Lifestyle changes for Alphas

When you have Alpha-1, your body's defenses against lung and liver diseases are down. While these lifestyle changes cannot cure Alpha-1, they can reduce the impact of low-AAT on your body.

Protect your lungs from irritants

Smoking increases the amount of neutrophil elastase in your lungs and may speed up the progression of lung damage. Quitting may not be easy, but it may be done. Don't give up if your first attempt to stop smoking fails. Keep trying and chances are you will be successful. Discuss therapeutic and behavior modification programs with your doctor.7

Smoking Accelerates Lung Function Decline in Alphas3

Second-hand smoke is dangerous if you have Alpha-1. If someone in your household smokes, ask them to stop, or step outside when smoking, or avoid them when they smoke.7

If you live in the United States, call the American Lung Association (800-LUNG-USA, 800-586-4872) or get more information about quitting on their Web site.

Prevent infections and treat them aggressively. Your body responds to infection by producing white blood cells that release neutrophil elastase, which can damage your lungs. Call your doctor at the earliest signs of cold or flu. Follow your doctor's advice for appropriate treatment. Ask about vaccines for flu and pneumonia. Avoid contact with people with colds. Washing your hands helps.7

Avoid environmental pollutants, both at home and in the workplace, if possible.

  • Formaldehyde is a common chemical found in glues for building and furnishing products, including carpets, upholstery, particle board, and plywood. These products can release formaldehyde into the air, causing lung irritation.4
  • Asbestos mineral fibers are used in roofing and flooring materials, wall and pipe insulation, spackling compounds, cement, coating materials, heating equipment, and acoustic insulation. Asbestos fibers are released into the air if the material is disturbed or disintegrates with age. When inhaled, asbestos fibers scar the lungs, causing cancer of the lungs or abdominal cavity.5,4
  • Pollutants in the workplace are harmful if you have Alpha-1. Wear a mask around dust or vapors. Ask to be moved to a place with more favorable conditions. Consult an environmental or occupational physician, if necessary.7
  • Air pollution can hurt if you have Alpha-1. Stay indoors when ozone values are too high. Consider moving to a less polluted area.7
  • Wood stoves generate harmful, lung-damaging smoke particles.7
  • Home heating and air conditioning filters must be changed on a regular basis to reduce inhaled particles.7

Try to achieve proper nutrition

Good nutrition helps maintain lung function 3 ways:

  1. Improves muscle function—Your supply of energy is lowered when you don't eat enough. Muscle tissue, including your breathing muscles, may be used to meet energy needs. This can lead to reduced strength, increased fatigue, and breathlessness. Bad nutrition can have an adverse effect on your diaphragm. A healthy diet helps preserve your body's energy and strength.1
  2. Increases "triggers" for breathing—With Alpha-1, you may have low levels of oxygen in your blood. This causes your central nervous system to tell your lungs to increase breathing. Poor nutrition adversely affects signaling and exacerbates your problem of low oxygen levels. Proper eating habits help avoid this.1
  3. Improves resistance to infection—Poor nutrition may increase your susceptibility to lung infections, which makes it harder for your lungs to remove bacteria . Avoiding infection in your lungs is important if you have Alpha-1. Good eating habits are an important way to maintain your health.6

You have special dietary needs

Because your body breaks down different nutrients in different ways, lung disease produces a lot of CO2 in your bloodstream. This leads to weakness in your diaphragm and other breathing muscles, which leads to respiratory failure . The goal is to decrease foods that produce CO2 and the resulting demand on your lungs.1

There are several guidelines that are common for people with Alpha-1:

  • Increase fats and decrease carbohydrates
  • Do not exceed caloric requirements
  • Avoid excessive fluid intake
  • Maintain adequate levels of phosphorous by eating dairy foods, lean meats, fish, beans, peas, whole grain cereals, and nuts

Consult your doctor or a registered dietitian before making any dietary changes.


Maintain proper weight

Weight loss is a common problem for people with chronic lung diseases such as Alpha-1; up to 40% of people with Alpha-1 have progressive weight loss . One theory is that people with Alpha-1 take in fewer calories. Another idea 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 a person with Alpha-1.1

While it's important to eat enough, eating too much can add to the burden on your lungs. Strike a balance between energy needs and food intake.

Consult with your doctor or registered dietician if you lose or gain weight for recommendations about maintaining your proper weight.


Exercise

Routine exercise helps improve mental outlook, stamina, and physical well-being. The long-term benefits are significant.1,2

Discuss your goals with your doctor, trainer, or exercise physiologist, and tailor an exercise program to your needs. Many people find that simple, routine stretching exercises, walking, or bicycling improves exercise tolerance.

The key to success is to follow the regimen that has been designed for you.

The right amount of exercise is different for each individual depending on your physical well-being and lung function. Start with low-intensity activity such as walking. Chart out a plan and keep a record of your exercise. After 3 to 4 weeks, you should see progress. Set realistic goals and refine them as your tolerance and stamina improve.1,2


Reduce stress

Managing stress may help you feel better and avoid adverse effects on your health. Stress management may offer benefits such as improved breathing, reduced shortness of breath, and relief of anxiety through relaxation techniques.7

Breathing exercises and muscle relaxation are common ways to relieve stress. Other techniques include biofeedback, visualization, hypnosis, systematic desensitization, positive thinking, and yoga.

Talk to your doctor for more information on specific techniques. The following is a brief summary of stress management techniques:7,8

  • Muscle relaxation can improve your overall sense of well-being and comfort. Through repetition, you will learn to recognize and respond to the signs of muscle tension
  • Biofeedback can reinforce proper breathing and relaxation techniques. This technique teaches you to use muscles properly to relieve stress
  • Visualization helps give negative feelings a mental representation and changes that image into something positive. Music and artwork may enhance the relaxation benefits of visualization
  • Hypnotherapy is guided relaxation that helps you recall a pleasurable experience and return to that moment during times of stress
  • Systemic desensitization can help you feel comfortable with stress-producing events
  • Positive thinking involves changing negative thoughts into positive ones
  • Yoga involves posture control and slow, deep breathing. With practice, you can make this beneficial breathing pattern an instinctive reflex

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. Goldstein SA, Askanazi J. Exercise, diet, and the rehabilitation of lung patients. In: Winick M, ed. Nutrition and Exercise. New York, NY: John Wiley & Sons; 1986:183-186.
  2. Bach JR, ed. Pulmonary Rehabilitation: the Obstructive and Paralytic Conditions. Philadelphia, PA: Hanley & Belfus; 1996.
  3. Alpha-1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Am J Respir Crit Care Med. 1998;158(1):49-59.
  4. Environmental Protection Agency. An Introduction to Air Quality: Formaldehyde. http://www.epa.gov/iaq/formalde.html. Accessed November 6, 2009.
  5. Environmental Protection Agency. An Introduction to Air Quality: Asbestos. http://www.epa.gov/iaq/asbestos.html. Accessed November 6, 2009.
  6. Preventing Infection. http://www.preventinfection.org /AM/Template.cfm?Section=Information_Center. Accessed December 4, 2009.
  7. Alpha-1 Antitrypsin Deficiency: A Guide For The Recently Diagnosed Individual. Alpha-1 Foundation. Version 1.6, December 2006.
  8. Coping with Alpha-1. Stress Management Tools. AlphaNet. Big Fat Resource Guide. April 2005. http://www.alphanetbfrg.org/BFRG.aspx?ID=1191. Accessed December 6, 2009.