Transition of Care for Patients With Hirschsprung Disease and Anorectal Malformations

NCT04106947 | Unknown

• 1 other identifier: 18/19101
• Study Type: observational
• Target: 1,000
• Locations: 1 country
• Sites: 1

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.

Conditions

Anorectal Malformations; Transition; Quality of Life; Hirschsprung Disease

Outcome Measures

Primary Outcomes (6)

• questionnaire: BFS

  Acquire knowledge about long-term bowel function in adult and adolescent H and ARM patients

  2 years

• questionnaire: DAN-PSS

  Acquire knowledge about urinary function in adult and adolescent H and ARM patients

  2 years

• urinary flow-residual

  Acquire knowledge about urinary function in adult and adolescent H and ARM patients

  2 years

• questionnaire: PIQS12 and IIEF-5

  Acquire knowledge about sexual function in adult and adolescent H and ARM patients

  2 years

• questionnaire

  Acquire knowledge about quality of life in adult and adolescent H and ARM patients

  2 years

• questionnaires

  Acquire knowledge about mental Health in adult and adolescent H and ARM patients

  2 years

Secondary Outcomes (4)

• Focus Group interviews With adult Hirschsprung's disease (HD) and anorectal malformations (ARM) patients

  2 years

• Questionnaires (impact of event scale) to adolescent and parent. Focus Group interviews will be applied for parents.

  2 years

• Focus Group interviews will be applied for parents.

  2 years

• Questionnaires to next of kin to syndromic patients

  2 years

Study Arms (1)

Patients with Hirschsprung and anorectal malformation

Patients with Hirschsprung and anorectal malformation living in Norway

Other: Questionnaires

Interventions

Questionnaires OTHER

Questionnaires

Patients with Hirschsprung and anorectal malformation

Eligibility Criteria

• Age: 12 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Alle patients with Hirschsprung and anorectal malformations living in Norway

You may qualify if:

• Alle patients with Hirschsprung and anorectal malformation

You may not qualify if:

• None

Sponsors & Collaborators

• Oslo University Hospital: lead
• University Hospital, Akershus: collaborator
• Skane University Hospital: collaborator
• St. Olavs Hospital: collaborator

Study Sites (1)

Oslo University Hospital

Oslo, Norway

Location

MeSH Terms

Conditions

Anorectal Malformations; Hirschsprung Disease

Interventions

Surveys and Questionnaires

Condition Hierarchy (Ancestors)

Digestive System Abnormalities; Digestive System Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Megacolon; Colonic Diseases; Intestinal Diseases; Gastrointestinal Diseases

Intervention Hierarchy (Ancestors)

Data Collection; Epidemiologic Methods; Investigative Techniques; Health Care Evaluation Mechanisms; Quality of Health Care; Health Care Quality, Access, and Evaluation; Public Health; Environment and Public Health

Study Officials

• Kristin Bjørnland, Prof

  Oslo University Hospital and University of Oslo

  STUDY CHAIR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Target Duration: 2 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Clinical Fellow

Study Record Dates

• First Submitted: September 20, 2019
• First Posted: September 27, 2019
• Study Start: August 12, 2019
• Primary Completion: August 1, 2021
• Study Completion: August 1, 2022
• Last Updated: February 23, 2021

Record last verified: 2021-02

Data Sharing

• IPD Sharing: Will not share

Locations

NO (1)