Cystic fibrosis (CF) is a chronic, multisystem disease with multiple comorbidities that can significantly affect nutrition and quality of life. Maintaining nutritional adequacy can be challenging in people with cystic fibrosis and has been directly associated with suboptimal clinical outcomes. Comorbidities of CF can result in significantly decreased nutritional intake and intestinal absorption, as well as increased metabolic demands. It is crucial to utilize a multidisciplinary team with expertise in CF to optimize growth and nutrition, where patients with CF and their loved ones are placed in the center of the care model. Additionally, with the advent of highly effective modulators (HEMs), CF providers have begun to identify previously unrecognized nutritional issues, such as obesity. Here, we will review and summarize commonly encountered comorbidities and their nutritional impact on this unique population.
Background/Objectives: Patients with spina bifida (SB) commonly experience neurogenic bowel dysfunction, characterized by defecation-related symptoms. While anorectal dysfunction and slow transit constipation (STC) have been implicated, the role of colonic motility in SB remains unclear. This study aimed to evaluate colonic motility in SB patients with refractory bowel dysfunction. Methods: This retrospective cohort study included SB patients who failed the repeated optimization of a bowel regimen including stimulant laxatives and subsequently underwent anorectal manometry (ARM), colonic transit time (CTT) studies, or colonic manometry (CM). Diagnostic findings were analyzed alongside treatment outcomes. Results: A total of 13 patients with myelomeningocele were included; one declined further treatment, and 12 underwent treatment optimization, with four achieving bowel continence. Of the five patients who proceeded with advanced motility testing, two had abnormal ARM findings, one of three had abnormal CTT results, and all five had normal CM findings. Conclusions: These findings suggest that anorectal dysfunction or STC may play a larger role in refractory bowel symptoms, while colonic motility appears to be preserved, and this highlights the importance of maximizing conservative therapies, particularly with stimulant laxatives, before pursuing invasive tests or surgical interventions for bowel dysfunction in this population.