Supporting Someone With Huntington’s: A Guide for Support Workers

Supporting someone with Huntington’s: a guide for support workers

If you are about to start supporting somebody with Huntington’s disease, five things will make you considerably better at it than most people, and none of them are obvious.

You are supporting three conditions at once. A movement disorder, a cognitive decline and a psychiatric illness, in the same person, interacting. Most of what goes wrong comes from treating it as one of the three.

Consistency is clinical, not a preference. A worker who knows this person’s early warning signs prevents incidents that a well-qualified stranger will trigger. You being the same person, doing things the same way, in the same order, is an intervention.

Arguing never works. Where insight is reduced and cognitive flexibility has gone, there is no belief that evidence could change. Every argument escalates and none resolve.

They understand you. Speech becomes slurred and hard to produce because of a motor problem, not a language one. Language and comprehension stay largely intact for a long time, and people with HD recognise everybody. Assume you are being fully understood, including the things said as though they were not in the room.

And the behaviour is the illness. Irritability, apathy, accusations and getting stuck on one subject are symptoms of brain damage in specific places. They are not aimed at you, and they are not choices.

This guide covers the practical detail. It does not replace your employer’s policies, the person’s support plan, their behaviour support plan or their mealtime management plan, all of which take priority and should be followed exactly.

The condition, briefly

Huntington’s is an inherited, progressive brain disease caused by a single faulty gene. It produces:

A movement disorder — chorea (involuntary flowing movements that cannot be suppressed), difficulty with voluntary movement and fine motor control, unsteady gait, slurred speech, and later dystonia, rigidity and slowness.

A cognitive decline affecting planning, sequencing, initiation, task-switching, inhibition and processing speed, with memory relatively less affected and language preserved until late.

A psychiatric illness — depression in around 40 per cent, anxiety, apathy, irritability, aggression, disinhibition, perseveration and sometimes paranoid delusions.

Onset is usually between 30 and 50, with a mean around 45. It runs a median of 15 to 18 years. Each child of an affected person has a 50 per cent chance of inheriting it — which means the family you are working with may include people who are at risk, waiting on results, or already becoming unwell. Around 2,600 Australians have it and about 9,000 more are at risk.

Late in the disease people are typically totally dependent, often unable to speak, and incontinent. Choking and aspiration pneumonia are a frequent cause of death.

Communication

Wait. Processing is slow and speech is physically hard to produce. The commonest failure in conversations with people who have HD is the other person filling the gap, answering their own question, or moving on. Count to ten. Then count again.

One thing at a time. One question, one instruction, one topic. A multi-part request fails — not because the person cannot do the parts but because holding the sequence has gone.

Do not raise your voice. Hearing is fine.

Speak to them, not about them. Never over their head to a family member, and never in the third person while they are present.

Be honest when you have not understood. “I got the first part — can you say the last word again?” is better than nodding.

Cue memory rather than testing it. Not “do you remember what we’re doing today?” Say “we’re going to the clinic today, we talked about it yesterday.” Retrieval is the problem; providing the cue solves it.

Reduce choices. Two options, not eight. An open question like “what do you want to do?” can be genuinely impossible.

Learn their communication system if speech has become hard to understand — the board, the device, the yes/no signals, the words only the family can interpret. Find this out on shift one, rather than at the moment it matters. And know that a touchscreen is hard to use accurately with chorea, so the access method may be a switch or a keyguard.

Keep a low-tech backup in reach — an alphabet board or whiteboard. Devices break and batteries die.

Behaviour: what to do and what never to do

Never argue. This is the single most important line in this guide. Where insight is reduced and cognitive flexibility is impaired, there is no mechanism by which evidence changes a fixed position. Agree where you can. Change the subject. Come back in twenty minutes. Do not try to win it.

Expect perseveration. The same question forty times, the same grievance, the same demand. It is impaired task-switching, not stubbornness. Answer calmly, the same way, as many times as it takes, or redirect.

Know the triggers, because almost every incident has one and they are predictable:

• Being rushed
• Being corrected or contradicted
• An unexpected change of plan
• Several instructions at once
• Noise, crowding, too much happening
• Fatigue, hunger, pain, needing the toilet, constipation
• Being helped with something they believe they can still do
• Late afternoon

Warn before any change. Not “we’re going now” — “in ten minutes we’ll head off,” then a reminder at five.

Rule out the physical first, every time. Pain, infection (especially urinary), constipation, hunger and sleep deprivation account for a very large share of behavioural incidents. A sudden change in behaviour over days is almost never the Huntington’s progressing — progression is slow. Sudden change means look for a cause and report it.

Give them space when they need it, where it is safe.

Do not take it personally, and this is easier said than done when somebody accuses you of stealing. Paranoid ideas in HD are commonly directed at the people providing care, because those are the people present. It is a symptom.

Follow the behaviour support plan exactly. If it does not work, report that it does not work rather than improvising — and note that plans built on teaching the person coping strategies tend not to work in HD, which is useful information for whoever reviews it.

And on apathy, which you will meet constantly: it is a loss of initiation, not laziness and not depression. The person is not refusing; the step from intention to action has been damaged. Start things for them. Do not ask “would you like a shower?” Say “the shower’s ready, let’s go,” with the towel in your hand. The difference in outcome is enormous.

Personal care and moving around

Explain before you touch, every time, even if you have done it a hundred times. Being handled without knowing what is about to happen is frightening and is a common trigger.

Use the hoist if the plan says hoist, and two workers if it says two. Every injury in this work comes from an improvised transfer that started as a reasonable shortcut.

Expect involuntary movement during transfers, and plan for it. Chorea worsens with stress, so a transfer somebody is anxious about is a transfer with more movement in it. Go slower, explain more, and never hurry.

Watch for injury from the environment. People with significant chorea bruise and occasionally fracture against ordinary furniture, bed ends and doorframes. Report anywhere this is happening so it can be padded.

Falls. HD falls are not mainly about balance — postural reflexes are impaired, so a stumble is not corrected, and impulsivity removes the pause in which somebody would normally assess before moving. Which means removing hazards works and reminding somebody to be careful does not. Keep floors clear, keep the route to the toilet clear and lit, and expect the person to stand up and go without warning.

Take the time the task takes. Rushing personal care in HD produces distress, aggression and injury. If the roster does not allow enough time, escalate the rostering rather than going faster.

Dignity. Knock. Close the door. Cover them up. Clean up without commentary.

Mealtimes: the part with the most risk

Read this section twice.

They need more calories, not fewer. People with HD typically need very high calorie intakes just to hold their weight, because chorea burns energy continuously and there are metabolic changes on top. Full-fat everything, fortified food, supplements between meals, snacks in reach. Never put somebody with HD on a reduced-calorie diet, and query it if somebody has.

Slow the pace — this is the main safety issue. Impaired inhibition means people with HD frequently eat too fast and take mouthfuls that are too large, cramming food in and taking the next bite before the first is cleared. Combined with an unreliable swallow, that is the mechanism behind most choking episodes. What works: small portions refilled rather than a full plate, one mouthful at a time, each one swallowed before the next, and you present and paying attention.

Quiet. Television off, one conversation, fewer people. Distraction stops the meal.

Positioning. Sitting upright with proper support, chin a little down and the head not tipped back. Keep them sitting up for half an hour afterwards.

Follow the mealtime management plan exactly if there is one — no variations, no “just a sip of water,” no family member’s preferred method over the speech pathologist’s.

Weighted cutlery is the most useful piece of equipment and dampens involuntary movement considerably.

Weigh them if asked, on the same scales at the same time of day, and record it. A documented weight trend changes clinical decisions. This is one of the most valuable things a support worker does in HD.

Report the same day: new coughing on food or fluids, a wet or gurgly voice after swallowing, food catching, meals being abandoned, weight loss, or a suspected chest infection.

And know that silent aspiration happens. No cough does not mean it was safe.

Independence and dignity

Prompt rather than do. If they can do a step once it is started, let them.

Ask before changing anything. How they like their coffee, where things sit, the order personal care happens in. In a condition that removes choices steadily, these matter out of all proportion.

Offer choices physically where speech or decision-making is hard. Hold up two shirts.

Keep the routine identical. Same order, same time, same words. In HD this is a clinical intervention, not a habit.

Keep the environment consistent. Same place for keys, wallet, glasses, phone.

Expect the “drunk” problem in public. Chorea, slurred speech and an unsteady gait look like intoxication, and people with HD get stared at, refused service and occasionally stopped by police. If you are out with somebody, you may need to explain. Ask the family whether they have an explanation card and carry one. Handle it calmly and in front of the person rather than over their head.

And talk to them like an adult. About the football, the weather, your week. People with HD are frequently fully aware of what is happening to them and consistently say that what they miss is ordinary conversation in which nobody is assessing them.

Restrictive practices

Know what counts, because HD generates these more than most conditions.

Five practices are regulated: seclusion, and chemical, mechanical, physical and environmental restraint. A locked front door or kitchen is environmental restraint. A lap belt or harness may be mechanical restraint, as distinct from prescribed postural positioning. Medication given to manage behaviour rather than treat a diagnosed condition is chemical restraint — and in HD this is genuinely nuanced, because antipsychotics are legitimately prescribed for chorea and for psychotic symptoms as well as sometimes for irritability.

Any of these requires a behaviour support plan written by an NDIS behaviour support practitioner, authorisation under state law, monthly reporting to the NDIS Quality and Safeguards Commission, and a strategy to reduce and eliminate it. Using one without authorisation or a plan is a reportable incident.

What this means for you: if you are being asked to lock a door, use a belt, hold somebody, or give a medication to settle behaviour, ask to see the plan and the authorisation. If they do not exist, say so in writing to your supervisor. That is not being difficult; it is the law, and it protects the person and you.

What to escalate, and how fast

Same day:

• Any sudden change in behaviour or cognition over days — look for infection, pain, constipation, a medication change
• New or worsening coughing on food or fluids, or a wet voice after swallowing
• Suspected chest infection
• A fall, or a near miss on a transfer
• Weight loss
• New injury or bruising, including from furniture
• Expressions of hopelessness or of not wanting to go on — do not wait, and do not keep it to yourself
• Any new or worsening depression
• Pain not covered by the plan
• A seizure, in anybody with juvenile-onset HD

Within the week, in writing:

• Function that has changed since the plan was written
• A behaviour support strategy that is not working
• Equipment that no longer suits the person
• Anything the person has told you they want that is not in the plan
• Rostered time that is not enough for the tasks
• Your own training gap on a task you are being asked to do

Report rather than absorb. If you are being asked to do a high intensity task — enteral feeding, dysphagia support, complex bowel care, seizure support — that you have not been trained and signed off for, say so in writing and do not do it.

Looking after yourself

HD support is hard in particular ways and it helps to know why rather than treating it as a personal failing.

You will be accused of things you did not do. You will have the same argument forty times. You will do everything right and get no response, because apathy looks exactly like indifference. You will work with a family who are exhausted, grieving, and sometimes at genetic risk themselves — and the anger in that household will occasionally land on you. You may support somebody for years and watch them lose everything. And your best work, the incident that did not happen because you noticed the constipation or arrived calmly or did things in the right order, will be invisible to everybody.

What helps:

Understand the mechanism. Workers who know that an accusation is a paranoid symptom, that apathy is a frontal lobe problem, and that perseveration is impaired task-switching, last considerably longer in this work than those who take it personally. That is not a platitude; it is the best predictor of who stays.

Debrief. Your supervisor, the team meeting, or somebody you work with at the end of a rough shift. What ends careers in this job is carrying it home on your own.

Keep your own boundaries. Consistency includes being consistent about limits.

Report rather than absorb. Being abused, threatened or put at risk belongs in an incident report and with your supervisor in writing, not in your own head.

Ask for HD-specific training in writing. This condition needs particular skills, and asking on the record is reasonable and creates a trail. Huntington’s Australia on 1800 244 735 provides information to workers, not only to families.

And notice what you achieved. A shower that happened without a fight. A meal eaten slowly and safely. An infection caught on a Thursday. A weight recorded that changed a treatment decision. An argument you did not have. A family who got an evening. Nobody outside the house will see any of it, and it is the whole of the work.