Functional aspects of spina bifida in childhood

Abstract

Due to advanced medical treatment, more children with spina bifida survive into adulthood. Most outcome studies in the field of spina bifida research focus on disease aspects (‘pathology’) and its relation to one of the domains in the ‘disablement process’ such as physical functioning, cognitive abilities, or psychosocial adjustment. From these studies it became clear that spina bifida has significant impact on the health of individuals, but little is known on ‘health related quality of life’ (HRQL). The main goal of this thesis is to get more insight into the different domains of the ‘disablement process’ in children with spina bifida. We were especially interested in how ‘pathology’, ‘impairments’,‘functional limitations’, ‘disability’ and HRQL relate to each other in order to set relevant and realistic goals for physical therapy treatment. Physical therapy traditionally focuses on improvement of muscle strength and muscle tone, and the prevention of contractures, to optimize childhood development and functioning. However, it is not clear how these impairments relate to functional independence and HRQL. As there is often an intense demand for commitment on children and their families regarding physical therapy, knowledge of determinants of independence is of utmost importance in order to focus “on realistic goals rather than wasting efforts on attempting the impossible”.

The following conclusions can be drawn from this thesis: 1. Parameters on the level of ‘impairments’ are statistically significantly associated with ‘disability’. 2. Ambulation is significantly positively associated with muscle strength of hip abductors and ankle dorso-flexors. 3. Good muscle strength, having no contractures and mental ability appears to be much more important for daily life functioning of children with spina bifida than other medical indicators. 4.With regard to independence in mobility, good muscle strength, especially of knee extensors, having no contractures, and good mental ability, appears to contribute more to independence than lesion level and shunt status. 5. Being independent in mobility appears to contribute more to HRQL than being independent in self-care or being wheelchairdependent. 6. In young children (= 7.5 years) with spina bifida, PEDI-scores were below the mean, but still within normal ranges with the exception of scores on mobility, whereas in older children (> 7.5 years) scores were deviant in all domains. 7. Most children with sacral level paralysis have gross motor problems, more severe in myelomeningocele than in lipomyelomenigocele. In both groups caregiver assistance was needed for a prolonged period, especially regarding bladder and bowel management. 8. Although patients with myelomeningocele were more disabled than those with other types of spina bifida, perceived competence in the latter group was not better. 9. Psycho-social support might be needed in children with other types of spina bifida and minor disabilities as well as in those with myelomeningocele with severe disabilities. 10. After neurosurgical untethering, late deterioration of motor andambulation level occurs in a minority of patients. Deterioration and retethering is mainly seen in patients with (lipo)myelomeningocele and not in patients with other types of spinal dysraphism. 11. Magnitude of curvature significantly decreases after spinal fusion. 12. Within the first six months after spinal fusion, more caregiver assistance is needed in self-care and mobility. It takes about 12 months to recover to pre-surgery level, while a small improvement is seen afterwards. 13. After spinal fusion, ambulation can become difficult in patients who are exercise walkers prior to surgery.