Huntington’s disease (HD) is now considered one of the most common hereditary brain disorders. The disease progresses slowly over a 10- to 20-year period and eventually robs the affected individual of the ability to walk, talk, think, and reason. Huntington’s disease usually appears between the ages of 30 and 50. It affects both the basal ganglia, which controls coordination, and the brain cortex, which serves as the center for thought, perception, and memory.
The most recognizable symptoms include involuntary jerking movements of the limbs, torso, and facial muscles. These are often accompanied by mood swings, depression, irritability, slurred speech, and clumsiness. As the disease progresses, common symptoms include difficulty swallowing, unsteady gait, loss of balance, impaired reasoning, and memory problems. Eventually, the individual becomes totally dependent on others for care, with death often due to pneumonia, heart failure, or another complication.
Diagnosis consists of a detailed clinical examination and family history. Brain scans may be helpful. The identification in 1993 of the gene that causes Huntington’s has simplified genetic testing, which can be used to help confirm a diagnosis. Researchers and genetic counselors, however, have established specific protocols for predictive genetic testing to ensure that the psychological and social consequences of a positive or negative result are understood. Predictive testing is available only for adults though children under the age of 18 may be tested to confirm a diagnosis of juvenile-onset Huntington’s disease. Prenatal testing may be performed. The ethical issues of testing must be considered, and the individual must be adequately informed, because there is no effective treatment or cure, although medications are available to help control some of the symptoms.
Cell and animal models can replicate many features of the disease and are now being used to test new theories and therapies. Although no effective treatments for slowing disease progression currently exist, clinical and observational trials are being conducted. Any of these may yield an effective treatment that would slow the progression or delay onset of the disease while researchers continue working toward a cure. — BrainFacts.org
All proceeds from my recordings benefit the good work of the Huntington’s Disease Program at the University of Connecticut Health Center. They are doing stem cell research to try to find treatments and help patients at the clinic. All eligible patients are treated free of charge. I get a tremendous amount of support there.
Huntington’s Disease is the hereditary disease that took the life of Woody Guthrie. It is a brain condition that causes problems with emotional control, memory, and involuntary movement, and eventually leads to dementia and death. There is no cure and the only treatments are an effort to manage symptoms. In the past, many HD patients went undiagnosed or misdiagnosed. Kathy’s mom was one such case.
More songs are on the way, but the tunes that are finished now are on my Bandcamp Page. I’ve never been great at figuring out how much my music is worth. So I made it all free. Well, for the time being it is called Name Your Price. That means if you like a song or a whole album; and free is all you’ve got, free it is. If you feel generous, or you really, really enjoy my work then any funds or tips will go to UConn. To date, you and I have raised over $600.00 for the fund and I hope to raise much more. Eventually, with your help, I’ll try to release the songs as an actual compact disc.
Thanks for listening and for all your support.