Motor neurone disease at home: staying one step ahead

Nobody can tell you the order in advance. The NHS says the symptoms affect everyone differently, that a person may not get all of them, and that how quickly they get worse varies from person to person. Some notice a weak hand or a foot catching on the stairs, and for others it starts with slurred speech. A plan built around a fixed sequence is the wrong shape, so what works better is knowing what to ask for at the first sign of each change.

That is where families lose time. A referral has to be made, an assessment booked, equipment ordered and delivered, and each step is measured in weeks while the condition is measured in the same weeks. NICE asks repeatedly for things to happen without delay, including equipment, wheelchair referrals and communication aid assessments, which tells you how often the two clocks fall out of step. What complex care means sets out the wider picture.

The MND team is how any of this gets moving. NICE says that at diagnosis the person should be given a single point of contact for the specialist team, and told what to do if there are concerns between appointments, out of hours, or if equipment fails. The team usually reviews things every two to three months, and NICE asks for a way to bring an assessment forward when something changes.

Speech changes because the muscles used for speaking weaken, and it can happen at any point. NICE asks for speech and communication needs to be assessed by a speech and language therapist without delay, and for that to cover the telephone, email and messaging as well as talking in person.

Two things sit under the heading of communication aids. The first is voice banking, which means recording a person's own voice while it still sounds the way they sound, so a speech device can later use it rather than a synthetic voice. It cannot be done once speech has gone. The second is the aids themselves. NICE covers low technology such as an alphabet or word board, and high technology such as a tablet that speaks what is typed. Where something complex is likely to be needed, including eye gaze, NICE asks the team to refer to a specialised NHS communication aid hub.

1. 1

   Ask for the speech and language therapist now

   At the first change, or before

   Ask the MND team or the GP for a referral, and say you want it before speech becomes difficult rather than after. NICE asks for it without delay, which is worth quoting if you have to chase it.
2. 2

   Ask about recording a voice

   While speech is still clear

   The therapist will explain what is involved and whether it suits your relative. Several short sessions work better than one long one.
3. 3

   Ask whether a specialist hub referral is needed

   Before the equipment is urgent

   Where eye gaze is likely to be needed, NICE asks the team to refer to a specialised NHS hub. Ask early, so the referral is in before the need arrives.
4. 4

   Keep something simple in the house, and show everybody

   Alongside anything electronic

   An alphabet or word board works when a battery is flat or somebody is too tired for a screen. NICE asks for ongoing training in using it for the family and carers, so build that into a new carer’s first shift.

Talking to somebody who uses a communication aid asks something of the listener too. It is slower, and the temptation to finish a sentence is strong when everybody is tired. Giving them the time to finish is part of the care, and worth saying to a carer.

The early signs are ordinary enough to be explained away: coughing on a drink, a meal that takes an hour when it used to take twenty minutes, weight coming off without anybody trying. NICE asks for weight, diet, fluid intake, feeding and swallowing to be assessed at diagnosis and at every team assessment, or sooner if anybody is concerned, and for a clinical swallowing assessment when problems are suspected.

A feeding tube does not have to replace eating. Many people keep eating what they can manage and use the tube for the rest, which takes pressure off the mealtime and off the person watching. It is also a decision that gets harder the longer it is left.

If a tube is placed, the feeds and flushes that follow are a clinical task. A registered nurse has to decide it is safe for that person, train the carer on your relative's own equipment, watch them do it and record that they are competent. Who can train a carer to do a PEG feed sets out who may hand that task over. Ask the community team before you book anybody, because the answer changes what you need.

The muscles used for breathing can weaken like any others, and the first signs are easy to miss because they look like tiredness. NICE lists breathlessness lying flat, daytime sleepiness, fatigue and an early morning headache among the effects. It asks for breathing tests soon after diagnosis to set a baseline, then roughly every two to three months.

Where the tests and symptoms suggest somebody would benefit, NICE says to offer a trial of non-invasive ventilation: a machine supporting breathing through a mask rather than a tube in the windpipe, usually started at night. The decision is made by the MND team with the respiratory ventilation service. Before it starts, NICE asks for a written care plan covering review, device maintenance, round-the-clock emergency support, secretion management, training for the person and the carers, what happens if the equipment fails, and night-time help for somebody who cannot remove or replace a mask.

The point of that list is not to make a carer sound limited. Breathing support at home works when the boundary is written down and everybody has read the same document. Our complex care pillar says the same about ventilator support: only with specific training and a written care plan from the respiratory team. If more clinical input is needed, nurse-led or carer-led complex care sets out what each covers.

The clinical side comes from the NHS and is free: the specialist MND team, the district nurses, the physiotherapist, the occupational therapist, the speech and language therapist and the dietitian. NICE names those in the core team, along with a professional who has expertise in palliative care, which here means symptom control and quality of life.

The paid care at home is separate, and the one a family organises and usually pays for until funding is agreed. It tends to begin with help at the hardest part of the day, then a second visit, then nights, and for some households live-in care or a team. 24-hour complex care teams sets out the shapes that can take, and transfers, hoists and two carers covers when a job needs two people.

When you are ready to look at paid care, you can search live-in and hourly carers near you and compare their rates by postcode and message the ones whose experience fits. Carers write their own account of the conditions and tasks they have worked with on their profile, and their reviews sit there too. What PrimeCarers checks before a carer appears is their identity, their right to work, an enhanced DBS on the Update Service, and an online interview. We do not check qualifications, training or references, so experience with this condition is for you to ask about and the clinical team to assess.

NHS Continuing Healthcare is health and social care arranged and funded by the NHS for people whose needs are primarily health needs. It is free and not means tested. The ordinary route is a checklist and then a full assessment by a multidisciplinary team, with a decision usually within twenty-eight days.

The fast-track route is different. Where an appropriate clinician judges that somebody has a rapidly deteriorating condition that may be entering a terminal phase, they complete a Fast Track Pathway Tool, and that document by itself establishes eligibility.

1. 1

   Ask the clinician who knows your relative best

   Consultant, MND nurse, GP or hospice clinician

   Only an appropriate clinician can complete the tool: under the National Framework, a registered nurse or medical practitioner responsible for the diagnosis, treatment or care of the person. Anybody else involved can raise it with them.
2. 2

   Ask before the need is at its worst

   In anticipation, not after the event

   The Framework says somebody may show few symptoms now and still meet the criteria where rapid deterioration is expected in the near future, and that the tool may be used then rather than later. It also says rapidly deteriorating does not mean a set time remaining.
3. 3

   Expect the package to be arranged quickly

   Usually within 48 hours of the tool arriving

   The integrated care board must accept and immediately action a properly completed tool, and the Framework says commissioning the care should not usually take more than 48 hours.
4. 4

   Ask who will provide the care

   The part families are surprised by

   Eligibility decides who pays, not who comes to the house. Ask the board what it will commission, and whether a personal health budget is possible so you can keep carers your relative knows.

Fast-track continuing healthcare covers the route and what to do if it is refused, and personal budgets and personal health budgets covers taking the funding as a budget you arrange.

The other decisions worth making while communication is easy are the legal ones. An advance decision to refuse treatment is legally binding in England and Wales when it is valid and applies to the situation, and NICE asks for people with motor neurone disease to be offered the chance to talk about one, and to review any they have, when a feeding tube or ventilation is planned. A lasting power of attorney is separate, and worth doing at the same time because it has to be made while somebody has the capacity to make it.