What Is Huntington's Disease

Huntington’s Disease
Haley Hamilton
January 4th, 2017
Honors Biology 3&4

The World in the eyes of everyday people do not see what awful things that are taking place in those around them. Huntington’s disease is a fatal illness in which those who develop this have no chance of survival. The disease deteriorates one's ability to function properly and their way of living. Not only does the disease harm the sufferer but it also affects the people around them as well, too. For, these people are the ones who will be needing to take care of the person, when they can no longer care for themselves. Huntington’s can take a lot out of people and it is important that together hope, courage, and strength can be found amongst those fighting
their everyday lives with this pitiful illness.
The central core or major components of the nervous system include the brain and spinal cord. These are protected within bony encasings known as the skull and the spinal column. From these main parts of the system, nerves branch out forming fiber linking systems for the information that is coming in from the environment and going out to the muscles, glands,and organs. Every cell in the human body is in some way affected by the nervous system. Hormones and other chemicals produced in the body heavily affect the ways in which the nervous system acts.The nervous system controls one's respiration, heart rate, body temperature, it controls one's movements, and even helps guide digestion(Evans-Martin, pg. 10 ). Neurons are the cells in which the nervous system receives and reacts to the needs or concerns for one's body to function properly. An 100 billion neurons and 1 billion neurons are estimated by a wide range of scientists(Evans-Martin, pg. 12). Neurons compare to other cells in the human body while they also differ from the other cell in the human body. Some similarities include a cell membrane, cytoplasm, nucleus, and organelles. Differing from the typical cell neurons do not divide to reproduce themselves. Neurons unlike others cells convey and electrochemical signal. The shape of neurons, also differs greatly, as they have extensions coming off of them. These make sending and receiving messages from other parts of the body possible. (Page 14)It is commonly known that each neuron is expected to have one axon that is used to transport messages out of the neuron. On the opposite side of the neuron cell extends dendrites that branch off in tree type patterns receiving the messages sent from other axons(Evans-Martin, pg. 15). The site in which the two (dendrite and axon) join is known as an axon hillock. The tiny space where these two join is called the synapse. This is where the neurotransmitters pass through the presynaptic nerve ending and connects to the postsynaptic neuron(Evans-Martin, pg. 21). This process allows the body to perform properly, safely and in a healthy manner. The deterioration of neurons and the wasting away of the cerebral cortex is what is commonly described as Huntington’s Disease.
Most damage takes place in the motor control regions of teh brain, and other areas making it difficult to functioning over years of damage(Huntington’s 2016).These things that are being damaged play an important role in simple movements and involuntary actions(Evans-Martin, pg.
139).
Huntington’s Disease is a progressive autosomal dominant neurodegenerative disorder.
This disorder is caused by a polyglutamine- repeat expansion in the huntingtin protein(Donley, 2016). An American Physician George Sumner Huntington who lived from 1850 to 1916 wrote an article in an 1872 paper titled “On Chorea”. George studied and practiced medicine on the eastern tip of Long Island, New York. Because of the families in his community who suffered from this disease he was able to draw a clear concise description of the illness. Huntington’s father and grandfather's experience with medicine and heredity gave him a different outlook on how the disease was contracted. It was also said that through witchcraft in Salem, Massachusetts some exhibited involuntary twitches like similar signs to Huntington’s in ancient myths(Marshal, 2014). Huntington’s disease was commonly known as Huntington chorea in past decades. Chorea also known as the uncontrollable movements of one body, like the typical symptoms of Huntington's. Once they found out it was passed on through genetics its name was then called Huntington's disease(Marshal, 2014). With only 4 to 10 cases per 100,000 this disease is classified as a rare disease. In the United States alone, there are more than 30,000 symptomatic patients(Huntington’s, 2016). Those at risk living in the U.S. accumulate to just over 200,000 people(Locate, 2016). Huntington’s typically affects the individual in their middle ages. It is an extremely
fatal disease in which affects the sufferer on a neurological and/or psychiatric level(Andersson, 2016). Although, there are 2 types of Huntington’s Adult-onset and juvenile-onset Huntington’s. The most cases are seen in adult-onset, while juvenile-onset is less likely to be seen(Huntington, 2016). These sufferers develop progressive motor cognitive and psychiatric deterioration the results from the degradation of cortico-striatal-thalamic circuits(Donley, 2016). Damage caused by this illness is almost always seen in the motor control regions of the brain, but it can also appear in other areas of the brain as well. Some symptoms of Huntington’s Disease include; abnormal and unusual movements, behavior changes, Dementia that worsens over time, and several others(Huntington, 2010). Early signs of adult-onset Huntington’s include irritability, depression, small involuntary movements, poor coordination, trouble making decisions, and difficulty learning new information. Those with adult onset are only estimated to live about 15 to 20 years after they begin to show troubling signs and symptoms. Juvenile-onset also involves motor skill difficulties, as well as mental and emotional effects. Some additional signs of the juvenile form of Huntington’s include slow movements, clumsiness, frequent falling, rigidity, slurred speech, and drooling. One may not the child's school performance decline as their ability to think and reason become impaired. Of the children affected with this disease 30% to 50% will experience seizures. Because of a child's tiny weak bodies, their symptoms seem to progress more rapidly that adult-onset, with a 10 to 15 year life expectancy after symptoms arise. In the early stages subtle changes may occur and the effects of the disease may make it difficult for the person to work at their typical functional level(Huntington, 2016). In the in between stage of the disease movements may become harder, and medications may be prescribed to deal with uncontrolled movements and discomfort. Therapy may also help with taking control of one's voluntary actions some specific therapies that may help include occupational, and physical therapy. Help from from a speech language pathologist may be needed as speech impairment is likely. In the latest stage of Huntington’s, total care from others is needed as breathing may become difficult and uncontrolled involuntary movements may become severe or may go away all together. Throughout all stages of this disease, the person's nutrition and diet must be maintained in order to keep the person's symptoms a little more under control(Locate, 2016). As symptoms of the disease worsen, its victims eventually die of respiratory failure or complications related to the neurodegenerative progression of the disease(Marshal, 2014).
Huntington’s disease is contracted in an autosomal dominant gene pattern. This is when the disease appears when at least one parent passes on the gene. With that in mind it is know that if the parents does indeed carry the gene, or is a sufferer, their child has at least a 50% chance of contracting the disease(Andersson, 2016). In rare circumstances neither parent of the sufferer is a sufferer themselves. As the HTT gene is passed on generation to generation, the CAG trinucleotide repeat often is shown to increase in size

Resources
Andersson, P. L., Peters-n, -., Graff, C., & Edberg, A. (2016). Ethical aspects of a predictive test for Huntington’s Disease. Nursing Ethics, 23(5), 565-575. doi:10.1177/0969733015576356 Donley, D. W., Olson, A. R., Raisbeck, M. F., Fox, J. H., & Gigley, J. P. (2016). Huntingtons
Disease Mice Infected with Toxoplasma gondii Demonstrate Early Kynurenine Pathway
Activation, Altered CD8+ T-Cell Responses, and Premature Mortality. Plos ONE, 11(9),
1-16. doi:10.1371/journal.pone.0162404 Evans-Martin, F. F. (2005). The nervous system. Philadelphia: Chelsea House.
Huntington Disease. (2010). In S. J. Judd (Ed.), Health Reference Series. Genetic Disorders
Sourcebook (4th ed., pp. 195-198). Detroit: Omnigraphics. Retrieved from http://go.galegroup.com Huntington disease - Genetics Home Reference. (2016). Retrieved January 02, 2017, from https://ghr.nlm.nih.gov/condition/huntington-disease Huntington's Disease Information Page. (2016). Retrieved January 02, 2017, from https://www.ninds.nih.gov/Disorders/All-Disorders/Huntingtons-Disease-Information-Pa ge
Locate Resources. (2016). Retrieved January 01, 2017, from http://hdsa.org/about-hdsa/locate-resources Marshal, L. (2014). Huntington Disease. In K. L. Lerner & B. W. Lerner (Eds.), The Gale
Encyclopedia of Science (5th ed., Vol. 4, pp. 2241-2244). Farmington Hills, MI: Gale.
Retrieved from http://go.galegroup.com