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Medical and nursing problems of children with neurodegenerative disease
Insights
This study details challenges faced by children with neurodegenerative diseases, highlighting common issues like impaired speech, mobility, feeding difficulties, and pain. Practical management strategies are suggested for these complex pediatric cases.
Area of Science:
- Pediatric Neurology
- Palliative Care
- Genetics
Background:
- Neurodegenerative diseases in children are rare individually but collectively significant.
- These conditions present substantial burdens on affected children, families, and communities.
- Limited literature exists on managing the complex needs of these pediatric patients.
Purpose of the Study:
- To describe the characteristics and problems of children with neurodegenerative diseases admitted to a children's hospice.
- To identify common medical and nursing challenges encountered in this patient group.
- To offer practical management suggestions based on clinical experience.
Main Methods:
- Retrospective case series analysis of 127 children admitted to Helen House hospice between November 1982 and December 1993.
- Data collection focused on diagnosis, family history, clinical problems, and outcomes.
- Analysis of recorded problems in 1993, including communication, mobility, feeding, and pain.
Main Results:
- The majority of conditions were genetic; 30% of families had multiple affected children.
- 58% of children died during the study period, with nearly half dying at home.
- Common issues included impaired speech (nearly all), reduced mobility (most), feeding difficulties (70%, with 25% requiring tube feeding), and pain (35%, often from muscle spasms).
Conclusions:
- Children with neurodegenerative diseases experience chronic, complex medical and nursing needs.
- Effective management requires addressing communication, mobility, nutritional support, and pain.
- Further research and practical guidelines are needed to support these children and their families.
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.