Motor neurone disease (MND) is a fatal, rapidly progressing disease that affects the brain and spinal cord. It attacks the nerves that control movement so muscles no longer work.
It does not affect the senses but can leave people locked in a failing body, unable to move, talk and eventually breathe. Some people may experience changes in thinking and behaviour, with a proportion experiencing a rare form of dementia. Currently it is incurable.
Here, Ammar Al-Chalabi (pictured), director of King’s MND Care and Research Centre, shares his expertise on the latest research, challenges and breakthroughs for people with the condition.
The search for a treatment
The underlying aim among researchers is to understand the causes of MND. If we can work out why it happens, we might be able to find a treatment.
We’re also looking into why it progresses more rapidly in some people than others. Th is may lead to a treatment which is able to at least slow its progression down. A lot of our research is genetics-based and there are quite a few studies which give us optimism in the search for a cure.
These studies are helping us to better understand the mechanism of MND and therefore increasing our chances of being able to treat it at some point in the future.
Outlook for people with MND
MND kills a third of people within a year and more than half within two years of diagnosis. An estimated one in 300 will die of the disease. Over the last decade we’ve found survival rates have improved slightly, but this is measured in weeks and months rather than years, so it is barely noticeable.
Strong multidisciplinary teams are shown to improve outcomes. In practice, this means care is managed by neurologists in conjunction with palliative care specialists, physiotherapists, dieticians, wheelchair therapists, clinical nurse specialists, care coordinators, respiratory consultants and occupational and speech and language therapists.
In our team at King’s we also involve a volunteer who has had first-hand experience of MND through a family member or loved one. is is important because they understand what the patients are facing in a different way from healthcare professionals.
In the last 15 years we’ve started intervening more quickly with non-invasive ventilation. Previously, if someone had respiratory failure, we would give a treatment to stop them feeling distressed about being breathless but it was not straightforward to offer any practical solution to the breathing difficulty, and so they would die soon after.
Now we are able to use a portable machine that can support breathing and take away breathlessness by putting air into their lungs. is relieves their symptoms for an extended period and gives a better quality of life for longer.
There is some evidence that it may even prolong survival. We also now intervene more quickly with nutritional support. ere is a lot of research going on currently into whether this needs to be a specific type, such as a high-calorie diet, for example. Hopefully we will get a greater understanding of the impact of diet on the condition in the coming years.
MND and the brain
We are now recognising that in up to 30% of people there is some involvement of thinking. MND can affect word finding and impact on personality in quite subtle ways; o en by making people more stubborn. As a result, patients may interact with relatives differently and may also reject healthcare options.
There is a lot of exploration going on into gene therapy. Although only about 5% of people have a family history of MND, there is probably a genetic component to the condition in everyone. By affecting the genetics, we may be able to slow the disease down.
Other strategies include trying to improve strength regardless of affecting the underlying disease. Increasingly we are seeing new drug treatments designed to make muscle contractions stronger. Another approach being looked into is immune therapy. ere is some evidence that inflammation might influence the disease’s progression. If you can affect the immune system, we might be able to change the course of the disease.
MND and NHS cutbacks
MND is an extremely distressing condition and you need time to be able to deal with all the multiple problems that patients face. When people are given the diagnosis it is a life-changing and usually very distressing experience. ere are many time pressures on people in the NHS.
So we have competing demands and it can be challenging to spend enough time with our patients to support them through this difficult situation.
Although the risk of developing MND is about the same as that of developing multiple sclerosis in the UK, MND affects life expectancy, so it is not as common. It is therefore perceived as a rare disease and it can be di cult to get funding for vital research. In the UK we’re fortunate to have the support of the Motor Neurone Disease Association, which is extremely effective and supportive of patients. Crucially, it also funds a lot of our research into MND.
Generally patients are extremely motivated to take part in research. Sometimes families can be reluctant to agree to get involved if there may be a genetic basis to their family’s condition, as that can have significant implications for them.
Raising public awareness
Perceptions and awareness of MND change depending on where you are in the world. In the UK, public awareness was very low but improved a lot with the Ice Bucket Challenge.
The problem with this viral social media campaign however, was that it referred to ALS rather than MND. A lot of people in the UK didn’t realise it was the same condition and donated funds to charities in the US.
Symptom variation over time – learning in real time from lived experience
In the UK, the average patient will see a GP around seven times a year, according to the Royal College of GPs. This is a rise from 20 years ago, when a patient would see their family doctor around three times a year.
With an average appointment time of 9.2 minutes, British patients see their family doctor for less time than patients in the United States, Sweden, Canada, Spain and Japan.
There are 8,760 hours in a common (not leap) year. In the UK, patients have about one hour per year on average with a UK GP. During the other 8,759 hours, symptoms come and go over weeks or months, so what is recalled at the consultation may not be accurate. Such symptoms sometimes just go away, but at other times these symptoms are a sign of something that needs treatment. We are often told “if it doesn’t go away within x days, see your GP”. Yet 2020/1 has made this more challenging to do, whether virtually or face-to-face.
So much to say – so little time
There’s the challenge of getting the phone call answered at the GP surgery, getting a consultation time that suits and the anxiety of getting there and waiting for your time slot. How easy is it to forget some of the detail of the illness? How can doctors solve problems with imperfect information and poor communication as well as recall error? Is “doorknob syndrome” becoming a greater problem?
Healthwatch UK advises: “Before you see your GP, think about what you want to say. You can always write down your symptoms, worries and any questions that you would like to ask, so you don’t forget.”
Similarly, Cancer Research UK and other organisations provide tips to help patients to get the most from such precious but brief appointments. Top of their list are:
- Write down your symptoms including when they started, when they happen and how often you have them.
- Write down anything that makes them worse or better.
How many patients do this, or have the capability or opportunity to do it even more effectively?
How can doctors feel empowered when their time is so pressurised, when there is a tsunami of data, information and knowledge let alone a mountain of paperwork and covid precautions on top of that? How can patients feel empowered when it is so difficult to see a doctor when they are most worried about their signs and symptoms?
COVID-19 makes things more complicated. Patients with multiple symptoms that vary from day to day find it especially hard to give a clear and concise summary. Even worse if they have fatigue and brain fog. Recall error is more of a problem. Could the patients be more empowered?
Symptom-tracking and real time data
There is a conundrum – how do we empower the patient-doctor relationship and strengthen the therapeutic alliance when there is less face-to-face time and longer waiting lists and more complex conditions? Achieving any of these might be easier if there was an increase in consultation time.
This draws attention back to the advice to patients to prepare to get the best from their consultation, how can digital technology help a good proportion of patients to do this ahead of the consultation? It is accepted that all patients will not use technology.
Accurate responses to questions about when symptoms started or when they happen and how often they occur could become digital real time data – provided this is sufficiently easy and well-structured. The acceleration of public health awareness and communication between doctors and patients via NHS Digital tools regarding symptoms has been a legacy of covid-19. While accepting that all patients won’t need or want digital, empowering those who do want or need it can relieve some pressure on the system as a whole and, over time, more will embrace it.
TIYGA™ – time is your greatest asset
So how do we manage to utilise time with GPs, nurses and specialists more efficiently? Patients and doctors must use their time together more productively and anything that can be captured, measured, calculated, and displayed in the right way ahead of the consultation must happen. Healthcare professionals are trained to deliver consultations, but patients are not trained in how to prepare to see different healthcare professionals with different specialities. In these time-poor days, it is more important than ever that patients present the right data to the right person at the right time and in the right place.
Personalised medicine is increasingly important in preventing, predicting, and managing many health conditions and we don’t just mean at genomic level, but more holistic treatment and accepting that not all one size fits all. In 2019, the UK had 55.5m smartphone users and this is expected to rise to 64.9m by 2025. According to Statista, in 2024, the smartphone user penetration rate is expected to reach 88.1 per cent of the total UK population – 93.7 per cent in 2025. In 2020, 70 per cent of 55–64-year-olds owned a smartphone. How can we justify not making better use of this high adoption rate?
Talk to many clinicians and they’ll tell you that all their patients don’t have a smartphone and don’t have access to broadband. In 2020, the overall share of households in the United Kingdom (UK) that had access to the internet was 97 per cent. The overall share of households in the United Kingdom (UK) with internet access was six percent higher than the average of the European Union (EU-27).
The challenge is not in the technology. We send people into space, we replace human vital organs, we can replace joints and even create and successfully rollout vaccines to fight novel virus at record speed. Do we encourage people to use popular consumer devices to record simple, routine health data in a way that can help our healthcare professionals to get more efficient input to consultations?
We will always need GPs, and nobody expects every poor pensioner to become a geek but symptom-tracking by smartphone could reduce the time many patients need to spend with a GP, fewer consultations might be required to make a diagnosis and the first consultation at secondary care could be more efficient. Taken together, these factors could reduce some pressure on waiting lists.
- By Katrina Delargy of TIYGA Health
Who am I? Self-identity following an Acquired Brain Injury
Dr Anita Rose, consultant clinical neuropsychologist at Renovo Care Group, explores the issue for NR Times
Who am I? Arguably life’s most defining problem. The answer as we all know is identity. But this is not the complete answer because “who I am”, my identity, is an all-encompassing system of memories, experiences, feelings, values, roles, thoughts and relationships.
When I am asked who I am my answer is likely to be multi-faceted and the answer will depend on the situation I am in. I might say. I am a mum, a wife, an Ouma (Grandmother), a sister, a clinical neuropsychologist, a friend, a regular church goer and so the list goes on.
This question permeates my working life as I sit with patients who want to know who they are now, how they can be who they used to be. They are scared, frustrated, anxious and believe that they can no longer be “me”. They face what the world calls an “identity crisis”. They are looking backwards prior to the traumatic, sudden, life changing experience they have gone through to this moment in time, to now after the brain injury.
This exploration highlights a gap, the gap between who they were before to who they are now. It is this gap that leads to the questions of “Who am I now?”, “What can I do now?”, “Where do I fit in?”.
Prior to their injury they had roles, “identities”, felt valued, helpful, independent, and confident in their life’s purpose. But now they report feeling lost, useless, frightened, insecure, stupid, lazy, and unreliable.
The gap seems insurmountable as they will report feeling they have gone from being kind, caring, and happy, to angry, sad and being a burden. These feelings are driven by the comparison of self-perceived capabilities pre- and post-injury. Such comparisons lead to the experience of “I am not me anymore” as the brain injury at a deeper level alters the sense of self and the qualities that define who they once were.
When I first started working in neuropsychology many years ago, I found the concept of loss of identity a real challenge. How can I support those whose lives have been dramatically changed “in an instant”, who maybe now very physically disabled and cannot return to their employment, their sport, their previous life?
I noted that many times individuals would turn to those around them to feed their self-worth. They would seek reassurance and praise from others to feel okay about themselves. Whilst in the moment such validation might support their emotional wellbeing, dependency on external validation is extremely detrimental.
Often patients would be perceived by caregivers as presenting with behaviours that challenge because they might be seen as demanding, for example constantly buzzing their call bell for reassurance. The reality is that sense of self should not come from other people but come from within. The most important relationship we can have is with ourself.
Over the years of clinical work, participating in research and listening to my patients stories I have learnt that the only way to support a person to develop that new sense of self is to recognise each patient brings with them their own unique life history, values and social context.
I have found that for many of my patients it has been about working together in a collaborative and supportive therapeutic relationship, a collaboration where the individual in is the centre. Based on mutually agreed goals we start out on a journey of rediscovery.
The initial stages of the journey are about grief and loss and enabling someone to express their grief and being given permission can be very powerful moment. The next stages are often around building awareness of the results of the brain injury as much raising self-awareness of the “me before” and the “new me”.
What is different? What is still the same? Can we build the bridge between the two parts of “me”?
Moving along the journey we start to look at the most important relationship in their lives, the one with themselves. When self-identity is challenged the ability to be compassionate towards one’s self is reduced. Finding news ways to promote self-compassion i.e. kindness and understanding towards one’s self can support the individual in finding meaning in their lives and achieve their personally valued goals.
Kenneth Gergen (1991) said: “One’s identity is continuously emergent, re-formed, and redirected as one moves through the sea of ever-changing relationships. In the case of ‘Who am I?’ it is a teeming world of provisional possibilities.”
The journey can be full of mountains and valleys, monsters to face but in my work at Renovo Care Group we recognise the various challenges these cause both the individual and their families.
We appreciate the need for a directed multi-disciplinary approach to work collaboratively with our patients by supporting them to re-engage in meaningful activities and to find meaning in their life following their brain injury. In other words, we are trying to re-establish the “bridge” across the gap between old and new. We support the patient along the journey leading to the “new me” after brain injury and look at how a comprehensive IDT evaluation and treatment of the “damaged sense of self” can lead to increased self-esteem, self-efficacy, and self-empowerment. All this leads to an improved quality of life for both our patients and their families.
But to end, it is the patient voice that speaks the loudest. A recent patient after going through this journey spontaneously said in his last session, whilst leaving the therapy room, “I have re-found myself”. He may still be profoundly disabled as a result of his brain injury and not met his original goal to walk again, but he has found an identity, a new sense of self that has emerged and reformed as he has found reconciliation between his self “before” and his “new me”. A bridge has been built across the gap.
It is for this reason that I find my role, and the role of the IDT (Interdisciplinary Team), in neurorehabilitation so fulfilling. As a patient learns to be self-compassionate, moves towards reconciliation and acceptance of the “new me”, regardless of whether or not their original goals have been met, the end of their rehabilitation process has been positive as they leave feeling empowered and able to believe they have a world of “teeming provisional possibilities”.
The importance of continuing rehabilitation in the home environment
The team at Neurokinex share insight into the role of rehab at home and how to maximise its potential
Community-based rehabilitation centres are powerful tools which can support you in your recovery journey.
As well as offering you access to expert advice and guidance, specialist equipment, novel and intensive training modalities, you will have the opportunity to receive client-centred sessions as part of structured rehabilitation programmes supported by neurorehabilitation experts. Together, they create the optimal environment for you to work towards goals and maximise your recovery potential.
In-centre rehabilitation sessions are a must have for any rehabilitation plan: those who can, should attend as much as possible to maximise their progress. However, for many people, barriers – such as travel and funding – stand in the way of them attending for the advised and required frequency. It is likely, therefore, that this time constitutes just a fractional part of the overall time most individuals should be committing to their rehabilitation.
Research has consistently shown that both exercise intensity and high repetition are essential to maximising recovery following neurological injury. Therefore, individuals should look to establish a strong home exercise routine as part of their rehabilitation plan to maximise on these principles. An established routine will also maximise the benefit of your other therapy sessions, keep you focused, active and committed to your rehabilitation journey.
However, building and dedicating to a home exercise programme can be challenging. Here is some advice from our team at Neurokinex for upgrading your home exercise programme based on our experiences with clients.
“What should I be doing at home?”
Firstly, make sure that your home exercise programme contains everything it needs.
Many of you will already be following a rigid and intensive stretching programme and this should continue to formulate the core of your home exercise routine to help manage spasticity and contractures. Dedicate time to moving your body into different positions, prone is great, which can be effective in allowing muscles to be stretched for prolonged periods of time and in alleviating pressure.
Standing should comprise a proportion of your daily routine. Standing for 30 to 60 minutes a day can help with bladder and bowel function, joint range of motion and bone demineralisation, along with promoting improved quality of life.
Decreased activity also increases the risk of developing cardiovascular disease and diabetes, therefore it’s important to find a way to incorporate some regular aerobic exercise to benefit cardiometabolic health. One guideline suggests 30 minutes of moderate to vigorous exercise three times per week.
Last but not least, dedicate additional time to completing any rehabilitation exercises prescribed by your trainer.
“The requirement seems like a lot, how do I fit it all in?”
Fitting all the above into your daily routine might feel impossible alongside incorporating the additional exercises prescribed by your trainer. The best way to start is by identifying if any of your rehab exercises can be integrated into time spent doing other things.
A couple of examples of this would be completing your shoulder or hand exercises whilst in your standing frame. Or completing your upper or lower limb exercises whilst spending time prone or as part of your morning stretching routine in bed. This would help maximise your efficiency, achieve those all-important repetitions without the feeling of having to go out of your way to put aside too much extra time.
Just like your sessions with your trainer, home exercise should be effectively programmed and this can be another helpful way to save you time. As well as helping you remain engaged and feel like you are progressing.
Typically, you’ll have many areas that you wish to improve on with many associated exercises to do. It is easy to fluctuate between these if you don’t have a plan and though rarely are these goal areas mutually exclusive, it is important to work each area independent of the other.
We often prescribe clients five exercises, taking no more than 30 to 45 minutes a day, that can be progressively tracked over four to eight weeks. This helps to promote overload, adaptation and better tracking of progress towards the goal area. It also means that you set a point of review and importantly introduce variation to keep things fresh and exciting.
Some common short-term goals that we use with clients include increasing the number of repetitions, timed seated or standing balance and physical exertion tests, such as a five-minute time trial.
“I over-did it last week”
Consistency beats intensity. Often, clients feel the need to work themselves to the maximum every session. Though this approach can produce short term results, it usually leaves them exhausted and they rarely maintain consistency long enough to see significant change.
Remember we’re playing the long game here. Our advice is to choose an exercise routine that you can execute even on your worst week.
“I’m worried about doing exercises incorrectly”
An unfortunate side effect of client-centred exercise is that people can become nervous to perform exercise without the keen eye of a trainer by their side.
If this is you, start with a few exercises that you are comfortable with and have completed in your rehabilitation sessions. Follow this up by challenging yourself to try a new exercise, film it and take it to your next therapy session for your trainer to okay.
Nine times out of ten they’ll be delighted you’re trying new things!
“I can’t remember what I did”
Keep a log! The most important piece of advice that we can give you is to start keeping a diary. In it note the rehabilitation you do each day and reflect upon how it went. Don’t forget to include your achievements and any significant milestones. This will form an essential part in your rehab journey and help you to stay committed and invested.
Most importantly, take this with you to your next session and show your trainer all the great rehab that you have been up to. We want to hear that you are continuing your rehabilitation at home.
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