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.
Taking time to look back – so the way ahead is clearer
Reflective practice within healthcare settings is widely talked about, but not always so easy to implement in the workplace. NR Times speaks to one neurological centre about how it benefits patients and staff there.
Reflective practice and discussion in healthcare settings is a professional requirement for nurses, as laid out by the Royal College of Nursing revalidation requirements as part of their continuous professional development.
It allows professionals to take time to pause and reflect, communicate and plan, which undoubtedly leads to better outcomes for patients and staff.
But in reality, reflective practice can often be left to the bottom of the pile, underneath many of the competing responsibilities facing staff who are often pressed for time.
It could be argued that this is also why reflective practice is so important – healthcare staff are facing so many pressures that it actually makes less sense to neglect the important work of individual and team reflection.
The Royal College of Nursing defines reflective practice as: A conscious effort to think about an activity or incident that allows us to consider what was positive or challenging and if appropriate
plan how it might be enhanced, improved or done differently in the future.
Staff at Elysium St Neots Neurological Centre in Cambridgeshire started doing regular, weekly reflective practices when its new hospital director, Fiona Box, came into the role a few months ago.
The nurses and healthcare assistants from a ward are invited into the meetings and in their absence the therapy staff monitor patients and provide activities.
“We thought it would be helpful for team members to give them the opportunity to think, learn, and to hear their opinions,” says charge nurse Jemima Vincent.
“If we have an incident with a patient, we discuss it in the session” she says.
Sessions are led by the management team, with added input from psychology teams on each ward.
They will talk through any strengths, weaknesses and opportunities, and work through an analysis to learn from the incident and create an action plan.
They talk about the worst-case scenario in relation to an individual situation and discuss how staff would manage that, so they’re better prepared in the event of it happening.
While they focus on one patient at a time, issues arise during conversations that bring in their wider experiences.
In an article* published in the Nursing Times in 2019, Andrea Sutcliffe, chief executive of the Nursing and Midwifery Council said: “In these challenging times for health and social care, it’s so important that collectively we do all we can to support our health and care professionals, and their employers, in devoting time to individual, reflective, personal and honest thinking.”
Fiona has received encouraging feedback from staff, who say the meetings help the staff feel much more involved in a patient’s care and allow the team to increase their knowledge and understanding resulting in a more consistent way of working.
“Healthcare workers often don’t fully understand patients’ diagnoses or why they’re reacting in a certain way, for example,” Jemima says.
“They know a patient presents with certain behaviours and may be taking medicine to help them cope but they’re not aware why the patient is showing signs of aggression and the best response to deescalate the situation,” she says.
“It’s a learning opportunity for staff, because reflective practice means that they can understand a patient’s diagnosis and why they behave how they do,” Jemima says.
“Reflective practice answers their ‘why’ questions, and gives them a more open mind.”
Jemima also benefits from the meetings; it’s a way for her to get to know staff better, especially when it comes to learning opportunities.
“I’m able to understand what level of support each member of the team requires, including training needs and if they need more knowledge on a specific topic.”
In her final year as a mental health nurse student on extended clinical placement at Elysium St. Neots, Jo took part in a reflective practice session.
She had just finished her dissertation, in which she looked at how settings can increase the opportunities and variety of reflective practices within hospital settings.
The aim of Jo’s session was to reflect on the recent deterioration in a patient’s mental state and the resulting impact on their well-being to ensure staff had a consistent approach to support the patient.
The hospital’s director Fiona asked the team about the patient’s care plan, diagnoses and needs and wishes.
Where staff were unsure of the answers to questions, Jo says Fiona gave them answers and encouraged the team to share their knowledge of the patient, problem solve and come up with an agreed plan to move forward with.
Jo found the session helpful and was impressed with how the healthcare assistants were so involved in the discussions about all aspects of the patient’s care, including the more clinical elements.
Healthcare assistants told her they found the session helpful too and that it made them feel like they had a better understanding of the patient’s changing mental state, behaviours and needs.
Jo says having the opportunity to reflect on practice is a crucial skill for all healthcare workers to help them learn from their experiences and increase self-awareness, which, in turn, can improve individual professional standards, strengthen teams and enhance patient-centred care and clinical outcomes.
For referrals to Elysium St Neots Neurological Centre or other Elysium centres visit: www.elysiumhealthcare.co.uk/neurological
Reference source: https://www.nursingtimes.net/news/ professional-regulation/nmc-highlights-importance-of-nurses- reflection-on-practice-18-06-2019/
Robots and resilience at Askham Rehab
NR Times reports on a new rehabilitation approach taking place in Cambridgeshire.
Despite a year of relentless change and upheaval for all involved in neuro-rehab, one provider in Cambridgeshire has been able to keep its ongoing development on track.
Askham Rehab, part of the Askham Village Community, is a recently-launched specialist rehabilitation service incorporating the latest in rehab robotics and sensor assisted technology.
While the firm has invested in state-of-the-art technology to do the heavy lifting, however, its rehab services remain person-centred, as director Aliyyah-Begum Nasser explains.
“We’re a specialist rehab centre in essence, and so, although the robotic technology helps us to get the most out of our patients and staff, we are very much family-focused.
The equipment is obviously fantastic but we know from experience that a person’s mindset, and their ability to sustain whatever improvements they make, comes down to the people who are supporting them – their family members.
“We’ve been on some real journeys with many of our family members who just didn’t understand the impact of a brain injury in terms of how it can impact behaviour or what it can do for cognition.
“Once they understand that, suddenly they become a lot more compassionate, and a lot more supportive; they become part of the recovery process, rather than being a frustrated observer.”
With recognition of the family’s paramount importance to recovery, Askham Rehab does everything within its power to harness this force – including by enabling families to stay together in specially-designed apartments on site.
Aliyyah-Begum says: “The flats are fully adapted, with cantilever cupboards, height-adjustable sinks in the bathroom and full wet room with turning spaces.
“We have the patients themselves participating in rehab, specifically to their programme, but relatives are also there from the beginning, seeing the improvement and being part of our process from the outset.
“We think of the centre as more of a rehab environment; it’s not a just care home with therapy as an added extra.
“So from the minute our patients wake up to the minute they go to bed, everything is based around their recovery goals, and everyone is working together towards achieving them.”
And robotics are an important tool in pursuing these goals through patient exercise. They help therapists to achieve the repetitions and intensity needed to progress their clients, as Aliyyah-Begum explains.
“The point of the robotics is that they respond to the patient. For example, if you set the machine on a left lower limb, but it senses that there is more pressure being exerted through the right limb than the left, it will automatically respond to make sure the patient is moving the correct part of their body.”
The centre’s head of rehab and nursing, Priscilla Masvipurwa, says: “This is a real a game changer in our approach to rehabilitation.
“Robotics help to bridge the gap, increasing the frequency and repetitiveness of treatment, something that’s an essential part of the process.
“We anticipate that this will enable us to support our patients in reaching their goals in a more efficient and sustainable way.
“The centre has so far invested in four items from robotic rehabilitation firm Tyromotion, but is looking to add more over time, as the benefit to both staff and patients becomes ever more evident.
Aliyyah-Begum says: “It’s really important to the team at the centre that the robotics aren’t just seen as an add on.
“There is a lot of nervousness about robots replacing therapists, but our service is still very much therapy-led.
“What this means in practice is that, where a resident would previously have had maybe an hour of therapy time in an afternoon, now you have an hour of therapy time, and then you can carry on exercising if you want to, or carry on playing games with other residents.
“For example, one of our machines, the Myro, enables patients to play games like bat and ball, or perform virtual tasks like sweeping leaves.
“However, because it is all sensor-assisted, if it senses that the patient needs to work a certain hand, it will alter what it is asking them to do accordingly, while they won’t even necessarily feel they’re having therapy – it’s all part of the game, and part of their socialising with other residents.”
Askham Rehab forms part of the Askham Village Community, on the edge of Doddington village, in Cambridgeshire.
It provides specialist care for people of all ages, offering day visits, respite care and continuing long-term support, both on-site or at home.
The site consists of five homes, three of which are specialist neurological facilities. In total, the neuro-rehab team can look after up to 52 patients at any one time, with 120 staff made up of rehab professionals and specialists.
The team comprises carers nurses, physiotherapists, occupational therapists, speech and language therapists and psychologists.
Aliyyah- Begum believes that the introduction of the robotic rehab services, combined with the patient-led therapy the group has been offering for 30 years, can only enhance the centre’s outcomes.
She adds: “We know that there is an increasing number of care homes that offer specialist therapy, but the difference with Askham Rehab is that we have embedded it into the whole culture of our setting – and the outcomes really speak for themselves.
“We often discharge people earlier than planned, and that’s a testament to the fact that the patients are really working hard with the team throughout their stay with us to achieve their goals – and that is the key.”
For more information about Askham Rehab, visit www.askhamrehab.com
Astrocytes identified as master ‘conductors’ of the brain
In the orchestra of the brain, the firing of each neuron is controlled by two notes – excitatory and inhibitory – that come from two distinct forms of a cellular structure called synapses.
Synapses are essentially the connections between neurons, transmitting information from one cell to the other. The synaptic harmonies come together to create the most exquisite music–at least most of the time.
When the music becomes discordant and a person is diagnosed with a brain disease, scientists typically look to the synapses between neurons to determine what went wrong. But a new study from Duke University neuroscientists suggests that it would be more useful to look at the white-gloved conductor of the orchestra – the astrocyte.
Astrocytes are star-shaped cells that form the glue-like framework of the brain. They are one kind of cell called glia, which is Greek for “glue.” Previously found to be involved in controlling excitatory synapses, a team of Duke scientists also found that astrocytes are involved in regulating inhibitory synapses by binding to neurons through an adhesion molecule called NrCAM. The astrocytes reach out thin, fine tentacles to the inhibitory synapse, and when they touch, the adhesion is formed by NrCAM. Their findings were published in Nature on November 11.
“We really discovered that the astrocytes are the conductors that orchestrate the notes that make up the music of the brain,” said Scott Soderling, PhD, chair of the Department of Cell Biology in the School of Medicine and senior author on the paper.
Excitatory synapses — the brain’s accelerator — and inhibitory synapses — the brain’s brakes — were previously thought to be the most important instruments in the brain. Too much excitation can lead to epilepsy, too much inhibition can lead to schizophrenia, and an imbalance either way can lead to autism.
However, this study shows that astrocytes are running the show in overall brain function, and could be important targets for brain therapies, said co-senior author Cagla Eroglu, PhD, associate professor of cell biology and neurobiology in the School of Medicine. Eroglu is a world expert in astrocytes and her lab discovered how astrocytes send their tentacles and connect to synapses in 2017.
“A lot of the time, studies that investigate molecular aspects of brain development and disease study gene function or molecular function in neurons, or they only consider neurons to be the primary cells that are affected,” said Eroglu. “However, here we were able to show that by simply changing the interaction between astrocytes and neurons — specifically by manipulating the astrocytes — we were able to dramatically alter the wiring of the neurons as well.”
Soderling and Eroglu collaborate often scientifically, and they hashed out the plan for the project over coffee and pastries. The plan was to apply a proteomic method developed in Soderling’s lab that was further developed by his postdoctoral associate Tetsuya Takano, who is the paper’s lead author.
Takano designed a new method that allowed scientists to use a virus to insert an enzyme into the brain of a mouse that labeled the proteins connecting astrocytes and neurons. Once tagged with this label, the scientists could pluck the tagged proteins from the brain tissue and use Duke’s mass spectrometry facility to identify the adhesion molecule NrCAM.
Then, Takano teamed up with Katie Baldwin, a postdoctoral associate in Eroglu’s lab, to run assays to determine how the adhesion molecule NrCAM plays a role in the connection between astrocyte and inhibitory synapses. Together the labs discovered NrCAM was a missing link that controlled how astrocytes influence inhibitory synapses, demonstrating they influence all of the ‘notes’ of the brain.
“We were very lucky that we had really cooperative team members,” said Eroglu. “They worked very hard and they were open to crazy ideas. I would call this a crazy idea.”
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