Beyond clinical outcomes: using a disease-specific pilot survey to explore parent perceptions of wellbeing for children with short bowel syndrome

ABSTRACT

Background: Pediatric patients with short bowel syndrome (SBS) have complex medical needs that may impact their quality of life (QoL). Research has generally found children with SBS/intestinal failure (IF) to have impaired health-related QoL, but the mechanisms by which SBS shapes QoL for these patients remain unclear. Methods: A disease-specific mixed-methods pilot survey investigating wellbeing for children with SBS and their families was developed collaboratively by community stakeholders and clinicians and distributed via convenience sampling. The survey included fourteen diseasespecific items (e.g., PN, EN, toileting, eating, medical procedures), each scored on a 5-point Likert scale (1=no negative impact on child's wellbeing, 5=high negative impact on child's wellbeing);a not applicable option was additionally included for each item. Upon completion of the matrix, respondents were prompted to explain their response for each item scored with a 4 or 5 (indicating a high negative impact on child QoL). An option to describe any additional items perceived as negatively impacting their child's QoL was provided. Descriptive analyses of closed-and open-ended responses were conducted to investigate parent-perceived impact of disease-specific items on child wellbeing. Results: A total of 21 parents completed the survey. Items least frequently reported by parents as having a strong negative impact on their child's wellbeing were pain/discomfort or vomiting (23%) and utilization of PN (29%). Items related to absorption, including oral eating (33%), output-related issues (38%), and enteral feeds (41%), were more frequently reported by parents as strongly negatively impacting their child's wellbeing. Half of survey respondents (50%) perceived sleep or fatigue to have a strong negative impact. Items outside of the medical/physical domain were reported by 62% of parents as having a strong negative impact on their child's wellbeing 39% of parents perceived their child's social life (independent of the impacts of the COVID-19 pandemic) to have suffered because of the condition;39% highlighted the impact of medical procedures on their child's mental health. Over half of respondents (53%) perceived two or fewer items to have a strong negative impact on their child's overall wellbeing. An additional 29% of respondents perceived 3-6 items, while 19% reported 7 or more items as having a strong negative impact on their child's wellbeing. Qualitative analyses of open-ended responses revealed no additional SBS-related items were perceived by parents as negatively shaping child wellbeing, suggesting the survey reached thematic saturation. Conclusion: These exploratory data provide insights into the parent-perceived factors shaping wellbeing for children with SBS. Better understanding how- and to what extent- these factors impact child QoL longitudinally is central to providing whole-person, patient-centered care. The conceptualization of overall wellbeing as a measurable outcome may provide members of the care team with important information to consider along with clinical factors and offer opportunities to discuss goals of care with children and their families. Future research should investigate the validity and reliability of a disease-specific child QoL measure.