Alpha 1-antitrypsin deficiency
Health Tips
The most important factor in preventing disease or slowing disease progression in individuals with Alpha-1 Antitrypsin Deficiency (Alpha-1) is avoidance of risk factors. The biggest risk factor for lung disease is cigarette smoking. In addition to smoking cessation, it is important to avoid second-hand smoke, avoid infection, and avoid occupational inhaled irritant exposures. While intravenous therapy with alpha-1 proteinase inhibitor is available for those with documented lung disease due to Alpha-1, this is only one part of the total management program for the genetic deficiency of alpha-1 antitrypsin.
The Big Fat Reference Guide or “BFRG” is the cornerstone of AlphaNet’s health management and disease prevention program for persons diagnosed with Alpha-1 Antitrypsin Deficiency. With its emphasis on educating patients, their families and their health care professionals, it is considered the most comprehensive guide to living with Alpha-1 available anywhere.
The BFRG provides detailed information specific to Alpha-1 including such topics as medical assessment, testing and treatment, diet and nutrition, activity and fitness, the management of environmental risk factors, coping with Alpha-1, insurance, disability and much more.
The Big Fat Reference Guide is quick and easy to use. After navigating to the AlphaNet web site at www.alphanet.org, click on BFRG. A simple registration allows the BFRG to be personalized to the specific needs of the user.
The following preventative recommendations have been listed in order of importance and practicality and can complement your physician’s plan of care. They are meant to be used as guidelines, and should be implemented and evaluated on an Alpha-by-Alpha basis.
1. PERSONAL HYGIENE
Hand washing is a very effective way to avoid contracting or spreading infectious diseases. The following are very specific guidelines for hand washing:
Use warm water to moisten hands.
Apply anti-bacterial soap.
Rub hands together for at least 20 seconds.
Rinse hands thoroughly.
Dry hands and apply anti-bacterial lotion as desired.
Wash hands after using the restroom, blowing nose, changing diapers, prior to meal preparation, etc.
If it is not possible to wash your hands, carry an antibacterial hand solution.
Avoiding dusty situations is important, including shaking rugs, vacuuming, sweeping and dusting. Avoid breathing airborne irritants, including smoke (second-hand tobacco, fireplace, etc.), construction/paint fumes, and any odorous cleaning products.
2. AVOIDANCE OF TRANSMITTABLE INFECTIOUS DISEASES
The following are situations you should attempt to avoid if possible. The use of a face mask may be of benefit in particularly high risk environments.
Crowds-movie theaters, public transportation, etc.
Airplanes
Children in daycare, school activities
Hospitals
Physician clinics
3. GET ALL VACCINES
(Pneumonia, Influenza, Hepatitis, Meningococcal, Tetanus)
Speak with your physician regarding the need for you to receive these immunizations. When you visit a clinic or a doctor’s office, try to schedule an appointment time that minimizes your exposure to infected patients. If you are receiving home infusions, immunizations may be administered safely by your home infusion nurse with an order from your physician.
4. NUTRITION
Diet plays a critical role in your ability to breathe. Foods low in carbon dioxide production make sense to a person who has trouble with carbon dioxide retention. Excessive carbohydrates in the diet can theoretically create more carbon dioxide than other energy sources.
Eating a number of smaller meals during the day rather than the customary three large meals keeps the digestive process from interfering adversely with the breathing process (meal-induced dyspnea). Vitamin supplements and natural additives to your diet may increase the effectiveness of the digestive process and the resulting energy derived.
Food Safety & Sanitation
The prevalence of food-borne illness seems to be on the rise. Food-borne pathogens and related illnesses are highly preventable with a few simple steps and some planning ahead.
Wash all fruits and vegetables before preparation.
Use separate cutting boards for raw and cooked foods. Wash and rinse cutting boards after each use.
Thoroughly cook meat and eggs before eating.
Thaw meat in the refrigerator or microwave (cook immediately after thawing in microwave). Do not thaw meats by setting them on the counter or putting in an oven.
Proteins and Amino Acids
The meat group supplies an excellent source of protein. A variety of foods are recommended: beef, pork, chicken, turkey, fish/shellfish. The meat group foods supply varying amounts of Iron, Zinc, and B Vitamins (Thiamin, Niacin, Vitamin B-6, and B-12).
Choose lean meats and skinless poultry. Include two to three servings of protein per day, equivalent to 5-7 ounces per day. (A serving is equal to 2-3 ounces of cooked lean meat, poultry or fish.)
Additional excellent sources of protein are eggs, dry beans, tofu, nuts and peanut butter. Other sources of protein that equal to 1 ounce of meat:
1/2 cup cooked lentils, peas or dry beans
1 egg or 1/4 cup egg substitute
2 tablespoons peanut butter or 1/3 cup nuts
4 ounces tofu
Calories
Be sure to consume sufficient calories to meet your energy demands. If you are losing weight undesirably or unexpectedly, you should increase your caloric intake. This keeps your energy level high!
Distribute your caloric intake evenly throughout the day. Four to five small meals per day would be recommended.
Vitamins/Minerals
Vitamin A – It is available in two forms. The first is in the form of retinol (comes from foods of animal origin such as liver, fish oil, eggs, milk fortified with Vitamin A and other Vitamin A fortified foods). The second is in the form of carotenoids (such as beta carotene that convert to Vitamin A in the body, which are found in red, yellow, orange, and many dark-green leafy vegetables).
Vitamin C – Most Vitamin C comes from plant sources. All citrus fruits, including oranges, grapefruit and tangerines, are good sources. Other good sources are berries, melons, peppers, dark-green leafy vegetables, potatoes and tomatoes.
Vitamin E – It is found most abundantly in vegetable oils, salad dressings and margarine. Vitamin E is also found in wheat germ, whole-grain products, seeds, nuts and peanut butter.
Vitamins A, B-6, C, D, and E, Copper, Folic Acid, Selenium and Zinc – These have been shown to regulate immune function. Many vitamins have antioxidant properties as well. Increase your consumption of these vitamins by improving your dietary intake of the food choices listed. Try to get your vitamins from food but supplements may be necessary.
For a versatile food product, try soy! Soybean products are excellent sources of nutrients. Some examples of food products include: soy milk, soy flour, texture soy protein (TSP), tofu, soy nuts and whole soybeans.
Check out a vegetarian cookbook and experiment. There are many soy products available in most grocery stores. If you do not find them, ask your supermarket to order them for you. To order a copy of the U.S. Soyfoods Directory call 1-800-TALKSOY.
5. LIFESTYLE CHANGES
Smoking Cessation – This should be a no brainer, but if you do smoke, it is never too late to quit! You need to STOP SMOKING!!!
Exercise – Understand that exercise is essential to all Alphas. Finely tuned muscles and a body with low body fat requires less oxygen to perform everyday tasks, leaving more oxygen for the greater chore of breathing. Stronger secondary body muscles help take up the slack of flattened diaphragm muscles and improve the success of the process of inhaling. Don’t make drastic changes in your activity level. Start out light. Light physical activities include: walking/strolling, swimming/slow treading and bowling. Also remember your warm up stretches prior to exercise and cool down stretches afterwards.
Stress Management – Seek ways to manage the stress in your life. For example, join a yoga class, practice deep relaxation/breathing, call a friend, play, watch a sunset, laugh or take a pottery class. Practice stress reduction daily and often. Through concentrated relaxation and breathing, stress can be more effectively managed and the breathing difficulties associated with high anxiety and panic can be better controlled.
Sleep Patterns – Strive for 6-8 hours of sleep per night. It may be time to evaluate your mattress for comfort and quality. Go to bed to sleep. Watching television or working while in bed may interrupt your desire to sleep. If you wake up, get out of bed and do an activity until you feel tired, then return to bed. Make sure you consult with your doctor if your sleep patterns are interrupted or not restful.
6. MEDICATIONS: STICK TO YOUR REGIMEN!
Please review the correct use of your Bronchodilators with your pharmacist and/or doctor.
Inhaled Corticosteroids: It is important to rinse your mouth thoroughly following each time you use your steroid inhaler or you may be at increased risk of an infection in your mouth.
Metered Dose Inhalers (MDI): Your doctor may prescribe a spacer to maximize the benefits and reduce side effects from these devices.
Initiate Prompt/Aggressive Treatment of upper and lower respiratory tract infections. The single most important factor in Alpha-1 disease progression is infection. During the flu and cold season it is very important that, if you begin to feel ill, you monitor your temperature. Likewise, if you develop increased shortness of breath, a fever or have a significantly increased amount of nasal drainage or a cough productive of sputum that is any color other than clear, you should seek medical attention as soon as possible to avoid the development of pneumonia.
7. PULMONARY HYGIENE
Oxygen Saturation Monitoring: If you are on oxygen therapy and you have an oxygen saturation monitor, changes in your oxygen saturations may be an early sign of infection or allergic reaction. Seek medical advice with changes in any of these parameters.
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