Towards a life worth living with Motor Neurone Disease

Photo: Eva Sundin with granddaughter - please do not re-use/republish this photo.

A late afternoon in October 2020, I recalled the neurologist's utterance, 'You have Motor Neurone Disease, Mrs Sundin… Are you sure you don't know what it is?'. I had been in a happy mood before the appointment, but that all changed when I understood that I have a life shortening illness.

Going back a few weeks, my Multiple Sclerosis (MS) specialist had told me that my voice problems were not due to acid reflux and probably not related to the MS I had lived with for some 30 years. When I read the letter that invited me to another appointment in the Neurology Department, I felt relieved. I would finally get some clarity. Surely the new condition was something like MS, which I managed with physical exercise; it was just a matter of finding the right techniques. When the neurologist informed me that ALS (Amyotrophic Lateral Sclerosis) is the most common type of Motor Neurone Disease (MND), I knew better what he was talking about.

I was unable to make sense of it all, and froze like a rabbit in the headlights. As a practising psychologist I worked with people who had experienced unwanted life events and reacted with hyperactivation (fight or flight) or numbness (freeze). This gave me some comfort, especially when combined with frequent communications with my family.

But my freeze reaction lasted, fuelled by unsuccessful attempts to get answers from the neurologist on questions that I had been unable to ask during the appointment. My family and the Motor Neurone Disease Association's website gave me some answers; for example: there is no diagnostic test for MND and therefore the clinical examination is a crucial component in the diagnostic assessment. Other questions remained unanswered, including which of my signs and symptoms that were indicative of MND and not of MS.  

My daughter suggested that I could request a second opinion from an independent neurologist. I welcomed the idea, and two months later, a second opinion appointment took place. At the start of the appointment, the neurologist invited me to describe my own health concerns, and throughout the clinical examination that followed she talked to me about her observations, some of which pointed to MND, and others pointed to MS. When the neurologist said that I probably have MND, although additional tests were needed for a conclusive diagnosis, it fitted with what she had told me during the clinical examination.

I felt a bit more ready to move on towards accepting that I have the disease. I began to read up about it and found that MND is characterised by many stressful uncertainties. This echoed Lazarus and Folkman's postulation that uncertainty goes hand in hand with stress. I found that I was not the only one who:

• had to live with MND a long time before I was diagnosed (event uncertainty)
• didn't know how quickly my MND would progress (temporal uncertainty)
• didn't know what muscular function I would lose next (event uncertainty).

Other stressful things, those of us living with MND can be certain of, include that little can be done to slow down disease progression and MND invariably has a fatal outcome (efficacy certainty).

Help from family in a changing reality

I tried to think about how I would manage a changing reality, move on from leading an independent life, living on my own with my dog, my work, and training six days per week with my personal trainer. So many aspects of my life had to change. Although I tried to take on one aspect at a time there were so many feelings, thoughts and bodily sensations that competed. Fortunately, my family stepped in and helped me engage with planning and putting in place transitions that needed to happen in my life. My daughter said to me 'now that you have MND we must move in together as soon as we can'. Hearing her saying that was one of few things that did not feel surreal.

I knew that family life would offer opportunities, but I did not know how I would be able to make use of them. At Christmas 2020, a few weeks before the third Covid-19 lockdown, I left my home and my dog in Nottingham and moved together with my daughter and her family in London.

It was a joy to be together with my family, although there were challenges too. I had a different role at home than I was used to. This became very clear during the moving days; while the most rational solution would have been to step aside during the unpacking, the endless tape, the boxes, and carrying heavy furniture around, I did not know how I could be actively engaged mentally and not physically. I soon realised that living together with my family afforded me the opportunity to look, through someone else's eyes, at what I had become when I got the MND diagnosis: someone who had fragile connections with herself and her close ones. It took more time than I would have liked to act on this realisation and begin the self-repair.

Professional identity and health professionals

It was useful to remember that when I am experiencing a life crisis I could ask myself 'Who am I?'. I then thought about how important my professional identity is to me. Throughout my career, I have been practicing as a clinical psychologist, seeking to make a difference in the lives of my clients through helping to find new ways through life's challenges. I specialised in encouraging adults and adolescents who struggled with feeling that they had lost their stability in life, helping them to find their purpose through recapturing their rhythm in daily life and rebuilding a frame which ensured that they stayed connected with their close ones while also trying to keep safe distances between themselves and others so they could preserve and develop themselves. I entered this field after witnessing the lack of mental health services organised to provide comprehensive services that allow people to preserve their mental health and avoid mental illness. I also built my career choice on my own experiences of attracting MS in 1984, which impacted on my health for over a decade before getting the diagnosis. The initial signs of the neurological illness were muscle fatigue and repeated optical nerve inflammations which impacted my energy levels, eyesight, and well-being. As neurological examinations did not identify the illness that caused the health problems, I lay focus on building my family life and professional career. I became a clinical psychologist, which was the platform that I then built my professional career from. It also helped in my work on creating a life worth living, first with MS and later with MND.

But it has been difficult for me to understand and accept myself as a person with MND, and therefore to build a life worth living with the disease. When I took up Acceptance and Commitment Therapy (ACT) it provided an opportunity to learn new ways of relating to myself and understanding my thoughts and behaviours. It was not possible to put MND on hold while I sorted out myself; during the first year after I learned that I have MND I lost my ability to speak, swallow, stand up and walk. It was a year full of taxing experiences for me and my family.

It was also a time when professionals in my MND team had a critical role. When swallowing became difficult, I had the benefit of having a speech and language therapist who talked to me about my experience of the problem, tested my capability to swallow, and encouraged me to make my own decision about eating. This psychologically informed intervention provided a safe space which helped me to find myself and to choose to go through with the insertion of a feeding tube, and a bit later give up eating and drinking while I still had a choice. I had a similar experience when I was hospitalised with a chest infection and had meaningful dialogues with a respiratory physician during ward rounds, which made it easier for me to think about using a non-invasive ventilator before it was medically necessary. These experiences helped me remember that I am a person who prefers to make her own decisions about her life, rather than being governed by experts. Because I am living together with my family, I have lots of opportunities to recognise that another of my most precious values is to behave caringly towards myself and others.

A better understanding

Another component of my work on finding my feet with MND is gaining a better understanding of the illness. For that purpose, I asked my MND consultant, Dr Cathy Ellis, to share her knowledge about the condition. Cathy wrote the following paragraphs, which I edited slightly.

MND is a life limiting neurological condition where nerves supplying muscles (the motor nerves) degenerate for reasons that are not yet fully understood. This leads to progressive weakness and wasting of muscles, which causes increasing disability over time. The lifetime risk of developing MND is 1 in 300, which is similar to the risk of developing MS. However, because MND progresses rapidly in some people, there are fewer who live with MND at any one time than with MS. This means that fewer people have personal experience of anyone with MND and people's general understanding of the condition is limited.

MND starts in different body regions, for example it may start in the legs, manifested in tripping, or may start in the speech muscles, initially showed as slurring of the speech. Each individual with MND has their own journey, and not every muscle is affected in everybody. In approximately 70% of people, weakness starts in the arms or legs, and in about 25% it starts in the muscles of speech and swallow. In a small number of people, MND presents with weakness of the respiratory muscles. Over time, everyone with MND will experience weakening in the respiratory muscles.

The loss of muscles and basic functions causes grief. Anecdotal evidence suggests that not every person with MND grieves the loss of specific muscles to the same extent. For example, a person whose MND starts with speech problems may hope that they will not need a wheelchair while someone whose symptoms starts in the legs may put hope on not losing their speech. One person with MND said: "my speech was the first area to be affected. I feel fortunate that I've still got my mobility because for me that's my independence."

My reading about MND showed that there has been a growing understanding of the condition recently, including its impact on psychological wellbeing. This has led to development of evidence-based standards of care that improve survival and quality of life in many countries. In England, NICE guidelines for assessment and management of MND includes instigation of multidisciplinary teams with neurologists, a dietitian, and allied health professionals, maintenance of weight and nutritional status, and early management of respiratory dysfunction with non-invasive ventilators. But NICE guidelines offer only general guidance for psychological treatment.

I therefore invited my neuropsychologist, Dr Masuma Rahim and her colleague, Dr Lorraine Haye, to discuss psychological treatment for people with MND. In the following, Masuma and Lorraine's reflections are merged with my thoughts about the role of psychological treatment for people who live with the disease.

MND is a condition with extremely distressing features. It is well known that stress impacts wellbeing and cognitive functioning, including decision making. In the case of MND, research shows that stress jeopardises treatment adherence, if left unmanaged. Psychological treatment promises to contribute to improved stress management and therefore to prolonged life expectancy. However extremely scarce research that evaluates the outcome of psychological therapies for MND has been published. Up until 2021, only ten outcome studies (of which three were randomised controlled trials [RCTs]) were published compared to more than 200 RCTs of psychological treatment for MS, which has similar prevalence to MND.  

Evidence from MND outcome studies has shown that

• the time period between symptoms first manifesting to receiving a confirmed diagnosis is often extended and might lead to crisis reactions which can hamper acceptance of the diagnosis and treatment adherence. Psychological support which addresses reactions to the unwanted news should be offered, including support in advanced decision-making.
• throughout the disease progression, stress, anxiety, depression, and hopelessness are more common than in the general public. Psychological treatments that address these problems should be offered to every individual with MND, not only to those with a known mental illness;
• psychological treatment for people with MND needs to flexibly adjust to the individual's needs that might fluctuate over time as the disease progresses;
• psychological treatments that support development of mindful acceptance of the disease and facilitate living with personal identity changes can boost treatment adherence and life quality

Treatments that have been trialled, with favourable results, include Mindfulness-Based Stress Reduction (MBSR) and Cognitive Behavioural Therapy (CBT). Acceptance and Commitment Therapy (ACT) may be another option but it has not been tried empirically with MND until now. However a large clinical trial of ACT adapted for MND, the COMMEND project, is currently being carried out across England, with the results due in Autumn 2023. In the project's first phase, ACT was adapted so that it would address the needs of people with MND. The adapted form of ACT was found to be acceptable for 29 people with MND. In the second, ongoing phase, 188 people with MND are chosen at random to either have ACT plus usual care or usual care only. The trial will find if ACT can improve quality of life in people living with MND and if ACT for MND is good value for money. For now, 159 people have taken part in the trial and we can also take a look at feedback from Jennie Starkey who has received ACT for MND: "ACT has helped me so much. Mainly to accept the way I feel – acknowledge it, but not hooked and caught up in my emotions. It is enabling me to mainly live without the future ruining the present."

Summing up

People who live with MND face a range of unique and severe challenges. Since there is no effective treatment to halt disease progression, care is focusing on boosting treatment adherence and preserving life quality. Psychological treatments must be offered to each individual who lives with MND, at every stage of the disease.

Very few empirical studies have been devoted to psychological health and treatment for people with MND; more research is needed to examine impact, coping and adjustment, and wellbeing across the illness. A handful of clinical trials have shown that mindfulness-based therapies such as MBSR and CBT can be useful for people with MND. Anecdotal evidence, including mine and Jennie Starkey's experiences, suggests that ACT is a promising approach. Hopefully the evidence from the COMMEND trial will encourage the use of ACT and a wide variety of other psychological treatments for people with MND.

• Clinical Psychologist Eva Sundin ([email protected]), Dr Cathy Ellis, Clinical Psychologist Lorraine Haye, Clinical Psychologist Masuma Rahim and Ms Emily Richardson

References

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