Medical and nursing problems of children with neurodegenerative disease

Abstract

Little is written on the management of problems encountered by children with neurodegenerative disease. Whilst the conditions are individually rare, as a group of diseases they pose a considerable burden on the child, the family and the community. This study describes the 127 children with neurodegenerative disease who were admitted to Helen House, a hospice for children, from the time of opening in November 1982 until the end of 1993. The great majority of the conditions were genetic in origin and nearly one-third of the families had had two or more children with the same disease. Seventy-four (58%) of the children have died, with nearly half dying at home. The course of the disease was in many cases chronic and these children presented with multiple medical and nursing problems. Examination of the problems recorded in 1993 revealed that nearly all the children had no speech, or speech was impaired. Most were either totally immobile or had considerably reduced mobility. Seventy per cent of the children had feeding problems and one-quarter were fed through a nasogastric tube or gastrostomy. Thirty-five per cent of the children suffered pain on occasions, with muscle spasm being the main identifiable source of pain. Practical suggestions are made for the management of these children.