Transitional health care for patients with Hirschsprung disease and anorectal malformations

Abstract

Background Hirschsprung disease (HD) and anorectal malformations (ARM) are congenital disorders with potentially lifelong consequences. Although follow-up is performed in most pediatric patients, transfer to adult health care is often problematic. This study assesses transitional care with the help of questionnaires in consultation with adult patients.MethodsThis study was conducted in an outpatient clinic of a pediatric surgical center in the Netherlands. All patients born and treated for ARM or HD before 1992 were invited to visit our clinic. Patients completed questionnaires concerning disease-specific functioning and quality of life at an initial visit to in response to which individual treatment plans were modified. Patients were reviewed 1 year later.Results Twenty-seven patients (17 ARM and 10 HD), mean age 27.9 years (range 17–64 years) of the 168 invited visited the transitional clinic (17%). Passive fecal incontinence was reported by 7/27, other defecatory problems, including urge incontinence and incomplete evacuation in 17/27 and anal or abdominal pain reported by 9/27. Quality of life was lower than a matched population. Only 13/27 returned for repeat assessment at 1 year; however, a further 8 reported that that their problems had resolved. In those attending follow-up, negative thoughts and feelings about their condition had decreased and one more patient was fully continent. There was no change in quality of life, bowel function or pain recorded. Twelve out of thirteen patients reported that they had found the transitional clinic satisfactory.ConclusionsThe transitional outpatient clinic provides care adapted to the needs and wishes of adult HD and ARM patients. It is a novel addition to quality of care of patients with complex congenital disorders.

Key components of successful transition for adolescents born with anorectal malformations–a Nordic focus group study

“…Since patients born with ARM might suffer from several somatic-and psychological conditions related to their congenital malformation, they depend on life-long treatment and follow-up by multiple medical specialties. There are a few studies exploring the transitional process from paediatric-to adult healthcare for patients born with ARM [15][16][17][18][19]. The transitional care is referred to as being inadequate, but the manner in which adolescents wish to make their transfer has not yet been explored.…”

Empowering Patients and Health Professionals to Address Sexual Health in the Context of Anorectal Malformations and Hirschsprung’s Disease

“…In addition, psychological problems are frequently observed, such as shame due to fecal incontinence. Moreover, wearing a stoma ( Grano et al, 2011 ; Leitner et al, 2017 ) or dilatations during childhood ( Athanasakos et al, 2006 ; Witvliet et al, 2017 ) may contribute to a negative body image. These psychological problems may make patients hesitant to seek friendships and sexual relationships in adulthood ( Witvliet et al, 2018 ) and cause sexual impairment such as erectile dysfunction, vaginism, or dyspareunia—genital pain that interferes with sexual intercourse ( Konuma et al, 2006 ; Schmidt et al, 2012 ; van den Hondel et al, 2015 ).…”

“…These psychological problems may make patients hesitant to seek friendships and sexual relationships in adulthood ( Witvliet et al, 2018 ) and cause sexual impairment such as erectile dysfunction, vaginism, or dyspareunia—genital pain that interferes with sexual intercourse ( Konuma et al, 2006 ; Schmidt et al, 2012 ; van den Hondel et al, 2015 ). Sexual impairment could also be the result of postoperative problems, such as scar tissue, or constrictions in the genital area due to urogenital anomalies ( Athanasakos et al., 2006 ; Manderson, 2005 ; Witvliet et al, 2017 ). 1 These problems can negatively affect the sexual health of these patient groups ( Kyrklund et al, 2016 ; Rigueros Springford et al, 2016 ; van den Hondel et al., 2015 ).…”

“…Of these patients, 60% (ARM) and 59% (HD) felt a need for education ( van den Hondel, 2015 ). In addition, Witvliet et al (2017) found that only one out of 168 patients with ARM and HD was referred to a specialist for sexual-related issues. Various authors suggested the need for better follow-up during adulthood to improve support ( Stenström et al, 2014 ; Witvliet et al, 2018 ).…”

“…A study by van den Hondel et al (2015) highlighted that patients with ARM and HD experience a need for support in managing their sexual problems. Nevertheless, there is a lack of attention to sexual health in clinical practice (Schmidt et al, 2012; Witvliet et al, 2017). van den Hondel et al (2015) showed that 93.5% of the patients they questioned did not receive any support—in the form of counseling or patient education—regarding (future) sexual problems.…”