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askthedoctor Huntington’s I


To Learn More The Huntington’s Dis- ease Society of America (HDSA) maintains local chapters and support groups. For more infor- mation on Huntington’s disease, visit www.hdsa .org, email hdsainfo@ hdsa.org, or call (888) HDSA-506 (437-2506).


50 MILITARY OFFICER MARCH 2014


A genetic disease that usually appears in middle age and progresses slowly, Huntington’s disease can be diffi cult to recognize. By Rear Adm. Joyce Johnson, D.O.


In 1872, Dr. George Huntington, a New York physician, identified a syn- drome he called hereditary chorea. (Chorea is an involuntary movement dis- order, often described as “writhing.”) The syndrome later was given the names Hun- tington’s chorea or Huntington’s disease. It aff ects about 1 in 10,000 people; 30,000 people currently show symptoms, and per- haps 200,000 might have the gene and later develop symptoms. Most people with Huntington’s will not


experience any symptoms until midlife. (A child/adolescent form aff ects less than 10 percent of Huntington’s patients.) Symp- toms — physical, cognitive, and emotional — usually begin between 30 and 50 years of age and worsen over the next 20 to 30 years. Huntington’s disease often is divided


into three stages as it progresses. The early stage features subtle changes in cognition (such as memory and problem-solving), emotional depression or instability, and in- voluntary movements. Medication can help address the emotional issues. Continued employment becomes more diffi cult as cog- nitive and physical symptoms worsen. In the second, or middle, stage of Hun-


tington’s, the movement disorder becomes more severe. Arms and legs might fl ail about, inhibiting mobility and activities of daily life. Swallowing and speech might be aff ected. Medication sometimes can address the movement disorders, while physical, oc- cupational, and speech therapy can help a patient manage many activities of daily life.


In the third, or late, stage of Hunting-


ton’s, independent living and self-care are no longer possible; full-time care is required. Swallowing impairment results in choking risks. The movement disorder makes it impossible to walk or talk. How- ever, patients still comprehend spoken lan- guage and are aware of family and friends. Death may result from choking or infection. Huntington’s disease raises diffi cult is- sues for those who have it and their family members. Support groups, as well as indi- vidual and family counseling, can be useful. Some of the challenges include the tragic course of Huntington’s, the need for custo- dial care, and end-of-life decisions. A diagnosis of Huntington’s also raises


questions regarding genetic testing. The gene mutation responsible for Huntington’s disease was identifi ed in 1993. A genetic test identifi es people with the gene but can- not predict how severe the disease will be. Huntington’s is autosomal dominant, so if one parent has the disease, each child has a 50-percent chance of inheriting the gene and eventually getting the disease. Will the children or siblings of someone with Hun- tington’s want to know that at some time (perhaps decades) later, they will develop the disease? Huntington’s disease is chal- lenging at many levels.


MO


— Rear Adm. Joyce Johnson, USPHS (Ret), D.O., M.A., is a health care consultant in Chevy Chase, Md. Find more health and wellness resources at www.moaa.org/wellness. For sub- mission information, see page 6.


PHOTO: STEVE BARRETT


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