Spotlight on Huntington’s Disease Awareness Month
Many diseases have tragic consequences, but Huntington’s disease has another side to it that is frightening. Because it is inherited, children of people with Huntington’s have a 50% chance of inheriting it.
Many symptoms of Huntington’s disease don’t appear until later in life, so many people who have inherited it won’t find out until they are already parents. Not only do their children have to watch a parent develop symptoms, but they also have to wonder if they will display those symptoms too.
Genetic testing is available for the disease, but it can be a difficult choice to determine if you really want to know: if you will get it, you will know, but you will not be able to do anything to prevent the onset of symptoms. Testing is only done for people eighteen years and older, because the results can be so life changing.
History of Huntington’s Chorea (aka: Huntington’s Disease)
Huntington’s disease was first called Huntington’s Chorea in the late 1800s, by a young doctor, only 22 years old, named George Huntington. Dr. Huntington saw the effects of the disease among a certain township on Long Island. Born into a family of physicians, Huntington first encountered the disease when he was a boy and was making doctor’s visits with his father. He and his father were on a road and observed two women in a “dancing mania.” The women were exhibiting signs of involuntary movements: twitching and grimacing, and Huntington was amazed and afraid. It led him to study the people in the town and write an abstract about the disease. In 1993, the gene causing the Huntington’s disease was identified, which led to the diagnostic test that is now available.
Care of Huntington’s Patients
Huntington’s is characterized by jerky, involuntary movements which develops into falling, swallowing difficulties and a type of dementia. Dementia can be expressed as personality changes, some people become short-tempered and emotionally unstable and in the latest stage. People with Huntington’s may not be able to walk or talk, and may require nursing home or hospice care. They usually pass away due to heart failure, malnutrition (because they cannot swallow food), or pneumonia. A person may live for ten years or so with the disease.
In cases where the family is estranged, especially in divorces or through long distances where one person removes themselves from interaction with their family, other family members may not know that their relative actually has Huntington’s. Families need to know the possibilities of inheriting it, since the decision to test for it is very important. The results of the test are life changing and affect every major decision a person could make about career, marriage, having children, and other life choices.
Huntington’s Disease in Pop Culture
Huntington’s disease awareness becomes more widespread as the disease is featured in popular culture.
One of the many fictional characters who has brought awareness to Huntington’s disease patients and includes “Thirteen” in the television series, House. The medical show featured actress Olivia Wilde as a resident, who Dr. House nicknames “Thirteen.” Wilde’s character had a mother who died of Huntington’s, so initially, she doesn’t want to know if she carries the gene.
Dr. House has Thirteen tested in secret and then substituted caffeinated coffee for her usual decaffeinated, and she develops some shakiness. Based on the shakiness, she capitulates and decides to find out whether she indeed carries the gene. It is revealed as part of the show’s plot that her brother also had Huntington’s disease, and Thirteen made a promise to him to assist his suicide. When the character finds out her diagnosis, she has some difficult life choices to face.
Huntington’s disease has also played a part in the award winning series, Breaking Bad. The main character, Walter White, shares with his son the story of his father’s diagnosis of Huntington’s chorea. Walt talks about how his father wasted away, and it still haunts him. The viewer wonders if Walt inherited the gene, especially because he has some odd behaviors, which could be characteristic of the disease.
A Cure for Huntington’s Disease?
Clinical drug trials are ongoing to find a way to neutralize the affected gene. A new pharmaceutical, which is injected into the lower spinal cord and is directly absorbed into the nervous system, is being tested in a joint venture between the drug companies Roche and Isis Pharmaceuticals. If it is proven to be safe, the possibility to an end of Huntington’s is on the horizon.
Do you have a story about someone with Huntington’s disease? Tell us! Leave a comment below or share your stories on Everlasting Footprint.