by the FDA for the treatment of Alpha-1 deficiency. Prolastin-C has been demonstrated to raise the plasma level of Alpha1-PI, but that the effect of this augmentation on pulmonary exacerbations and on the rate of progression of emphysema has not been demonstrated in adequately powered, randomized, controlled clinical trials for any Alpha1-PI product. Discuss augmentation therapy with your doctor.³

New PROLASTIN-C is now approved for chronic augmentation and maintenance therapy in adults with emphysema due to AAT deficiency. PROLASTIN-C is a more purified and more concentrated version of PROLASTIN®, the leading Alpha-1 therapy in the US for more than 20 years.

You can have augmentation administered in a doctor's office, hospital, clinic, at home or anywhere IV infusions are given. Prolastin Direct^® coordinates infusion services anywhere you need it. Check with your insurance carrier; criteria for services and benefits vary.

If you are motivated and physically able, you may be taught to self-infuse and administer your own augmentation therapy. A willing family member or significant other may also be trained. If you are interested in self-infusion, discuss it with your doctor and develop a plan of care. You must identify a nurse willing to work with you on a consistent basis to complete training on all aspects of therapy. Your safety with home self-infusion is always the primary goal

Drug therapy for lung diseases

Bronchodilators

Your doctor may prescribe bronchodilators for your Alpha-1 lung-related symptoms. Bronchodialtors dilate or open up your airways so more air flows in and out.

There are 2 main types of bronchodilators: beta-agonists and anticholinergics. Beta-agonists can be either quick-acting "rescue" medications that work in 5 to 15 minutes and last from 4 to 6 hours or long-acting maintenance medicines that work in 30-40 minutes and last 12 hours. Anticholinergics can also be short-acting or long-acting maintenance medicines.²

Corticosteroids

Your doctor may prescribe oral or inhaled corticosteroids for your Alpha-1 lung-related symptoms. Corticosteroids can be used as a preventive treatment or used to treat flare-ups, also known as "exacerbations."²

Supplemental oxygen

Some people with Alpha-1 need supplemental oxygen. Oxygen is important if you have low blood oxygen levels, during active infections, and/or with progressive destruction of the lung tissue. Supplemental oxygen may be needed during exercise, during sleep, and when traveling by air, because cabin pressure changes with altitude.¹

Learn more about using supplemental oxygen therapy and supplies on the Web site of the Alpha-1 Association.

Pulmonary rehabilitation⁵

Pulmonary rehabilitation is a program of education and exercise classes that teaches you about your lungs, how to exercise and do activities with less shortness of breath, and how to "live" better with your lung condition.

By attending education classes, you will learn many things about your lungs, such as: what is wrong with your lungs, what your medicines do, when to call your health care provider, and how to keep from being hospitalized.

The exercise classes will help you be more active with less shortness of breath. Usually, you will be exercising both your arms and legs. The exercise classes will help you feel better and become stronger by helping you get into better shape.

Some programs offer help with quitting smoking as part of the pulmonary rehabilitation program. Others require that you stop smoking before beginning the program.

Talk to your doctor about a pulmonary rehabilitation program to see if it might be a good choice for you.

For more information about programs in the United States, contact:

• American Lung Association, telephone 1-800-LUNGUSA, or www.lungusa.org or contact your State or local chapter of the Lung Association.
• American Association for Cardiovascular and Pulmonary Rehabilitation (AACVPR), telephone 312-644-6610 or www.aacvpr.org

Vaccinations

If you have Alpha-1, your lungs are vulnerable to pollutants and infections, and vaccines can help you protect them. Get a yearly flu vaccine and a Pneumovax® shot, which protects against certain infections, every 5 to 6 years. Ask your doctor if you need a vaccine for hepatitis A and B. These are especially important if you have established liver disease. Recommendations:¹

• Annual flu vaccine
• Pneumovax vaccine (every 5 to 6 years)
• Hepatitis A vaccine
• Hepatitis B vaccine

Treatment of lung infections

As an Alpha, you need to treat infections and control lung inflammation promptly and aggressively. When you have an infection anywhere in your body, you produce additional neutrophil elastase. Your lungs attract more leukocytes when an infection is present, and leukocytes release neutrophil elastase. Notify your doctor immediately if you suspect a lung infection and ask if antibiotics could help speed recovery. ¹

Watch carefully for these symptoms:

• Fever
• Increased shortness of breath
• Increased coughing (may not be productive)
• Chills with fever
• Changes in color of phlegm

Evaluation of liver complications

If you have Alpha-1, make sure that your family, and caregivers know about complications related to liver disease. Carefully read the labels on over-the-counter medications. Inform your doctor or other healthcare provider if any alternative medicines or vitamin supplements are being taken.

Tell your doctor if you experience any symptoms of liver disease such as increased swelling of your abdomen or extremities, purplish or blackish colored stools, change in appearance or jaundice or other symptoms.

For more information on what symptoms may suggest liver complications, refer to the educational materials below.

A Guide for the Individual Recently Diagnosed With Alpha-1

AlphaNet Guide to Staying Healthy

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