Until a medical cure for juvenile Huntington’s disease (JHD) is identified, the optimal management of a child with this condition will require a sensitive team of health professionals working with the family and the community to minimize the impact of disease symptoms, share the psychological and social burden of care that the family must shoulder, and promote mutually beneficial interactions between the child and the world around him or her. There is not a single medication of proven benefit in JHD; because this is an uncommon presentation of a relatively uncommon disease, the medical literature includes, at best, case reports suggesting that one or another treatment might be of benefit. This can lead some physicians, with an attitude of therapeutic nihilism, to suggest to families through their words or actions, that ‘there is nothing we can do to help’.
The authors of this volume take strong exception to that attitude, and suggest that if the physician works with the family, and rallies the help of nurses, social workers, therapists, counsellors, clergy, and schools, that much can be done to improve a child’s course with JHD. The physician must assume the role of team leader, providing education and guidance to new team members as they join in the child’s care, and must also be willing to work creatively with the family to use medications as well as non-pharmacological therapies. The medical management of JHD has three parts: coordinating the ‘big picture’, including anticipation of upcoming changes and problems; managing individual symptoms as they arise; and ensuring that age-appropriate paediatric care is not neglected because of the overwhelming nature of the neurological disease.
Chapter. 5565 words.
Subjects: Neurology ; Clinical Pharmacology and Therapeutics
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