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Why is nutrition so important for people with alpha1-antitrypsin (AAT) deficiency?
Proper eating habits are essential to maintaining healthy lung function. Without appropriate intake of calories and a proper mix of nutritional foods, problems with weight control can occur, which may add to existing problems with lung function. Below you will find information about:
Good Nutrition and Lung Function
Good nutrition can help maintain lung function in 3 important ways:
1. Improve muscle function
The body's supply of energy is lowered greatly when there is not enough nutrient intake. Under these conditions muscle tissue, including muscles involved in breathing, may be used to meet energy needs. Eventually, this process can lead to reduced strength, increased fatigue, and breathlessness. Improper eating can also have an adverse effect on the muscle most involved in breathing, the diaphragm. A healthy diet helps to preserve the body's energy and strength.
2. Increase "triggers" for breathing
People with alpha1-antitrypsin (AAT) deficiency often have low levels of oxygen in the blood. This causes the central nervous system to send signals to the lungs to activate, or "trigger," increased breathing.1 Poor nutrition can adversely affect this signaling system and exacerbate the problem of low oxygen (O2) levels. Proper eating habits help avoid these adverse effects.
3. Improve resistance to infection
Poor nutrition increases the body's susceptibility to lung infections, which makes it even more difficult for the lungs to remove bacteria. Avoiding infection in the lungs is particularly important to people with AAT deficiency. Good eating habits are an important way to maintain your health.
Why Do So Many People With AAT Deficiency Lose Weight?
Weight loss is a common problem in people with chronic lung diseases such as AAT deficiency, with as many as 40% of people experiencing progressive weight loss. Unfortunately, the reasons for this are not well understood. It may be that people with AAT deficiency and other lung diseases ingest fewer calories. However, it is more likely that people with lung disease use up more energy for breathing. Therefore, what may be enough food for a person with normal lung function may not be sufficient for a person with AAT deficiency.
Even at rest, people with chronic lung diseases have increased energy demands as measured in a test called Resting Energy Expenditure (REE). It seems that people with AAT deficiency and other lung diseases burn more calories because of the increased energy requirements associated with their breathing.
Consult with your doctor or registered dietician if you find that you are losing weight. They can provide specific recommendations to help you gain and maintain the weight that is healthiest for you.
Can Gaining Too Much Weight Cause Problems As Well?
Yes. While it is important for people with AAT deficiency to eat enough, eating too much can also place added demand on the lungs. People with AAT deficiency should strike a balance between their energy requirements and their food intake.
Are The Kinds Of Food I Eat Important?
Yes, very much so. People with AAT deficiency have very specific nutritional needs. This is because the body metabolizes (breaks down) different kinds of nutrients in different ways.
Obstructive lung disease produces an unusually high level of carbon dioxide in the bloodstream. This can lead to weakness in the diaphragm and other muscles used for breathing, which in turn may lead to respiratory failure. The goal of proper dietary management for people with AAT deficiency is to provide a balance of foods that decrease carbon dioxide production and the resulting demand on the lungs. While specific needs vary, there are several dietary guidelines that are common to all people with obstructive lung disease:
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Increase fat and decrease carbohydrate consumption
High-fat, low-carbohydrate diets minimize production of carbon dioxide, which decreases the demand on the lungs.
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Meet but do not exceed caloric requirements
An insufficient intake of calories increases the demand on the lungs. Excess calories cause a similar effect by increasing carbon dioxide production.
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Avoid excessive fluid intake
Excess fluids put a strain on the lungs by increasing pressure within the blood vessels of the lungs.
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Maintain adequate levels of phosphorous
Very low levels of phosphorous have been reported to cause respiratory failure. Your doctor should monitor phosphorous levels and prescribe supplementation if necessary. Good dietary sources of phosphorous include dairy foods, lean meats, fish, beans, peas, whole grain cereals, and nuts. |
Before making any dietary changes, be sure to consult your doctor or a registered dietitian.
What Kinds Of Foods Are Best At Lowering Carbon Dioxide Production?
Foods are rated by their respiratory quotient (RQ). RQ measures the amount of carbon dioxide produced versus the amount of oxygen consumed when certain foods are broken down by the body.
| RQ = |
Amount of carbon dioxide produced
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Amount of oxygen consumed |
The lower the RQ, the lower the amount of carbon dioxide produced. Because the goal of dietary management for people with obstructive lung disease is to reduce the amount of carbon dioxide in the bloodstream, it is important to eat foods with a low RQ. The RQs of the 3 major kinds of nutrients are:
Carbohydrates
Protein
Fat |
1.0
0.8
0.7 |
This means that for a given amount of oxygen consumed, fats produce the lowest amount of carbon dioxide and carbohydrates produce the highest.
What Kind Of Diet Is Right For Me?
In general, a diet that is somewhat higher in fat and lower in carbohydrates is best for most people with AAT deficiency. It's important, however, to take in only the level of calories needed, because too much food can lead to obesity, which puts an added strain on the lungs. It's always best to speak with your doctor or a registered dietician to determine an optimal nutritional plan for you.
Should I Consider Nutritional Supplements?
There are nutritional supplements designed specifically for people with obstructive lung diseases. They may be beneficial in helping you achieve an optimal balance of nutrients. Speak with your doctor or dietitian to determine if dietary supplements are right for you.
Key Points to Remember:
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Proper nutrition is vital to maintaining adequate lung function
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People with AAT deficiency have specific dietary needs
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Work with your doctor or a registered dietitian to develop a nutritional plan that is right for you |
Reference:
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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. |
Important Safety Information
Prolastin®, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic replacement therapy of individuals having congenital deficiency of alpha-1 PI (alpha1-antitrypsin deficiency) with clinically demonstrable panacinar emphysema. Weekly Prolastin® therapy has demonstrated a low occurrence of side effects. In clinical studies with Prolastin®, reactions were observed in 1.16% of infusions, the most common events being fever (0.77%), light-headedness (0.19%), and dizziness (0.19%). As with all plasma-derived therapeutics, the potential to transmit infectious agents cannot be totally eliminated. Individuals with selective IgA deficiencies who have known antibody against IgA (anti-IgA antibody) should not receive Prolastin®, since these patients may experience severe reactions, including anaphylaxis, to IgA which may be present.
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.
Please click here for Prolastin® full Prescribing Information.
Remember, your doctor or healthcare provider is the single best source of information regarding you and your health. Please consult your doctor or healthcare provider if you have any questions about your health or any of your medications.
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