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 inside 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 can also 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, which means that the
enzymes created in the pancreas that digest fat, starch, and
protein aren't properly passed into the intestines. This causes
problems in 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 may 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.
In addition to more calories, kids with CF have some specific
nutritional needs to help them thrive:
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. During hot weather and when your child plays sports
or has phys ed, the dietitian may recommend sending sports drinks
along.
Zinc.
Important for growth, healing, and fighting infection, zinc is
found in meats, liver, eggs, and seafood.
Meals at Home and Away
To meet your child's nutrition needs, you'll work with
the CF dietitian to create meal plans that include plenty of
snacks, especially for toddlers who eat smaller meals.
For a picky eater, pay attention to the foods your child
especially likes and adapt the menu as these preferences change.
Ask an older child with CF to try one new food a week. It's
also important to model good behavior for kids of all ages. Eat a
well-balanced diet with a variety of foods and make sure you try
new things yourself.
To make meals a pleasant experience:
- Try to keep meals to about 20-30 minutes for toddlers and
young kids, who can't sit still for extended periods.
- Eat in a comfortable environment with few distractions.
- Don't give huge portions that might seem
overwhelming.
- Offer praise for eating well, but don't force plates of
food to be finished; encourage kids to eat as much as they can
comfortably.
- Keep food choices simple, especially for younger kids.
- Make foods as attractive and appealing as possible.
All child-care providers and teachers should know that your
child has CF and be aware of his or her nutritional and caloric
needs. Work with the staff to plan high-calorie meals and snacks or
send food if the menu can't accommodate your child's needs.
Young kids need help taking their enzymes and supplements, and the
staff should understand that they need to be taken before all
meals.
As kids with CF grow into teens, they'll probably begin
eating more meals away from home and may feel pressured to eat in
certain ways. Work with your teen, emphasizing the positive and
monitoring any weight changes that are of concern. If you're
packing lunch, include high-calorie options like ranch dressing and
peanut butter crackers.
Increasing Caloric Intake
It can be tricky to make sure that kids with CF eat enough fat
and calories, especially if they're sensitive to what's on
the plates of siblings and peers. One way to increase calories
without creating an entirely separate menu is to increase the
calories and fat in one part of the meal.
Here are some simple ways to do so:
- Add extra butter or margarine to sandwiches, sauces, and
potatoes.
- Use dressings on salads or vegetables; add extra oil to the
dressing.
- Prepare entrees with gravies and creamy sauces.
- Add bacon to burgers and chicken.
- Add dried skim milk powder to sauces and beverages.
- Add extra cheese to scalloped potatoes or macaroni and
cheese; order extra cheese on pizza.
- Top salads and sandwiches with avocados or guacamole.
- Add nuts to cookies, cakes, pancakes, and salads.
- Add extra cheese and deli meats to sandwiches.
- Grill sandwiches in butter or margarine.
- Use heavy whipping cream and whole milk when cooking.
- Make milkshakes.
- Add instant breakfast mixes to milk-based beverages.
- Make high-calorie smoothies.
- Prepare calorie-rich desserts such as pudding and
cheesecake.
- Top hot chocolate, pudding, and other desserts with whipped
cream.
- Provide high-calorie snacks like peanut butter crackers or
trail mix.
- Prepare high-calorie versions of popular family recipes.
When you're shopping, be sure to compare the labels on
similar prepared foods and purchase those that have higher fat and
calorie content. Avoid diet foods - anything that claims to be
nonfat, low fat, reduced calorie, or light.
Look for whole-fat versions of dairy products such as sour
cream, cottage cheese, and yogurt (many natural food stores carry a
good variety of these). Don't forget to check baby food labels.
You'd be surprised at how the caloric level varies between
brands.
Beyond Food: Enzymes and Tube Feeding
Enzymes
A child with pancreatic insufficiency will need to take enzymes
with meals and snacks to help digest food properly and to get the
nutrition and fat needed to grow and gain weight.
Signs that your child may need enzymes or an enzyme dose
adjustment include:
- failure to gain weight, in spite of a strong appetite
- frequent, large, greasy, or smelly bowel movements
- bloating or gas
The CF dietitian or doctor will prescribe enzymes based on
weight, growth, and how much a child eats at a time. An increased
enzyme dose doesn't necessarily mean that a child is doing
poorly. It may mean that he or she has gained weight and now
requires more enzymes.
Enzymes need to be taken with every meal and most snacks. They
come in capsules, full of tiny beads, that can be broken open for
kids who are too young to swallow entire capsules. They should only
be mixed with foods that are acidic, like applesauce. They should
not be chewed or crushed. Never change the dose of enzymes without
first consulting your child's dietitian or doctor.
Tube Feedings
In some cases, enzymes and high-calorie meals may not be enough
to help a child thrive, and feedings through a tube into the
stomach may be needed. It's not always physically possible for
kids with CF to meet their increased energy needs by eating by
mouth alone. Tube feedings, which can be done at night, are an
excellent way to help kids who have trouble gaining weight,
especially when they're ill.
Many kids and their parents are resistant to having a tube put
in. But tube feedings are only prescribed when dietary
interventions, calorie-boosting strategies, and enzymes haven't
been enough, and tube insertion usually is an uncomplicated
outpatient procedure.
Tube feedings do not mean that you've failed to feed your
child well or that he or she isn't a good eater - often they
just mean that a child needs so many calories a day that it's
impossible to keep up without them. Your child's doctor and
dietitian will make recommendations about tube feedings.
It can be difficult for kids with CF to eat enough in a day to
meet their needs, especially when they aren't feeling well.
Although we all think we'd like to be able to eat a high-fat,
high-calorie diet, many kids with CF, especially teens, find it
difficult to do so in our diet-conscious culture.
Keeping your eye on the positive - your child's growth and
weight gain - will help your child with CF eat well, stay healthy,
and thrive.
Reviewed by:
Allison Brinkley, RD, LD/N, CNSD
Date reviewed: February 2007
Note: All information is for educational purposes only. For specific medical advice,
diagnoses, and treatment, consult your doctor.
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