Wilschanski et al (JPGN 2016; 63: 671-5) provide a summary (“highlights”) of a full report (Turck D et al. Clin Nutr 2016; 35: 557-77) on nutritional recommendations for infant and children with cystic fibrosis.

What’s in here:

Table 1: criteria for adequate nutritional status including

• Age <2 yrs: 50% for weight & height compared to healthy-age peers
• Age 2-18 yrs: 50% BMI compared to healthy peers

Table 2: nutritional assessment and followup

• Assess elastase-1 annually if pancreatic sufficient
• Assess pancreatic enzyme supplementation
• Annual blood tests: CBC/d, iron status, fat-soluble vitamins, LFTs.  Possibly: fatty acids
• If older than 10 years, annual glucose tolerance
• Dietary review every 3 months
• Bone density assessment between 8-10 yrs and then every 1-5 yrs

Table 3: Energy requirements

• Anticipate need for 110-200% compared with healthy peers

Table 4: Pancreatic enzyme replacement therapy (PERT)

• 0-1 yr: 2000-4000 units lipase/120 mL of formula/breast milk & 2000 units lipase/gram of dietary fat
• 1-4 yrs: 2000-4000 units of lipase/gram of dietary fat (max 10,000 units lipase/kg/day)
• >4 yrs: starting dose; 500 units lipase/kg/meal -titrate up to 1000-2500 units lipase/kg/meal (max 10,000 units lipase/kg/day)

Table 5: Fat-soluble vitamin/vitamin guidelines

Table 6: Sodium supplementation

• 0-6 months: 1-2 mmol/kg/day –give salt in small portions throughout the day, “diluted in water or fruit juice”.  In some infants, up to 4 mmol/kg/day if increased losses (eg. due to heat, gastrointestinal losses)
• Older children: anticipate need for additional salty foods or use sodium chloride capsules, especially when excessive sweating (eg. fever, sports, hot weather)

Related blog posts:

• Complex Family of CFTR-Associated Disorders | gutsandgrowth
• N2U -Part 3: EoE, IBD, and Cystic Fibrosis | gutsandgrowth
• “Origins of Cystic Fibrosis Lung Disease” | gutsandgrowth
• Breaking down lung defenses in Cystic Fibrosis | gutsandgrowth
• Fluctuating Elastase Levels in Infants with Cystic Fibrosis …

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

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