A, B, C, D, G, I, L, M, O, P, R, S, T
Aerobic exercise (also known as cardio) is physical exercise of low to high intensity that depends primarily on the aerobic energy-generating process. Aerobic exercise provides cardiovascular (heart and lung) conditioning. The term aerobic actually means “with oxygen,” which means that breathing controls the amount of oxygen that can make it to the muscles to help them burn fuel and move. Aerobic exercise improves heart and lung function so that more oxygen can make it to the muscles for use as fuel. Benefits of aerobic exercise include: improves cardiovascular conditioning, decreases risk of heart disease, lowers blood pressure, increases HDL or “good” cholesterol, helps to better control blood sugar, assists in weight management and/or weight loss, improves lung function, and decreases resting heart rate.
Physical exercise of low to high intensity that depends primarily on the aerobic energy-generating process.
Aggression is hostile or violent behaviors or attitudes toward another. People with HD may have difficulty controlling their emotions due to injury or loss of brain cells (neurons). For example, a person with HD may over-express a feeling of slight frustration or irritation in the form of a temper tantrum or aggressive behavior. Although the emotion itself is often a legitimate response to something in the person’s environment, they are not able to regulate the proper amount to be expressed. Anger and aggression can be frightening and may become dangerous. Strategies for dealing with this behavior emphasize safety first. Strategies for family members and caregivers to use if a person with HD is showing aggressive behaviors include: 1) try to figure out what is causing the temper tantrums and aggressive behavior and whenever possible avoid or change them; 2) try to redirect the person away from the source of anger; do not scold or show anger towards the person as this could make the situation worse; 3) take steps to provide a safe environment by removing potential weapons and posting emergency numbers near the telephone; and 4) if the situation continues to escalate despite efforts to calm the person down, family members and caregivers should leave the situation, call for assistance (family, friend, neighbor), and call the police. If there is a threat of danger to anyone, calling the authorities is the best thing to do.
Hostile or violent behaviors or attitudes toward another.
Posture is the position in which you hold your body upright against gravity while standing, sitting or lying down. Good posture involves training your body to stand, walk, sit and lie in positions where the least strain is placed on supporting muscles and ligaments during movement. Individuals with Huntington’s disease may begin to sit and stand leaning to one side and leaning forward. This is an altered posture due to weakness and poor balance.
Individuals with Huntington’s disease may sit and stand leaning to one side and leaning forward.
Anti-social behaviors are disruptive behaviors which cause, or are likely to cause, alarm or distress to others. As the disease progresses, patients with Huntington’s disease may exhibit behaviors that are hostile or aggressive such as stealing, trespassing, using or threatening violence, abusive language, and socially unacceptable behaviors such as urinating in public. People with HD may feel embarrassed by the behaviors in social situations. The anti-social behaviors can put a strain on their relationships. Family members and caregivers can help the person with HD who is exhibiting anti-social behaviors by explaining that the behaviors are due to changes in the person’s brain and by seeking to find out if there are any specific circumstances (e.g., disappointment, grief, frustration with failing capabilities) that trigger the behaviors so that they can be prevented or reduced. People with HD may benefit from medications and/or behavioural therapy to help them deal with frustration, irritability, and anger.
Disruptive behaviors which cause, or are likely to cause, alarm or distress to others.
Anxiety is a feeling of worry, nervousness, or unease. Many people with HD say that they worry more than they used to, even about seemingly trivial matters. Anxiety in people with HD may be a reaction to challenging and changing situations in their lives such as waiting for results from genetic testing or experiencing increasing difficulty at work, changing roles within the family, or symptom progression. Anxiety is also caused by changes in their brains caused by the disease. Medications and cognitive-behavioral treatments by a mental health care provider can help prevent or decrease the likelihood of a person with HD becoming anxious. Family members and caregivers can help people with HD to be prevent or reduce anxiety symptoms by: 1) helping the person with HD to establish and stick to regular routines and schedules which minimize stress and make the person with HD feel secure; 2) keeping the home environment simple by turning the television off when leaving the room, reducing clutter, etc., to reduce potential triggers for stress; 3) simplifying requests and demands on the person with HD; 4) not discussing future events until the day before the event is to occur or gently introducing an event in advance and adding details over time if the person tends to worry and perseverate; 5) planning ahead for changes in routine and telling the person with HD about upcoming modifications in routine that will be needed to make the change; and 5) identifying “triggers” that provoke anxiety and recommending that the person with HD discontinue the activity that is making them feel anxious.
A feeling of worry, nervousness, or unease.
Apathy is a lack of feeling, emotion, interest, and concern. Apathy is a state of indifference, or the suppression of emotions such as concern, excitement, motivation, and/or passion. An apathetic individual has an absence of interest in or concern about emotional, social, spiritual, philosophical and/or physical life and the world. Apathy is caused by damage to specific areas of the brain by Huntington’s disease. It is not purposeful. Apathy is different from depression. The depressed person feels sad and unhappy. The apathetic person just doesn’t care or doesn’t feel like he/she has the energy to engage in activities that are interesting and fun. The apathetic may lack a sense of purpose or meaning in their life.
A lack of feeling, emotion, interest, and concern.
Attention is the awareness of someone or something and concentration is the ability to focus one’s attention or mental effort. People with HD will usually be able to focus their attention on one activity, but may have difficulty sustaining their attention if distracted as well as being able to divide their attention. Divided attention is the ability to attend to more than one thing at the same time. Because of declines in mental abilities, tasks that were once automatic (e.g., walking) may require intense concentration. This makes dividing one’s attention very difficult. For example, it may be difficult for a person with HD to walk while carrying on a conversation (called dual tasking because the person is doing two tasks simultaneously). If the person with HD is not able to walk safely while doing another activity, the person needs to concentrate on only doing one activity at a time. “One thing at a time” is good advice for people with HD who are having difficulty with dual tasking.
The awareness of someone or something.
Bradykinesia literally means ‘slow movement.’ Movement can be very slow for a person with HD. The movement can be hard to get started and will also be slow once movement has been initiated. It is vital that the person with HD uses the correct technique to help him/her be successful when moving (e.g. standing up from sitting) and that the caregiver allows the person time to be as independent as possible. This symptom can develop overtime as the HD progresses and may be controlled by medication.
Breathing control (e.g., endurance) Endurance is the ability to do something difficult for a long time. Endurance is improved by improving lung function and breathing control. To inhale adequate air for an activity a person has to be able to control the muscles of breathing so that breathing in and then breathing out are coordinated. When breathing control is poor the person may spend too little time breathing in oxygenated air or exhaling (blowing out) the “old” or unoxygenated air.
Endurance is improved by improving lung function and breathing control.
Poor balance may result from the primary symptoms of HD (e.g. Chorea, Dystonia, and Bradykinesia). These symptoms can directly affect the person with HD’s ability to balance. These symptoms may also result in the person being less active due to fear of falling or general lack of confidence. Therefore balance may also be affected by secondary symptoms that may occur over time because of the person being less active. For example, muscle weakness and joint stiffness are secondary symptoms and may have a negative impact on balance. Although poor balance may increase the risk of falls, it is vital that the person remains active to gain many health benefits. Poor balance can be observed as a symptom through all stages of HD. Balance can be improved through physical activity and exercise.
Chest complicatons (e.g. aspiration) The primary chest complicatin associated with Hntington’s disease is aspiration. Aspiration is the entry of material (such as saliva, food or drink) from the throat into the respiratory tract (the tubes leading from the throat to the lungs). A person inhales the material during swallowing. When lung aspiration occurs during eating and drinking, the aspirated material is often colloquially referred to as “going down the wrong pipe.” Consequences of lung aspiration range from no injury at all, to pneumonia, to death within minutes from asphyxiation (blockage of the windpipe preventing air from entering or leaving the lungs).
The primary chest complicatin associated with Hntington’s disease is aspiration.
Complex thinking is a separate set of mental processes that facilitates your ability to do complex, sophisticated thinking, such as understanding concepts, generating original ideas, and using logical approaches to address complicated problems. Thinking skills are characterized on a continuum of levels. Lower levels of thinking are simpler (memorizing, identifying, etc.) than higher levels (synthesizing, judging, analyzing, etc.) Complex thinking refers to these higher level skills of pulling together more than one fact or concept to come to a decision or analyzing a situation and coming to a conclusion. Situations that involve complex thinking include things like crossing a busy street, driving a car and deciding what to do in an emergency such as a fire.
A set of mental processes that facilitates the ability to do complex, sophisticated thinking.
Chorea literally means irregular, purposeless, involuntary movements which may affect the limbs, (arms and legs), torso and face. Movements are rapid and jerky and do not seem to have any purpose. The movements are involuntary, which means the person cannot control the movement. These movements are often upsetting and disturbing to people observing. However people with HD often report that they are not concerned by these movements. In fact, research in this area indicates that chorea does not seem to have a major impact on the person’s ability to balance, walk or function in his or her daily life. This symptom may be controlled by medication and is usually first seen early on in HD.
Coordination is the ability to use different parts of the body together smoothly and efficiently. Fine motor coordination of the hands and fingers deteriorates early in the disease while gross-motor coordination of the legs for walking and balancing deteriorates later in the disease. Coordination can be improved by doing specific exercises. Practicing activities that are meaningful can help to maintain coordination for these activities.
The ability to use different parts of the body together smoothly and efficiently.
CVS fitness is the ability of the heart and lungs to supply oxygen-rich blood to the brain, muscles and organs of your body. It is also the ability of the muscles to use oxygen to produce energy for movement. This type of fitness is a health-related component of physical fitness that is brought about by sustained physical activity. Like all muscles, the heart becomes stronger as a result of exercise, so it can pump more blood through the body with every beat and continue working at maximum level, if needed, with less strain. The resting heart rate of those who exercise is also slower because less effort is needed to pump blood. Regular exercise has a favorable effect on many of the established risk factors for cardiovascular disease. For example, exercise promotes weight reduction and can help reduce blood pressure.
The ability of the heart and lungs to supply oxygen-rich blood to the brain, muscles and organs of your body.
Delirium is an acute, transient, fluctuating disturbance in attention, mental abilities, and consciousness level. Individuals with HD may experience delirium especially in later stages of the disease and typically due to medications, dehydration, and/or respiratory or urinary tract infections. The main symptom of delirium is difficulty focusing, maintaining, or shifting attention. Other symptoms include confusion, disorientation, changes in personality and affect (agitated and hyperactive or quiet and withdrawn), disorganized thinking, impaired speech (slurred, rapid, and nonintelligible), delusions, and hallucinations. Symptoms of delirium change over minutes to hours; they may lessen during the day and worsen at night. Delirium is usually reversible but mental deficits often take weeks or months to go away after the acute illness. Medications are often used to treat delirium. Suggestions for how family members and caregivers can help the person with HD who is experiencing delirium are the following: 1) maintain a stable, quiet, and well-lit environment and provide visual cues to orient the person (e.g., calendar, clocks, family photographs); 2) provide frequent reorientation (place, time, person, and events) and reassurance that the person will get better; and 3) minimize sensory deficits by encouraging the person with HD who needs eyeglasses or hearing aids to use them.
An acute, transient, fluctuating disturbance in attention, mental abilities, and consciousness level.
Delusions and Hallucinations
Delusions and hallucinations are disorders of thought experienced by some individuals with HD in which the person loses contact with reality. Delusions are irrational and false beliefs about reality. Examples of delusions are when a person believes that someone else is living in his or her house when the person actually lives alone or when a person thinks that someone is out to get him or her or is watching the person. Hallucinations are when the person sees, hears, smells, tastes, or feels something that is not there. They seem real to the person experiencing them but cannot be verified by anyone else. For example, a visual hallucination could be seeing bugs crawling over the bed that aren’t actually there. Both of these symptoms occur more frequently as the disease progresses and may be distressing for the person with HD and for families. Some tips for family members and caregivers to cope with delusions and hallucinations that a person with HD is having are the following: 1) tell the person’s physician about the delusions and hallucinations as antipsychotic medications are often required for these severe symptoms; 2) try not to argue with the person about what he or she sees or hears and provide comfort if the person is afraid; 3) distract the person; sometimes moving to another room or going outside for a walk helps; and 4) make sure the person is safe and can’t reach anything that the person could use to hurt someone or him or herself.
Disorders of thought in which the person loses contact with reality.
Dementia is a general term for loss of mental abilities severe enough to interfere with daily life. It occurs in the later stages of Huntington’s disease and is caused by injury or loss of brain cells (neurons). Common symptoms of dementia in HD include a severe decline in thinking and reasoning skills, irritability, depression and other mood changes, impaired memory, and problems with completing daily activities. Physicians can diagnose dementia using specific tests and recommend medications and/or other treatments to reduce symptoms. Family members and caregivers can help people with HD with dementia to remain as functional as possible by maintaining a calm, predictable, routine environment that allows the person to organize daily tasks and follow a specific schedule. They can also assist them to be physically active (e.g., walking, chair exercises), to engage in activities that they enjoy (e.g., listening or singing along to music, helping in the kitchen, playing bingo) and to eat a healthy diet and drink adequate amounts of water.
Loss of mental abilities severe enough to interfere with daily life.
Depression is a mood disorder that causes a continuing feeling of sadness and loss of interest. People with HD can experience depression at any time during the course of the disease. Common depressive symptoms include lack of interest in usual activities, sleeping most of the day or rarely sleeping, sad mood most of the day nearly every day, weight loss, feelings of worthlessness, social withdrawal, inability to concentrate, recurrent suicidal thoughts, and loss of sex drive. The causes of depression in people with HD may be from the disease itself or from reasons such as adjustment to life changes, increased disability and grief. Depression is very treatable. Physicians may recommend medications, and different types of psychological and behavioral therapies. Family members and caregivers can help people with HD relieve depressive symptoms by encouraging them to stay active and exercise regularly as regular aerobic exercise (e.g., walking, cycling) has been shown to improve mood in people with mild to moderate depression. They can also talk to the person with HD to find out what might be triggering his or her depression and give the person support and assistance as needed.
A mood disorder that causes a continuing feeling of sadness and loss of interest.
Problems with walking can result from a combination of primary symptoms of HD and secondary symptoms that occur as a result of the primary symptoms (e.g. muscles get weaker because the person is less active). The person may walk with shorter strides and may lack exercise tolerance (endurance), which will limit how far he/she can walk. People with HD may have more difficulty walking up and down slopes and curbs and particularly uneven surfaces outside. Walking up and down stairs may also be a problem. Walking ability can be improved with assistance and /or advice from a physiotherapist and endurance can be increased by physical activity and exercise. For example, findings from a recent exercise study showed that people with early stage HD improved their endurance/fitness through completing a 12 week exercise programme where they exercised on a static bike three times a week.
Dual tasking is when a person performs more than one activity at a time. Examples are walking and talking, walking and carrying an object and talking while typing. When a person engages in two activities at the same time the brain has to monitor and run both activities simultaneously. The area of the brain that is involved in dual-tasking is the basal ganglia. The basal-ganglia is damaged by Huntington’s disease. Individuals with Huntington’s disease will have progressively more difficulty performing dual-tasks (doing two things at the same time) as the disease progresses. It may be helpful to wait to converse with the person with Huntington’s disease until he/she is sitting. Physical and occupational therapy may be helpful in improving the ability to dual-task or in finding ways to avoid dual-tasking and improve safety.
Performing more than one activity at a time.
Dysarthria is a condition in which the muscles you use for talking are weak or you have difficulty controlling them. Dysarthria often is characterized by slurred or slow speech that can be difficult to understand. Common causes of dysarthria include nervous system (neurological) disorders such as Huntington’s disease. Huntington’s disease causes tongue and throat muscle weakness. There may also be weakness of the muscles that control mouth movements. Dysarthria treatment is usually involves speech therapy to help improve strengthen the muscles and to teach you how to better control the muscles used for speech and swallowing.
Where the muscles you use for talking are weak or you have difficulty controlling them.
Dysphagia is difficulty swallowing. It takes more time and effort to move food or liquid from your mouth to your stomach. Dysphagia may also be associated with pain. In some cases, swallowing may be impossible. In Huntington’s disease dysphagia is caused by poor control of weak muscles in the mouth and throat. It can lead to food and liquids going down the “wrong pipe” and ending up in the lungs. When food or liquids enter the lungs it creates an environment for germs to grow and leads to pneumonia. Speech therapy is usually prescribed to improve swallowing and to determine types and textures of foods and liquids that can be swallowed easily. For most individuals with Huntington’s disease it becomes difficult to swallow liquids. The liquids go into the tube leading to the lungs and cause choking and coughing. A swallow test is something the doctor will order to learn more about any swallowing issues you may be having. Some common recommendations for individuals who are having trouble with swallowing are to use a straw for liquids or to use a thickener in liquids to make them easier to swallow. Some foods such as very dry or sticky foods may be harder to swallow. Foods that are difficult to be swallowed should be avoided. A speech therapist can help you with suggestions on how to prepare your favorite foods so that they are easier to swallow. Chewing more thoroughly and eating slowly are other common instructions given to individuals with dysphagia.
Difficulty with swallowing.
Dystonia literally means a sustained muscle contraction. This abnormal posturing can occur anywhere in the body. In HD it is common around the face, mouth, tongue, torso, limbs, feet and hands. This sustained muscle contraction and abnormal postures can result in pain for the person with HD. Posturing of the feet and legs may cause problems with balance and walking and maybe a primary cause of falls. This symptom may be controlled by medication and is usually seen from mid-late stage of HD.
We call HD an ‘autosomal dominant condition’ which means a child of an affected parent has a 50% chance of also being affected. The gene that causes HD is on chromosome 4 and the problem is with the increased length of a portion of the chromosome called the CAG (cytosine-adenine-guanine) triplet repeat. This increased length (or expansion) is measured by the number of repeats of the CAG triplet: 0-35 repeats means that the person will not develop HD; 36-39 repeats means that the person has an increased risk of developing HD and; 40 or more repeats means that the individual will develop HD.
Impulsivity involves a tendency to act on a whim, displaying behavior characterized by little or no forethought, reflection, or consideration of the consequences. Individuals with Huntington’s disease are often impulsive. The impulsivity is caused by damage to specific areas of the brain. Examples of impulsive behavior would be buying something he/she likes even though there is not enough money to buy the object.
A tendency to act on a whim, displaying behavior with little or no forethought, reflection, or consideration.
Incontinence is the involuntary leakage of urine; in simple terms, to wee when you don’t intend to. It is the inability to hold urine in the bladder because voluntary control over urinary closure is either lost or weakened. Urinary incontinence is a much more common problem than most people realize. The severity ranges from occasionally leaking urine when you cough or sneeze to having an urge to urinate that’s so sudden and strong you don’t get to a toilet in time. If urinary incontinence affects your daily activities, don’t hesitate to see your doctor. For most people, simple lifestyle changes or medical treatment can ease discomfort or stop urinary incontinence. There are exercises that can help minimize the leakage of urine. In Huntington’s disease the incontinence can be due to damage the nerves that control the bladder. Treatment with medication prescribed by a doctor may be necessary. In some cases individuals have severe bladder “spasms” that lead to leakage of urine. Sometimes it is helpful to treat this type of problem with Botox injections.
To wee when you don’t intend to.
Intellectual decline is a general term for an abnormal condition in which a person experiences progressive or sudden worsening of their mental abilities. Common symptoms of intellectual decline that may develop in people with HD include increasing forgetfulness, confusion, disorientation, behavior changes, irritability, falls, and depression. People with HD with intellectual decline can also experience difficulty with speaking, language, concentration, organizational skills, and learning and memory. Physicians can diagnose intellectual decline using specific tests and recommend medications and/or other treatments depending on the cause. Family members and caregivers can help to reduce intellectual decline in people with HD by encouraging them to be physically active and to exercise regularly, by playing games, doing puzzles, or having conversations with them that stimulate them to think and remember things, by helping and encouraging them to eat a healthy diet, and by ensuring that they drink enough water and stay well hydrated.
An abnormal condition in which a person experiences progressive or sudden worsening of their mental abilities.
Irritability is when a person is easily annoyed, excited, or angered. It is a common symptom in Huntington’s disease that occurs at all stages of the disease. Irritability can lead to aggressive behaviors when the emotional response escalates and cannot be controlled. Physicians can prescribe medications to help reduce irritability. Family members and caregivers can help the person with HD to reduce irritability by helping the person to identify and avoid situations of stress or “triggers” that can make irritability worse and set off more aggressive behaviors. Close attention should be paid to verbal or nonverbal signals that the person is frustrated. Verbal signs include the person stating that he or she feels a frustration about something. Nonverbal cues include a higher volume or speed of speech or sometimes when a person stops talking. Often people with HD are frustrated because they can’t do something they want to do. If the person with HD is capable, family members and caregivers should encourage the person to be as independent as possible. Giving people with HD some responsibilities around the house that are appropriate to their level of function (e.g., watering the plants in the front yard, sweeping a room) but not overwhelming can help the person with HD avoid frustration. For people with HD living in a care facility, including them in making decisions as much as possible can help to avoid frustrations.
When a person is easily annoyed, excited, or angered.
Lack of Insight
Lack of insight is the inability to recognize that a problem exists. The individual is not in denial but due to damage in the brain the person has an impaired awareness of their illness and the deficits it causes. A person with Huntington’s disease may have severely impaired balance and still believe that he or she can walk safely up or down a flight of stairs. Individuals with Huntington’s disease often refuse to give up driving or doing other activities even though he/she has had many accidents or problems completing the activity safely. This lack of insight means the person with HD has an impaired awareness of illness caused by damage to specific parts of the brain.
The inability to recognize that a problem exists.
Memory is the ability to learn and remember information. People with HD have problems with learning new information and problems with recalling the remembered information. Learning may become difficult because they are not able to organize (put similar items together) and sequence (put tasks in correct order) the information to be learned or because they have problems with divided attention (doing more than one thing at once). Therefore it may take longer for the person with HD to learn new things and they may need to concentrate on the information or task that they are trying to learn and not try to do other things at the same time. If a person with HD is having difficulty recalling information such as what he or she had for dinner, it is best to give the person a choice from which the person can recognize (rather than recall) the information (e.g., ask the person if he or she had pizza or chicken for dinner).
The ability to learn and remember information.
Understanding your personal abilities and limitations is important. If you in any doubt you should consult a professional. Current guidelines recommend 150 minutes of moderate physical activity per week. Moderate exercise stimulates the body’s heart, lungs and muscles overtime, causing them to adapt and become more efficient. People can tell when their activity is moderate intensity because they will experience:-
- Breathing faster
- An increase in heart rate
- Feel warmer
- They may sweat on hot or humid days NB.
Working at a moderate intensity means that you should still be able to hold a conversation. The amount of activity needed to reach this state varies from one person to another. An unfit person may only have to walk up a slope, whereas a very fit person maybe have to walk quite fast or travel a longer distance to get the same effect. Over time the person’s fitness will improve so that when walking, for example, they will be able to walk further or faster while still working at moderate intensity. A person working at vigorous intensity will experience:
- Breathing very hard
- Rapid heartbeat
- Will be unable to hold a conversation
Organising is the process of arranging thoughts or actions into a purposeful and orderly way. Problems with organisation can result in people with HD not being able to easily find things when they need them, taking longer to do things or not being able to complete activities that they start, and having difficulty planning their days (e.g., figuring out the families’ schedules and where each person needs to be at a specific time). Behavioral techniques can be used to address problems with organisation. Family members and caregivers can help people with HD with problems with organisation by: 1) reducing the amount of information given to a person with HD at one time to allow for the brain’s slower speed of processing information; 2) maintaining regular schedules and routines which make it easier for a person with HD to stay organised; 3) using calendars, to-do lists, and white boards that the person with HD can refer to throughout the day; and 4) review the person’s schedule/calendar at least once a week and make sure it is up to date.
The process of arranging thoughts or actions into a purposeful and orderly way.
Paranoia involves intense anxious or fearful feelings and thoughts often related to persecution, threat, or conspiracy. Paranoia can occur in Huntington’s disease due to damage to specific areas of the brain. Paranoia can become delusions, when irrational thoughts and beliefs become so fixed that nothing (including contrary evidence) can convince a person that what they think or feel is not true. Symptoms of paranoia and delusional disorders include intense and irrational mistrust or suspicion, which can bring on sense of fear, anger, and betrayal. Some identifiable beliefs and behaviors of individuals with symptoms of paranoia include mistrust, hypervigilence, difficulty with forgiveness, defensive attitude in response to imagined criticism, preoccupation with hidden motives, fear of being deceived or taken advantage of, inability to relax, or are argumentative.
Intense anxious or fearful feelings and thoughts often related to persecution, threat, or conspiracy.
Pedometers are small portable devices that measure a person’s physical activity level throughout the day. When worn at the waist attached to a belt they can be used to count the number of steps a person takes. People with HD tend to be sedentary and have low levels of physical activity throughout a day. Pedometers can be worn by people with HD to motivate them to be more physically active. A recommended goal for adults is to reach 10,000 steps per day. However, the person with HD may have to start at a much lower goal and gradually work up to the recommended level. Family members and caregivers can help people with HD to be more active by: 1) making sure that they put on and start the pedometer properly in the morning and that they wear it all day, 2) recording the steps at the end of the day and repeating this process for one week’s time, 3) calculating an average of the person’s daily steps at the end of the week, and 4) identifying a starting goal for daily physical activity that is acceptable to the person with HD. Daily step counts can be recorded using a calendar, notebook, daily organizer, or log on a refrigerator or bulletin board so that the person with HD can refer to the counts and see how much he or she is progressing over time.
Portable devices that measure a person’s physical activity level throughout the day.
Perseveration or “getting stuck” is an uncontrollable repetitive emotion, thought, or action. People with HD with perseveration often repeat questions or get “stuck” or “fixated” on certain topics. The behavior is a consequence of the disease; the individual with HD is not able to control the behavior and often is not aware of being “stuck” on things. A physician may be able to prescribe medications to reduce the perseveration. Family members and caregivers may need to choose their battles is regards to changing the person’s behavior. Not all perseverative behaviors are harmful and family members and caregivers may be able to adjust to and tolerate some of them. If perseverative behaviors are disruptive to family life or distressing to the person with HD behavioral techniques may help to reduce the perseveration to a level that the family members and caregivers can manage. Redirection or distraction is the most common technique that breaks the behavioral cycle. Family members and caregivers can use calming or pleasant activities for redirection such as changing the subject, starting a new activity, moving to a different place, giving the person a new job (folding laundry, etc), offering the person’s his or her favorite food, going for a walk together, bringing up pleasant memories, or playing soothing music.
An uncontrollable repetitive emotion, thought, or action.
Planning is the process of deciding how to do something before actually doing it. People with HD often have difficulty planning things such as completing errands or putting on a party for friends. Poor planning leads to inadequate preparation, unexpected problems, and poor execution. Suggestions for helping people with HD with planning problems are to make lists to help them organize the individual tasks in the order needed to do an activity, cue them in each step of an activity, and emphasize routine daily activities that are well learned and easier for people with HD to start or continue on their own.
The process of deciding how to do something before actually doing it.
Prioritizing is the process of determining the order for dealing with a series of items or tasks according to their relative importance. People with HD have difficulty deciding what tasks are more important at a certain time and giving those tasks more of their attention, energy, and time than others. Poor prioritizing can lead into crisis or troubling situations if the person has not completed important urgent tasks before completing less important tasks. To help people with HD with prioritizing problems number their to-do lists so that activities that are most important to do are labelled “1.” Tasks that are of intermediate importance that should be done only after the person with HD finishes the “1” tasks are designated “2”. Finally, tasks that are less important and should be done only if the person has time after finishing with the “2” tasks are labelled “3.”
Determining the order for dealing with a series of items or tasks according to their relative importance.
Rigidity is literally stiffness of the muscles. Stiffness will make movement difficult for the person with HD. Movement will be slow and the person may find it difficult to bend at the waist when sitting down, or to twist when reaching for an object. Rigidity can also be felt by the caregiver, when he/she attempts to move the person’s arm or leg. The caregiver will feel a resistance to the movement. Rigidity can occur in all muscles, including the face and muscles which allow us to speak. This can result in a person with HD having difficulty with speech and facial expressions. Slowness of speech is particularly a problem for effective communication and it is important that the person with HD is given time and allowed to speak for himself/herself. This symptom may be controlled using medication and tends to be observed later in the disease.
Sequencing is the process of arranging thoughts or actions into a particular order. People with HD have difficulty with sequencing of individual tasks in the order needed to complete an activity such as when preparing a meal or completing household chores. Problems with sequencing could lead to safety issues if for example the person turns on the stove burner under a pot and leaves it without first putting the soup in the pot. Suggestions for helping people with HD with sequencing problems are to make lists to help them organize the individual tasks in the correct order to complete an activity, cue them in each step of an activity, and emphasize routine daily activities that are easier for people with HD to start and complete on their own.
The process of arranging thoughts or actions into a particular order.
Strength is the ability to do things that need a lot of physical or mental effort. Muscle strength is measured as the size of the load a person can move or lift using specific muscles. To have a strong muscle contraction the person must be able to use the brain to send a signal through the nerves to tell the muscle to contract. Individuals with Huntington’s disease have some weakness associated with damage to the brain from the disease. Lack of strength also occurs due to inactivity and not using muscles. Individuals with Huntington’s disease often lack interest in doing activities and so become weak due to inactivity.
The ability to do things that need a lot of physical or mental effort.
Total Functional Capacity Score
The Total Functional Capacity score (TFC) 12 is a measure of general function which will be taken by a healthcare professional (usually a Doctor) and involves a series of questions to the person with HD and/or their caregiver. The TFC score indicates the level of independence of the person with HD in 5 aspects of life (Occupation, Finances, Domestic Chores, Activities of Daily Living, and Care Level). A higher TFC score means the person with HD is more independent. The score produced allows the person to be classified at a specific stage of HD: Stage I (11-13) is early stage; Stage II (7-10) and Stage III (3-6) are middle stage and Stages IV (1-2) and V (0) are late stage.
Table of Symptoms
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