About Cystic Fibrosis
Cystic fibrosis (CF) is an inherited disease that affects the respiratory and digestive systems. CF makes
children sick by disrupting the normal function of epithelial cells — cells
that make up the sweat glands in the skin and that also line passageways in the lungs,
liver, pancreas, and digestive and reproductive systems.
A defect in these cells causes problems with the balance of salt and water in the
body, leading it to produce thick mucus, which clogs the lungs, setting the stage
for infections and other breathing problems. In kids with CF, this mucus also can
prevent the normal absorption of key nutrients and fat in the intestines, leading
to poor digestion, slow growth, difficulty gaining weight, greasy bowel movements,
and a decreased ability to fight infection.
At the time of diagnosis, the majority of patients have a condition called pancreatic
insufficiency. This means that enzymes created in the pancreas that digest fat, starch,
and protein aren't properly passed into the intestines. As a result, the body has
problems absorbing the necessary amounts of several important nutrients.
By providing good overall nutrition along with extra fat and calories and prescribed
enzymes, you can help your child with CF grow healthy and strong.
Your Child's Nutritional Needs
In general, kids with CF need more calories than other kids in their age group.
The amount of additional calories they need will vary according to each child's lung
function, activity level, and illness.
A child's calorie needs might be even higher during an illness (even a low-grade
infection can greatly increase the calories required). A CF dietitian can help you
determine how many calories your child needs each day, and track growth and weight
gain over time in order to provide a good nutrition plan.
Kids with CF also have some specific nutritional needs:
Calcium. Patients with pancreatic insufficiency have difficulty
absorbing calcium and are especially at risk for developing osteoporosis (weak, brittle
bones), as are all kids with CF. Dairy products are good sources of calcium (and the
full-fat varieties also are good sources of fat and calories). Many juices also are
fortified with calcium.
Essential fatty acids. Patients with cystic fibrosis have altered
levels of plasma fatty acids. Found in plant oils, safflower oil, and soybean oils,
they help in the building of cell membranes and may play a role in lung function.
It is suggested that CF patients try to incorporate more omega 3 fatty acids into
their diet, which can be done with either food sources or supplements. Salmon, flax
seeds, and walnuts are excellent food sources of omega 3 fatty acids. Before giving
your child any over-the-counter supplements, discuss this with your CF dietitian or
doctor.
Fat-soluble vitamins (vitamins A, D, E, and K). Important for
immune function, growth, and healing, these vitamins are absorbed along with fat.
And because most kids with CF have trouble digesting fat, they often have low levels
of fat-soluble vitamins and need to take specialized prescription supplements.
Iron. Many children with CF are at risk for developing iron-deficiency
anemia, which can cause fatigue and poor resistance to infection. Fortified cereal,
meats, dried fruits, and dark green vegetables are good sources of iron.
Salt. Kids with CF lose a lot of salt in their sweat, especially
during hot weather and when they exercise. So a CF dietitian may suggest adding salt
to an infant's formula and giving an older child salty snacks. The dietitian may recommend
sending sports drinks along when your child plays sports or has phys ed, and during
hot weather.
Zinc. Important for growth, healing, and fighting infection, zinc
is found in meats, liver, eggs, and seafood.