Pediatric Nutrition in Practice

Nutrition in Cystic Fibrosis 245

3

tritional deficits at all ages. This has led to the
publication of nutritional guidelines in Europe
and in North America [7, 8].

I n f a n t s

The evaluation of an infant with CF should be ex-
pedited. If PI is established by tests for steatorrhea
and indirect pancreatic function tests, pancreatic
enzyme replacement therapy should be initiated as
soon as possible. Breast milk can provide complete
nutritional support to infants with CF for the first
4–6 months of age, though supplemental energy
may sometimes be required by fortifying a portion
of the breast milk feeds with formula or by fortify-
ing formulas to a more concentrated energy level
for those infants on a combination of breast milk
and formula or on formula alone [9]. Regular
cow’s milk-based infant formulas can be used if
breastfeeding is not an option or if supplementa-
tion is required; there is no need for a predigested
formula in most instances. Enzymes are given with
all foods and milk products including predigested
formulas containing medium-chain triglyceride.
Infants require powder, which should be taken
with fruit sauce with lubricant pretreatment to the
mouth and perianal area to avoid skin excoriation.
The enzymes should be administered at the begin-
ning of and during the meal. The initial dose of
enzymes should be approximately 5,000 IU lipase/
kg/day. The dose may be gradually increased ac-
cording to symptoms and objective assessment of
growth and fat absorption. In many instances, the
caloric density needs to be increased, and this may
be achieved by fortifying breast milk, adding fat or
carbohydrate or concentrating the formula. Once
solid food is introduced, enzymes should be titrat-
ed by fat intake. The maximum dose is 10,000 IU
lipase/kg/day. Fat-soluble vitamin (ADEK) sup-
plementation should be initiated according to the
current recommendations [7, 8].
New guidelines from the North American
Cystic Fibrosis Foundation Committee on Vita-

min D advise higher supplemental amounts of vi-

tamin D than are found in the currently available

CF vitamin supplements. Additional vitamin D

supplements are therefore recommended to keep

the lower limit of 25-hydroxyvitamin D at 30 ng/

ml (75 nmol/l) [10]. Yearly monitoring of serum

vitamin levels is recommended for vitamin A, vi-

tamin E and vitamin D. Hyponatremic alkalosis

may occur in infants, especially during the sum-

mer months; supplementation with sodium chlo-

ride is recommended. Zinc supplementation may

be considered in the child who is not thriving [9].

Toddlers

As infants are introduced to table foods, it is im-

portant that the diet be balanced, with moderately

increased fat and protein content ( table 1 ). Parents

need to be in control, routinely adding calories to

maintain growth. The child with CF should avoid

low-fat food and ‘grazing’. The dietician should

promote positive interactions around meals. The

mealtime must not turn into a battleground which

is the catalyst for poor feeding behavior.

School-Age Children

School age is the age at which encourage the child

to obtain a basic knowledge of the physiological

processes, eventually leading to taking increasing

responsibility for practical enzyme and nutrition-

al management.

Ta b l e 1. Recommended dietary macronutrient composi-

tion in CF and non-CF patients (% of energy intake)

Non-CF CF

Protein 10 – 15 15

Carbohydrate 55 – 60 35 – 40

Fat 30 45 – 50

Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249
DOI: 10.1159/000367876