Transition of Care for Patients With Hirschsprung Disease and Anorectal Malformations

Quality of Life Clinical Trial

— NOHARM  

Official title:

Transition of Care for Patients With Hirschsprung Disease and Anorectal Malformations

Clinical Trial Details — Status: Enrolling by invitation

Administrative data

• NCT number: NCT04106947
• Other study ID #: 18/19101
• Status: Enrolling by invitation
• Start date: August 12, 2019
• Est. completion date: August 2022

Study information

• Verified date: February 2021
• Source: Oslo University Hospital
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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.

Recruitment information / eligibility

• Status: Enrolling by invitation
• Enrollment: 1000
• Est. completion date: August 2022
• Est. primary completion date: August 2021
• Accepts healthy volunteers: No
• Gender: All
• Age group: 12 Years and older

Eligibility

Inclusion Criteria:

Alle patients with Hirschsprung and anorectal malformation

Exclusion Criteria:

None

Related Conditions & MeSH terms

• Anorectal Malformations
• Congenital Abnormalities
• Hirschsprung Disease
• Quality of Life
• Transition

Intervention

Other:
• Questionnaires
Questionnaires

Locations

Country	Name	City	State
Norway	Oslo University Hospital	Oslo

Sponsors (4)

Lead Sponsor	Collaborator
Oslo University Hospital	Skane University Hospital, St. Olavs Hospital, University Hospital, Akershus

Country where clinical trial is conducted

Norway, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	questionnaire: BFS	Acquire knowledge about long-term bowel function in adult and adolescent H and ARM patients	2 years
Primary	questionnaire: DAN-PSS	Acquire knowledge about urinary function in adult and adolescent H and ARM patients	2 years
Primary	urinary flow-residual	Acquire knowledge about urinary function in adult and adolescent H and ARM patients	2 years
Primary	questionnaire: PIQS12 and IIEF-5	Acquire knowledge about sexual function in adult and adolescent H and ARM patients	2 years
Primary	questionnaire	Acquire knowledge about quality of life in adult and adolescent H and ARM patients	2 years
Primary	questionnaires	Acquire knowledge about mental Health in adult and adolescent H and ARM patients	2 years
Secondary	Focus Group interviews With adult Hirschsprung's disease (HD) and anorectal malformations (ARM) patients	Acquire knowledge about how adult H and ARM patients have experienced and would design transition from paediatric to adult health care	2 years
Secondary	Questionnaires (impact of event scale) to adolescent and parent. Focus Group interviews will be applied for parents.	Acquire knowledge about the influence of anal dilatations and regular rectal enemas on psychosocial and mental health in adolescents	2 years
Secondary	Focus Group interviews will be applied for parents.	Acquire knowledge about the influence of anal dilatations and regular rectal enemas on psychosocial and mental health in adolescents	2 years
Secondary	Questionnaires to next of kin to syndromic patients	Acquire knowledge about bowel function in adult syndromic H and ARM patients	2 years