Motor Neurone Disease (MND) is a progressive neurological disease that attacks the motor neurones / nerves in the brain and spinal cord – meaning neurological messages stop reaching the muscles – which can lead to muscle weakness and wasting.
It is generally considered a disease of adulthood (diagnoses peak from approx. 50 – 70 years old) but age of onset can vary significantly.
Motor Neurone disease symptoms present and progress very differently for each person living with Motor Neurone Disease. If you read up on Motor Neurone Disease; it is unlikely that you will experience every symptom listed on reputable websites such as www.IMNDA.ie or www.rmn.ie. It is unlikely that you will be able to predict the way in which your symptoms will progress. Sometimes, reading around the condition can be overwhelming as “Motor Neurone Disease” really is an umbrella term to describe a number of different types of conditions which are broadly described as “Motor Neurone Disease”. Your Neurology team will explain more to you about the particular type of Motor Neurone Disease that you present with.
A Speech and Language Therapist helps people with MND to address and compensate for communication changes
Different subtypes of MND can include but are not limited to
Further information on the subtypes of MND is available from the Irish Motor Neurone Disease Association www.imnda.ie.
The onset of Motor Neurone Disease can vary significantly. Some people may experience tingling or weakness in their hand, others may notice slurring of their speech or the onset of swallowing difficulties or changes in saliva management. Everyone experiences Motor Neurone Disease differently.
There are a number of other conditions that present similarly to Motor Neurone Disease; these will need to be excluded as part of working towards an accurate diagnosis. For example; in certain cases fasciculations can be “benign” and caused by other conditions.
Fasciculations are a particular type of visible, involuntary and irregular muscle twitching or contraction of muscle fibres that can be experienced by people who are living with MND. They are the result of the ongoing disruption of nerve signals to the muscles).
Speech and Language Therapists often play an important role as part of the team working towards a diagnosis of MND. This is because for 1/3 of “Classic MND” cases; there is a bulbar onset – meaning muscles of speech and swallowing are impacted first or the initial symptoms present in the face/ head or neck.
If you or your relative has recently received a diagnosis of Motor Neurone Disease; it is recommended that you read this information only as you are ready to do so and it is hoped that this information will prove empowering in terms of what symptoms can be supported and managed.
The causes of MND continue to be widely researched around the world. See www.rmn.ie for the most up to date information on international research taking place in Ireland and for information on the most up to date findings. 90-95% of MND cases are deemed to be of “sporadic” origin. This means we are uncertain of the cause. In 5-10% of cases of MND; the cause is considered to be genetic (13+ genes identified). It is thought that for those with genetic susceptibility that environmental factors may also play a role regarding the onset of MND.
If you have recently been diagnosed with MND; you will be aware that it can take a long time to achieve your diagnosis. This is because there is no “one test” to confirm a diagnosis of MND. On average it can take approximately one year or more to achieve a diagnosis of MND. It can be a stressful process working towards achieving an accurate diagnosis. If you have recently received a diagnosis of MND; you will likely have undergone a number of tests under the care of a Specialist Neurology team. Such tests include (but are not limited to); EMG studies, nerve conduction studies, blood tests, brain scans (to exclude other conditions) and/or lumbar puncture (to exclude other conditions).
If you are living with MND in Ireland; you are not alone. There are approximately 400 people with MND in the Republic of Ireland at any one time. You will be aware that a diagnosis of MND does impact on your life expectancy however the prognosis is different for every person with MND; so it is important to be aware of this.
Approximately 140 people in the Republic of Ireland develop MND each year (IMNDA, 2020).
MND as a condition is experienced differently for each person living with it. It is well acknowledged in the research literature that a diagnosis of MND is associated with significant variation in clinical course. The impact of MND is very individual.
Classic MND (ALS type) accounts for approximately 85% of cases. 1/3 of Classic MND is associated with a Bulbar onset (ie. 1st signs include speech changes and / or swallowing difficulties).
Image courtesy of: www.mndandme.com.au
The symptoms of MND depend on where it starts. Upper motor neurone signs include high tone/stiffness and resistance to movement, speech changes, swallow changes, changes re: breathing system, clumsy hand, tripping, limping, falls and cramping. Lower motor neurone signs can include muscle wasting, muscle weakness and fasciculations (see description above).
MND is also associated at times with “thinking changes”. It is helpful to be aware of these and advise your clinical team; should you have concerns. Speech and Language Therapists, alongside Neuropsychologists, Occupational Therapists, Clinical Nurse Specialists and other MDT members will be able to provide support in terms of strategies to help you to understand these changes and compensate for them. A smaller percentage of people with MND who are living with “thinking changes” experience more significant difficulties with memory and behavioural changes. If you have a strong multidisciplinary team and appropriate person-centred supports in place; it is possible to compensate well and continue to live well, despite these changes.
If you/your loved one is living with MND; emotional support is available and is a natural part of the package of recommended supports for people who are living with MND – should they require it. See www.IMNDA.ie for emotional supports available or Freephone 1800 403403 / email: services@imnda.ie. Your Health Care Multidisciplinary team will be able to access Psychological supports for you also through your clinical teams (Neurology or Palliative Care) or local HSE services. Anxiety, depression and significant emotional burden are commonly experienced by people living with MND and their families. It is important that you seek support when required.
Emotional lability can be associated with laughter or crying that is not in proportion to the underlying mood or emotion. If you are experiencing emotional lability; it can be challenging to control bouts of laughter or crying.
This can happen for some people with MND (associated with function of the prefrontal cortex in the brain). Sometimes pharmacologic or behavioural interventions can help reduce symptoms of emotional lability. It is helpful to be open about emotional lability should you experience it; as help is available from your multidisciplinary team in order to help you to manage these symptoms.
Clinical professionals typically involved in supporting people to live well with MND include (but are not limited to):
Multidisciplinary (MDT) team care has been proven in multiple research studies to enhance quality of life and improve survival for people living with MND. It is recommended that you work with a multidisciplinary team in order to optimise your management of MND.
Early intervention has been shown in the research to have positive impact on symptom management and therefore achieving a diagnosis can actually be a positive step towards getting involved with the appropriate MDT clinics and clincians. Riluzole is an evidence based medication used to treat MND. It works by changing the activity of certain natural substances in the body that affect nerves and muscles. It is commonly known by the brandname Rilutek. Your Neurology Team will be best placed to discuss suitable pharmacologic interventions for your condition.
Collaborative and proactive symptom management is the gold standard for clinicians who are working with people with MND. If you have MND; you will benefit from strong integrated teamwork by your MDT. Your package of care should be tailored to your personal needs and wishes and should help to empower you to live well; despite your diagnosis with MND. Members of your professional clinical team should communicate with one another across sites (eg acute hospital, primary care, home care). Therapists will work with you to support your current clinical needs and in anticipation of potential future needs. Therapists should also work to support family members/relatives re: your condition and how best to offer support. Your wishes and priorities regarding what your needs are will be central to your management.
This may be described as advance healthcare planning
Everyone living with MND is different
Most people who are living with MND will experience some symptoms of dysphagia at different stages of their condition. These can range from mild to severe. Some commonly observed swallow symptoms include (but are not limited to):
A Speech & Language Therapist can work with you & your MDT to help to compensate for these issues; should they arise. Strategies can help to improve comfort, enjoyment & safety when eating & drinking.
Be aware that some literature re: swallowing difficulties in MND will not be relevant to your situation. A Speech & Language Therapist will help to empower you with information that is relevant to your own situation. Try to avoid general online searches where possible – due to the diverse nature of peoples’ presentations – the impact of MND on your swallow needs to be evaluated carefully 1:1 by a Registered SLT.
If you have done your own research on MND; you may be aware of the option of non-oral supplementary nutrition/hydration. A Registered Dietitian; alongside your Multidisciplinary team (eg Neurologist, Clinical Nurse Specialist, Speech and Language Therapist, Physiotherapist) will be able to offer you person-centred advice around the most appropriate options for you; in the context of your clinical presentation, your prognosis and most importantly; your own wishes.
Even if you are not experiencing many speech, communication/language, or swallow symptoms; it is recommended that you work with a Speech and Language Therapist early in your condition, in order to optimise your symptom management and function, to inform you how to self-monitor/compensate and overall; to support maintenance of communication, nutrition and hydration, to help benefit your overall quality of life and to support informed, person-centred decision making by you; around how you choose to manage your condition.
Everyone with Motor Neurone Disease experiences a different combination of impacts and different rates of progression but some of the following are most commonly reported communication changes:
A Speech and Language Therapist will work with you to help you to address and compensate for your communication changes; which can range from very mild to severe.
Even if your speech and communication are very good and your difficulties are very mild, there is value in working with a Speech & Language Therapist as early as possible in your condition; order to help to maintain your communication function regardless of disease progression and in order for options such as voice or message banking to be open to you
A Registered SLT will help you by assessing your communication (both speech and language) and evaluating the best person-centred treatment for your communication changes. This plan will take into consideration your own goals and preferences, your own individual needs and the needs of your family/support network.
A Speech and Language Therapist will empower you to help you to future-proof your ability to communicate. A Speech and Language Therapist will provide information for you and your communication partners on how best to support your communication and on options for forward planning including options such as message or voice banking
Communication intervention for people living with MND is generally compensatory in nature. Behavioural speech intervention can include techniques to optimise the intelligibility of your speech function (eg. over-articulation, optimising respiratory support for speech, environmental optimisation, collaborative work with other professionals on positioning, promoting oral comfort and hygiene, strategies to manage fatigue, educating others re how to support you etc).
Work to optimise your respiratory function completed with the support of a Registered Physiotherapist will compliment work to optimise respiratory support for speech and therefore Physiotherapists and Speech and Language Therapists tend to work very collaboratively with people who are living with MND.
SLTs also work closely with Occupational Therapists particularly around issues regarding optimisation of positioning for communication and facilitating ongoing communication access and environmental control through the use of assistive technology etc.
Some helpful “text to speech” apps include (but are not limited to): Predictable, Gridplayer and Speech Assistant AAC. Your Speech and Language Therapist will help to advise you on high-tech paid for and cost effective options from an assistive technology perspective.
Some people living with MND supplement their communication through the use of lower tech augmentative and alternative communication. This can work very successfully for some people living with MND and their families. Options include (but are not limited to): LCD tablet and stylus, pen and notebook, small whiteboard and marker, alphabet boards and partner assisted tracking. If you require more information about any of these intervention options; it is recommended that you discuss it with your Registered Speech and Language Therapist.
Speech & Language Therapists work alongside people living with MND to ensure their ongoing access to functional communication – so that the person living with MND will maintain the ability to guide and direct their medical and holistic life management, regardless of the stage of their condition.
Speech & language therapists work alongside people living with MND to ensure their ongoing access to functional communication
In cases where a person with MND develops a moderate or severe communication difficulty; a Speech and Language Therapist will help to empower the person with MND and their family/significant others with options around set up and use of augmentative or alternative communication means (e.g. alphabet boards, text to speech electronic devices via headmouse/eye gaze, handwriting props etc).
Not everyone will MND will need to use alternative communication systems; your Speech and Language Therapist will provide more person-centred guidance around the best plans for you. Some people with MND find it helpful to use both verbal and alternative communication systems at once, with the option of text to speech when they are feeling more fatigued for example.
Use your therapy strategies – some of which may include (but are not limited to):
A Registered Speech & Language Therapist will provide bespoke, person-centred recommendations based on your swallow presentation which may change over time. Each person experiences dysphagia symptoms differently and therefore it is essential to consult with your own Speech & Language Therapist for person-centred recommendations that are specific to your own presentation.
A Speech and Language Therapist will help you to empower yourself to optimise your swallow function, efficiency and comfort when living with Motor Neurone Disease. A Speech and Language Therapist will also provide evidence based information for you and your family/friends (as appropriate), on how best to compensate for your swallowing difficulties.
Intervention for swallowing difficulties for people with MND tends to be primarily compensatory in nature. Your Speech & Language Therapist will offer you strategies to help to improve your comfort and safety when swallowing; following a comprehensive assessment.
Your SLT may:
● offer advice re: how to enhance pleasure/variety and choice within your diet.
introduce aids and special utensils to improve your comfort and control whilst eating/drinking/swallowing.
● recommend you to use certain compensatory strategies, posture and positions while swallowing.
● recommend strategies to improve sensory stimulation when swallowing. These strategies may include using hot/cold temperatures to stimulate the senses, increasing flavour, using carbonated fluids).
● recommend strategies to manage fatigue while swallowing and how best to compensate for this.
Provide advice to help support improved saliva management.
Help to support you to make informed decisions (alongside the MDT) regarding eating/drinking.
A Registered Speech & Language Therapist will provide bespoke, person-centred recommendations based on your swallow presentation which may change over time. Each person experiences dysphagia symptoms differently and therefore it is essential to consult with your own Speech & Language Therapist for person-centred recommendations that are specific to your own presentation.
This support includes but is not restricted to;
What is aspiration?
Aspiration is when food, drink or saliva unintentionally enters a persons’ airway or lungs.
What is silent aspiration?
Silent aspiration is when food, drink or saliva unintentionally enters a persons’ airway or lungs without any sensory awareness or response- e.g. coughing, throat-clearing or other reactive response.
What is aspiration pneumonia?
Aspiration pneumonia occurs when food, fluids,saliva or vomit/stomach contents are inhaled into the lungs / airway resulting in infection, which can become very serious if left untreated.
What is a Videofluoroscopy?
A Videofluoroscopy is a moving x-ray of your swallow; conducted by a Speech and Language Therapist and Radiographer/Radiologist. As part of this assessment; you will be given some items to eat and drink. The food and drink will contain barium (either as a liquid or powder). The barium gives a clear picture of what happens to the food and drink when you swallow. It also gives a Speech and Language Therapist information on how best to manage your swallowing difficulties.
What is a FEES Study?
A FEES study is also known as a Fiberoptic Endoscopic Evaluation of Swallowing study which involves passing a thin, flexible camera through your nose in order to view parts of your throat as you swallow. It provides a Speech and Language Therapist with information about how to help to manage your swallowing difficulties.
“I was referred to Professor Hardiman in Beaumont who advised me life wasn’t over and all my plans and life should go ahead and to try and live as normally as possible. Yes, there are changes to what I can do and what I can’t do but I have learned to adapt to each circumstance. Things like sometimes not being able to zip up my jacket, close my buttons or even tie my laces don’t frustrate me anymore. I’ve learned its ok to ask for help. I don’t work now, which was very hard to get used to after working the last 40 years of my life but again I have accepted not been able to lift things and am getting used to not over doing things that would get me out of breath”.
“I continue to live as normal a life as possible and we are busy now planning our wedding for this summer. I try in every way not to let MND change my life; instead I try to adapt to new challenges”
(Written by G; aged 55; living with MND).
“Don’t look down the road at what’s coming. Once you put a dark picture in your head; you cannot lose it. Some people want to know every single thing but if you are like me – you want to meet each day as it happens. Look for the very best you can out of every day. You get really bad days where you say why should I bother and you’ll have days where you’ll be amazed you got up and had a few laughs” (Written by E; aged 70; living with MND).
Advice for other people living with MND from a person living with MND; “When you get down and feel defeated; the next thought in your head should be; I’m damned if I’m going to let this get the better of me!” (Recorded by E; aged 70; living with MND).
“The support of the team of professionals ranging from my Neurology team to the IMNDA Nurse and local HSE Community team has been an excellent support when living with progressive challenges. When I was diagnosed; the prognosis was presented as very poor. Working with the team has given me a sense of hope that there are things I can do to help” (Recorded by P; aged 69; living with MND)
“I found meeting with a Speech and Language Therapist gave me a sense of improved control re: swallowing as I was more aware of how to reduce risks and began to pace myself better. I became more aware of how swallowing and breathing are related” (Recorded by J; aged 75, living with MND)
“Message banking was important to me as it was a way of preserving my own voice for a time when I may need it” (Reported by F; aged 68; living with MND)
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