Growth and Nutrition in Cystic Fibrosis

Abstract

Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study

“…More recently, evidence of higher quality, including meta-analyses, contradicts this narrative, indicating that SFA intake has either positive, or null effect on cardiovascular health based on dose-response analyses [ 39 , 40 ]. On the other hand, children with CF often demonstrate essential fatty acid deficiencies, with some being non-responsive to supplementation treatment due to multiple potential impediments leading to malabsorption issues, indicating that the disease is associated with a cascade of health issues requiring adequacy in the intake of fat [ 41 , 42 , 43 ]. In parallel, despite the reported high intakes of fat, serum lipid levels are often within normal ranges, even among adults with CF who have adhered to a high-fat diet for a longer period of time [ 44 ].…”

Cistinės Fibrozės Komplikacijos Ir Jų Gydymas

“…Augimo sutrikimų gydymas yra paremtas tinkamos plaučių funkcijos palaikymu, malabsorbcijos gydymu bei tinkama, daug kalorijų (kasdienis kalorijų vartojimas turėtų būti didesnis, nei sveiko žmogaus) bei riebalų turinčia dieta su vitaminų papildais ir, jei reikia, egzogeninių kasos fermentų vartojimu [27,28].…”

Promoting emotional wellness in children with cystic fibrosis, Part I: Child and family resilience

“…Another dimension to life with CF has been the reliance on carbohydrate‐rich, prepared, and fast foods to achieve energy needs. Evidence is accumulating that children and adolescents with CF, independent of their weight or body mass index (BMI), too often carry excess body fat and less skeletal muscle than is considered healthy 58‐60 . As physical and emotional health are dependent on healthy sleep, exercise, and nutrition, it appears that outcomes for children and their families can be enhanced by more attention to these lifestyle factors in the CF care model.…”

“…Early childhood helps set the stage for a lifetime of nutritional health for children with CF, fostering lifelong enjoyment of food, and laying the groundwork to reduce the likelihood of future nutrition problems or disordered eating. CF care teams can foster resilience and food‐enjoyment for young children with CF by providing families with education about normative eating development, alongside implementation of CF‐specific nutrition 60 …”