Huntington's Disease


Why Communication and Swallowing Symptoms Arise

As brain cells become depleted in Huntington' s Disease (HD), problems may develop in the following three areas: motor control (movement); cognition (thinking); and behavior. Speech and swallowing problems arise when the centers of motor or cognitive control are affected that cause muscle weakness or discoordination, chorea, and problems with memory, sequencing, new learning ability, reasoning, and problem solving.

Typically, speech and language functions are primarily controlled in the left side of the brain and swallowing function is controlled in the brainstem (at the base of the brain). Cognitive function is believed to be controlled in the right side of the brain. HD typically begins in the caudate nucleus and putamen, which are located in the central part of the brain (core), and spreads to these other control centers, causing communication and swallowing problems as the disease progresses.

Communication

Problems with communication and cognition vary in nature and severity from person to person. Although there are commonalties, no two people with HD are exactly alike, and as the disease progresses, the variability continues. The following list summarizes problems that people with HD may experience at different stages of the disease. In many cases, a person with HD will experience the same areas of difficulty throughout the course of the disease, with severity varying from stage to stage.

Communication problems

  • Dysarthria (muscular weakness, slowness, or incoordination of the lips, tongue, throat, and jaw)
  • Apraxia (disruption in programming and sequencing muscle movements for speech)
  • Diminished rate of control (talking too fast or too slowly)
  • Poor voice quality (hoarse/harsh, breathy, volume too low or too high)
  • Discoordination of breathing and voice
  • Word finding difficulties
  • Short length of utterance (person only responds with one or two words)
  • Misarticulations (incorrect pronunciation of sounds)
  • Lack of initiation (inability to initiate conversation)
  • Perseveration (person gets "stuck" on certain words or phrases, repeating them often and at inappropriate times).
  • Echolalia (person keeps repeating back what you've just said)
  • Difficulty with monitoring pragmatic skills (turn-taking in conversation; reduced ability to maintain a topic or to switch topics
        appropriately
  • Inability to speak
  • Stuttering (difficulty beginning a word or sentence, with repetition of sounds)
  • Difficulty understanding information
  • Difficulty reading and writing

    Cognitive problems

  • Diminished memory, immediate and short-term (long-term memory usually remains intact)
  • Poor reasoning/judgment
  • Reduced problem-solving ability
  • Difficulty sequencing/organizing ideas
  • Concentration problems/distractibility/short attention span
  • New learning ability diminished
  • Problems with numbers and mathematics computations

    How the speech-language pathologist can help

    The speech-language pathologist (SLP) can be helpful at all stages of the disease. In early stages, he/she can assist with problem solving and developing strategies to help persons with HD compensate for some of the problems they might be experiencing. As the disease progresses, the role of the SLP evolves into helping preserve and maintain the person's highest level of communication and swallowing. Early intervention and involvement with professionals is best because people can learn compensatory strategies more successfully during the early stages of HD and can then apply them throughout the course of the disease.

    The SLP can also evaluate a person's ability to use augmentative or alternative communication devices and techniques, which can be as simple as an electronic device that speaks for the person. After determining a person's level of ability for using such techniques, the SLP begins to focus on personalizing the technique or method of communication.

    For example, the SLP might work with a person with HD and his or her family to create a word/picture board tailored to the person's environment (whether it be a nursing home or private residence) or flexible enough to be carried around. Where an electronic device might be beneficial, the interest and motivation of the person with HD to use it should be evaluated. If there is evident motivation, then the device should be made easily accessible.

    Suggestions for Improving Communication

    Although alternative methods of communication are available, people with HD generally prefer to attempt verbal communication for as long as possible, even if their speech becomes hard to understand. The SLP and family members can often help by encouraging the speaker to:

  • Speak more slowly
  • Say one word at a time
  • Repeat the word or sentence when necessary
  • Rephrase the sentence
  • Exaggerate the sounds
  • Speak louder (taking a deep breath before speaking)
  • Describe what he/she is trying to say if he/she can' t think of the word
  • Indicate the first letter of the word
  • Use gestures
  • Keep sentences short
  • As a supplement to speech, use alternative techniques such as word boards, alphabet boards, picture boards, electronic devices


    Some suggestions for the listener are:

  • Eliminate distractions (TV' s, radio, large groups of people)
  • Keep questions/statements simple
  • Ask one question at a time
  • Use yes/no question format as much as possible
  • Pay attention to gestures and facial expressions/changes
  • If you do not understand what is being said, don't pretend that you do. Ask for clarification, or repeat what you think was said in the
        form of a question, such as, "Did you say& ?"
  • Try to keep to familiar topics
  • Encourage the speaker to use his/her specific compensatory strategies
  • Allow enough time for the person to convey his/her message
  • Most important, be patient with the speaker

    People with HD should seek out an SLP at the point when the areas of memory, thought processing and organization of thought become problematic.

    There are also compensatory strategies for cognitive problems that can be implemented in the home. Some examples are:

    Problem: Poor orientation to time and place

        Strategy:

       

  • Keep a large calendar visible
       
  • Display a large, visible clock
       
  • Post signs on walls stating location

    Problem: Diminished Memory

        Strategy:

       

  • Post a schedule of daily routine
       
  • Post a schedule of daily routine
       
  • Establish routines for all activities, e.g., place keys or glasses in same place daily.
       
  • Label cabinets and drawers
       
  • Keep a memory log book like a diary in which the person with HD can write down and refer back to what he/she has done, with      whom, and when.
       
  • Keep an appointment book for social events, doctor's visits, and other dates and occasions to be remembered.
       
  • Reduced problem-solving ability

    The person with HD should discuss with a family member possible problems that could occur in the home. Solutions or steps to be followed should be anticipated, written down, and kept in an obvious place.

    Follow this set guidelines for problem solving.

    These are just a few examples of how a person experiencing cognitive problems can make life easier at home. An SLP can provide assessment, guidance, and further suggestions on the use of compensatory strategies.

    Swallowing

    Swallowing problems (also known as dysphagia) are common among people with HD. Statistics have repeatedly shown that the number one cause of death among persons with HD is aspiration pneumonia. This can occur when food or liquid enters the airway rather than the esophagus during eating or drinking, and then forms a collection in the lung that can become a pneumonia. The involvement of an SLP can be helpful at all stages of the disease in maintaining the highest possible level of swallowing function. This intervention will help to prolong the quality of life and may lower the risk of death caused by pneumonia. There are many steps that can be taken to preserve safe swallowing for as long as possible. First, let' s look at the problems that people with HD frequently encounter:

    Swallowing Problems Associated with HD

       

  • Impulsivity or difficulty controlling amount of food or liquid intake    
  • Difficulty controlling rate of food or liquid intake    
  • Difficulty chewing or manipulating food    
  • Delayed swallow reflex (doesn' t kick in even when food moves to the back of the throat)    
  • Holding food/liquid in the mouth    
  • Difficulty initiating a swallow at all    
  • Inability to swallow    
  • Incomplete swallows where food or liquid is left in the mouth and/or throat    
  • Lack of coordination between swallowing process and breathing or speaking    
  • The need to swallow repeatedly for each bite/sip    
  • Chorea of the oral or pharyngeal muscles (tongue, lips, throat, esophagus)    
  • Drooling and/or spillage of food or liquid from the mouth

    The SLP can evaluate a person's swallowing function and make recommendations that involve positioning issues, feeding techniques, diet consistency changes, and education of the person with HD, family members, or caregivers. Special testing known as videofluoroscopy (or a modified barium swallow) can be done by a speech-language pathologist and a radiologist to determine if a person is actually aspirating a particular consistency. This test provides an inside view of a person in the act of swallowing food or liquid and can be a useful tool in developing strategies for safe swallowing.

    Warning Signs

    The following signs at mealtime may indicate swallowing problems:

       

  • Coughing    
  • Choking    
  • Gurgly voice quality    
  • Wet sounding breathing    
  • Spillage of food and liquid from the mouth    
  • Frequent throat clearing    
  • Progressively slower rate of food intake    
  • Regurgitation of food after it has been swallowed*    
  • Food or liquid left in the mouth after swallowing    
  • Difficulty manipulating food or liquid in the mouth    
  • Frequent congestion*    
  • Frequent temperatures*    
  • Consistent or significant weight loss*

    Signs marked * could be indicative of a serious, and possibly unrelated, medical condition and should be monitored by a physician. In general, if a person with HD experiences any one or a combination of the above problems, he/she should contact a physician and seek out an SLP for evaluation.

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