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St. Louis Chapter of the
Huntington's Disease Society of
America 314-647-4372
Huntington's Disease Society of
America or by participating in some
of the walks or fundraising efforts
put forth by HDSA.
stepson was also diagnosed at age 22 with early onset. Not many people know about this devasting illness and
I thought I would put this on my site to make more people aware. I was inspired by a local couple who were
recently on our local news, Channel 5 News in St. Louis, who came forward with a "Breaking The Silence"
segment about Huntington's Disease.
There is no cure for Huntington's Disease and it is a brain disease which consists of progressive mental and
physical deterioration. For more information about the disease and to learn how you can help, go to
www.hdsa.org and roll down to to read Information about Huntington's Disease.
It is a very difficult family situation to live through and I just feel the more people that know about it, the better I
and my family can cope with it.
breaking the silence.
Some of you might know Huntington's as a disease that affects one of the characters on the popular TV show "House." The Turner family of St. Charles knows it as the devastating
brain disease that's affected several members of their family for generations.
"I'd never heard of it before," said Kathie Turner.
Katie and her husband, Pat, said Huntington's can be clearly traced through the Turner family tree. Pat's grandmother, father, and aunts had it.
"It's kind of a hush-hush, you know; people really don't say too much about it," Pat said. "They called it the 'shaking disease' back then, you know."
In 2003, his brother, John, died from this cruel genetic disorder that causes progressive mental and physical deterioration.
Symptoms typically first appear in the 30s and 40s. The early symptoms are lack of coordination and an unsteady gait. They progress to a decline in mental abilities and sometimes
behavioral disorders.
A year after John's death, the Turner family started the John Turner Memorial Walk to raise awareness and money for research.
The story does not end there, unfortunately. In 2005, Kathie began noticing similar changes in Pat.
"My movements in my hands and my feet and legs and that," Pat said.
Kathie hesitated at first to talk with Pat about it. When she did, he decided to do nothing.
"I didn't want to get tested," Pat said, "because I didn't want my kids to know and that, and then they'd have to watch me go through all of that too."
But in September 2005, Pat received a positive diagnosis. He also began testing experimental drugs to help find a treatment. He and Kathie had to sell their jewelry business, but the
hardest part has been talking with their son and daughter. Children born to those with Huntington's have a 50 percent chance of inheriting it themselves.
"I'm afraid for my kids now," Pat said. "I pray for them all the time that they don't have the gene."
Pat said that's his biggest fear and one of the biggest motivations for him to break the silence about Huntington's disease.
"I just made up my mind I'm going to go with this, and do the best I can with it," he said.
The John Turner Memorial Walk is set for Saturday, April 25 at 9:30 a.m. in Tilles Park, at McKnight and Litzsinger roads in Ladue.
Call the St. Louis Chapter of the Huntington's Disease Society of America at 314-647-4372 for more information about the walk and the disease. Or visit the web link on this page.
KSDK
from brain cells in the caudate nucleus destroying them. It takes several years before symptoms begin to show and can have devastating
effects both on the pHD (person with Huntington's disease) and on their families and friends, co-workers and communities.
The Huntington's disease gene was identified in 1993 and is located near the top of the fourth chromosome. Since one chromosome from
each parent is passed on to a child, the chance is random that either the healthy or the HD gene is inherited by the child. The only way to
get this disease is through birth. It is a dominant gene, meaning that each child of a Huntington parent has a 50/50 chance of inheriting the
gene. That means that all, none or some of your children could inherit HD if it is in the family. However, because of lack of knowledge and
the stigma attached to the people with the disease in the past, many families hid the pHD or blamed it on mental illness, alcoholism or
dementia. Many other families adopted out children or marriages broke up resulting in the family chain being broken and along with it, the
history of their illnesses.
Huntington's disease can be identified with a DNA test but testing procedures are stringent, and most centers require a consultation with a
neurologist, geneticist and psychiatrist so that the person will have as much support as possible while going through the testing period
and be counseled for a positive or negative result.
What does the disease do to a person?
There are 3 main areas that are affected by Huntington's Disease:
EMOTIONAL:
The early stages of the disease are hard to diagnose or to recognize as the emotional changes can be subtle at first and slow progressing
so that it may take years of depression, anxiety and sleeplessness before a person with Huntington's disease (pHD) - starts to show
physical signs.
Many pHDs do not know what is wrong and will chalk it up to stress, lack of sleep or depression. Families usually agree and indeed these
are the very first symptoms of HD and serve also to make the disease worse. Some pHDs will experience severe rage or inappropriate
behavior in situations, paranoia and on rare occasions, hallucinations.
Nervousness and anxiety are common during the early stages of Huntington's. Depression is the main cause of emotional problems in
pHD's and can easily be treated. Left un-treated, the pHD gets worse and can show signs of paranoia, withdrawal from society and
inappropriate social behavior.
Many of these emotional symptoms create other problems and a circle of emotional grief. Lack of self-esteem, the sense of loss of one's
control over one's life, and becoming more and more dependant on others serves to rid the person of their sense of pride and can cause a
depressive state in itself.
It is at this time of the pHD's life where difficulties on the job occur, difficulties remembering names, numbers, sequences, directions, etc.
can cause lower performance on the job and often result in loss of jobs or demotions.
As the disease progress, the pHD finds it increasingly challenging to follow instructions, to be organized, plan ahead and keep track of
themselves. Their sense of direction may also be affected. Short term memory and instant recall become very difficult and the ability to
complete thoughts into actions is slower.
These emotional symptoms of Huntington's may occur years before any physical symptoms and usually get quite severe before the
person seeks medical help. Often anti-depressants are prescribed which help.
PHYSICAL
Some problems with coordination may start to show and even involuntary movements, falling, clumsiness, shrugging, facial tics, and neck
problems are common. This "chorea" or dance-like movement is constant and the pHD has little to no control over it.
Originally known as St. Vitus' Dance, the movements are regular and repetitive in the person but each person's movements may differ from
someone else with HD. Because the brain is not sending the signals to the muscles properly, frequent falling is a danger to a pHD. This
staggering 'gait' along with slurry speech is often mistaken for drunkenness.
Some pHDs become rigid and cannot move their muscles. Speech and swallowing become difficult and choking is a real problem. Speech
therapists can help somewhat at this stage. Not all pHDs manifest the same symptoms at the same stages of the disease as there are
many variables but they do share many symptoms in common. If the person with HD lives long enough, he or she will get dementia and
rigidity. This usually takes 15-20 years from the onset of the physical symptoms.
Although Huntington's Disease usually does not appear until the mid-thirties to mid-forties and later, there are about 1% who contract the
disease in their teens or younger. This is known as juvenile Huntington's disease.
COGNITIVE
Some of the more devastating cognitive symptoms have already been mentioned. For example, instant or short-term recall, decreased
ability to organize thoughts, words or actions, organizational or multi-level skills. These include following directions or giving directions,
sometimes a loss of sense of direction or getting confused easily and not being able to snap out of it.
pHDs suffer from loss of the ability to figure out complex situations, often not being able to think where there are minor distractions. Simple
tasks such as bathing or brushing teeth, or getting dressed become stressful and a 'big deal' because of the many steps involved and the
step by step process which has to be thought out one step at a time.
Planning ahead or having plans changed is extremely difficult for persons with HD, as it requires more abstract concepts than say, making
a sandwich. pHDs often have to have things set out for them and a list to follow.
The most important thing to remember with this disease is not to confuse it with Alzheimer's, Multiple Sclerosis, ALS (Lou Gehrig's
disease) or Parkinson's Disease even though all of the symptoms of the above diseases do occur. This is because it is the central brain
that is malfunctioning and causing all systems and organs and abilities to malfunction.
A person with Huntington's disease is completely aware of what is happening to their body and mind right up until the end - they can hear,
understand and feel even though they may not be able to communicate or move their muscles.
If the devil, himself could create a disease, Huntington's disease would be it.