A standard of care for Huntington's disease: who, what and why?
By Sheila A Simpson, Daniela Rae
Neurodegenerative disease management, February 2012, Vol. 2, No. 1, pages 1-5
Reviewed by Carol Moskowitz, APRN-C
This introductory paper describes the inheritance, cardinal signs, standard evaluation tools, and the current lack of lay and professional awareness of the positive impact of expert care on improving the lives of Huntington’s disease patients and their families. While there is a need for standards of care, few peer-reviewed articles exist to provide evidence for best practice. The role of the European Huntington’s Disease Network (EHDN), their recently established working groups which seek to define the essentials of care and the dissemination of this knowledge through the EHDN website provides a good foundation for auditing care, evaluating therapeutic interventions and future research.
This paper, written on behalf of the European Huntington’s Disease Network (EHDN), introduces the reader to an entire special on-line issue on Huntington’s disease (HD) care. This hallmark publication includes peer reviewed papers on physical therapy, nutritional management, oral feeding, oral health, speech, language and communication, and occupational therapy. These papers are the combined efforts of some of the over 20 EHDN working groups of HD experts world-wide who were asked to develop basic guidelines to help improve the care provided to HD families now and stimulate future research to refine non-pharmacologic approaches. Not included in this special issue are papers from the counseling professions: clinical psychology, psychiatry, social work, genetics, advance practice nursing and neurology.
While the contributions of the counseling professions are woven in the fabric of the other papers, many readers would benefit from an in-depth exploration of the scope of practice of the counseling professions in HD. The references provide an excellent starting point for any clinician who wants to develop a comprehensive patient/family centered-care program for HD. The website references, while Euro-centric, are an excellent means for maintaining current clinician knowledge.
NOTE: This special issue complements the recent paper on the treatment of chorea, a sign which is often a source of personal embarrassment to the patient and family, although rarely a disabling feature of HD.
Evidence-based guideline: Pharmacologic treatment of chorea in Huntington's disease: Report of the Guideline Development Subcommittee of the American Academy of Neurology, Melissa J. Armstrong and Janis M. Miyasaki, Neurology, published online before print July 18, 2012.
About Carol Moskowitz, APRN-C
Carol Moskowitz is a board certified neuroscience nurse specialist and nurse practitioner, specializing in movement disorders since 1981. As a clinician, she has been the triage nurse and nurse consultant for Columbia University’s Center for Parkinson’s Disease and other Movement Disorders since 1981, established a multi-disciplinary outpatient service for people with little or no insurance, the nurse practitioner in the Columbia Health Sciences HDSA Center of Excellence at the New York State Psychiatric Institute since its inception in 1991 and director of a HD summer camp from 1994-2008.
As an educator, she established a worldwide educational program on movement disorders (WE MOVE), co-chaired a week-long conference which encouraged new HD centers across the USA, started three organizations to bring neuroscience nurses together to learn, network and do research on improving care for people with movement disorders, and established an annual week-long clinical practicum on Huntington’s disease for graduate students in physical therapy, speech pathology, neurology and nursing. As a researcher, she assisted physicians conducting clinical genetics research in the Philippines, Venezuela, Russia, Poland and Albania which contributed to the discovery of the HD gene and the genes for dystonia-1, dystonia-3 and dystonia-5. She coordinates tissue and brain banks for basic neuroscientists. She has published on standards of care for these disorders and written a musical about HD study coordinators, transgenic mice and the basic scientists.