Occurring once in approximately 5000 live births, Hirschsprung disease is a congenital abnormality characterized by the absence of autonomic parasympathetic ganglion cells in the colon preventing peristalsis and resulting in intestinal obstruction and abdominal distension. Also known as congenital aganglionic megacolon, this medical malady is an unfortunate anomaly of a very vulnerable population – infants and children. Hirschsprung disease can occur in otherwise healthy babies, but is commonly diagnosed in combination with Down’s syndrome and congenital heart defects. Functional outcomes, thanks to contemporary medical advances, are generally successful. As medical professionals, nurses play a significant role in helping patients achieve therapeutic goals. Therapeutic goals also include a level of psychological wellness in living with persistent bowel dysfunction after Hirschsprung surgery but few, if any, studies have addressed this likelihood.
Focusing on the results after definitive surgical repair of Hirschsprung disease, the journal article, “An example of psychological adjustment in chronic illness: Hirschsprung’s disease” by Athanasakos, Starling, Ross, Nunn, and Cass, identifies outcomes in terms of psychiatric health. The primary aim of this study was to report overall outcomes after Hirschsprung surgery and to address the impact of the disease on the optimism for a normal life. Fecal soiling was identified as the most common problem for children after surgery because incontinence is embarrassing and generally regarded as socially unacceptable. If the quality of life is negatively affected and manifests long term emotional or psychological problems for the Hirschsprung child and family, then possibly medical professionals can approach patient care differently considering therapy to promote a positive self-image.
This quantitative study was designed so that children, grouped in categories such as pre-school and school age, could be evaluated for bowel function, as well as to raise awareness of the psychosocial aspects of life with Hirschsprung disease. The sample included 131 children between the ages of 1 and 24 years and excluded those with limited medical histories, without contact information (37%), and those who had died (2%). Nine (11%) families declined to participate. Studies conducted earlier had excluded children with Down’s syndrome and/or children with total colonic aganglionosis to prevent reporting bias. Of the remaining 72 children, 82% were male and 18% were female, and this is consistent with the male to female birth rate of 4:1 born with Hirschsprung disease. A questionnaire was developed to collect data to measure bowel function and psychosocial outcome which included defining subjective complications such as what is considered fecal soiling and constipation. Psychiatric health in children over the age of seven and the child’s parent was measured using the existing assessment tool, the...