Pediatric Nutrition in Practice

Nutrition in Cystic Fibrosis 247

3

guidelines have been established. It is recom-
mended that monitoring of BMD and ensuring
factors related to bone health be addressed at rou-
tine visits.

Follow-Up

A formal dietary assessment should be undertak-
en annually. This should incorporate a review of
nutritional intake, the enzyme dose, the timing of
administration as well as vitamin supplement in-
take. Anthropometry should be performed regu-
larly and at length, and BMI percentile charts
should be used for the interpretation of nutrition-
al status. Bone health is an increasing concern in
CF [14]. BMD and body composition should be
assessed by dual-energy X-ray absorptiometry
[15].

When Things Go Wrong

Figure 1 demonstrates the pathogenesis of mal-
nutrition in CF [16]. As pulmonary disease wors-
ens and resting energy expenditure increases,
other factors predispose to an energy deficit. The
frequency and severity of infections increase, in-
ducing anorexia and/or vomiting, in turn reduc-
ing intake. Weight loss results in causing loss of
muscle tissue; respiratory muscle wasting reduces
effective coughing, further contributing to the de-
terioration in lung function. Malnutrition is
known to cause immune dysfunction. Taken to-
gether, a vicious cycle is established, leading to
further deterioration.

Management of the Malnourished Child

Once poor growth is identified, patients must be
evaluated more frequently. The visits must in-
clude medical, nutritional and behavioral input.
Figure 2 shows an algorithm for the work-up.

Nutritional Intervention

If the reason for the poor weight gain is poor in-

take, the first strategy must be to gradually increase

calories at mealtime. At the same time, nutritional

intervention with high-calorie supplements may

be made. The long-term effect of supplements is

controversial and they must not take the place of

meals [17]. If this fails, enteral feeding should be

commenced [18]. The choice of access should be

made together with the family, but, generally, na-

sogastric feeding is started before gastrostomy

Poor weight gain

Assess pulmonary status/disease activity

Nutritional assessment

Feeding behavior

evaluation

Maximize dietary intake

Psychosocial consultation

(adherence)

72-hour fecal fat balance

Other etiologies:

• GERD


• CFRD


• Constipation


• DIOS


• Bacterial overgrowth


• IBD


• Etc.

Consider acid suppression

Assess enzyme dose

Enteral feeding

Fig. 2. Plan of action for poor weight gain in CF. GERD =

Gastroesophageal reflux disease; CFRD = CF-related dia-

betes; DIOS = distal intestinal obstruction syndrome;

IBD = inflammatory bowel disease.

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