Michael Wilschanski
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tritional deficits at all ages. This has led to the publication of nutritional guidelines in Europe and in North America [7, 8] .
Infants
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.
Table 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
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Adolescence
This stage is associated with increased growth, puberty and increased physical activity. This adds up to markedly increased nutritional require- ments which are often difficult to attain. Pulmo- nary infections are more common, as is the onset of CF-related diabetes and, in a small minority of cases, CF-related liver disease. Female patients are at a greater risk of nutritional failure at this age [11] . This may be partly due to dissatisfaction with weight and body shape in healthy adolescent females. Growth retardation and pubertal delay occur with increased social pressure and psycho- social stress. These factors must be considered when nutritional advice is provided to teenagers.
Ideally, dietary advice should be passed on before conception as a low prepregnancy BMI is associ-
ated with reduced birth weight. Nutrition should be optimized throughout pregnancy and vitamin levels should be monitored [12] .
Bone Health
A decrease in bone mineral density (BMD) in pa- tients with CF may begin at a young age [13] . There are many factors that influence bone health both in healthy individuals and in patients with CF; these include nutritional status, calcium, vita- mins D and K, pulmonary infection, exercise, glu- cocorticoids and the class of CFTR mutation. The causes of poor BMD, a reflection of bone health, are thus multifactorial. Evidence for the efficacy of treatments for maintaining and improving bone health is lacking in CF; however, consensus
Cellular
defect? Psychogenic
Gastrointestinal Biliary
Vomiting Energy deficit
Needs Losses Intake
Weight loss Pulmonary
infections Immune
dysfunction
Deteriorating lung function Pancreatic
Intestinal Iatrogenic
Respiratory
muscles Lung
parenchyma
+ +
Anorexia
Fig. 1. The pathogenesis of energy imbalance in CF.
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
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.
248 Wilschanski
placement. Calorically dense formulas (1.5–2.0 kcal/ml) are well tolerated, and, initially, nocturnal infusion is encouraged to promote normal eating behavior during the day. Our experience is that once families see a success after 6–8 weeks of naso- gastric feeding, gastrostomy placement is wel- comed. Patients with excessive nausea, bloating or vomiting may benefit from prokinetic drugs or semielemental or elemental formula.
Growth Hormone and Appetite Stimulants The efficacy of growth hormone therapy in CF has recently been reviewed [19] . While growth parameters and pulmonary function seem to im- prove in treated patients, the overall benefits to health cannot be determined from the moderate evidence available. One recent multicenter trial in which growth hormone was administered for 12 months to patients with reduced growth and bone age indicated its effectiveness in improving growth and lung volumes [20] . Larger trials with appropriate patient selection are needed in order to establish its safety and effectiveness.
Appetite stimulants (AS) are often requested by individuals with CF, or by parents of a child with CF, in anticipation of an enhanced appetite
and increased energy intake to promote weight gain [21] . While megestrol acetate is one of the most studied AS in CF, results are not conclusive on the use of AS in patients with CF at this time, and larger, controlled studies are needed.
Conclusions
• The overall goal is that every patient with CF should achieve normal growth. This requires regular surveillance including age-specific in- dividualized expert advice with nutritional care plans to suit each patient. Nutritional in- tervention should be appropriately timed to influence the evolution of the disease
• Nutritional support is an integral part of the care of patients with CF
• At diagnosis, all patients require pancreatic and nutritional assessment
• Patients must be carefully monitored and di- etary counseling provided
• Nutritional evaluation and support is age re- lated
• Patients who fail to respond require enteral supplementation
• Nutritional status impacts on the progression of CF
6 Snell GI, Bennetts K, Bartolo J, et al:
Body mass index as a predictor of sur- vival in adults with cystic fibrosis re- ferred for lung transplantation. J Heart Lung Transplant 1998; 17: 1097–1103.
7 Sinaasappel M, Stern M, Littlewood J, et al: Nutrition in patients with cystic fi- brosis: a European consensus. J Cyst Fibros 2002; 2: 51–75.
8 Borowitz D, Baker RD, Stallings V: Con- sensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002; 35: 246–259.
References
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Cystic fibrosis diagnosed after 2 months of age leads to worse outcomes and re- quires more therapy. Pediatrics 2007;
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Wasting as an independent predictor of mortality in patients with cystic fibrosis.
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9 Borowitz D, Robinson KA, Rosenfeld M, et al: Cystic Fibrosis Foundation evi- dence-based guidelines for management of infants with cystic fibrosis. J Pediatr 2009; 155(suppl):S73–S93.
10 Tangpricha V, Kelly A, Stephenson A, et al: An update on the screening, diagno- sis, management, and treatment of vita- min D deficiency in individuals with cystic fibrosis: evidence-based recom- mendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab 2012; 97: 1082–1093.
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Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evalua- tion of various criteria used to identify malnutrition. J Pediatr 1988; 132: 478–
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3 Nutritional Challenges in Special Conditions and Diseases
Key Words
Congenital heart disease ã Reflux ã Growth ã Nutrition ã Lactation consultation ã Breastfeeding
Key Messages
• Assess and maximize growth promotion in every encounter/visit
• Utilize breast milk if available and tolerated • Encourage breastfeeding/nonnutritive sucking if
safe
• Treat clinical symptoms of reflux, constipation and formula/milk intolerance
• Close communication concerning goals of growth and development © 2015 S. Karger AG, Basel
Introduction
Congenital heart disease (CHD) and its impact on infant growth and development has been well doc- umented in the literature over the years. The most risky cardiac lesions are cyanotic CHD lesions, and they are outlined in table 1 . The goal of this chapter is to focus on those infants with single-ventricle physiology at high risk for growth failure, such as double outlet right ventricle, tricuspid atresia and hypoplastic left heart syndrome, who have under- gone stage 1 palliation (Norwood or Sano proce- dure). Practical guidance will be provided to those practitioners caring for fragile and at-risk neonates
between stage 1 and stage 2 palliation (Glenn pro- cedure). However, the principles outlined can be applied to other conditions associated with poor growth listed in table 1 .
Outlined here are key concepts essential for optimizing growth; they include the following:
utilization of breast milk; lactation consultation;
practical caloric density increases; hands-on rec- ipe education; and promotion of normal growth and development. Speech and otolaryngology involvement in the care team is also key to pro- moting optimal growth and developmental milestones. Equally important is acknowledging reflux, constipation, and milk and protein intol- erance, and treating these issues early, as they are essential to optimal growth success. Figure 1 demonstrates the complexity of the many com- mon growth variables interacting in CHD.
Impact of Home Surveillance Program on Nutrition and CHD
With the advent of home surveillance in 2000 [1] , postnatal attention to nutrition following dis- charge from stage 1 palliation has become height- ened and a main center of focus until stage 2 pal- liation, typically at 3–6 months of age [2–5] . In 2003 the first multicenter quality improvement collaborative within the USA, the Joint Council
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 250–253 DOI: 10.1159/000360346