Huntington's Disease Interview

Many individuals fortunately haven't had to undergo the painful process of assisting a loved one diagnosed with Huntington's Disease. It is an extremely hard obstacle to overcome, but many people don't have a choice. It significantly effects those involved in that patients life and changes many lives all together.

Kit McFarlane (M.S.W., L.C.S.W.), who works at Baltimore Huntington's Disease Center & HDSA Center of Excellence, was a major provider of useful information through an interview via email. She expressed, "I'm glad young people are learning about Huntington's Disease. It's a very terrible disease, but it varies radically from one person to another." She goes on to forward us onto a Huntington's Disease Forum, giving us primary sources into the lives of individuals with relatives diagnosed with Huntington's Disease.

In this Forum, we are able to conclude the actuality of everyday life for these people. A devastated husband states, "Tania "my love" passed away on 11-28-2010 at the age of 37, from a long, courageous battle against Huntington's Disease." He talks about how even when accompanied by this disease, she never gave up hope and despite a devastating final year and a half of her life, she did not allow Huntington's to consume that spirit. He spoke of how hard it was to finally let her go and how this disease, even though it did not end his life, he felt as though it did. He expressed the limitations she had to live with, such as not being even being able to function properly as to get dressed in the mornings. At the end of his forum post, he states, "Tania will be missed, but never forgotten and now the angel that walked on earth is looking down at us and smiling."

Huntington's Disease Forum: http://www.hdsa.org/living-with-huntingtons/wallrem.html

Successful People Affected by Huntington's Disease

There are many people who were affected by Huntington's disease, however the most famous person who was affected by the genetic disease of Huntington's Disease was Woody Guthrie. Woody Guthrie was a folk singer who was well known during the 1930s and 1940s for his songs about the life of migrant workers and poor city people. He shared his experiences about the Great Depression. Among his best known songs are "This Land Is Your Land" and "So Long, It's Been Good to Know You." Woody Guthrie died of Huntington's disease. When he first exhibited symptoms of the disease, people thought he was drunk. Only later was his behavior understood. His son Arlo Guthrie is also a famous singer. Arlo will not develop Huntington's disease because he did not inherit the gene from his father.

Another famous person who suffered from Huntington's disease is a drummer named Trey Gray. He plays for Brooks and Dunn. Gray claims that playing the drums helps him to cope with the disease. He is 39 years old and is presently also concentrating on creating awareness about the disease and raising funds for Oklahoma Chapter of the Huntington's Disease Society of America.

 

Huntington's Disease Statistics

Prevalance of Huntington's Disease: estimated 3-7 per 100,000 people of European ancestry suffer from Huntington disease

Prevalance Rate: approx 1 in 33,333 or 0.00% or 8,160 people in USA

Incidence Rate: approx 1 in 175,390 or 0.00% or 1,550 people in USA

Incidence extrapolations for USA for Huntington's Disease: 1,550 per year, 129 per month, 29 per week, 4 per day, 0 per hour, 0 per minute, 0 per second. Note: this extrapolation calculation uses the incidence statistic: 26 annual cases in Victoria 1996 (DHS-VIC)

Prevalance of Huntington's Disease: About 30,000 Americans have HD and about 150,000 more are at risk of inheriting the disease from a parent. (Source: Genes and Disease by the National Center for Biotechnology)

 

Fact Source: http://www.rightdiagnosis.com/h/huntingtons_disease/stats.htm

 

This information, taken from rightdiagnosis.com, illiterates how rare Huntington's Disease really is. But, though this disease may only effect a small percentage of people in the world, you could be one of them. The results from this disease are deadly and could result in a loss of a family member or friend. Being aware of the causes and taking the right precautions to point out any symptoms of the disease could lead to quicker diagnosis and treatment.

 

Background information on Huntington's Disease

Background Information on Huntington's Disease

During the 1800's, people who suffered from Huntington's Disease may have been thought to be possessed by spirits or persecuted as witches, and were exiled by society. Some were more accepting, for example, George Huntington openly accommodate those who exhibited symptoms of Huntington's disease. In 1846, Charles, Gorman observed how higher prevalence seemed to occur in localized regions.

 The first thorough description of the disease was by George Huntington in 1872. Examining the combined medical history of several generations of a family exhibiting similar symptoms, he realized their conditions must be linked; he presented his detailed and accurate definition of the disease as his first paper.

Treatments for Huntington's Disease

Treatments

Unfortunately there is no cure for Huntington's Disease. There is no known way to stop the disease from getting worse. The goal of treatment is to slow the symptoms and help the person function for as long as possible. If an individual would go to a doctor to seek help, the doctor would most likely perform a physical exam and may ask about the individual's family history and symptoms. An exam of the nervous system will also be done. The doctor may see signs of: Dementia, abnormal reflexes, abnormal movements, hesitant speech or poor pronunciation and even difficulty walking. Medicines can be prescribed, depending on the symptoms. Dopamine blockers may help reduce abnormal behaviors and movements. Drugs such as diamante and tetrabenazine are used to try to control extra movements. Depression and suicide are common among persons with Huntington disease. It is important for caregivers to monitor for symptoms and seek medical help for the person right away. As the disease progresses, the person will need assistance and supervision, and may eventually need 24-hour care. Keep in kind, not all treatments will produce the same results because each patient may require different treatments. 

For additional information please click on the links below:

http://www.nlm.nih.gov/medlineplus/ency/article/000770.htm

http://www.mayoclinic.org/diseases-conditions/huntingtons-disease/basics/treatment/con-20030685

Symptoms of Huntington's Disease

Symptoms of Huntington's Disease 

The genetic disorder of Huntington's Disease has numerous symptoms, however, probably the most prominent of the symptoms would be involuntary jerking or spastic movements, Involuntary or sustained contractions of muscles, slow uncoordinated movements, difficulty with the production of speech, abnormal eye movements, and even difficulty swallowing. 

The genetic disorder of Huntington's disease doesn't only have physical symptoms. There are also serious cognitive symptoms, the most common symptoms of Huntington's disease would be difficulty organizing and prioritizing tasks, inability to start a task or conversation, the tendency to get stuck on a thought, acting without thinking, can cause difficulty in focusing on tasks for a long time, difficulty in learning new information and much more. 

For more information on these symptoms please visit:

 http://www.mayoclinic.org/diseases-conditions/huntingtons-disease/basics/symptoms/con-20030685 

Nuerological Effects of Huntington's Disease

This diagram depicts the affected areas of the brain in someone with Huntington's Disease:

 

As you can see, a large portion of the brain is greatly effected, causing extreme amounts of damage.

Here is a list of the functions of each part of the brain effected, showing you the detrimental losses an individual with Huntington's Disease undergoes.

 

Primary Motor Cortex - tells us that various regions of the cerebral cortex are involved in planning of the voluntary motor functions in the human body.

Premotor Cortex - to send messages that direct movements of the body to accomplish specific tasks. The premotor cortex has a lateral and medial area.

Primary Somatosensory Cortex -  It comprises essential processing centres, or sensory modalities, such as proprioception, tactilation or mechanoception (touch), thermoception (temperature), and nociception (pain). The sensory receptors cover the skin and epithelia, skeletal muscles, bones and joints, internal organs, and the cardiovascular system.

Wernicke's Area - the region of the brain where spoken language is understood.

Broca's Area - Broca's area is one of the main areas of the cerebral cortex responsible for producing language.

Primary Auditory Cortex - The function of the primary auditory cortex is to process sound. It processes such information as pitch, volume and the location of a sound, and is crucial for understanding language.

Primary Visual Cortex -  the portion of the cerebral cortex of the brain that receives and processes impulses from the optic nerves.

The Effect on Loved Ones

If your looking to understand the basics of Huntington's Disease and the personal effect on loved ones, this is a great watch:
http://www.youtube.com/watch?feature=player_embedded&v=imgn3NuTQMc

What is Huntington's Disease and How is it Caused?

Huntington's Disease is a common inherited disease spread all throughout the World, prevalent in about 1 in 10,000 individuals. Huntington's Disease damages the nerve cells in the brain causing deterioration and gradual loss of function throughout areas of the brain, affecting movement, cognition (perception, awareness, thinking, judgment) and behavior. Both men and women are vulnerable to inheriting the disease, usually developing around adulthood but is present, very rarely, in children. Until recently, scientists new little to nothing about the disease, as they watched it pass from generation to generation. Loved ones suffered as they had to undergo the painful process of watching the disease destroy their ability to feel, think, and move.

Huntington's Disease is cause by genetically programmed degeneration of nerve cells, called neurons, in areas of the brain. Some areas affected are the brain's outer surface, or cortex, which controls thought, perception, and memory. This degeneration causes loss of many important functions and results in uncontrolled movements, loss of intellectual faculties, and emotional disturbance. Huntington's Disease is caused by a defect in a gene on chromosome number 4, known as the Huntington disease gene or HD gene. When a defect occurs in the HD gene, it leads to the body creating an abnormal Huntington protein, disrupting the normal function of certain nerve cells and ultimately leads to their premature death.

Want to know more about symptoms, causes, treatments, and testing of Huntington's Disease?

Visit:
http://www.rightdiagnosis.com/h/huntingtons_disease/intro.htm
http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm