Clinical trial NCT04106947: Transition of Care for Patients With Hirschsprung Disease and Anorectal Malformations
Official title: Transition of Care for Patients With Hirschsprung Disease and Anorectal Malformations
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Anorectal Malformations
Transition
Quality of Life
Hirschsprung Disease
Lead sponsor: Oslo University Hospital
Sponsor class: OTHER
Collaborators: name: University Hospital, Akershus
name: Skane University Hospital
name: St. Olavs Hospital
First posted: 2019-09-27
Enrollment: 1000
Sex: ALL
Minimum age (years): 12
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: CROSS_SECTIONAL
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Alle patients with Hirschsprung and anorectal malformations living in Norway

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.


Eligibility criteria: Inclusion Criteria:

Alle patients with Hirschsprung and anorectal malformation

Exclusion Criteria:

None

Primary outcomes:
measure: questionnaire: BFS
timeFrame: 2 years
measure: questionnaire: DAN-PSS
timeFrame: 2 years
measure: urinary flow-residual
timeFrame: 2 years
measure: questionnaire: PIQS12 and IIEF-5
timeFrame: 2 years
measure: questionnaire
timeFrame: 2 years
measure: questionnaires
timeFrame: 2 years
Secondary outcomes:
measure: Focus Group interviews With adult Hirschsprung's disease (HD) and anorectal malformations (ARM) patients
timeFrame: 2 years
measure: Questionnaires (impact of event scale) to adolescent and parent. Focus Group interviews will be applied for parents.
timeFrame: 2 years
measure: Focus Group interviews will be applied for parents.
timeFrame: 2 years
measure: Questionnaires to next of kin to syndromic patients
timeFrame: 2 years
Other outcomes:
Locations:
city: Oslo
country: Norway
facility: Oslo University Hospital
Study officials:
affiliation: Oslo University Hospital and University of Oslo
name: Kristin Bjørnland, Prof
role: STUDY_CHAIR
References: