ࡱ> []Z '<bjbj 4P'4R R <}+0"   *******$-O0R"+,,,"+7+,*,*)|*AfY,**M+0}+>*~00$*0*,,,,,,,,"+"+,,,}+,,,,0,,,,,,,,,R [: A Physicians Guide to the Management of Huntingtons Disease (EXTRACTS FOR SALT & DIETICIAN) Swallowing difficulties Dysphagia is, directly or indirectly, the most common cause of death in people with late stage Huntington's disease, whether through choking, aspiration, or malnutrition. Dysphagia results from impaired voluntary control of the mouth and tongue, impaired respiratory control due to chorea, and impaired judgment, resulting in eating too rapidly, or taking overly large bites of food and gulps of liquid. Dry mouth, which can be brought on by neuroleptics, antidepressants, and anticholinergics, may worsen the problem. TABLE 4: Swallowing TipsEat slowly and without distractions. Prepare foods with appropriate size and texture. Eating may need to be supervised. Caretakers should know the Heimlich manoeuvre. No medications are known to improve swallowing directly. Early referral to a speech-language pathologist will help identify swallowing difficulties, and periodic reassessment can identify changes in swallowing ability and suggest appropriate non-pharmacologic interventions such as a change in food consistency. Devices such as enlarged grips tor silverware and nonslip plates with raised edges to prevent spilling may prolong independent eating. Huntington's disease affected individuals should be instructed early in the disease, before the onset of dysphagia, to eat slowly and deliberately, to sit in an upright position during and after meals, to take small bites, and to clear the mouth of food after each bite by taking sips of liquid. Individuals with dysphagia should avoid doing other activities while eating, in order to concentrate on chewing and swallowing. For instance, patients should not talk while eating, nor be distracted by television or ambient noise. Those who tend to hyperextend the neck due to chorea or dystonia should be encouraged and reminded to use a "chin-tuck" position. Drinking fluid through a straw may be easier than drinking directly from a cup, and the use of a covered cup or mug, like a "sippy cup" used by young children, may prevent spillage due to chorea. Grainy items, such as ground beef or rice, may irritate the pharynx and cause choking. Foods such as steak, which are hard to chew, should also be avoided, or ground to a puree. Patients may have difficulty adjusting to different textures of food, and may do better if they finish each item on the plate in turn. In late Huntington's disease, when even liquids may.be difficult to swallow, the texture of food should be soft and smooth, and liquids may be thickened with an additive (see  HYPERLINK "http://www.uihealthcare.com/depts/huntingtonsdisease/physiciansguide/appendix3.html" Appendix 3). For those patients who may be unable to follow instructions reliably, a caregiver can cut the food in advance, and ensure that each mouthful has been completely chewed and swallowed before the next bite is begun. Supervision throughout the meal may be necessary, and the family or caregiver should be taught to perform the Heimlich manoeuvre. In some cases, eating eventually requires so much energy and concentration that the patient becomes tired and frustrated before consuming adequate amounts of food. Weight loss, very prolonged mealtimes or an inability to handle utensils may be the signal that he will need to be fed for at least part of the meal. Self-feeding may be prolonged by having the patient eat more frequent, but smaller meals, and by using "finger foods." The transition to assisted feeding does not have to be all or nothing, as patients may still be able to eat unassisted at certain times and be fed at other times. Choking may decrease once self-feeding is stopped, because the caregiver will have greater control over the size and frequency of the bites. The caregiver should still promote eating slowly, and not talking while eating, and should make sure the mouth is empty before each bite. With supervision, most patients are able to assist with feeding and to take adequate amounts of food by mouth quite far into the illness. However, before dysphagia and communication difficulties become severe, the issue of feeding tubes should be discussed with the patient and family, to ensure that appropriate nutrition can be maintained throughout the illness. A gastrostomy tube can clearly improve nutritional status in a debilitated person with severe dysphagia, and may prolong life. However, patients and families may not desire this intervention late in the course of Huntington's disease. The question of whether to use a gastrostomy tube, and other end of life issues are discussed in the final section of chapter 6. Nutrition The main aims of dietary treatment in HD are to encourage people to achieve a well balanced diet, which includes all the essential nutrients, to prevent or minimise the weight loss frequently seen in the mid to later stages of the disease and to help people regain lost weight where possible. It has been well documented that many people with HD find it difficult to maintain their body weight and therefore need a higher than normal calorie intake. While the exact reasons for this are unknown, several studies have shown some people have a higher metabolic rate. This is frequently, but not always, associated with increased chorea movements. In the later stages of the disease increasing swallowing problems contribute to weight loss as mealtimes take longer and it can be difficult to ensure a pureed diet provides adequate nutrition. Maintaining a healthy body weight is essential because people who are underweight (i.e. BMI of less than 18.5) lose muscle mass and therefore feel weaker, become apathetic and depressed, are more prone to catch infections, develop pressure ulcers if their mobility is compromised and take longer to recover from illness, operations or wounds. There is some anecdotal evidence that shows that providing a high calorie intake can help to reduce chorea movements, improve cognition and improve speech & swallowing. Given the consequences associated with a low BMI it is important to monitor weight on a regular basis, but the frequency will vary from person to person, depending on where they are in the disease process and their weight history. As a guide a someone in the early to mid stages whose weight is stable should be monitored as below BMI > 27 yearly, BMI 23-26 6 monthly, BMI 20-23, 3 monthly BMI 18.5 19 2 monthly BMI <18.5 monthly. When the patient is in the later stages of the disease or if they are losing weight it should be monitored monthly where possible. Dietary advice When a patients BMI is above 23 and stable then it is appropriate to offer general healthy eating advice Aim for a regular meal pattern of 3 meals per day and include starchy foods at each meal. Aim for 5 portions of fruit and vegetables per day Include some form of meat, fish, eggs, cheese, pulses or similar foods at least twice a day Aim for 1 pint of milk per day or its equivalent in other forms i.e. 1/3 pint milk = 1 oz cheese or 1 x 125 gm yoghurt If desired include foods containing fats or sugars in moderation as extras to meals. Drink 8-10 cups of fluid per day e.g. water, tea, coffee, fruit juice, milk, squash. Once a patient starts to lose weight or if they are underweight then they need information on how to increase their calorie intake and preferably should be referred to a Registered Dietitian for specialist advice. However if this is not possible then the guidelines below can be followed Step one Gradual weight loss and BMI above 22 Encourage a regular meal pattern with high calorie snacks between meals e.g. Cheese and biscuits, sweet biscuits & cakes, sandwiches with fillings such as: cream cheese, peanut butter, jam, thick and creamy yoghurts, mousses, fromage frais. Encourage milky drinks e.g. drinking chocolate, fruit smoothies, milkshakes Advise on the use of full fat products e.g. full fat milk, butter rather than reduced fat margarine Fortify meals by Adding plenty of butter, cheese, evaporated milk, salad cream to potatoes and vegetables Use plenty of butter, jam etc on bread, toast, crumpets etc Encourage high calorie puddings and add extra cream, evaporated milk, ice cream etc. Review after 2 months If weight has stabilised advise patients to maintain changes and continue to monitor weight on a regular basis Step 2 If weight loss continues or BMI is less than 22 Advise as above plus introduce supplement drinks. These can be Either nutritionally complete supplements e.g. Ensure Plus, Fortisip, Clinutren 1.5, Resource Shake, Fresubin Energy which are ready to drink and Complan Shake which needs to be mixed with whole milk Or milk shake powders e.g. Scandishake, Calshake, and Enshake which need to be made up with whole milk and are not nutritionally complete All of the above are prescribable under NICE guidelines in order to prevent/treat the disease related malnutrition which often occurs in Huntingtons Disease To ensure compliance and therefore save wastage it is essential to find a supplement that the patient will tolerate. Therefore they should be prescribed a weeks trial of either 2 nutritionally complete supplements daily or 1 sachet of milk shake powder supplement. Tastes vary from person to person so try a variety of brands until you find one they like which can then be prescribed on a monthly basis, Nutricia and Abbott, who manufacture Fortisip and Ensure Plus respectively, produce variety starter packs of 10-12 cartons, which are useful as an initial prescription. Step 3 Patients should continue with the supplements until their weight has stabilised and/or they have regained any weight lost. After this they can be gradually reduced but their weight must continue to be monitored closely, particularly if they have swallowing problems and have been recommended to change to a modified texture diet by a Speech and Language Therapist. Step 4 If a patient continues to lose weight they should be referred to Registered Dietitian as soon as possible as they have the skills, expertise and knowledge of more specialist products, which can be used to prevent further weight loss. They will also be able to discuss issues such as PEG feeding with patients and their carers if required. In conclusion it is now recognised that nutrition plays an important role in the treatment of HD. Therefore it is essential that all people who are diagnosed with HD should have their weight and dietary intake monitored regularly at the same time as their other symptoms are reviewed. This enables suitable action to be taken as soon as problems appear rather than trying to reverse significant weight loss. It is much easier to prevent weight loss and its consequences then it is to help people to regain lost weight. Dysarthria Dysarthria, a difficulty with the physical production of speech, results largely from impairment of voluntary movement. Speech becomes slurred, dysrhythmic, variable in volume due to inconsistent breath support, and increasingly difficult to understand. TABLE 5: Coping Strategies for CommunicationAllow the person enough time to answer questions. Offer cues and prompts to get the person started. Give choices. For example, rather than asking "what do you want for dinner?" ask "do you want hamburgers or meatloaf?" Break the task or instructions down into small steps. If the person is confused, speak more simply and use visual cues to demonstrate what you are saying. Ask the person to repeat phrases you did not understand, or spell the words. Alphabet boards, yes-no cards, or other communication devices may be helpful. Furthermore, just as patients do not always appreciate the presence or degree of chorea, some patients do not seem to be aware of distortions in their speech. For others, articulation is a constant source of frustration. No medications are known to be helpful, and dysarthria may be worsened by agents which suppress chorea. However, several interventions may enhance communication in these patients. The listener must do everything possible to promote successful communication, beginning with allowing enough time. Many Huntington's disease patients thought to be incapable of communication can be understood if the listener is patient enough. Patients may need to be moved to a quieter, calmer environment, and urged to speak slowly. Patients can be asked to spell difficult to understand words. A communication board can also be useful in some cases. A speech-language pathologist may be able to provide additional insights and management strategies. Dysarthria may be compounded by cognitive problems found in Huntington's disease, such as word-finding difficulty, difficulty initiating speech, or difficulty completing a sentence. Even those with severe cognitive impairments often respond to cues, such as asking for the size, shape or colour of an object. Even severely impaired patients may be able to respond accurately to a series of yes and no questions. If unsuccessful attempts at communication become very frustrating, it may be better to take a break. 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