Exercise recommendations, tailored to each individual's needs, can be made by any member of the treatment team. Exercise in moderation can help control weight and maintain cardiovascular fitness. Certain exercises strengthen less affected muscles that compensate for the more affected muscles, maintain flexibility and reduce contractures. Exercise programs like gentle yoga can be beneficial and adaptable to each individual.
If the hands have significant weakness and/or joint deformity, an occupational therapist can suggest alternative ways to perform daily activities to promote joint and energy conservation, and provide assistive devices, such as buttonhooks, key turners and jar openers.
A caution regarding treatments: People with CMT should be informed vincristine (a chemotherapy agent known to be harmful) and other neurotoxic medications may worsen symptoms.5 Vincristine can cause profound peripheral neuropathy symptoms particularly in those with demyelinating types of CMT including those who are asymptomatic. A comprehensive list of these medications is available at www.cmtausa.org/med_alert.php.
Although there is limited direct evidence these medications exacerbate CMT, it is important to be aware of this potential.
The Nurse's Role
Assuring patients receive appropriate care is fundamental; preparing patients to face the lifelong challenge of coping with CMT is equally important. With extensive patient contact, nurses are ideally positioned to help coordinate the care of CMT patients, enable them to develop a healthy attitude and provide them with information needed to manage their condition over the long term.
Education is primary and often begins prior to the patient's first clinic visit. First contact can include answering questions about CMT. Sending a pamphlet about CMT prior to the first visit is helpful. Encourage the patient to keep a running written list of questions to discuss to maximize every visit.
Other educational needs include basic CMT information, its inheritance, community resources, current research as well as specific information on foot care, exercise and pain management. Schedule time during each visit to address individual education issues. A one-page handout that includes information on organizations, support groups and community resources is beneficial.
After diagnosis, CMT patients are frequently overwhelmed. Nurses can offer educational materials for home review and reference. Excellent low-cost or free materials are available from The National Institute of Neurological Disorders and Stroke (www.ninds.nih.gov), the Muscular Dystrophy Association (MDA, www.mda.org), the CMTA (www.cmtausa.org), or the Hereditary Neuropathy Foundation (HNF, www.hnf-cure.org).
Many are unaware CMT is under the MDA umbrella of diseases/disorders. By registering with the local MDA office, a person with CMT may receive services, including reimbursement for cost of leg braces not covered by third party payers.
The Promise of STAR
Research in genetics has increased optimism among those seeking to treat and cure CMT. "We now are able to replicate this disorder in the laboratory and. can begin testing new treatments that will ultimately lead to clinical trials in people," according to Michael E. Shy, MD, chairman of the CMTA Medical Advisory board, director of the CMT Clinic and co-director of the Neuromuscular Program at Wayne State University, Detroit.
Scientific and technological advances have enabled the CMTA to implement an initiative, the Strategy to Accelerate Research (STAR), designed to lead to new treatments for CMT within 3-5 years and possibly to a cure within 10 years.
Spearheaded by an international collaboration of researchers, the STAR initiative will initially focus on CMT, type 1A.
1. Gondim, F., de Oliveria, G., & Thomas, F. (2007). Hereditary neuropathies of the Charcot-Marie-Tooth disease type. Retrieved Nov. 21, 2008 from the World Wide Web: http://emedicine.medscape.com/article/1173484-overview
2. Hashmat, A., Daud, Z., & Brannagan, T. (2007). Charcot-Marie-Tooth and other hereditary motor and sensory neuropathies. Retrieved Nov. 21, 2008 from the World Wide Web: http://emedicine.medscape.com/article/1173104-overview
3. National Institute of Neurological Disorders and Stroke. (2007). Charcot-Marie-Tooth disease fact sheet. Retrieved Jan. 21, 2009 from the World Wide Web: http://www.ninds.nih.gov/disorders/charcot_marie_tooth/detail_charcot_marie_tooth.htm
4. Kedlaya, D. (2005). Charcot-Marie-Tooth disease. Retrieved Jan. 21, 2009 from the World Wide Web: http://emedicine.medscape.com/article/1232386-overview
5. Weimer, L., & Podwall, D. (2006). Medication-induced exacerbation of neuropathy in Charcot-Marie-Tooth disease. Journal of Neurological Sciences, 242(1-2), 47-54.
Janet Goodard is a nurse at the John P. Murtha Neuroscience & Pain Institute, Johnstown, PA. Dana Schwertfeger, director of member services at the Charcot-Marie-Tooth Association, contributed to this article.