- Alpha-1 Antitrypsin Deficiency
- Prevalence
- Signs/Manifestations
- Alpha-1 differential:
- Emphysema
- COPD
- Asthma
- Bronchitis
- Etiology & Heredity
The leading alpha1 augmentation therapy for 20 years is now improved
While lifestyle changes cannot cure Alpha-1, they can reduce the impact of low-AAT on your patient’s body.
Smoking increases the amount of neutrophil elastase in lungs and may speed up the progression of lung damage. Assure your patients that quitting may not be easy, but it may be done. Discuss therapeutic and behavior modification programs with your patients.
If your patients live in the United States, they can call the American Lung Association (800-LUNG-USA, 800-586-4872) or get more information on quitting.
Learn more about how smoking can accelerate lung function decline.
Prevent infections and treat them aggressively. Your patient responds to infection by producing neutrophil elastase, which can damage lungs.
Avoid environmental pollutants, both at home and in the workplace, if possible.
Second-hand smoke is dangerous if your patient has Alpha-1. If someone in the household smokes, your patient should ask them to stop, or step outside when smoking, or avoid them when they smoke.
Pollutants in the workplace are harmful. If your patient has Alpha-1, he or she should wear a mask around dust or vapors. They can ask to be moved to a place with more favorable conditions. Together, you can consult an environmental or occupational physician, if necessary.
Air pollution can hurt patients with Alpha-1. Advise them to stay indoors when ozone values are too high, and consider moving to a less polluted area.
Wood stoves generate harmful, lung-damaging particles.
Home heating and air conditioning filters must be changed on a regular basis to reduce inhaled particulates.
Help Alphas stay healthy. Managing Environmental Risk Factors, a brochure from AlphaNet that you can share with your patients.
Good nutrition helps maintain lung function 3 ways:
Lung disease produces a lot of CO2 in the bloodstream. This leads to weakness in the diaphragm and other breathing muscles, which can lead to respiratory failure. The goal is to decrease foods that produce CO2 and the resulting demand on the lungs.
There are several guidelines that are common for Alphas:
Nutrients have respiratory quotients (RQ): the amount of CO2 produced versus the amount of oxygen consumed. The lower the RQ, the lower the CO2. The RQs of the 3 major kinds of nutrients are:
A diet higher in fat and lower in carbohydrates is best for Alphas, but only to provide calories they need. Obesity puts added strain on lungs.
There are nutritional supplements designed specifically for people with obstructive lung diseases. They may help your patients balance their nutrients.
Weight loss is a common problem in patients with chronic lung diseases such as Alpha-1; up to 40% of patients have progressive weight loss. One theory is that people with Alpha-1 take in fewer calories. Another is that people with Alpha-1 burn more calories because they use more energy breathing. What may be enough for a person with normal lung function may not be enough for an Alpha.
Routine exercise improves mental outlook, stamina, and physical well-being. The long-term benefits are significant.1,2
Discuss goals with your Alphas, and tailor an exercise program to their needs. Many patients find that simple, routine stretching exercises, walking, or bicycling improves exercise tolerance.
The right amount of exercise is different for each individual depending on physical well-being and lung function. Advise them to start with low-intensity activity such as walking. Chart out a plan and encourage them to record their progress. After 3 to 4 weeks, they should see progress. Set realistic goals and redefine them as tolerance and stamina improve.1,2
Managing stress will help Alphas feel better and avoid adverse effects on health. Stress management offers benefits such as improved breathing, reduced shortness of breath, and relief of anxiety through relaxation techniques.
Breathing exercises and muscle relaxation are the most common ways to relieve stress. Other techniques include biofeedback, visualization, hypnosis, systematic desensitization, positive thinking, and yoga.
The following is a brief summary of stress management techniques:
PROLASTIN-C, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic augmentation and maintenance therapy in adults with emphysema due to deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency). The effect of augmentation therapy with any alpha1-proteinase inhibitor (alpha1-PI) on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials. PROLASTIN-C is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.
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 reactions during clinical trials in ≥ 1% of subjects were chills, malaise, headache, rash, hot flush, and pruritus.
PROLASTIN-C is made from human plasma. Products made from human plasma may carry a risk of transmitting infectious agents, e.g., viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
Please see accompanying PROLASTIN-C Full Prescribing Information for complete prescribing details.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.