When jubilant researchers announced at a news conference that they had found the gene responsible for cystic fibrosis, an inherited disease that begins to clog the lungs with a thick, life-threatening mucus in early childhood, the news sent a shock wave of excitement throughout the dozens of cystic fibrosis centers across the nation. It might take another decade of work to figure out how to repair the gene, the researchers concluded, but clearly, a cure is at hand.
“Everybody who is working with cystic fibrosis children and their families today does it with a different sense of hope than we had a few years ago,” says Virginia Stallings, M.D., nutrition chief at the University of Pennsylvania’s Children’s Hospital of Philadelphia. “This used to be thought of as a fatal disease. Now we know that the more years we can add to the window of excellent health, the better the chance at one of the new therapies.”
And some experts believe that nutrition is a big part of getting that opportunity.
| Food Factors Some of the dietary recommendations suitable for the general population may be downright harmful for people with cystic fibrosis. "Everything is reversed," explains Donna Mueller, R.D., Ph.D., associate professor of nutrition and foods at Drexel University in Philadelphia. "Good nutrition in cystic fibrosis can sometimes appear to be the antithesis of how we were taught." In fact, says Dr. Mueller, "we tell patients that going to fast-food restaurants is great, because the food is high-fat, high-sodium and high-protein!" Here are some other "radical" recommendations for those with cystic fibrosis. Pig out. "Eat lots of calories," says Dr. Mueller. Cystic fibrosis demands a lot of metabolic energy. And the only way to get it is to fill yourself with high-calorie foods. Go light on salads, which have few calories, and instead reach for calorie-dense foods such as hamburgers, milk shakes and cheesecake. Eat foods with fat. "People with cystic fibrosis need fat," says Dr. Mueller. "It would be great if instead of trying to eat 30 percent or less of calories from fat, people with cystic fibrosis would eat 30 percent or more of calories from fat." Put an extra dollop of butter or margarine on breads, vegetables, pastas, potatoes and rice. Add whipped cream to desserts, coffee and hot Avoid fried foods. Even though high-fat foods are great, don't try to get more fat into your diet by eating greasy fried foods, says Dr. Mueller. Fried foods require more bile, which helps keep that extra fat isolated so that digestive enzymes can work on the food. And bile is in short supply in those with cystic fibrosis. Salt foods. "When people have cystic fibrosis, the sweat glands are also impaired," says Dr. Mueller. "Instead of being reabsorbed, which is what happens for most of us when we sweat, the sodium and chloride just come out and stay on the skin. That's why people with cystic fibrosis have to supplement their diets with salty foods or salt. Never use salt tablets, though. They are much too concentrated." The amount? "About one-quarter to one teaspoon (1,375 to 5,500 milligrams) a day, depending on what kinds of foods are eaten," replies Dr. Mueller. That means babies, too, she adds. "When parents are advised 'Add salt to the baby food,' they just stare at us. Again, it appears to be the antithesis of good nutrition. But each person truly has individual needs, and baby foods no longer have salt added to them." Drink lots of water. "People with cystic fibrosis should be taking in around two quarts of water daily, plus what's in the foods they eat," says Dr. Mueller. The excess loss of salt as they sweat causes them to dehydrate easily, particularly in hot weather. |
Starving in a Land of Plenty
Fifteen years ago, most children born with cystic fibrosis never made it to adulthood. The thick mucus produced by their secretion glands blocked their airways, clogged their digestive tracts and encouraged the growth of bacteria that led to frequent life-threatening infections in their lungs.
Today at least half of these children grow up and make it into their thirties and beyond. And as women with the disease begin to live longer, healthier lives, some are even becoming pregnant, a landmark that is sending waves of both ecstasy and concern into a medical community that must now learn how to help them.
The problem is that anyone who has cystic fibrosis has a lot of trouble getting just the basic nutrients to sustain herself, much less enough to provide for another life.
In healthy people, mucus in the digestive tract is slippery and light, so food can easily slide along the digestive tract and nutrients can pass from digested food through the intestinal wall, into the bloodstream and on to the rest of the body, explains Donna Mueller, R.D., Ph.D., associate professor of nutrition and foods at Drexel University in Philadelphia. But in people with cystic fibrosis, the digestive tract is covered with such thick mucus that many, if not most, nutrients can’t get through the intestinal wall and into the bloodstream. That’s why people with cystic fibrosis are frequently at risk for malnutrition. They are literally starving in a land of plenty.
Complicating the situation is the fact that the pancreas, which produces enzymes that help your body digest protein, fat and carbohydrates, is also affected by the thick mucus. “The enzymes produced in the pancreas’s cells leave the pancreas through little canals that empty out into the small intestine,” explains Dr. Mueller. But the canals get so clogged with mucus that most enzymes never reach the food. So most of the food eaten by someone with cystic fibrosis is simply not digested.
“Some nutrients are going to get through,” says Dr. Mueller. “But this condition is a walking textbook on nutrition, because every nutrient is affected: protein, fat, carbohydrates, vitamins and minerals.”
Meeting the Body’s Increasing Demands
Unfortunately, just as the body is least likely to get the nutrients it requires, its need for those nutrients will increase by 20 percent or more.
This is a chronic, progressive disease that hits the airways particularly hard, explains Dr. Mueller. The lungs degenerate. People get sicker and sicker while their bodies work harder and harder. That’s why nutrition is always important. As the lungs become more involved, the body is working harder, and energy requirements increase.
“Unfortunately, when someone doesn’t feel well, one doesn’t feel like eating, either,” says Dr. Mueller. “So just when there are greater body needs, there’s less of an appetite.”
But tough as it is for adults with cystic fibrosis to meet their daily nutritional needs, children with cystic fibrosis also have to meet the demands of growing bodies.
“You know the growth charts doctors use?” asks Dr. Stallings. Children with cystic fibrosis are often at the bottom, somewhere around the tenth percentile, she says. That means that 90 percent of everybody else their age is bigger. That’s why the goal of most doctors, including Dr. Stallings, is to give kids the nutritional support they need to grow as big as their brothers and sisters and to get them through these growth phases so they go into adulthood as strong and as well-nourished as possible.
On average, Dr. Stallings is successful. “We have a little trouble with adolescent girls because of the body image issues in our society,” says Dr. Stallings. With all of the cultural emphasis on thinness as an ideal, “they don’t mind being the skinniest kids in class,” she says. “But of course, that may be harmful to their health.”
| Prescriptions for Healing To keep the body as strong as possible while new gene therapies are under development, a person with cystic fibrosis is encouraged to eat a well-balanced diet and to take specially prepared supplemental pancreatic digestive enzymes (these break down food so nutrients can be better absorbed) and an over-the-counter balanced multivitamin/ mineral supplement that provides the Recommended Dietary Allowances plus other vitamin and mineral supplements based on individualized blood tests and in consultation with a cystic fibrosis nutrition specialist, says Donna Mueller, R.D., Ph.D., associate professor of nutrition and foods at Drexel University in Philadelphia. For example, vitamin K may be added if someone is also on antibiotics or has liver disease. MEDICAL ALERT: Anyone who has cystic fibrosis should be taking vitamin and mineral supplements only after discussing it with his physician. |
Winning with Prevention Power
Once children have completed their adolescent growth spurts, doctors and nutritionists generally breathe a sigh of relief. But only for a moment. They still need to help the adults with cystic fibrosis store up enough nutritional support to withstand the frequent infections associated with the disease without losing ground.
To help meet the nutritional demands of their bodies, most people with cystic fibrosis are encouraged to eat a well-balanced diet and—after very careful personal evaluation by their doctors and nutritionists—to take specially prepared supplemental pancreatic digestive enzymes and a general multivitamin/mineral supplement plus other vitamins and minerals prescribed just for them as their conditions indicate at that time, says Dr. Mueller.
“The major issue is calories,” adds Dr. Stallings. “If anything affects growth, energy, quality of life and being able to fight off infection, the big thing is absorbing adequate calories. If you can get enough calories to keep up your body weight, then almost everything else that you can do nutritionally follows.”
In fact, she adds, “if someone with cystic fibrosis is consuming adequate calories and has maintained normal body weight, I try to not prescribe any extra supplements besides what has been determined to be absolutely necessary. Since these are people who may be taking 60 pills a day, they don’t need to be taking another pill if they don’t have to.”
Dr. Mueller agrees with the need to keep the food and pill regimens as simple as possible. But she also feels that “even with a well-balanced diet and pancreatic enzyme replacements, most people with cystic fibrosis need extra vitamins and minerals.”
Her reasoning is based on the hit-and-miss effectiveness of the enzymes. “There’s no good way of knowing exactly how much of these enzymes is necessary at any particular time,” she explains. Pancreatic enzyme pre scriptions are based on general guidelines, and the enzymes are not 100 percent effective. The thickness of the mucus constantly varies—thicker one day, thinner the next—and people eat different things on different days. With that much variability, how much of the nutrients gets through can vary a lot.
That is also why it’s important to have blood levels of target nutrients checked at least once a year, says Dr. Mueller. People with cystic fibrosis have changing needs, and what was fine last year may not be so this year. Some vitamins and minerals might need to be decreased, and some might need to be increased. And because deficiencies of these nutrients may make the body even more vulnerable to infection and disease, “vitamin and mineral levels are as important as drug level tests,” says Dr. Mueller.
It is important to remember that anyone with cystic fibrosis should be under a doctor’s care. Only a doctor or nutritionist can recommend the right types and amounts of vitamin and mineral supplements for each person. To locate a cystic fibrosis center near you, contact the Cystic Fibrosis Foundation at 6931 Arlington Road, Bethesda, MD 20814 (1-800-FIGHTCF).
