Overweight and cystic fibrosis: An unexpected challenge.

Achieving a healthy weight balance has been a central focus of care for people who have cystic fibrosis (CF). Over the years, the emphasis has primarily been on promoting weight gain to optimize pulmonary outcomes. With continued improvements in CF care, including highly effective CF modulators available for many people, the CF community is now experiencing a new challenge: addressing the concern that some people are gaining weight excessively. While at this time, we do not know to what extent overweight and obesity will affect health outcomes for people with CF, it is likely that excessive weight gain may have negative health impacts similar to those seen in the general population. In this paper, we review the history of nutritional guidelines for people with CF, as well as more recent trends toward overweight and obesity for some. A multidisciplinary approach is needed to collaboratively start the oftentimes difficult conversation regarding excessive weight gain, and to identify resources to help people achieve and maintain a healthy weight through diet, exercise, and behavioral modification.

Nutrition in cystic fibrosis: From the past to the present and into the future.

Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition care.

Relationship of Initial Pancreatic Enzyme Replacement Therapy Dose With Weight Gain in Infants With Cystic Fibrosis.

OBJECTIVE: The aim of the study is to test the hypothesis of a positive relationship between initial dose of pancreatic enzyme replacement therapy (PERT) in infants with cystic fibrosis (CF) and optimal weight gain over the first 2 years of life. METHODS: Using the CF Foundation Patient Registry, we identified 502 children born in 2010 and used multivariable models to compare as our primary analysis their 2-year changes in weight-for-age z score (WAZ) and as our secondary analysis weight-for-length percentile (W/L%) by initial PERT dose. We focused on initial dose without reference to subsequent changes in treatment to avoid confounding by indication (severity). RESULTS: Initial PERT dose demonstrated a linear relationship to change in WAZ and W/L% at age 2 years. An initial dose of >1500 lipase units/kg/largest meal resulted in a higher likelihood of attaining WAZ at 2 years at or above the birth WAZ (adjusted odds ratio [aOR] 1.87, 95% confidence interval [CI] 1.22-2.86) and at the top quartile for improvement over 2 years in WAZ (aOR 1.90, 95% CI 1.19-3.05). There was no correlation between initial PERT dose and weight at initial PERT encounter (P = 0.35). Findings were similar for W/L% and when the cohort was restricted to infants who began PERT in the first 3 months of life. CONCLUSIONS: Infants receiving higher initial PERT dose demonstrate better weight-related outcomes, as reflected by attainment of favorable changes in WAZ and W/L%, at age 2 years.

Nutrition Management of Cystic Fibrosis in the 21st Century.

Despite significant advancements made in life expectancy over the past century, cystic fibrosis remains a life-threatening genetic disease that affects the gastrointestinal tract, and it has significant impact on the nutrition status of those with the disease. Nutrition management includes a high-calorie/high-fat diet, pancreatic enzyme replacement therapy, vitamin and mineral replacement, and enteral support as needed. As patients are living longer, clinicians may encounter patients with cystic fibrosis in obstetrician offices, endocrine clinics, or hospital settings, owing to lung transplantation or for treatment for distal intestinal obstruction syndrome.

Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis.

BACKGROUND: Vitamin D deficiency is common in CF. Whether vitamin D affects pulmonary function in CF is unknown. METHODS: Data were abstracted from clinically stable CF patients who had pulmonary function studies and serum 25-hydroxyvitamin D [25(OH)D, ng/ml] levels drawn within 2 months of each other. Findings were adjusted for multiple variables known to affect pulmonary function in CF. RESULTS: Enrollees totaled 597. Overall mean 25(OH)D level was 29.6±12.8 ng/ml (SD). Serum 25(OH)D levels showed a significant correlation with forced expiratory volume in 1s (FEV1) % predicted (r=0.20, p<0.0001) and forced vital capacity % predicted (r=0.13, p=0.0019). Multivariate analysis revealed that serum 25(OH)D remained an independent predictor of FEV1 % predicted even after controlling for multiple other factors known to affect CF lung function. CONCLUSIONS: Serum 25(OH)D levels are significantly associated with pulmonary function in CF. Further study is required to determine whether this association is causal.

Nutrition management of pediatric patients who have cystic fibrosis.

Since the identification of cystic fibrosis (CF) in the 1940s, nutrition care of patients who have CF has been a challenge. Through optimal caloric intake and careful management of malabsorption, patients are expected to meet genetic potential for growth. Yet factors beyond malabsorption, including nutrient activity at the cellular level, may influence growth and health. This article reviews nutrition topics frequently discussed in relationship to CF and presents intriguing new information describing nutrients currently being studied for their impact on overall health of patients who have CF.

Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed individual. The focus of management is on maintaining health by preventing nutritional and respiratory complications. The CF Foundation convened a committee to develop recommendations based on a systematic review of the evidence and expert opinion. These guidelines encompass monitoring and treatment recommendations for infants diagnosed with CF and are intended to help guide families, primary care providers, and specialty care centers in the care of infants with CF.

Overweight among young children in the Philadelphia health care centers: incidence and prevalence.

OBJECTIVES: To estimate the prevalence and incidence of overweight among low-income, inner-city children aged 3 to 7 years and to determine predictors of changes in body mass index (BMI) (calculated as weight in kilograms divided by height in meters squared) percentile. DESIGN: Retrospective cohort study using administrative and medical records. SETTING: The Philadelphia Health Care Centers, 1996 through 2003. PARTICIPANTS: Three hundred eighty-six patients who had at least 2 well-child visits between the ages of 3 and 7 years, had at least 1 visit between September 2001 and 2003, and were between the ages of 5 and 7 years at the most recent visit. Mean follow-up time was 2.4 years. MAIN EXPOSURES: Age, sex, race/ethnicity, and BMI percentile at baseline. MAIN OUTCOME MEASURES: Prevalence and incidence of overweight and at risk of overweight and change in BMI percentile. RESULTS: Prevalence of overweight at the initial visit was 18%, with 16% at risk of overweight. At the last visit, the prevalence of overweight was 19%, with 15% at risk of overweight. Based on maximum BMI percentile, 29% were overweight at some point and an additional 19% were at risk of overweight at least once. Annualized incidence of overweight among those not overweight at baseline was 5% per year: 2% per year for normal-weight children and 14% per year for children in the at-risk category. The outcomes were not associated with sex, race/ethnicity, or age at first or last visit. Incident overweight was positively associated with BMI percentile at baseline. CONCLUSION: The early onset and frequent persistence of overweight demonstrated herein underline the need to prevent overweight among very young children.