Nutrition and behavioral health in cystic fibrosis: Eating and body image.

People with CF (pwCF) are at high risk for malnutrition, making nutritional management a critical aspect of CF care. Over the past several decades, optimal nutritional status for pwCF has been defined by body mass index (BMI) based on evidence linking suboptimal BMI to decreased lung function and life expectancy, although more recent changes in CF care may also bring changes to how nutritional health is defined. The historical focus on weight, BMI, and nutrition as key parts of multidisciplinary CF care starting at an early age places pwCF at increased risk for body image concerns and disordered eating. The landscape of CF care is evolving with the approval of highly effective modulator therapies (HEMT) and resulting improvements in growth; however, issues related to body image and eating remain important to consider, especially as past difficulties gaining weight may shift to discomfort with one's weight gain and/or physical appearance. This review aims to describe how body image concerns and disordered eating occur in pwCF across the lifespan; to discuss evidence-based approaches to addressing these concerns; and to identify future directions for research and clinical practice in assessing and treating eating disorders and body image concerns in this population.

Clinician perspectives on assessing for disordered eating and body image disturbance in adolescents and young adults with cystic fibrosis.

BACKGROUND: Maintaining a healthy weight is a focus of Cystic Fibrosis (CF) care. With the increased use of highly effective CFTR modulators, many people with CF are gaining weight more easily, which may affect eating habits and body image. This study investigates providers' understanding and current practices surrounding body image disturbance and disordered eating in people with CF. METHODS: We distributed a one-time web-based survey to United States (U.S.)-based CF healthcare providers via CF Foundation list servs. The survey investigated providers' understanding and perceived importance of issues surrounding disordered eating and body image disturbance in adolescent and young adults (AYA) with CF as well as current screening practices. We used descriptive statistics to analyze participants' characteristics and practices. RESULTS: A total of 232 healthcare providers completed the survey. While most participants felt that screening for both body image disturbance and disordered eating should be standardized in CF care (79% and 82%, respectively), fewer than one third felt comfortable screening, and only one quarter actually screened for various eating disordered behaviors in daily practice. Only 2.7% reported using a formal screening tool. Participants reported provider assessment tools (86%), standardized partnerships with eating disorder specialists (80%), and CFF or national guidelines (79%) would be helpful to improve screening and counseling. CONCLUSION: While most CF providers believe that body image disturbance and disordered eating are important topics in AYA with CF, few address these topics with their patients. The development of educational sessions and national guidelines may improve screening and counseling practices.

Eating disorders in adolescents and young adults with cystic fibrosis.

INTRODUCTION: There is evidence for increased risk of eating disorders in individuals with diet-treated chronic illnesses, however, data in patients with cystic fibrosis (CF) is less clear. No studies have evaluated avoidant/restrictive food intake disorder (ARFID) in the CF population. We investigated the prevalence of eating disorders, including ARFID, in adolescents and young adults with CF. METHODS: Patients with CF aged 14-35 years were recruited to complete three validated surveys: (1) Eating Disorder Examination Questionnaire (EDE-Q), (2) Nine-Item Avoidant/Restrictive Food Intake Disorder Scale (NIAS), and (3) Cystic Fibrosis Questionnaire-Revised (CFQ-R). Univariate linear regression analysis identified baseline risk factors associated with these survey scores. Variables with univariate p < 0.20 were considered for inclusion in a multivariable linear regression model. Backwards stepwise linear regression was used to identify the final model. RESULTS: A total of 52 patients enrolled. The prevalence of a positive screen on the EDE-Q was 9.6%, and on the NIAS was 13.5%. The CFQ-R eating and weight subscales were associated with scores on the EDE-Q, and CFQ-R eating subscale and being dF508 homozygous were correlated with the NIAS total score. DISCUSSION: A clinically significant number of participants screened positive for eating disorders on the EDE-Q and NIAS. Scores on the eating and weight scales of the CFQ-R were associated with the scores on these surveys. Further work is needed to better understand the optimal way to use such tools to screen and treat for eating disorders in individuals with CF.

Gastrointestinal Manifestations of Cystic Fibrosis.

Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis.

Influence of body mass index on outcome of pediatric chronic hepatitis C virus infection.

BACKGROUND AND AIMS: Evidence demonstrates that obesity is associated with progression of chronic hepatitis C virus (HCV) infection and poor response to interferon therapy among HCV-infected adults. However, this evidence has been confounded by multiple comorbidities present in adult cohorts and the use of single adult doses. PATIENTS AND METHODS: We performed a retrospective investigation to evaluate the role of body mass index (BMI) in chronic HCV progression and response to therapy in the children. One hundred twenty-three children and teenagers studied at Children's Hospital Boston for HCV infection between 1998 and 2007 were included. Patients' weight and height at the time of liver biopsy or before and after HCV therapy were obtained and BMI was calculated. RESULTS: The presence of steatosis was statistically associated with higher mean (+/-SE) BMI percentiles (72nd +/- 5.8 vs 58th +/- 3.5) percentile; F(1,101) = 4.2, P = 0.04. Nonresponders to treatment had a higher mean (+/-SE) BMI percentile (70th +/- 7.4) when compared with responders (50th +/- 6.5) in univariate and multivariate analyses (P = 0.04, P = 0.02, respectively). Using a multivariate model, it was calculated that 1 standard deviation (1 z-score unit) increase in baseline BMI z score is associated with a 12% decrease in the probability of sustained virologic response. CONCLUSIONS: Overweight adversely affects the progression of chronic HCV liver disease and is associated with diminished response to antiviral therapy using weight-based dosing in a cohort with minimal comorbidities.