By Gary E. Eddey, MD
Vice President for Medical Affairs and Chief Medical Officer
Protective devices are, at times, controversial, but they are essential to use for individuals with Lesch-Nyhan disease.
Professionals who are unfamiliar with LND may perceive the use of these protective devices as a form of restraint. But when protective devices are requested by the patient and used to safeguard the patient from him or herself, the outcome is an extraordinary feeling of comfort and safety. Not allowing the patients to use protective devices, in fact, would violate their autonomous rights.
Individuals with LND appear at times to be very high functioning in an intellectual way, although they are limited in some aspects of their cognition. Their self-injurious behavior goes against what they want to do. So, the behaviors they exhibit to parents, caregivers and to themselves can be very difficult to understand. Caregivers need continuing support on how to best cope with and care for those individuals in order to promote the positive aspects of the person. As mentioned previously, treatment of individuals with LND should include use of a behavioral technique commonly referred to as selective ignoring, with redirection of activities.
Second in a series of four articles on Lesch-Nyhan Disease. For information, feel free to call Dr. Eddey at 908 234-0011, ext. 737.