People with lung disease related to AAT deficiency have a number of therapy options available. Talk to your healthcare provider about the options that are best for you.
Take Care of Your Lungs
It is very important for Alpha-1 patients to do everything possible to take care of their lungs. Some protective measures your doctor may recommend include:
- Stop smoking – the first and most essential protective step
- Avoid all forms of tobacco smoke, including second-hand smoke from other smokers
- Avoid occupational and environmental pollutants, including dust and pollen
- Avoid wood-burning stoves
- Avoid fumes from household cleaning products
- Avoid paint and/or other toxic agents
Stay as Healthy as Possible
It is also important for Alpha-1 patients to maintain overall good health. Your doctor may recommend that you:
- Participate in a pulmonary rehabilitation program
- Follow a good nutrition and exercise program
- Stay current with immunizations for flu and pneumonia
- Reduce alcohol consumption
- Keep regular physician appointments, take prescribed medications, and follow professional advice
- Avoid exposure to people who are sick
- Develop a stress-management program
“Pulmonary rehabilitation” combines many of the lifestyle and lung-protection recommendations into a structured program. Ask your doctor whether a pulmonary rehabilitation program could help you control symptoms and achieve your highest level of activity.
For Treatment of Respiratory Symptoms
Some medications are available to help people who have breathing problems associated with asthma, emphysema, and COPD:
- Bronchodilators (to reduce airway constriction)
- Corticosteroids (to reduce inflammation)
- Supplemental oxygen
For Treatment of Infections
To help prevent further lung damage, your doctor may also want to treat any upper respiratory infections with antibiotics as soon as possible. Be sure to contact your doctor at the first sign of an infection.
For Treatment of Mental Health Symptoms
Sometimes people with breathing difficulties experience depression, panic, or anxiety. There are therapies and/or medications that your doctor can prescribe to treat these conditions. There are also medications and devices available to help manage sleep disorders.
Some Alpha-1 patients with advanced lung disease may be candidates for lung transplantation. This option may be considered if there is advanced lung disease that has not responded to more conservative therapy.
AAT augmentation with an alpha1-proteinase inhibitor (A1-PI) such as GLASSIA or ARALAST NP may be appropriate for you if you have alpha-1 antitrypsin (AAT) deficiency with clinically evident emphysema. AAT augmentation can help increase the low levels of AAT in the blood and in the lungs. AAT augmentation is not a cure for AAT deficiency. It cannot repair damage that has already occurred in your lungs.1,2
Clinical data demonstrating the long term effects of chronic augmentation or replacement therapy of individuals with GLASSIA or ARALAST NP are not available.
AAT augmentation is administered intravenously (in a vein) once a week. You can receive this infusion in your home or workplace.
GLASSIA [Alpha1-Proteinase Inhibitor (Human)]
GLASSIA is indicated for chronic augmentation and maintenance therapy in individuals with emphysema due to congenital deficiency of alpha1-proteinase inhibitor (Alpha1-PI), also known as alpha1-antitrypsin (AAT) deficiency.
- The effect of augmentation therapy with GLASSIA or any Alpha1-PI product on pulmonary exacerbations and on the progression of emphysema in Alpha1-PI deficiency has not been demonstrated in randomized, controlled clinical trials.
- Clinical data demonstrating the long term effects of chronic augmentation and maintenance therapy of individuals with GLASSIA are not available.
- GLASSIA is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.
Detailed Important Risk Information for GLASSIA
- GLASSIA is contraindicated in IgA deficient patients with antibodies against IgA. GLASSIA is contraindicated in individuals with a history of severe immediate hypersensitivity reactions, including anaphylaxis, to Alpha1-PI products.
- GLASSIA is made from human plasma. It may carry a risk of transmitting infectious agents, such as viruses, and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
- Administer GLASSIA at room temperature at a rate not greater than 0.04 mL/kg body weight per minute. If anaphylactic or severe anaphylactoid reactions occur, discontinue the infusion immediately.
- Administer GLASSIA within 3 hours of entering the vials.
- Safety and effectiveness in patients over 65 years of age have not been established.
- Two serious adverse reactions observed on two separate occasions during clinical studies with GLASSIA were cholangitis and exacerbation of chronic obstructive pulmonary disease (COPD).
- The most common product-related adverse reactions in clinical studies were headache and dizziness.
Please see GLASSIA Full Prescribing Information for full prescribing details.
ARALAST NP [Alpha1-Proteinase Inhibitor (Human)]
ARALAST NP is indicated for chronic augmentation therapy in patients having congenital deficiency of A1-PI with clinically evident emphysema.
- The effect of augmentation therapy with ARALAST NP on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials.
- Clinical data demonstrating the long-term effects of chronic augmentation or replacement therapy of individuals with ARALAST NP or ARALAST are not available.
- ARALAST NP is not indicated as therapy for lung disease patients in whom congenital A1-PI deficiency has not been established.
Detailed Important Risk Information for ARALAST NP
- ARALAST NP is contraindicated in IgA deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity.
- ARALAST NP is derived from pooled human plasma. It may carry a risk of transmitting infectious agents, e.g., viruses and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
- The recommended rate of administration (≤0.08 mL/kg/min) should be closely followed and vital signs monitored continuously. If anaphylactic or severe anaphylactoid reactions occur, the infusion should be discontinued immediately.
- Safety and effectiveness in patients over age 65 years of age have not been established.
- ARALAST NP should be administered at room temperature within three (3) hours after reconstitution and should be administered alone, without mixing with other agents or diluting solutions.
- The safety of ARALAST NP was evaluated with ARALAST in a crossover clinical PK comparability study. The most common adverse events deemed related to ARALAST NP included headache and musculoskeletal discomfort. No serious adverse reactions or deaths were reported in the study. In the ARALAST pivotal study, the most common adverse events were headache and somnolence.
Please see ARALAST NP Full Prescribing Information for full prescribing details.
- 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;168:818-900.
- ARALAST NP [Alpha1–Proteinase Inhibitor (Human)] Prescribing Information, Baxter International Inc., Westlake Village, CA; April 2010.