Spina bifida: implications for cognitive functioning, disability and health in young adults

Abstract

Spina bifida is a developmental birth defect involving the neural tube. It can result in a variety of problems, amongst them incontinence, restrictions of mobility and restrictions of cognitive functioning, depending on the severity of the defect. Due to improvements in medical care, the life expectancy of children with spina bifida has improved considerably and nowadays most children reach the adult age. However, little is known about the problems regarding the medical condition and the quality of life of adolescents and young adults with spina bifida and about their needs for medical care. The aim of the study was describe the functional limitations, social participation and subjective quality of life of Dutch young adults with spina bifida in order to make recommendations concerning the care and support for this group of young adults. Participants were persons with spina bifida aperta or spina bifida occulta and were between 16 and 25 years of age. A total of 179 (response 51%) participated in this study. Results Many spina bifida patients suffer from secondary impairments such as incontinence, constipation, foot deformities, scoliosis and loss of mobility. The groups most likely to suffer from these impairments are young adults with hydrocephalus or a high level of lesion. Persons without hydrocephalus have a cognitive status similar to that of healthy peers. The cognitive status of the group of young adults with hydrocephalus is lower than that of the general population. Roughly half of the young adults with hydrocephalus suffer from specific or global cognitive impairments. Persons without hydrocephalus have an educational outcome similar to that of healthy peers. The educational outcome of the group of young adults with hydrocephalus is lower than that of the general population. Only half of the young adults with hydrocephalus attend regular secondary school. Intelligence level was the main predictor of educational outcome, but hydrocephalus and wheelchair dependence were additional significant predictors. Many young adults with spina bifida experience participation restrictions for the domains of educational career, vocational status and residential status. Severity of spina bifida and to a lesser extent activity limitations were negatively related to participation. Perceived restrictions in participation in relation to accessibility of buildings and long distance transport were most noticeable for wheelchair dependent patients. Perceived restrictions in relation to physical impairments and emotional stress were found for all patients with spina bifida, regardless of the severity of the spina bifida. The overall life satisfaction of young adults with spina bifida is similar to that of healthy peers. The domains of self care ability and partnership relations are negatively affected by spina bifida, but overall spina bifida does not seem to be an important determinant of life satisfaction. Executive functioning was associated with how young adults with spina bifida and hydrocephalus rated their happiness and satisfaction in life as well as their quality of life. Recommendation The most important recommendation is that the medical and psychosocial care for young adults with spina bifida is preferable organized in specialized multidisciplinary teams and should be available in every part of The Netherlands.