From abstracts to posters, pushpins to ribbons, it takes a whole year to get to this day – no, not Christmas, but the 28th International Symposium on ALS/MND. In this and the following ‘catch-up’ blog we will summarise what went on at the Symposium and where you can find out more information. To begin with, you can read about what goes into organising the biggest meeting of its kind on our blog: It’s that time of year again … #alssymp.
Because of the diversity of the talks presented at the Symposium, we categorised them into five key themes that follow the timeline ‘from bench to bedside’; biomedical research, diagnosis and prognosis, causes of MND, clinical trials and treatments, and improving wellbeing and quality of life. You can read more about each of these themes on our Symposium LIVE webpages.
Throughout the article, you will see codes next to the speakers (eg C1) – these correspond to the abstract of that talk, which you can read in the official abstract book.
In the Joint Opening Session, Jeffrey Rosenfeld (C1) discussed the need for a dramatic change in the way we define and study ALS/MND. Specifically, considerations need to be made on how we separate ALS/MND due to its heterogeneity – that is, the different forms it takes based on the causes, genetic involvement or disease progression – in order to develop successful treatment trials.
Autonomy & Quality of Life
Autonomy and quality of life are important to all of us but when communication and physical ability become difficult, as is the case in MND, what might be done to help those affected?
Empathy, something that many clinicians might struggle with when faced with emotive subjects (such as end-of-life care), and honesty have been found as vital for patients’ wellbeing. James Tulsky (C7) suggests that clinicians can improve their communication skills using the ‘REMAP’ technique – Reframe, Expect emotion, Map out the future, Align the values and Plan treatments that match values.
Quality of life is a complex multifactorial concept that is not necessarily about longevity. In a small group of patients in Portugal, Susana Pinto (C8) found that patients were more likely to have a lower perception of quality of life if, prior to diagnosis, they experienced physical difficulties rather than difficulties with speech or swallowing. This was also more prevalent in females. Their findings suggest that independent function relates more to quality of life in the early stages of the disease.
A small study in Poland, carried out by Dorothée Lulé (C9), looked at patients with locked-in syndrome and found that, at this later stage, wellbeing was not associated with physical function by the patients. When questioned, patients with locked-in syndrome reported that they mostly had a satisfactory quality of life, would not choose to hasten death and, with hindsight, would still choose the same interventions. Interestingly, caregivers overestimated the depressiveness and underestimated the quality of life of those in their care.
Geraldine Foley (C10) carried out a systematic review of the literature between 2007 and 2017 to look at involvement of carers and family in the decision-making process relating to care. Wider family can have an impact on decisions such as interventions through direct or indirect influence. This is primarily due to protective instincts and wanting to minimise distress for those close to you. People with MND and care givers are co-dependent in decision making but are swayed by wider family. More work is needed to understand this and enable professionals to better involve wider family in the decision making process. How to include people with MND who have cognitive impairment in making decisions also needs to be considered.
So, good empathy skills, evaluation of health status before diagnosis, good social support and recognising how the MND patient/family dynamic may influence decision-making are all areas for improvement to help people with MND and their families achieve autonomy and good quality of life.
Technology & ALS
Technology plays a big part in all our lives. One session was dedicated to exploring how technology can improve patient care using telehealth – a remote data exchange between individuals and their clinicians to assist in the diagnosis and management of a condition, and decrease the frequency of clinic visits – a game changer for people with mobility issues.
Proximity to a multidisciplinary clinic can have a great impact on patient care. In the first study of its kind in the United States, Kevin Horton (C60) considered how close patients lived to a multidisciplinary clinic. It was seen that almost half lived more than 50 miles from a clinic and a quarter lived more than 100 miles away. Recommendations were to look at satellite clinics, telemedicine and training for rural neurologists (these might however not have internet access).
Brain-computer interfaces may once have been the stuff of sci-fi films but this technology may mean that, one day, people with MND will never lose their ability to communicate. People with limited movement will be able to convert thought into output and make assistive technology work for them.
You can read more about telehealth, brain-computer interface and MND on our blog.
Respiratory Assessment and Management
Respiratory changes are one of the symptoms that lead to a severe disability due to weakening of the muscles that control breathing. As these are not able to work properly, lung capacity decreases over time. Research in this area is now focusing on predicting survival based on the progression of respiratory scores over time.
Slow (SVC) and forced (FVC) vital capacities are the most commonly used pulmonary function tests in MND, however, it is unknown whether they can reliably predict survival. Susana Pinto (C40) compared both measures in predicting survival in people with MND and concluded that FVC and SVC are strongly correlated and interchangeable in predicting survival. This was relevant not only for discussions over monitoring and treatment of the disease, but could also help evaluate potential drug effectiveness and sensitivity in clinical trials. A trend towards using SVC might be observed in the future.
Nimish Thakore (C41) reported on a study involving 1,874 patients which observed that the respiratory sub-score of the ALS Functional Rating Scale-Revised (ALSFRS-R) is unreliable as a marker of early respiratory dysfunction in ALS. A more detailed assessment is required, and the recommendation was to eliminate Q12 on use of NIV, as it is too subjective to patient preference.
A Cough Assist Device is a safe, non-invasive alternative to suctioning that removes secretions from lungs and assist the patients cough. Jon Nilsestuen (C42) recommended that using flow and pressure graphics for cough assist (CA) should become a standard of care. The graphics help with customisation of settings on CA to suit individual needs. Prior to this it would appear that the vast majority of CA settings were made without any objective methods for evaluating efficacy. This approach allows for a smoother continuous process, without pauses during the cough process. It maximises the benefit of the device to clear secretions by significantly improving peak cough flow.
Rachel McConnell (C43) reported that, because both mechanical and non-mechanical CA techniques were comfortable to use, there should be a much earlier intervention. However, professionals would feel more confident about prescribing this if they had appropriate literature for patients. Patient and carer experiences need more study and evaluation.
HeadUp collar – cervical orthosis
People living with MND can experience weakness of the neck muscles with consequent head drop. This can be associated with pain, social embarrassment and difficulties with communication and swallowing. Standard practice is to use a cervical orthosis, or neck collar, but these were generally designed for other conditions and considered to be quite uncomfortable. This led to a project, led by Christopher McDermott (C75), involving people with MND, engineers, designers and healthcare professionals, resulting in the design of the HeadUp collar. The collar will soon be available on NHS and you can read about it in more detail in a blog article.
What lessons have we learnt?
- More thought needs to be given to how ALS/MND is separated into its different forms so that more successful treatment trials might be developed.
- Some clinicians need to be more empathetic and honest in order to deliver a better patient experience.
- Quality of life is perceived differently depending on the stage of disease and this needs to be taken into account when disease management strategies are being decided.
- Both patients and caregivers, and even the wider family, should be involved in any decision making.
- Technology plays an increasing role in supporting people living with MND meaning that they may never completely lose the ability to communicate.
- Several new approaches to aid respiration and assessing need were discussed. Professionals are happy to consider earlier intervention but feel that communication and support of patients needs to be improved.
- The HeadUp collar has been trialled and will be available to buy in 2018.
You can also watch a series of short videos on the Symposium website from professionals involved in the care of people with MND.
We would like to thank the staff that kindly provided their session summaries to allow us to keep you informed via a number of blogs, some of which are still on their way. Many thanks to Rachel Boothman, Kaye Stevens, Kirsten Kelly, and Mandy Spencer for collating these into an article.