Motor Neurone Disease is a heart-breaking condition that affects an entire family and for which at the time of writing this blog there is no known cure, when one is first diagnosed with this dreadful illness it is as if a bolt of lightning has suddenly emerged from what were clear blue skies to strike at the very core of one’s heart with all the force of a nuclear warhead. Subsequently those clear blue skies are gone and all you are left with is a darkness hanging above one’s head, much like the fabled sword of Damocles.
There is at present around 5,000 people in the United Kingdom who suffer from this horrendous condition with new cases diagnosed every single day. Motor Neurone Disease is classed as a rare condition, We are aware that there will be people out there reading this blog that have no understanding of what Motor Neurone Disease actually is so let me try and elucidate.
MNDs roots lie in the early 1800s when Sir Charles Bell made the discovery of the sensory nerves and the Motor Nerves, but it was not until 1874 when French physician Jean-Martin Charcot, a pioneer in the field of neurology made the link to a group of conditions which affect the nerves in both the brain and the spinal cord; this condition later became known as Amyotrophic Lateral Sclerosis (ALS) /Motor Neurone Disease (MND). In the human body we have what are known as Motor Nerves these nerves carry the electrical messages to the muscles that allow said muscles to contract, in our daily lives we tend to move around a lot and we tend not give a second thought as to how this occurs.
With Motor Neurone Disease the motor nerves begin to malfunction to a point that they eventually stop functioning hence the electrical messages are no longer sent to the muscles and because the muscles are no longer receiving these messages over time muscles become weaker and eventually muscle wastage begins to occur. Motor Neurone Disease is not one condition it is a title that is given to a group of diseases; these diseases include Amyotrophic Lateral Sclerosis (ALS, Progressive Muscular Atrophy (PMA), Primary Lateral Sclerosis (PLS) and Progressive Bulbar Palsy (PBP).
The most common form of Motor Neurone Disease and the one that most people may be aware of is Amyotrophic Lateral Sclerosis of because of Professor Stephen Hawking who was diagnose with this form of Motor Neurone Disease in 1963 at the tender age of just twenty-one and at that time was given a prognosis of just two years life expectancy, Professor Stephen Hawking has so far defied the odds and is still with us today at the age of seventy-five.
However, I digress from the reason for writing about Motor Neurone Disease, In April 2017 the woman of whom I gave my heart and soul to was diagnosed with the form of Motor Neurone Disease known as Progressive Bulbar Palsy (PBP) with PBP it is the stem of the brain that is affected as well as the nerves associated with the bulbar muscles, the bulbar muscles are what we use for swallowing, eating, chewing and speech. As a result of this speech is slurred making it somewhat difficult to understand and swallowing food can also be troublesome which can result in choking so a person’s dietary requirements have to be adjusted to accommodate this.
Progressive Bulbar Palsy also tends to cause issues with the breathing muscles, this means that the breathing muscles become weaker and allows for a build-up of Carbon Dioxide in the body which as we know is unsafe so regular checks are performed to monitor the increases in Carbon Dioxide levels, but unlike Amyotrophic Lateral Sclerosis, Progressive Bulbar Palsy does not tend to affect the limbs although over time Progressive Bulbar Palsy can turn into Amyotrophic Lateral Sclerosis which does.
We were advised by the hospital to watch for signs that Carbon Dioxide building in her body these signs include headaches in the morning and feeling sleepy during the day, if we notice any of these signs we have to contact the hospital, of course this could be something simple like she is just tired, but in my option it’s better to be safe than sorry. Sadly the life expectancy of someone that has Progressive Bulbar Palsy is between Six months to Three years.
Motor Neurone Disease destroys lives; it is a little known disease that has such a big impact on a family. More money is needed to not only help fund research into the condition that will one day vanish this horrible disease once and for all, but also to help families who suffer from Motor Neurone Disease. If you wish to help you can help by making other people aware of the condition or you can make a donation to the Motor Neurone Disease Association…
MND, The pain behind the laughter 