Abstract
The aim of this study was to investigate the outcomes after definitive surgical correction for children with Hirschsprung’s disease (HD) and the psychosocial impact of HD on the child and family. The total sample comprised 72 children with HD along with their families. The development of a condition-specific questionnaire measured the functional and psychosocial outcomes for children with HD with parental perception of their child’s condition. Psychiatric measures were also examined to assess psychiatric morbidity. The greatest functional problem after definitive surgery for HD was faecal soiling (76%). The principle findings of the study were that (1) HD did not have a significant impact on the child’s rate of psychiatric morbidity and levels of hopefulness in comparison to the normal population, (2) surgical and psychosocial functioning improved with increasing age and, (3) families remain troubled about their future with HD and dealing with psychosocial difficulties related to the condition (such as distress because of faecal soiling). Specifically, faecal soiling was found to be physically, emotionally and psychosocially distressing complication. Bowel functioning and psychosocial distress improves with increasing age and parental and medical professional support. Despite the significant impairment of faecal continence, we found that children/young adults with HD have minimal psychiatric morbidity, yet experience condition-specific psychosocial problems (e.g. embarrassment and distress/discomfort). HD does not increase the rate of clinical psychiatric morbidity in children and families with HD, but does determine the context of their daily distress and concern.
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Abbreviations
- HD:
-
Hirschsprung’s disease
References
Pless I, Pinkerton P (1975) Chronic childhood disorder promoting patterns of adjustment. Henry Kimpton, London
Wallander JL, Varni JW (1998) Effects of pediatric chronic physical disorder on child and family adjustment. J Child Psychol Psychiatry 39:29–46
Gershon MD (1997) Genes and lineages in the formation of the enteric nervous system. Curr Opin Neurobiol 7:101–109
Pomeranz HD, Gershon MD (1990) Colonisation of the avian hindgut by cells derived from the sacral neural crest. Dev Biol 137:378–394
Serbedzija GN, Burgan S, Fraser SE (1991) Vital dye labelling demonstrates a sacral neural crest contribution to the enteric nervous system of chick and mouse embryos. Development 111:857–866
Young HM, Hearn CJ, Ciampoli D (1998) A single rostrocaudal colonisation of the rodent intestine by enteric neuron precursors is revealed by the expression of Phox2b, Ret, and p75 and by explants grown under the kidney capsule or in organ culture. Dev Biol 202:67–84
Catto-Smith AG, Coffey CMM, Nolan TM, Hutson JM (1995) Faecal incontinence after the surgical treatment of Hirschsprung disease. J Pediatr 127:954–957
Hsu WM, Chen CC (1999) Clinical and manometric evaluation of postoperative faecal soiling in patients with Hirschsprung’s disease. J Formos Med Assoc 98:410–414
Yanchar NL, Soucy P (1999) Long-term outcome after Hirschsprung’s disease: patient’s perspective. J Pediatr Surg 34:1152–1160
Bjornland K, Diseth TH, Emblem R (1998) Long-term, functional, manometric, and endosonographic evaluation of patients operated upon with the Duhamel technique. Pediatr Surg Int 13:24–28
Heij JA, Vries X, Bremer I, Ekkelkamp S, Vos A (1995) Long-term anorectal function after Duhamel operation for Hirschsprung’s disease. J Paediatr Surg 3:430–432
Shono K, Hutson JM (1994) The treatment and postoperative complications of Hirschsprung’s disease. Pediatr Surg Int 9:362–365
Teitelbaum DH, Cilley RE, Sherman NJ et al (2000) A decade of experience with the Primary pull-through for Hirschsprung’s disease in the newborn period. Ann Surg 232:372–380
Diseth TH, Bjornland K, Novik TS, Emblem R (1997) Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung’s disease. Archiv Dis Childhood 76:100–106
Moore SW, Albertyn R, Cywes S (1996) Clinical outcome and long-term quality of life after surgical correction of Hirschsprung’s disease. J Pediatr Surg 31:1496–1502
Badner JA, Seiber WK, Garver KL, Chakravarti A (1990): A genetic study of Hirschsprung’s disease. Am J Hum Genet 46:568–580
Sherman JO, Snyder ME, Weitzman JJ (1989) A 40-year multinational retrospective study of 880 Swenson’s procedure. J Pediatr Surg 24:833–838
Smith VV (1992) Isolated intestinal neuronal dysplasia. A descriptive histological entity? In: Hadziselimvic F, Herzog B (eds) Inflammatory bowel disease and Morbus Hirschsprung’s disease. Kluwer, Dordrecht, The Netherlands, pp 203–214
Fortuna RS, Weber TR, Tracy TF, Silen ML, Cradock TV (1996) Critical analysis of the operative treatment of Hirschsprung’s disease. Arch Surg 131:520–525
Marty TL, Seo T, Matlak ME, Sullivan JJ, Black RE, Johnson DG (1995) Gastrointestinal function after surgical correction of Hirschsprung’s disease: long-term follow up in 135 patients. J Pediatr Surg 30:655–658
Sarioglu A, Tanyel CF, Senocak ME (2001) Complications of the two major operations of Hirschsprung’s disease: a single centre experience. Turk J Pediatr 43:219–222
Beck AT, Steer RA, Brown GK (1996) Beck depression inventory manual. The Psychological Cooperation Harcourt Brace and Company, San Antonio
Weiss B, Weiz JR, Politano M (1991) Developmental differences in the factor structure of the children’s depression inventory. Psychol Assess 3:38–45
Spielberger CD, Edwards CD, Lushene RE (1973) Preliminary test manual for the state-trait anxiety inventory for children. Consulting Psychologist Press, California
Cross RW, Huberty TJ (1993) Factor analysis of the state-trait anxiety inventory for children with a sample of seventh-and-eighth-grade students. J Psychoeduc Assess 11:232–241
Marsh HW (1990) Self description questionnaire manual. The University of Western Sydney, Australia
Boersma FJ, Chapman JW (1992) Perception of ability scale for students manual. Western Psychological Services, Los Angeles
Hay I, Ashman A, van Kraayenoord CE (1997) Investigating the influence of achievement in self-concept using an intra-class design and a comparison of the PASS and SDQ-1 self-concept tests. Br J Educ Psychol 67:311–321
Nunn KP, Lewin TJ, Walton JM (1996a) Hunter opinions and personal expectations scale (HOPES). University of Newcastle, Australia
Nunn KP, Lewin TJ, Walton JM et al (1996b) The construction and characteristics of an instrument to measure personal hopefulness. Psychol Med 26:531–545
Moos RH, Moos BS (1994) A social climate scale: family environmental scale manual. Development, application, research. Consulting Psychologist Press, California
Asarnow JR, Carlson GA, Guthrie D (1987) Coping strategies, self-perceptions, hopelessness, and perceived family environments in depressed and suicidal children. J Consult Clin Psychol 53:361–366
Finney J, Moos R, Mewborn C (1980) Post-traumatic experiences and treatment outcome in alcoholic patients six months and two year after hospitalisation. J Consult Psychol 48:17–29
Achenbach TM (1991) Manual for the child behaviour checklist/4–18 and 1991 profile. Department of Psychiatry University of Vermont, Vermont
Albrecht G, Veerman JW, Damen H et al (2001) The child behaviour checklist for group care workers: a study regarding the factor structure. J Abnorm Child Psychol 29:83–89
Goldberg D (1978) Manual of the general health questionnaire. NFER Publishing, Windsor
Tennant C (1977). The general health questionnaire—a valid index of psychological impairment in Australian populations. Med J Aust 2:392–394
Coakes SJ, Steed L (2001) SPSS: analysis without anguish (version 10.0 for Windows). Wiley, Australia
Livaditis A (1981) Hirschsprung’s disease: long-term results of the original Duhamel operation. J Pediatr Surg 16:484–486
Rescorla FJ, Morrison AM, Engles D (1992) Hirschsprung’s disease evaluation of mortality and long-term functions in 260 cases. Arch Surg 127:934–942
Mishalany HG, Woolley MM (1987) Postoperative functional and manometric evaluation of patients with Hirschsprung’s disease. J Pediatr Surg 22:443–446
Tariq GM, Breton RJ, Wright VM (1991) Complications of endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 26:1202–1206
Ludman L, Spitz L, Tsuji H, Pierro A (2002) Hirschsprung’s disease: functional and psychological follow-up comparing total colonic and rectosigmoid aganglionosis. Arch Dis Child 86:348–351
Baillie CT, Kenny SE, Rintala RJ (1999) Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung’s disease. J Pediatr Surg 34:325–329
Diseth TH, Egeland T, Emblem R (1998) Effects of anal invasive treatment and incontinence on mental health and psychosocial functioning of adolescents with Hirschsprung’s disease and low-anorectal anomalies. J Pediatr Surg 33:468–475
Bai Y, Chen H, Hao J, Huang Y, Wang W (2002) Long-term outcome and quality of life after the Swenson procedure for Hirschsprung’s disease. J Pediatr Surg 37:639–642
Eiser C (1990) Chronic childhood disease: an introduction to psychological theory and research. Cambridge University Press, New York
Lavigne JV, Faier-Routman J (1992) Psychological adjustment to paediatric physical disorders: a meta-analytic review. J Pediatr Psychol 17:133–157
Thompson RJ Jr, Gustafson KE (1996) Adaptation to chronic childhood illness. American Psychological Association, Washington
Soliday E, Kool E, Lande MB (2001) Family environment, child behaviour and medical indicators in children with kidney disease. Child Psychiatr Hum Dev 31:279–295
Berg I, Butler A, Ellis M (1993) Psychiatric aspects of epilepsy in childhood treated with carbamazephine, phenytoin or sodium valproate: a random trial. Dev Med Child Neurol 35:149–157
Acknowledgements
We would like to thank Dr Heather Swanston’s support for the assisting in the construction of the “HD Family Impact Questionnaire”. We would also like to thank all the patients and their families for participating and sharing their experience with us.
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Athanasakos, E., Starling, J., Ross, F. et al. An example of psychological adjustment in chronic illness: Hirschsprung’s disease. Ped Surgery Int 22, 319–325 (2006). https://doi.org/10.1007/s00383-006-1651-6
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DOI: https://doi.org/10.1007/s00383-006-1651-6