Quality of life measures in the study of neurogenic bowel dysfunction among children and adolescents with spina bifida.

INTRODUCTION: Neurogenic bowel dysfunction (NBD) affects many individuals with spina bifida (SB) and is associated with substantial physical, social, and emotional burdens. Although quality of life (QoL) is recognized as an important outcome in SB care, the tools used to measure the impact of NBD on QoL in the pediatric SB population have not been systematically evaluated.

OBJECTIVE: This systematic review aimed to identify instruments used to assess the impact of NBD on QoL of children and adolescents with SB and to document the specific domains evaluated.

STUDY DESIGN: A methodological systematic review was conducted consistent with Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines. The inclusion criteria were: (1) participants: children and adolescents (0-21 years of age) with SB and/or myelomeningocele (MMC), (2) outcome: utilization of an author-reported "QoL tool" to measure the impact of NBD on QoL, and (3) English-language publication. Data regarding study characteristics, respondent type, evaluation tools used, whether the tool focused on measurement of QoL or functioning, and tool domains were extracted and synthesized.

RESULTS: Twenty studies met the inclusion criteria and were conducted across the United States, Europe, Asia, and Australia, with most authorship represented by surgical subspecialties. Sixteen unique tools were identified with six tools being classified as condition-specific tools and ten as generic tools. The Fecal Incontinence and Constipation Quality of Life questionnaire was the most used instrument and was used in six of the 20 studies. Half of the studies did not specify who completed the QoL instrument, and only one study relied exclusively on self-report. Notably, only two of the identified instruments primarily measured QoL rather than function. Generic QoL tools included broad domains that covered physical, emotional, and social functioning regardless of diagnosis, whereas condition-specific tools included additional domains assessing concepts such as financial impact and independence.

CONCLUSION: There was significant variability observed in measurement approaches, professionals conducting QoL research, and respondents completing the QoL tools. An ideal QoL instrument is simple, condition-specific, clinically relevant, age-appropriate, available in both self- and proxy-report formats, and able to capture the multidimensional nature of QoL separate from function. The QUALAS emerged as a tool which fulfills these criteria for evaluation of the impact of NBD on QoL among individuals with SB. Given the global prevalence of SB and the growing international focus on promoting QoL, it is important that QoL instruments are culturally adaptable and available in multiple languages.