Prospective Multisite Study of Quality of Life in Pediatric Intestinal Failure

Health related quality of life is becoming an increasingly important topic as medical advancements continue to increase life expectancies in various illnesses. Quality of life (QoL) encompasses the physical, psychological, and social well-being of a person. Studies focusing on health related QoL show that providers have a limited understanding of the QoL of their patients, demonstrating a need for further research. Mortality rates in pediatric intestinal failure (PIF) have significantly dropped over the past 3-4 decades and long-term morbidity rather than survival has come into focus as the outcome of interest. However, there remains a significant knowledge gap regarding the specific factors that contribute to QoL among PIF patients and their families. Insight into the challenges these patients face can promote enhanced support services and psycho-education for caregivers of these children as well as help to tailor medical and surgical treatment with a focus on improved QoL. Due to the rare nature of PIF, and despite a recent bloom in multidisciplinary PIF centers, experience is limited in individual centers and a multi-site study with a robust and diverse patient population is optimal to study QoL in pediatric intestinal failure.

Inclusion criteria

• Participants will be followed in a participating institution's intestinal rehabilitation program
• Participants will have a diagnosis of intestinal failure due to functional or structural intestinal dysfunction with current or prior history of specialized nutritional support (parenteral nutrition requirement for 60 out of 74 consecutive days)
• Participants will be age 6 months to 25 years old.
• Parents/caregivers must be able to complete questionnaire without assistance.
• English or Spanish speaking

Exclusion criteria

• Participants aged less than 6 months or greater than 25 years will not be included in this study
• Participants will not be enrolled less than 3 months from index admission or initial outpatient evaluation
• Participants will not be enrolled less than 1 month from inpatient admission (any admission greater than 24 hours in duration)
• Participants will not be enrolled less than 1 month from any operative intervention requiring general anesthesia.
• Primary language other than English or Spanish.
• Participants who have a currently functional small bowel, liver/small bowel or multivisceral transplant
• While other major co-morbidities may be excluded at a later time during data analysis, or may be analyzed as a specific sub-group, they will not be excluded outright. For example, participants with global developmental delay may have parent surveys only, and therefore would need to be excluded from paired analysis of proxy vs. participant perception of HRQOL