Doctors must grasp the key elements of living with progressive disability

All doctors receive some training in physical disability, but many will not come face to face with the realities. Managing people with increasing disability is done largely outside hospital by a multidisciplinary team. Even as a physician working in rheumatologya specialty allied to rehabilitationI was amazed to discover the range of expertise available in the community for people who are on an inexorable downward trajectory. The experience of having Motor Neurone Disease has provided insights into optimal management of chronic disease and disability which would have been helpful earlier on in my career. This journey has been informative, and surprisingly, not entirely negative. 

I was fortunate to receive a clear diagnosis and an empathic guide to the prognosis at the outset. This was crucial to predicting the likely trajectory. Getting it right at the initial consultation, with time for respectful, truthful, and sensitive communication, conferred huge dividends further down the track. Although it was made clear there was no effective medical treatment, the value of management by the community teams was emphasized as a positive therapeutic approach. “Good doctoring,” with the doctor being effective “medicine,” treating the “person” not just the “condition,” allowed my family and me to “get our act together” and make the most of our time. 

The advice to consult a hospice at the first post-diagnosis neurology consultation came as a shock, but it was clear that once the diagnostic ball had been set rolling, the ending was both clear and inevitable. It was reassuring that the hospice itself had much to offer in terms of palliative care. 

While I was still ostensibly healthy, a referral was made to a specialist respiratory team.  Although my respiratory function was normal, a non-invasive ventilator (NIPPV3) was provided and advice to have a Percutaneous endoscopic gastrostomy (PEG) given. Both seemed very premature or even unnecessary at the time, but of course, both eventually became essential. I was grateful to have the opportunity to participate in a clinical trial, which allowed me to continue to contribute, even though not in the way to which I had been accustomed.

An occupational therapist (OT) recommended dramatic changes to my home to be made straight away and put the wheels in motion to communicate with a variety of other colleagues and companies. Her blunt honesty with the much to be repeated phrase “You don’t need it now, but you will,” persuaded me to plan realistically. The OT became the team leader for my community care, as a single, invaluable point of contact, coordinating the involvement of others and the provision of equipment.  

A footdrop led the physiotherapist to advise use of a walking stick and footdrop splint. These were adopted only after several falls. When discussing stairs, my expectation of a stairlift was resolutely ruled out. Only a “through floor lift” would accommodate me throughout the whole disease course. After careful consideration, the lift was installed, just as I was no longer able to climb the stairs.  

The prescription of a manual wheelchair was a shock to the system, but was the necessary prelude to the provision of an individually designed, electrically powered, indoor/outdoor chair which was delivered several months later. Within a short while it was essential and an extraordinarily positive means of maintaining mobility; but it did however require the acquisition of a wheelchair-accessible vehicle.  

The bathroom required substantial modification by a specialist disabled bathroom team. The bath was declared redundant in favour of a shower. I was given the option to consider an automatic wash and dry lavatory. I had never heard of one, but was told that they were “de rigeur” in many other countries. A Swiss device was selected which turned out to be a valuable addition to our home over the course of my illness. Predictably, by the time the work had been completed, it was necessary.

Since the onset of MND, a remarkable amount of equipment has been provided by the local authority, including hoists, a shower chair, suitable cushions, a hospital bed, and a riser-recliner armchair. In addition to input from the OT and physiotherapist, the dietician and speech therapist were also crucial, arranging swallow assessment and voice banking in collaboration with the MND Association in case a speech synthesizer should become necessary. The assistive technologies unit provided computerised technology for operating mobile phones, TV, emergency alarms, and positioning of the bed, which can be operated by mouse or eye contact. Inexpensive smart voice-activated devices (Alexa/Siri) and mobile phone Apps have been invaluable in playing audio books, radio, and television, as well as providing an intercom. This greatly helped independence and provided reassurance.

The intensity of the heavy burden born by a loving spouse cannot be overestimated, but it was inevitable that carers would be needed eventually. Gradually increasing support from an independent carer agency has allowed some respite from unremitting hard physical work and allowed family involvement to continue in a manageable way.   

I was surprised by much of what I have learned, on a personal and professional level, not all of it bad. Important elements have been gained thanks to the phenomenal amount of support which underpinned my resilience and determination to continue to live a rewarding life. The increased opportunities to spend time with family and friends, often taken for granted in normal life circumstances, became of immense importance.

Being managed by clinicians at several hospital sites and in the community, made communication potentially challenging, but the community services formed a cohesive MDT with a clearly identified “lead”, streamlining communication, minimizing hospital visits and timely actioning aspects of the joint agreed care plan. Nevertheless, it was apparent that the hospital and community services operated quite separately, with potential challenges. 

The pandemic has changed the way that hospital services have had to work. Telephone communication rather than face to face clinics, have been a distinct advantage. With increasing disability, where no physical examination is needed, it was helpful not to attend hospital clinics, with the attendant practical difficulties and added risk of exposure to covid-19.  

Clarity about the disease course early on has two advantages. Firstly, in addition to the practical measures described above, it provides the opportunity for family and friends to come to terms with the reality and become a support “bubble.” Moreover, involving a hospice enables access to ongoing day to day care, with streamlined communication with the local authority and General Practitioner (GP). As a palliative care team, the hospice professionals working with the GP can ensure that appropriate medication is available for use at times of distress and extremis.   

Many other experts are involved but we, as hospital doctors, should fully grasp the key elements of dealing with evolving disability, if we are to give realistic, truthful information and support to our patients and their families. Of key importance is mutual trust between doctor and patient, with adequate time in consultations and access to a wider team for all concerns to be addressed. 

Andrew Keat is a retired consultant rheumatologist and has motor neurone disease.

Liz van Rossen is a consultant physiotherapist.

Competing interests: none declared.