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Facing Unwelcome Truths

Sometimes the rare and grim retains its power to shock. Bandolier was struck by the moving account of the management of children with neurodegenerative disease in a hospice for children [1]. Over 40% of the 307 children admitted to the hospice over an eleven year period had a neurodegenerative disease. A quarter of the affected families had more than one child with the disease.

The special clinical problems were communication, feeding, respiration, excess secretions, seizures, constipation, pain, movement disorders, sleep disorders and skin care. Three quarters of the children could not speak. The use of signs, initially formal then simpler, was vital for those with retained intellectual skill despite speech impairment. Again three quarters had feeding problems. Thin liquids were harder to manage than thick. The watershed decision to move to feeding by tube or by gastrostomy is clearly emotive for all involved. Respiratory distress was managed with opioids. Hyoscine patches were used to control excess secretions, and most of the 60% who had seizure problems were managed with an oral anticonvulsant regime. Constipation, affecting 40% and compounded by the liquid diet, was dealt with by a hierarchy of lactulose, senna, microenemas and then phosphate enemas.

The difficulty in identifying and treating pain in children who cannot say they hurt or describe their pain is clear. The frequent causes of the pain identified in 35% of the children were muscle spasm, joint pain and pain from gastrointestinal causes. The movement disorders (a third of the children) were managed with antiparkinsonian, muscle relaxant and anticholinergic drugs. The severe sleep disruption, children with Sanfilippo syndrome not sleeping for many consecutive nights was better managed by practical rather than drug measures. Although most of the children were immobile none developed pressure sores.

Thinking sideways the management of these rare disorders has to be based on evidence obtained in other contexts. Improving the evidence for managing movement disorders [2] and constipation is important. A second problem is how this care is provided within the context of an internal market. Reading this account the harrowing impact on the child, the family and the community comes through. What happens when this level of charitable funding is not available?
Henry McQuay
Pain Relief Unit, The Churchill, Oxford


  1. A Hunt, R Burne. Medical and nursing problems of children with neurodegenerative disease. Palliative Medicine 1995 9:19-26.
  2. N Quinn. Drug treatment of Parkinson's disease. British Medical Journal 1995 310: 575-9.

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