Providing bespoke care nationwide for clients with spinal injuries and neurological conditions
Motor Neurone Disease is a chronic, degenerative condition which affects the nerve cells that control movement.
Motor Neurones are the nerve cells in the brain and spinal cord that are responsible for movement control. In Motor Neurone Disease, these nerve cells become damaged, and this damage is irreversible and worsens over time. Without healthy motor neurones, we lose our ability to control movement.
At the start of the disease, this may have a minimal effect on people. In about two thirds of people, the first symptoms are some weakness in the arms or legs. Over time, MND begins to affect all movement. This includes all movement of the limbs, and progresses to restrict a person’s ability to speak, swallow, and breathe.
Everyone’s experience of MND is a little different, and the pattern of disease progression may be slightly different from one person to another. The speed at which the disease progresses can also vary significantly – some people have very swift disease progression, and reach the end of their lives within only a few months.
Others may have very slow disease progression, and sometimes find that their symptoms plateau for periods of time. The average life expectancy after diagnosis of MND is around 3 years[i]. There are a few people who have lived for many decades after a diagnosis of MND, with some supportive care and life adjustments.
Motor Neurone Disease is a disorder that affects movement. In the early stages, it can present as weakness of the muscles. Some of the first symptoms someone with MND might notice include:
As the disease progresses, the symptoms worsen. Over time, weakness progresses to total lack of control over movement. People with MND lose the ability to walk, talk, and look after themselves without considerable help and support.
The muscles that control breathing and swallowing are affected just as much as those that control mobility. The effect of the disease on these functions is usually the cause of decline at the end stage of MND. Without effective control over breathing, coughing and clearing the throat, respiratory problems can develop quickly. With a compromised ability to actively or reflexively swallow, nutrition and hydration can become a serious issue. Swallowing difficulties combined with reduced lung function make a person at very high risk of aspiration pneumonia.
We know that some – around 10%[ii] – of people who develop MND have a genetic predisposition. People with this form of MND in the family may be offered genetic counselling – this means they can be offered a test to look for genes known to be associated with the familial form of MND. They will receive support and counselling for themselves and their family if they are found to be at high risk of developing MND.
For the 90% of cases of MND that don’t seem to follow a genetic pattern, it isn’t usually possible to anticipate the development of the disease. There are lots of theories and research is ongoing into the different effects that some viruses, inflammatory responses, environmental toxins and other possible culprits might have on the motor neurones. There is not, however, a single clear explanation as to why some people develop MND.
MND is more likely to develop as we get older, and most cases are diagnosed when people are in their 50s or 60s; the average age of diagnosis is 55[iii]. People with familial MND usually begin to develop symptoms a little younger, in their 40s to 60s.
Some of the earliest symptoms of Motor Neurone Disease can overlap with the symptoms of other conditions, so some investigations are needed before a definite diagnosis is made.
There is no one simple test that gives a definitive diagnosis of MND, so a diagnosis is reached by collating different pieces of information[iv]. This may include:
This can look for other causes of MND-type symptoms, like infections or inflammation affecting the brain and spinal cord.
There is no cure for Motor Neurone Disease. It is a progressive disease which gets worse over time, and it is eventually fatal. The progression of the disease cannot be reversed, so the main aim of treatment for MND is to promote good quality of life for as long as possible throughout the disease, and to control symptoms. Having discussions early about what treatment would be appropriate or desirable as the disease progresses can help loved ones make difficult decisions.
Any disorder which significantly impairs a person’s ability to move, communicate, eat and drink, and independently respond to their body’s own needs comes with a high risk of causing certain other problems and conditions. Treatment for MND largely takes the form of symptom management, good nursing care, and the prevention of associated problems. Some of the issues that a person with MND might be at high risk of include:
Having a ventilator will not stop or slow the progression of the disease, but can help to bypass some of the breathing problems that come with MND, controlling some symptoms and supporting quality of life for as long as possible. It’s a good idea to have discussions about what level of breathing support would be appropriate and acceptable at the latter stages of MND.
In the later stages of MND, care may be referred to as ‘palliative care’; this means that the aim is symptom control, comfort, and support for the person with MND and those close to them. Any symptoms a person experiences at the end stage of a terminal disease can be well controlled so they remain comfortable and calm as they reach the end of their life.
Receiving a diagnosis of Motor Neurone Disease can be overwhelming; it means a huge change in the way we live and in the lives of the people closest to us. A diagnosis of a terminal disease raises uncertainties and questions – from the emotional, psychological and physical changes to expect, to the practicalities of reaching the end of a person’s life.
Conversations about the later stages of MND can be difficult to begin. It is hard to talk about terminal illness, death, and the reality of living with a disease that will become so completely limiting. It can be very helpful for everyone involved, however, if a person’s wishes surrounding their care and treatment in the later stages of the disease are shared and understood[v].
Motor Neurone Disease is a terminal disease which severely limits a person’s function, independence, lifestyle, and longevity. The kind of care and support a person with MND needs will change over the course of the disease. Generally, at some point after diagnosis, someone with MND will begin to need some minimal assistance with their symptoms; this might be mobility or communication support. As the disease progresses, and towards the later stages, carers – whether family, formal carers, or a mix of both – will be needed for support with all activities of daily living.
People with MND can expect to have ongoing support from teams made up of specialist doctors; nurses; dieticians; occupational, physical, and speech and language therapists; clinical psychologists and counsellors, and more.[vi] In the later stages of the disease people with MND usually have significant or total care needs, and a formal care package is usually necessary. The complications that can arise with movement disorders – continence issues, risk of pressure damage, nutrition and communication challenges – require very specialised care, and some people need support round-the-clock to help keep them safe, comfortable, and content in the later stages of their disease.
Family members and close friends often take on an increasingly demanding role in the care of a person with MND – the gradual progressive nature of the disease means that it can be difficult to pinpoint the exact time when an informal carer needs more support. Arranging a care package early helps ensure that no one reaches a crisis point before they receive the support they need. The difference a supportive full-time care package can make to quality of life for everyone affected by MND is immeasurable.
[i] Turner MR, Parton MJ, Shaw CE, et al (2003) Prolonged survival in motor neuron disease: a descriptive study of the King’s database 1990–2002 Journal of Neurology, Neurosurgery & Psychiatry;74:995-997. https://jnnp.bmj.com/content/74/7/995
[iii] Azam, S & Leigh, P.N. (2007) Motor Neuron Disease and the Elderly. Geriatric Medicine https://www.gmjournal.co.uk/media/21414/mar07p61.pdf
[vi] Leigh PN, Abrahams S, Al-Chalabi A, et al (2003) The management of motor neurone disease. Journal of Neurology, Neurosurgery & Psychiatry74:iv32-iv47. http://dx.doi.org/10.1136/jnnp.74.suppl_4.iv32