If you knew that you would develop a dehabilitating, eventually fatal illness, would you choose to know? This is the question that current technology is bringing to the minds of many people who may have inherited a cruel and deadly disease. Some choose knowing, but with the result can come a high price. Knowing the future can sometimes be a scary option. Huntington's disease brings disability and eventually a cruel death.
Huntington's disease consists of the degeneration of brain cells called neurons. The disease typically strikes in middle age and ravages the mind for decades before death. HD is a familial disease that is passed from parent to child in most cases. There is a 50-50 chance that if one parent has Huntington's, they will pass the gene on to their child. If the child does not carry the mutated gene, there is no chance of passing it on to any future offspring.
With current genetic testing available, more and more young people face the dilemma of whether they would like to know what lies in the future for them. The gene sits on the short arm of everyone's fourth chromosone where the letters of the genetic alphabet normally repeat. C-A-G as many as 35 times in a row. In people who who develop Huntington's however there are moe than 35 repeats. No one knows why this is this DNA hiccup causes cell death in the brain, leading Huntington's patients to jerk and twitch uncontrollably and rendering them progressiviley unable to walk, talk, think and swallow. The more repeats in the abnormality, the sooner the symptoms appear and the faster they progress.
One young woman, age 23, had to deal with the results of the test that she had done. After much agonizing about whether she should test or not she felt that she should be tested. Was she ready to handle the results, if the results came back positive? She felt she was and received a positive result. She would develop HD. She had watched her grandfather deal with the incurable disorder. She had pleaded for months with a clinic in Manhattan to do her testing. They were worried that it would be too much for the young woman to deal with, if she found that she carried the defect. A counselor helped her with the devastating results. After months of turmoil the girl, realized that marriage or bearing children would not be in her future. She made the decision to devote herself to fund-raising, in the hopes that someone would find a cure. She claims she has never regretted being tested.
Scientists have become aware of increasingly younger numbers of young people seeking to be tested for Huntington's. No one collects much data about who seeks testing and their ages. At Huntington's Disease Center at Columbia, Michael Hayden a professor of human genetics says, "Younger people are coming in for testing now, people in their 20s and 30s. Before this shift such testing was quite rare. They feel it is part of their heritage and feel it is possible to lead a life that will not be defined by this gene."
Symptoms of Huntington's can be, mood swings, depression, trouble driving and leaning new things. They also can include remembering facts and trouble making decisions. Tremors and lack of muscle control follow.There are similar symptoms with many other disorders.
In 1 to 3 precent of people with HD no family history can be found. There is no current cure for Huntington's at this time, though some symptoms can be helped with medication.
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