Nutritional Issues and CF

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• published: 21 May 2015
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NUTRITION AND CYSTIC FIBROSIS: HOW TO HELP MY CHILD WITH CF ACHIEVE THEIR NUTRITION GOALS. Presented by Julie Matel, MS, RD, CDE Cystic Fibrosis (CF) is an autosomal recessive disorder, which affects approximately 30,000 adults and children in the United States and 70,000 individuals worldwide.1 CF occurs as a result of a mutation in the CF transmembrane conductance regulator (CFTR), a sodium chloride channel, and presents in many systems, including the pulmonary, gastrointestinal, and reproductive organs.2,3 Individuals eventually succumb to the pulmonary manifestations of the disease, however, maintaining an optimal nutritional status, especially early in life may have a significant impact on the trajectory of CF. A connection between nutrition and lung function in CF has been established through numerous studies. A classic study comparing outcomes at 2 CF centers, Toronto and Boston, recognized that, despite similarities in the medical management of the CF patients, a distinct survival advantage was realized at the Toronto center. The distinguishing feature at the Toronto center was the more aggressive approach to nutrition management and the promotion of high-fat diets, during a time when low-fat diets were prescribed as a standard of care.4 Data from the Cystic Fibrosis Foundation registry database suggest further supporting evidence. When body mass index (BMI), a weight to height comparison, is compared with lung function, as measured by forced expiratory volume in 1 second (FEV1), there is a correlation between improved BMI percentiles and lung function. This relationship between improved BMI and improved lung function exists until a BMI of the 50th percentile is reached.5 From the body of literature that exists, it appears that not only does nutrition affect outcomes in CF, but that better nutritional status early in infancy and childhood and its positive effect on growth parameters is particularly important. With the expansion of newborn screening programs for cystic fibrosis, early nutrition intervention is now possible. Data from Wisconsin’s CF neonatal screening project demonstrated a decrease in the proportion of patients with a weight and height at less than the 10th percentile in the newborn screening group compared to infants that did not receive their CF diagnosis through newborn screening.6 We know that achieving good nutrition for individuals with CF is important, mainly in the form of maintaining an optimal BMI status. However, achieving this goal presents challenges for parents of kids with CF at every stage of childhood, from infancy through the teenage years. Each stage has its own set of issues from challenging food behaviors, increased energy requirements, to enzyme refusal. These barriers can make meeting nutritional goals difficult. However, there are strategies that parents can employ that will help children to meet their weight gain goals. References 1. Cystic Fibrosis Foundation. About cystic fibrosis. http://www.cff. Org/About CF/. Published 2009. Accessed May 31, 2011. 2. Davies JC, Alton EWFW, Bush A. Cystic fibrosis. BMJ. 2007; 335:1255-1259. 3. Davies PB, Drumm M, Konstan MW. Cystic fibrosis. Am J Respir Crit Care Med. 1996; 154:1229-1256. 4. Corey M, McLaughlin FJ, Williams M, et al. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988-41-581-591. 5. Cystic Fibrosis Foundation patient data registry. Bethesda, MD: Cystic Fibrosis Foundation, 2009. 6. Wagener J, Farrell PM, Corey M. A debate on why my state (province) should or should not conduct newborn screening for cystic fibrosis. North American CF Conference, 14th Annual. Pediatr Pulmonol. 2001. Nov, 32 (5): 385-96. Julie Matel, MS, RD, CDE, has been a Registered Dietitian for 18 years, and has worked at Lucile Packard Children’s Hospital Stanford for the past 15 years. Julie is also a Certified Diabetes Educator. Julie works with children and adolescents with cystic fibrosis at Stanford Children’s Health/LPCH, and has been credited with improving nutrition-related benchmarks at the CF center. In addition, Julie is an instructor at San Francisco State University, where she teaches courses in Medical Nutrition Therapy.