Working with motor neurone disease

Catherine Turnbull writes: Around two people in every 100,000 of the general population will develop motor neurone disease (MND) each year; a progressive and ultimately fatal condition.  

This means many physiotherapists may only come across a few people living with the disease during their professional lifetimes, unless they specialise in MND, or work in palliative care, neurology or a respiratory team.  

However, as we speak with physiotherapists who work as members of primary care teams with patients with MND, it is evident that physios make a great difference to the quality of life of people with the disease – often up until their death – by providing treatments, care, symptom management and support. 

Consultant physiotherapist in respiratory 

Rachael Moses is consultant respiratory physiotherapist at Lancashire Teaching Hospitals NHS Trust. She works with a wide spectrum of patients who have respiratory muscle weakness and need long-term ventilation to survive. She works with a home ventilation service and is a specialist in complex ventilation including weaning, invasive and non-invasive ventilation (NIV), airway clearance and tracheostomy care.  

The NICE clinical guideline around use of NIV in MND states that multidisciplinary teams (MDT) should coordinate and provide on-going management and treatment for patients, including regular respiratory assessment and provision of non-invasive ventilation,  and that the team should include a specialist physiotherapist. 

‘We offer a gold star service for people diagnosed with the most devastating disease on the planet,’ Rachael says. ‘They can visit our clinic, where a respiratory specialist carries out an assessment. And the MND physio is a super specialist and aims for home care as far as possible.  

‘Identifying respiratory failure and surveillance monitoring is important, and we can do this using remote technology. We are one of the centres leading on this in the UK.’ 

She adds that a respiratory physiotherapist may also give advice on positioning, breath stacking, or assisted cough techniques to try to prevent rapid decline and respiratory emergencies that may normally require intubation. 

One of the most challenging areas Rachael leads on is ventilation withdrawal.  

‘Patients do sometimes ask us about assisted dying and travel to Switzerland, but we reassure people that we can provide supportive holistic care alongside palliative care and specialist MND teams.  It is important to know that people can always be in control of their healthcare decisions and this means deciding they no longer want to continue with assisted ventilation. Sadly some people do see elective ventilation withdrawal as euthanasia but it’s not, it’s simply a patient removing the consent they give to have home ventilation.’ she says.  

‘In this type of scenario, MND patients ‘pick a death day’ and we work together with supporting teams to ensure ventilator withdrawal is carried out peacefully giving someone a dignified death which is so important to us. 

‘Many patients don’t want to go on ventilation, but it’s very important that we have conversations about advanced care planning as well as dying as part of our consultations,’ Rachael explains.  

We have to be clear about what the patient wants early on because, as MND progresses, people may develop communication problems

NICE guidelines for MND (NG 42, Feb 2016) advocate offering people the opportunity to discuss their preferences and concerns about care at the end of life, at trigger points such as: at diagnosis, if there is a significant change in respiratory function, or if interventions such as gastrostomy or non-invasive ventilation are needed.  

The guidelines also advise that clinicians should be sensitive about the timing of discussions and consider the person’s current communication ability, cognitive status and mental capacity.

Advance Decisions to Refuse Treatment (ADRT) and Do Not Attempt Resuscitation (DNACPR) orders, and Lasting Power of Attorney should be explained. 

‘I’ve led on elective ventilation withdrawal,’ Rachael says. ‘Some people want to plan their death day and the location. There is a lot of emotion for the team and the carers and families, which is when the palliative care team really helps. 

‘I find it is important to hold my emotions together for the patient. All my MND patients will die and feeling emotion is what makes us human, so I may need to let the tears go when I am outside of the patient’s home or hospital.’  

MND specialist physiotherapist 

Hospice physio

Sue Rossitter works with Emma Hartley as joint day therapy team lead at Arthur Rank Hospice Charity in Cambridge and they manage the physio team who work across three clinical areas - inpatient unit, day therapy and community (specialist palliative care home team), as part of MDT support to patients.  

The physios have close links with the MND clinic and physiotherapy team in the community, managing neuro patients, so are often able to provide continuity to the patients. 

The MND clinic is run by a team at Addenbrookes Hospital, but they use the outpatient facilities at the hospice once a week.  

‘It helps MND patients and their families to acknowledge that this is a palliative condition and address specific rehabilitative palliative care needs,’ Sue says.  

‘We pride ourselves on having a holistic approach- considering the whole person, physical, psychological, spiritual and social.’ 

Royal Papworth Hospital teams are the respiratory experts in the region and their physios assess needs and provide teaching and necessary equipment for these patients but the physios at ARHC support with breathlessness management and knowledge of technology, such as manually assisted cough, LVR (lung volume recruiter) bags to facilitate breath stacking, cough assist, non-invasive positive pressure ventilation (NIPPV) and suctioning. 

‘We also advise about and deliver exercise and passive movement programmes – we have a gym with Medimotion bikes – and our physio in the lymphoedema clinic can advise on the management of dependent oedema. We work closely with OTs and advise on fatigue and anxiety management,’ Sue says.  

‘And we can offer advice about collars and equipment to facilitate mobility, and offer pain management with positioning/posture, equipment and acupuncture. 

‘As physios we offer continuity to patients along their pathway and use the rehab palliative care approach to facilitate the patients in parallel planning, managing current and future issues.'

Staff are supported by close MDT working, they can attend a neuro MDT case review monthly at ARHC and have access to the CiC external counselling service, staff wellbeing support and mindfulness sessions. 

‘It’s really important when you are working with life-limiting conditions that the staff have access to support too,’ Sue adds. 

The hospice’s CEO Sharon Allen says that one of the most important, valuable and valued features of hospice care is the multidisciplinary team (MDT) approach, focused on the whole person – their physical, psychological and emotional self and their family and friends.  

‘Our physiotherapy colleagues are essential in this MDT, bringing their specialist skills, knowledge and experience related to movement, exercise and advice for people newly diagnosed with a life limiting condition.  

‘They give hope, encouragement and holistic support to people to live well, as much as they support people at the end of their life.  

‘At Arthur Rank Hospice Charity this is particularly through their leadership of our day therapy and lymphoedema services and we’re proud they are part of #TeamArthur.’ 

Palliative care 

Emily Stowe is a specialist physiotherapist in palliative care working in a hospice. She is also chair of The Association of Chartered Physiotherapists in Oncology and Palliative Care (ACPOPC), one of the CSP’s smaller professional networks.  

The network provides peer support, ideas and knowledge exchange, while providing opportunities for continuing professional development. 

The network is research-active and influences cancer and palliative care policy and strategy at both local and national level, including clinical guidelines for NICE. 

‘ACPOPC provides an opportunity for networking and support.’ Emily explains. 

‘As hospice physiotherapists we get support within our MDTs, but it’s important to meet up or share ideas with other physios, as we are often working alone or in small teams within our hospices.  

She adds that the network strives to help its members with self-care. 

Nothing quite prepares you for the complexities of working with patients with MND, whose condition can progress and deteriorate quickly - It’s emotionally challenging,’ she says. 

‘I’ve been working in palliative care for 10 years and have seen that some clinicians need time to talk, some need time and space and others need to let out their emotions in other ways. 

‘In the hospice situation we work in small teams and are affected together, and there is support and a level of acceptance. We look out for our colleagues. 

‘It has helped me personally to see patients through to the end of their journeys, when appropriate, even just to sit and chat or visit to say goodbye. It helps me process their death and can also provide comfort to the patient and their family, who have built up trust in me over time.’ 

Emily previously worked at St Clare Hospice in Harlow, Essex, where she typically saw about ten people a year with MND. 

‘We had a holistic approach to assess all their needs and practiced rehabilitative palliative care, right from diagnosis.’ 

The patients attended specialist MND centres at Addenbrookes in Cambridge or Barts in London, but relied on local teams to provide physiotherapy in between their specialist appointments, which might involve positioning, exercises, respiratory interventions, aids, support and end of life care, in the hospice or at home. 

Now, as Emily prepares to begin work in another hospice, she says: ‘I am lucky to have found a speciality I love and which is so rewarding.’ 

A patient’s experience: Len Johnrose  

Len Johnrose - a former midfielder who made more than 400 Football League appearances for Blackburn Rovers, Hartlepool, Bury, Burnley and Swansea City – has spoken publicly about living with the condition since he was diagnosed in March 2017, and has a campaign to raise awareness of MND. 

Len, 50, was working as a primary school teacher in Burnley, Lancashire, when he realised something was wrong.  

‘Around 2014, I had been experiencing slight weakness in my right hand,’ he says.   

‘I had a number of tests which all proved inconclusive.  A year or so later, I broke my left hand. After a few months it still hadn’t healed. Particularly in the cold weather, I was struggling to grip things. I was referred to a physio who massaged and manipulated it. This would ease the stiffness, but only for a couple of hours.’ 

A few months later, while playing golf, his right hand seized up. He was referred to a consultant who referred him to a neurologist in July 2016, a year after he broke his left hand and two years after he first experienced weakness in his right. It is often difficult to make a diagnosis of MND, as there is no specific test. 

‘I was finally diagnosed in March 2017, although at that point, after exploring other avenues, we had already come to that conclusion,’ he remembers. 

‘The consultant was fairly clinical with his diagnosis, and we were signposted to the MND team in Preston, which is really helpful.  About a week after diagnosis, I was introduced to the physio – Amy Parkes. 

‘For the first few sessions, Amy was more like a confidante. Physically, I was okay, but mentally I was completely shot. She was absolutely amazing; not at all prescriptive but provided me with what I needed at that time. When we began the physio sessions, she assessed my range of movement, my gait and my posture. She then planned treatment around these issues.’ 

Amy left the MND team about six months later and, since then, he sees a physio on request when he needs to. Len has acupuncture for pain relief and has begun a series of massage, which he plans to continue for as long as he is able.  

‘This involves trying to improve my range of movement, looking at trying to improve my posture, and pain relief of specific areas,’ he says.  

‘My legs are really stiff, and the massage relieves some of that stiffness. Also, the main pain that I am experiencing is in my right bicep, and again this helps with the pain.’ 

He set up the Len Johnrose Trust and Project 92, which aims to visit 92 football clubs and share information to players about links between MND and contact sports. Research led by Mike Hutton, consultant spine surgeon at Exeter in Nuffield Health Exeter Hospital and Royal Devon and Exeter NHS Trust, suggests those playing a top-level contact sport and sustained repeated blows to the head and spine throughout their career are more than eight times more likely to develop the condition. 

Freelance physio working with MND Scotland 

MND Scotland advertises a physio service that allows people to self-refer to provate physios or may be referred by an MND specialist.  

One of these is Giridhar Ravi (known as Ravi), whose core job is working as a Macmillan physio at Highland Hospice in Inverness, and who sees private patients in the evenings and at weekends. 

‘Some people with MND don’t wish or are unable to attend our day therapy service, outpatient service or in-patient service at the hospice.’ 

‘Some prefer to see a physio in their homes. I see patients, who have self-referred through MND Scotland and help shorten the waiting list.’ 

Ravi travels to patients’ homes in the Highlands, within a 20-mile radius of Inverness. His main contact during private patients’ treatment is the specialist MND nurse.  

‘I see some patients as part of my hospice job and I have many years of experience in end of life care. However, one of my biggest challenges, with either hospice or privately referred patients, is managing their expectations. They often hope that I can fix things, but the nature of the conditions I work in are hard to fix, mainly with patients with MND.’ 

Ravi’s first task is to explore the patient’s aims and make a management plan.  

‘It’s hard, but I often have to bring down their expectations so I can ensure they understand the limitations they face,’ he explains. 

‘I help them maximise and maintain their function as far as possible, but also help to understand their limitations. Sometimes it may be telling someone they will never walk again.’ Ravi mainly works on moving and handling to maintain mobility as well as planning ahead.  

‘Sometimes the condition will deteriorate faster than expected, and it is important that the patient has equipment they require at home in place. I contact the MND nurse with updates and to make appropriate referrals.’ 

The later stages can be challenging, Ravi says, as hospice patients can become breathless and anxious and often feel frustrated and angry.  

‘Sometimes I’ve known a patient or a long time, from the outpatient stage to end of life. 

‘But we can’t afford to allow emotion to cloud our judgment, as we need to help other patients and stay clear and focussed. We support each other at the hospice and have debriefs. And we aim to make death a positive experience. 

‘I see my hospice role then as making the patient as comfortable as possible. In a way it is not natural for a physio to see their patient decline, as usually the aim is to fix things to improve/maintain mobility and independence. But that’s not the case with MND.’ 

Ravi says the key to his work is communication and listening to what the patients’ goals are.  

‘Learning to listen is something we can struggle with as clinicians. I have worked in palliative care for over 11 years and I am still learning – and learning to listen to understand rather than just listen to respond.’ 

MND Scotland is looking for more physios to help provide freelance services to people with MND in their homes. 

The organisation also offers a professional development grant, which allows professionals to meet the costs of attending relevant MND-related conferences and training courses, study days through an education service and books, journals and audio-visual materials through its library service. mndscotland.org.uk 

The MND Association