1. Nutritional Challenges in Huntington’s Disease Huntington’s Disease Association

2. What is Huntington’s Disease? A hereditary degenerative neuro- psychiatric disorder Symptoms of HD usually start between the ages of 30-50 years, although late onset and juvenile manifestation does occur The prognosis is usually between 15-20 years from onset of symptoms Currently no cure

3. Introduction: The Social Importance of Eating Eating is one of the primary pleasures in life Most celebrations involve eating and drinking Consumption of food and drink is important in many social interactions

4. Introduction: How HD affects eating HD PhysicalEmotional Cognitive (thinking)

5. Physical Impairments Problems with Co-ordination Accessing food – shopping/mobility Packaging Preparation – takes a lot of time & energy Using utensils Swallowing- dysphagia – consistency of food

6. Physical Impairment Involuntary movements Loss of fine muscle control and coordination Muscle weakness – poor posture Fatigue – it takes a great deal of effort Impaired swallow

7. Problems with thinking Planning & sequencing Organisation Problems with concentration Cognitive Impairment & Eating

8. Cognitive Impairment and Eating Impaired Executive Function Difficult to organise Difficult to plan ahead Difficult to make decisions Loss of Automatic Pilot Hand eye coordination no longer automatic Chewing and swallowing action no longer automatic

9. Emotional Aspects Family History – seen problems, lodged in mind/memories Fear of choking/ explosive coughing fit/ aspiration Fear of starving – seen family member lose weight Embarrassment / social situation/ messy

10. Emotional Aspects The preparation and consumption of food and liquids can be a huge source of anxiety for an individual with Huntington’s Disease and their family

11. Someone with HD is likely to experience loss of enjoyment, dread and/or decreased participation in many social events.

12. Swallowing – how it works Normal Swallow A person usually swallows approximately 590 times each day: 146– eating 394– awake not eating 50– asleep Fast, automatic, coordinated No need to concentrate 4 phases: 1.Oral preparation 2.Oral phase 3.pharyngeal phase 4.Oesophageal phase

13. Summary of Effect of HD on Swallowing Impaired pacing of the intake of food Uncoordinated extra effort Poor posture Muscle weakness Explosive coughing Aspiration Chest infections

14. Towards Minimising the Problems Promote independence for as long as possible Know the person well Only teaspoons of food Avoid distractions Good positioning and posture Posture after meal

15. High Calorie Diet Nutrition is still important – input from dietician Likes/dislikes – choice still important Extra calories needed Little and often – more frequent meal times, high calories snacks Don’t waste energy on consumption of low calorie foods Use food supplements

16. Modified texture/consistency of food and drink Importance of regular monitoring by S&LT Normal Food (possibly chopped) Naturally soft food (Fork) mashed food Pureed Food Normal Fluids Slightly thickened fluids Moderately thickened fluids Very thick fluids NB Avoid mixed textures

17. The Environment People with HD may need extra time to eat – food may need to be re-heated or eating postponed People with HD may find the environment around them distracting – provide a quiet, calm environment Positioning – upright seating position

18. Summary Food is important for us all HD can cause difficulties with eating –Process of preparing and eating food –Calorie intake There are a number of things we can do to make eating easier and to support people with HD

19. Thank you for listening If you would like any further information or would like to contact me, my details are below Name; Mandy Ledbury Tel: 01483 285231 Email; mandy.ledbury@hda.org.uk

20. Further Information Suite 24 Liverpool Science Park Innovation Centre 1 131 Mount Pleasant Liverpool L3 5TF Tel: 0151 331 5444 E mail: info@hda.org.ukinfo@hda.org.uk Web: www.hda.org.ukwww.hda.org.uk