Touching Lives - November 2007
Taking on Huntington's
In this fellowship, Dr Ben Underwood is focusing on ways to delay the onset of Huntington’s disease, a devastating inherited condition.Typically, sufferers die in middle age after enduring a 15-year deterioration in their physical and mental well-being. The first symptoms of Huntington’s disease, which include subtle changes in personality or mood, and occasional jerks or spasms, normally begin around age 40. Sufferers gradually become increasingly disabled, and eventually have to give up work.They lose the ability to walk. Abnormal movements increase and talking becomes difficult. When dementia becomes more advanced, most need full-time care.There is currently no cure, and most people die within 15 to 20 years of first noticing symptoms.
“Particularly cruelly, Huntington’s disease runs in families,” says Dr Underwood.”Many sufferers have seen a parent die of the same disease and have some idea of their own fate. Worse still, they have a 50:50 chance of passing the disease on to their own children. One can only imagine the distress that they endure.”
Researchers identified the faulty gene that causes Huntington’s disease in 1993. Dr Underwood is now trying to identify genes that may also affect how old people are when they first develop symptoms, how quickly the disease progresses and how severe it is.
“My research is in the early stages, with a huge amount of work needed before patients might directly benefit,” explains Dr Underwood. “But ultimately, it may be possible to use a combination of drugs to delay the onset of Huntington’s disease by several decades — possibly even beyond the normal human lifespan.This would effectively provide a cure.”