Spina bifida is the most common defect of the central nervous system. It is a congenital malformation of the spine with abnormal neural tube closure occurring between the third and fourth weeks of gestation, and most frequently affecting the lumbar and sacral regions. Most children with spina bifida have a normal urinary tract at birth, although renal damage and renal failure are among the most severe complications of spina bifida. Before ventricular shunting, survival rates for children with spina bifida were low, but most patients can now be expected to live into adulthood, thus prevention of urologic complications and promotion of continence have become critical. This article reviews the literature regarding urinary continence, and discusses issues across the lifespan, and implications for clinical practice and the pediatrician’s role in the urologic care of children with spina bifida.
Spina bifida is the most common defect of the central nervous system, affecting approximately 1000 newborns each year in the United States. The Centers for Disease Control and Prevention (CDC) estimates that as many as 166,000 individuals with spina bifida live in the United States. Spina bifida is a congenital malformation of the spine with abnormal neural tube closure occurring between the third and fourth weeks of gestation, and most frequently affecting the lumbar and sacral regions. Liptak and El Samra characterize it as the most complex birth defect compatible with survival. Most children with spina bifida have a normal urinary tract at birth, although renal damage and renal failure are among the most severe complications of spina bifida, resulting from lack of appropriate innervation of the bladder, and the effects of the neurogenic bladder on the kidneys. Thirty to forty percent of individuals with spina bifida exhibit varying degrees of renal dysfunction throughout life, and renal failure is the most common cause of death. Before ventricular shunting, survival rates for children with spina bifida were low, and therefore “urologic intervention was rarely necessary.” (p 72) With improving technologies and clinical care, most patients can be expected to live into adulthood, thus prevention of urologic complications, in particular renal failure, and promotion of continence have become critical.
This article reviews the literature regarding urinary continence, and discusses issues across the lifespan and implications for clinical practice. The pediatrician’s role in the urologic care of children with spina bifida focuses on serving as the medical home for the child, assisting the child and family to become independent in care, promoting urinary continence, and monitoring for the development of urologic complications that threaten the health of the kidneys, in collaboration with the spina bifida special care center and other specialists. In addition, the medical home coordinates care with providers and services in the community, and works with the child and family as they transition to adult care.
Goals of urologic care
More than 90% of children with spina bifida have normal upper tracts at birth, although, if unattended, 50% will experience deterioration. There are 4 goals of urologic care for the individual with spina bifida: (1) prevention of urinary tract infection, (2) preservation of renal function to avoid chronic renal failure and end-stage renal disease, (3) prevention of decubitus ulcers through promotion of continence, and (4) facilitation of urinary continence and independence in bladder care. Achievement of these goals is assumed to enable meaningful participation in activities (including adult sexual experiences, and typical vocations and avocations), promote positive self-esteem, and improve quality of life. Successful therapeutic interventions require a working knowledge of the pathophysiology, effective treatments, normal developmental processes, and effective means of empowering parents and children to master urinary continence as a means to promote health and greatly improve activity and participation in typical societal roles as adults.
Urinary continence is a significant factor in estimating the quality of life for children with spina bifida and their families. Percentages of children who are continent range from 78% to 90%. Children with spina bifida are at risk for symptoms of depression and anxiety, as well as lower levels of self-concept compared with their nonaffected peers. This may be because of teasing, feeling self-conscious about their bodies and physical appearance, and social isolation. Daily bladder management can become burdensome for parents and children, and, as children struggle with negative feelings, parents may experience frustration with their child’s bladder and bowel management programs, or adherence to these programs, resulting in tension between parents, children, and team members. Previous research conducted with other chronic conditions suggests that family cohesion is related to better treatment adherence, whereas family conflict has been associated with poorer treatment adherence. Siblings may also experience frustration with their brother’s or sister’s bladder or bowel management programs, especially if they attend the same school or share mutual friends. We have heard siblings complain about being embarrassed about catheters being left in the bathroom, where friends may discover them. However, the research on the adjustment of siblings of spina bifida patients has been mixed, and not focused on the effect of incontinence on siblings’ emotional functioning. Nevertheless, because spina bifida care affects the entire family, it is important to overall family functioning and quality of life to identify the factors that promote successful bladder and bowel continence programs for children who have spina bifida.
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