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

PROLASTIN-C 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). PROLASTIN-C is a more purified and more concentrated version of PROLASTIN®, the leading Alpha-1 therapy in the US for more than 25 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


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. Alpha-1 Antitrypsin Deficiency: A Guide For The Recently Diagnosed Individual. Alpha-1 Foundation. Version 1.6, December 2006.
  2. Meds for Alpha-1. AlphaNet. Big Fat Resource Guide. April 2005. http://www.alphanetbfrg.org/pdfs/ Medications-for-Alpha-1-Lung-Disease.pdf. Accessed December 7, 2009.
  3. Augmentation Therapy. AlphaNet. Big Fat Resource Guide. 2008. http://www.alphanetbfrg.org/pdfs/ Augmentation-Therapy.pdf. Accessed December 7, 2009.
  4. PROLASTIN-C [package insert]. Grifols
  5. American Thoracic Society. Patient Information: Pulmonary Rehabilitation. January 2009. http://www.thoracic.org/sections/clinical-information/pulmonary-rehabilitation/patient-information/pages/pulmonary-rehabilitation.html