Huntington’s disease

Huntington’s disease is known to be fatal disease which causes chorea (involuntary movements), language and speech dysfunctions and even dementia (cognitive decline). It is necessary to admit that Huntington’s disease or HD is considered to be inherited genetically caused by mutation passing down from generation to generation.  HD is characterized by psychiatric and neurobiological features meaning that disease is able to affect certain structures such as, for example, brain and basal ganglia which is responsible for performing important body functions – speech function, coordination and movement functions. (Smith 2003)  HD affects not only speech functions, but also perception, memory and thought because the basal ganglia are linked to frontal lobes. It may result in uncontrolled movements, speech problems and difficulties, loss of intellectual abilities, behavioral and emotional misbalances. (Walker 2007)

HD’s symptoms are varying from patient to patient. However, it is suggested that the earlier the symptoms appear, the faster the illness progresses. Disease symptoms cause personality changing, for example, changes in mood – depressions, apathy, irritability, aggression and anger. Cognitive decline is cause by problems with communication abilities, learning difficulties, difficulties with answering questions, judgment impairment, and difficulties in making decisions. With the progression of disease, the patient fell more difficulties with intellectual tasks and communication processes. (Walker 2007)

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Because of HD, person’ brain becomes depleted and it leads to disabilities in motor control and movements, cognition and thinking and, finally, speech and swallowing problems. HD affects speech and language abilities because the centers of cognitive or motor control become affected and it may result in weakness of human muscles, chorea, discoordination, memory problems, etc. It is known that language and speech function are under control of the left side of human brain, whereas swallowing function is controlled at the base of the brain. HD starts from affecting caudate nucleus and putamen located in the brain core and in such a way causing speech and language problems with disease progression.

Physicians and scientists state that communication problems are different in nature and their severity varies from person to person. Nevertheless, the commonalities are present, though no two people with HD exactly alike symptoms. The variability continues with disease progression. In different stages of HD progression people may suffer from number of speech and language problems. Generally, people are suffering from the same areas of difficulties during disease progression. (Smith 2003)

Firstly, HD may cause dysarthria meaning person’s speech problems are caused by muscular weakness, improper coordination of lips and muscle’s slowness. One more problem is apraxia i.e. disruption on sequencing and programming of muscle movements necessary for speech. Furthermore, it becomes more difficult for a person to find necessary words because of memory decline. Communication process is also affected by loss of coordination of voice and breathing and very often a person is even unable to utter words because of poor voice quality meaning that person’ volume is either too high or too low. Person may have difficulties not only in perceiving and understanding pf information, but also in writing and reading. Other problems are:

§  Improper articulation meaning that the person pronounce words and sounds incorrectly;

§  Lack of initiation – person experiencing speech problems finds it difficult to start communication;

§  Shorter length of utterance when a person is bale to respond only with no more than a couple of words;

§  Changed rate of control meaning that a person speaks either too fast or to slow;

§  Difficulties leading to repeating the same words and phrases and inabilities to proceed (perseveration);

§  Stuttering when it is difficult to start a word or sentence repeating the same sounds;

§  Echolalia meaning that person repeats what he has just said;

§  Reduced abilities to highlight topic appropriately and to maintain it;

§  Difficulties monitoring pragmatic skills. (Bates et al. 2002)

It goes without saying that speech-language pathologist will help to cope with appearing communication difficulties as well as to work out strategies how to compensate for some of the problems which may appear in future. With disease progression the role of the pathologists is to maintain and to preserve possibly the highest level of communication abilities. It is better to ask professionals for involvement and intervention, because patients suffering from HD will feel more comfortable during the early stages of disease progression. Actually, it is recommended to evaluate person’ ability “to use augmentative or alternative communication devices and techniques, which can be as simple as an electronic device that speaks for the person”. It is known that HD is incurable, though certain techniques are present to maintain patient’s health and to find better methods of communication. It is possible to turn to alternative and augmentative communication.

Despite availability of alternative communication methods, people with HD tend to refer to verbal communication as long as possible, though very often their speech becomes hardly understandable. Family members and doctors should encourage speakers to say one word at a time; to speak slower; to repeat word if necessary; to exaggerate the sounds; to rephrase the sentence or phrase; to take a deep breath before speaking in order to speak louder; to describe what he is trying to say; to use gestures and to make sentences shorter; to indicate the first letter of the words, etc. (Hope Through Research 2003)