Nutritional Challenges in Huntington’s Disease

Huntington’s Disease Association

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

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

Introduction: How HD affects eating

Cognitive (thinking)

Cognitive (thinking) EmotionalEmotional

PhysicalPhysical

HDHD

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

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

• Problems with thinking

• Planning & sequencing

• Organisation

• Problems with concentration

Cognitive Impairment & Eating

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

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

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

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

social events.

Swallowing – how it worksNormal 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 preparation2. Oral phase3. pharyngeal phase4. Oesophageal phase

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

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

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

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

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

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

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

Further Information

Suite 24 Liverpool Science ParkInnovation Centre 1131 Mount PleasantLiverpoolL3 5TF

Tel: 0151 331 5444

E mail: info@hda.org.uk

Web: www.hda.org.uk