According to a story from abc.net.au, Cyril Wise watched in horror as his wife Kaye died of Huntington’s disease in 2014. However, before his wife’s death, Cyril was struck with the realization that he would have to endure this tragedy all over again when his daughter Bec was diagnosed with the illness also. Getting good care for Bec, who has had the disease for seven years, has been a serious challenge.
About Huntington’s Disease
Huntington’s disease is a heritable disorder that causes brain cells to die. This is a long term, progressive, and ultimately lethal disease that causes severe debilitation over time. The disease is caused by a genetic mutation that affects the HTT gene. It normally appears between 30 and 50 years, but in rare cases is can occur before age 20. Symptoms of Huntington’s may first appear as subtle mood and behavioral changes and loss of coordination. Other symptoms include random movements called chorea, abnormal posture, sleep issues, trouble chewing, swallowing, and speaking, dementia, anxiety, depression, and impulsivity. Nine percent of deaths are the result of suicide. Treatment for Huntington’s disease is symptomatic, with no cure or disease altering therapies available. Most patients die around 15 to 20 years after their diagnosis. To learn more about Huntington’s disease, click here.
Inadequate Treatment Access
In an effort to bring awareness to this problem, Cyril and Bec, who now can barely speak, are inviting Australian MPs to come to Tasmania, the island where they live, to meet with Bec and other patients so that they can understand the extent of the problem for themselves. Tasmania has one of the highest rates of Huntington’s disease in the world. Cyril and Bec are far from the only people on the island who are desperately seeking the specialist care that they need. The prevalence of the disease can be linked to a woman named Mary who first settled on Tasmania in 1842. All people on the island with the disease are almost certainly her descendants.
Too Little Too Late
A temporary Huntington’s clinic is operated on the island three times a year, but the clinic is simply too small to see all of the patients on the island, who also need more steady monitoring and care. The recruitment of new specialists and neurologists is under way, but it is unclear when they will begin work.
As a whole, the medical system of Tasmania is not sufficiently equipped to meet the medical demands of the population, and the lack of Huntington’s disease caregivers is reflective a broader, systemic problem.