Transition of Care for Patients With Hirschsprung Disease and Anorectal Malformations

• Conditions: Anorectal Malformations; Quality of Life; Transition; Hirschsprung Disease
• Interventions: Other: Questionnaires

• Registration Number: NCT04106947
• Lead Sponsor: Oslo University Hospital

Brief Summary

Transition from paediatric to adult health care is crucial for preventing deterioration of chronic diseases. At present, transitional care (TC) is not established for patients with the Hirschsprung disease (H) and Anorectal Malformations (ARM). To set up a program for TC and to treat persisting symptoms in adults, data on outcome in adult patients are needed. At present such data are very limited. Therefore, we want to investigate clinical and PROM in H and ARM adolescents and adults. A cross sectional study in all H and ARM patients operated in Norway from 1970-2000 and in all adolescents operated at Oslo University Hospital from 2002-2006 will examine somatic, psychosocial and mental health, and quality of life (QoL). In children operated for H and ARM a large body of evidence shows that bowel problems, reduced QoL and impaired psychosocial and mental health are common. There are papers on sexual and urological impairment in these patients, but large studies on the topic is missing. It is a general assumption among paediatric surgeons that both somatic and mental health problems related to H and ARM improve during adolescence and adult life. Therefore, no standardized guidelines for TC in these patients have been established. Interestingly, very few studies have actually examined H and ARM patients beyond adolescence. Reports from patient organizations showing significant long-term sequels and inadequate understanding of the unique problems of H and ARM patients among health professionals treating adults. Hypothesis:H and ARM adults and adolescents have bowel, urinary and sexual difficulties and reduced QoL, psychosocial and mental health. H and ARM adults receive insufficient treatment of their chronic congenital disease. H and ARM patients with syndromes have particularly bad functional outcome. Anal dilatations and repeated rectal enemas have a negative impact on adolescent psychosocial and mental health. Main aim: Acquire knowledge about long-term bowel, urinary and sexual function, QoL and psychosocial and mental health in adult and adolescent H and ARM patients. Results: from this large study of H and ARM patients will have significant influence on treatment and follow-up, both nationally and internationally. Since very few countries except the Nordic countries have the possibility to follow patients with congenital malformations into adulthood, it is important that studies like this are done.

Detailed Description

Not available

Study Timeline

• Study Start: 2019-08-12
• Study First Submitted: 2019-09-20
• Study First Submitted QC: 2019-09-25
• Study First Posted: 2019-09-27
• Status Verified: 2021-02
• Last Update Submitted: 2021-02-22
• Last Update Posted: 2021-02-23
• Primary Completion: 2021-08
• Study Completion: 2022-08

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 1000

Inclusion Criteria

Alle patients with Hirschsprung and anorectal malformation

Exclusion Criteria

None

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Patients with Hirschsprung and anorectal malformation	Questionnaires	Patients with Hirschsprung and anorectal malformation living in Norway

Primary Outcome Measures

Name	Time	Method
questionnaire	2 years	Acquire knowledge about quality of life in adult and adolescent H and ARM patients
questionnaires	2 years	Acquire knowledge about mental Health in adult and adolescent H and ARM patients
questionnaire: BFS	2 years	Acquire knowledge about long-term bowel function in adult and adolescent H and ARM patients
urinary flow-residual	2 years	Acquire knowledge about urinary function in adult and adolescent H and ARM patients
questionnaire: PIQS12 and IIEF-5	2 years	Acquire knowledge about sexual function in adult and adolescent H and ARM patients
questionnaire: DAN-PSS	2 years	Acquire knowledge about urinary function in adult and adolescent H and ARM patients

Secondary Outcome Measures

Name	Time	Method
Focus Group interviews will be applied for parents.	2 years	Acquire knowledge about the influence of anal dilatations and regular rectal enemas on psychosocial and mental health in adolescents
Focus Group interviews With adult Hirschsprung's disease (HD) and anorectal malformations (ARM) patients	2 years	Acquire knowledge about how adult H and ARM patients have experienced and would design transition from paediatric to adult health care
Questionnaires (impact of event scale) to adolescent and parent. Focus Group interviews will be applied for parents.	2 years	Acquire knowledge about the influence of anal dilatations and regular rectal enemas on psychosocial and mental health in adolescents
Questionnaires to next of kin to syndromic patients	2 years	Acquire knowledge about bowel function in adult syndromic H and ARM patients

Trial Locations

Locations (1)

Oslo University Hospital; 🇳🇴 Oslo, Norway