29th Annual International Symposium on ALS/MND Patient Fellow Program

Background: “The key to defeating MND lies in fostering strong collaboration between leading researchers around the world, and sharing new understanding of the disease as rapidly as possible. This was the MND Association’s rationale behind the creation of the International Symposium on ALS/MND.” – MND Association

Objective: “This fellowship is as much about bringing the individual person with ALS/MND to the research meetings as it is about sharing that experience with the rest of the ALS community” – ALS/MND Patient Fellows Application

Methods: An online application process yielded 3 patient fellows from varying demographics, disease progression, and life stages that attended the international symposium in Glasgow, Scotland. Prior to the conference, social media posts were made announcing selection and participation in the program. During the conference, social media posts were made to engage others in the conversations and presentations taking place. The busy, three day conference was divided between the patient fellows according to interest and each reported out their thoughts, feelings, and takeaways from each session through each fellow’s personal social media accounts. Post conference reports on the experience are being collected from the patient fellows and their caregivers. Reported below is my conference schedule, highlights and takeaways from myself and my caregiver and sister, Carlie Brous, input from social media and any pictures that were taken during the session.

Results: Attendance to the sessions was critical for our reporting, though one afternoon we retired to bed due to mental and physical exhaustion and with the permission of his Highness, Guðjón Sigurðsson. The sessions missed are indicated below and input is based on speculation only. It was mentioned numerous times throughout the conference that patient input and questions in the sessions remains a crucial part of the success of this symposium. Opportunities to utilize this platform were well executed by the patient fellows and other pALS in the audience.

Discussion and Conclusions:

As a rule of thumb for my personal interests in session selection, we mostly attended those that fell under the “People” and “Symptoms and disease management” sections. Below you’ll find:

• the sessions I attended, or intended to attend,
• any significant attendees, awards, or announcements from the session,
• a piece of the abstract that influenced my attendance,
• any social media engagement from the session on my personal platforms,
• and my personal input on the session topic, findings, or application to my own ALS journey.

Friday, December 7, 2018

• JOINT OPENING SESSION – Welcome on behalf of MND Scotland, International Alliance Humanitarian and Forbes Norris Award, IPG Award and winner’s research presentation
  • Her Royal Highness, the Princess Royal Welcomed Us
    • I’m embarrassed to admit that I had to google who this honored guest was. It was very interesting to learn her ties to MND, her history with this symposium, and her passion for helping those of us living daily with this disease.
  • Dr. Brian Dickie Received the Humanitarian Award
    • Getting to meet Dr. Dickie after receiving his award and learning more about his passions through his social media account was a true honor. Our picture together is below, and has been officially added to my “Celebrity Photos” collection
  • Dr. Angela Genge – Forbes Norris Award Winner
    • We had the opportunity to hear from Dr. Genge early Saturday morning at the Cytokentics session. Her ability to discuss complex topic in a casual, humorous way was inspiring. I got the opportunity to ask a question during her session, and that info is below.
  • Dr. Rickie Patani – IPG Award Win
  • Social Media Engagement: 1200+ across all platforms

• Defining ALS/MND: The Big Debate (C3)
  • “Few issues generate as much controversy as to whether our current approach for diagnosing ALS/MND needs to be seriously reconsidered.” Platform Communications, page 3
  • Social Media Engagement: 200+ across all platforms
    • “Of course it does” – fb comment
  • I preferred this presentation format because it gave us an opportunity to explore the topic in its entirety, with points of view coming from both sides. It was obvious to me that the stances taken on this subject by the presenters were assigned rather than truly believed, which made the arguments weaker.
  • My personal journey to diagnosis took more than 18 months, countless doctor visits, examinations and co-pays. I saw many specialists from different fields of study, had gallons of blood taken for various tests, 4 EMG tests, a spinal tap, and more that I’m sure that I’m forgetting. I realize that a lot of that wild goose chase was due in large part to my age (27) and my gender (female). My second opinion came from Dr. Elliott at UTSW and he told me that if I was a 60 year old man, he’d diagnosis me today and be able to sleep that night but because of my age and gender, we were going to explore and leave no stone left unturned.
  • My diagnosis process took so long and involved so many appointments that when I was in Dr. Philip’s office at Texas Neurology I was by myself. 27 and alone when I found out that I had ALS. No one could miss work that often for my appointments, I had no significant other in my life, and my mom was over an hour and a half away. Never would I have imagined that she was going to diagnose me during that appointment, I even was dressed to return to work after the appointment.
• Lifetime sport practice and brain metabolism in ALS: a [18F] FDG-PET study (C12)
  • “Background: The impact of physical activity on the risk of Amyotrophic Lateral Sclerosis (ALS) has been investigated in several studies with controversial results. Objectives: The aim of the present study was to evaluate the relationship between the practice of sport activities during life and brain metabolism assessed by 18F-FDG-PET at ALS diagnosis.” Platform Communications, page 10
  • Social Media Engagement: 120+ across all platforms
  • This is a topic of conversation that presents itself often, mainly because most people only know of ALS through media coverage surrounding athletes. At the ALSA Advocacy conference in May 2018 in Washington, DC it was explained to us that the working theory amongst some doctors is that the enhanced genetic makeup that produces professional athletes is so elite that one would have to assume the bad with the good; because their bodies are able to preform at Herculean levels, so would the diseases.
  • I was an athlete, not a great athlete but I was tall, consistent, and somewhat coordinated. I have no major injuries from my time playing sports, head or otherwise. I was playing softball when I acknowledged my symptoms. I do not think “my ALS” has anything to do with sports.
• Advance care planning in ALS: Representation and realisation of individual treatment goals through care management schemes (C10)
  • “Background: In Amyotrophic Lateral Sclerosis (ALS) a fundamental problem of choice arises between palliative care and life-prolonging measures such as non-invasive ventilation (NIV), invasive ventilation (IV) and percutaneous endoscopic gastrostomy (PEG). The corresponding decision-making process is embedded in the Advance Care Planning (ACP). ACP defines a patient’s individual treatment goal. When opting for palliation, a distinction is made between withholding therapy and withdrawing therapy. ACP concepts are being increasingly applied in ALS. However, few systematic evaluations of the implementation of ACP in real-life care settings have been conducted. Objectives: Investigation of the implementation of an ACP concept by means of a care management scheme. How often do key players opt for withholding or withdrawing therapy as part of the ACP concept realised in a palliative care management scheme?” Platform Communications, page 8
  • Social Media Engagement: 210+ across all platforms
  • I signed my first Advanced Directive at my first multidisciplinary clinic in February 2015, and I amended it immediately following my marriage to Kenneth in November 2016. He became my primary advocate and decision maker in the chance that I am unable to do so for myself. This was a thorough conversation that I had with my social worker (see Sheilah Storch), and she is fully aware of my ACP.
  • In August 2018 I signed a “Do Not Resuscitate” directive. It is my belief that the physical trauma sustained during resuscitation would greatly impact my quality of life, and is not a step I am willing to take.
  • These documents are on file with my doctors, my family members have electronic copies for quick access in case of emergency, and my husband has a copy in the refrigerator. I am open and honest about my ACP in my blog, on social media, and when meeting people. My biggest fear with this disease is no that I’m going to die, it’s that someone I love will be forced to make a decision on my behalf and not be confident in the process.
  • We’ll discuss in more detail my desire to never have a tracheotomy, and that has been my feelings from the very beginning. I am willing to go as far as PEG tube for feeding but that is all.
• Decision support tools for motor neurone disease multidisciplinary care (C11)
  • MND Patient Decision Support
  • Social Media Engagement: 140+ across all platforms
  • I’m a big advocate for multidisciplinary care, especially in the clinic setting. I have access to multiple clinics with different formats, and for that I am very thankful. Both clinics provide multidisciplinary care, but at UTSW the appointment takes place in an individual patient room whereas at Texas Neurology the appointment takes place in an infusion suite with 10-15 other ALS families. I attend Dr. Heitzman’s clinic at Texas Neurology 4 times a year, but I would attend every appointment if they would let me. This serves as my support group, we engage in conversations during clinic and beyond. We rely on each other for tips, tricks, encouragement, and hugs.
  • All of the therapists there are on the same page with my decisions and progression plans, we make educated and quantitative benchmarks for intervention, and celebrate successes together every appointment.
• Improving outcomes as rapidly as possible for patients: Multi-arm, multi-stage platform, umbrella and basket protocols (C20)
  • “There is real need to change how we do some of our clinical trials, as currently the testing and development process is too slow, too costly and too failure-prone. Ultimately, we often find that a new treatment is no better
    than the current standard.” Platform Communications, page 16
  • Social Media Engagement: 160+ across all platforms
  • This was a “duh” session for me but it was a relief to know that others feel the same way about finding the most effective and efficient ways to approach research. I’ve participated in many different studies: one drug, most PGB or questionnaire. Research contribution is very important to me, and aligns greatly with my intrinsic needs to find a positive in this diagnosis.
• Cognitive deficits in ALS are a marker of localized TDP-43 cerebral pathology (C27)
  • “Background: Approximately 35% of amyotrophic lateral sclerosis (ALS) patients exhibit mild cognitive deficits in, executive functions, language and fluency, without dementia. The precise pathology of these extra-motor
    symptoms has remained unknown.” Platform Communications, page 21 
  • No social media engagement to report
  • When I was first diagnosed the idea of cognitive impairments scared me, as most of the women on my maternal side have died from some form of dementia or were on their way there when they passed. Then, I went through a sick humor phase that welcomed the idea of losing my mind while losing everything else. Now, I’m not entirely confident in my true feelings on this topic. It’s one that I find intriguing, and in the right mental state enjoy exploring the pros and cons of this side effect. Though more times than not, it’s a topic that fast tracks depression and sends me into a funk that is difficult to recover from.

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Saturday, December 8, 2018

• INNOVATION LANDSCAPE IN ALS/MND: DESIGNING TOMORROW’S CLINICAL TRIALS TODAY INCLUSION AND EXCLUSION CRITERIA: A LIVE DEBATE
  • My notes: early early morning meeting discussing designing tomorrow’s clinical trials today, inclusion and exclusion criteria. y’all know I’m all about research, game to be the guinea pig if it means helping others. no busted lip or goose egg will slow us down 💪🏼 what’s your opinion: should clinical trials be more broad or more specific?
  • My comment to the panel was: why should I have to choose between NIV, which is proven to extend life in people with ALS, and participating in clinical research?
  • Social Media Engagement: 370+ across all platforms
    • “broad” – instagram comment
    • “Should also discuss whether trial design places unnecessary burden on patients and their carers. Reducing assessments, remote monitoring and involvement of pts in trial design would help improve equality of access and ultimately more relevant results.” – @Drestherhobson
    • “In past decades exclusion criteria have seemed like copy/paste & arbitrary to many people w ALS. Moving fwd, important to explain scientific rationale for each. That will increase enthusiasm for trials, especially if criteria are narrow.” – @alsadvocacy
• Computer assessment of speech in a successful ALS treatment trial (C32)
  • “The results of this study demonstrate that even patients who did not substantially improve while on DM/Q, as determined by the patient based
    and clinician-administered assessments, exhibited quantifiable improvements in speech that could be detected using objective measures of speech. Objective
    speech measures may provide sensitive, unbiased outcomes in ALS therapeutic drug trials targeting bulbar motor function. Furthermore, these findings provide additional evidence of the positive impact of DM/Q on
    one or more of the neural systems that control bulbar motor function and production of spoken language.” Platform Communications, page 25
  • This session opened a later conversation between myself and Bruce Virgo about my use of Nudexta and it’s unavailability in the UK. I take 2, 20-10mg capsules daily and have seen significant increase in my ability to control over-acted emotions.
    • e.g. I once went to the movies with some girlfriends, and on the way to our seat one of the girls dropped the entire bag of popcorn – that we were all to share – on the ground. As the movie progressed, in times that I should have had an emotional response (in this case fear or weepy) I began to laugh loudly and uncontrollably. Because we were nearing the end of my first dose of Nudexta of the day, my onset of emotion was exaggerated and met with manic laughter that was not appreciated by anyone else in the theater, even after numerous apologies.
    • I have many other examples like this one, and of the majority that suffer from PBA report either laughing or crying, I’m thankful that my response is laughing.
    • I do find that this medication helps me to feel more in control of my face and mouth, allowing for better understanding of my speech by others especially late in day.
• Causal effects of presymptomatic lifestyle on ALS, stratified by C9orf72 genotype (C38)
  • I went into this session thinking it was going to be a broad discussion on pre-diagnosis lifestyle choices – working out too much/little, surgeries, meat vs. vegetable diets, etc. – but I guess I stopped reading at “stratified…”
  • One conversation I enjoy having with fellow pALS is, what do YOU think caused your ALS? Some are specific, others reach desperately for supporting studies to group them somehow, others have never even thought about it. It’s always an interesting, insightful conversation that inevitably enables me to further wrap my head around this disease.
  • As a sporadic, limb onset, no family history anywhere, diagnosed at 27 female, my answer is this: When I was 23 or 24 I went to see a “fat doctor” for help in losing excess weight. For the first time in my life I was sitting more than standing and the sedentary lifestyle was taking a toll on me physically. Ultimately I was prescribed the maximum dose of Phentermine with no results – positive or negative. I can’t remember any ALS syptoms prior to taking this medication. This is just my opinion, it’s never been explored further.
• Is psychological stress a predisposing factor for ALS? An online international case-control study of premorbid stressful life events, resilience and anxiety (C40)
  • “Background: Psychological stress has been suggested to be relevant to the pathogenesis of neurodegenerative disorders such as ALS, possibly via the generation of oxygen free radicals.
    Objectives: We sought to determine whether people with ALS had been subjected to more stressful life events than controls, and whether they had differences in resilience or trait anxiety that could adversely affect their responses to these stressors.” Platform Communications, page 31
  • Social Media Engagement: 40+ across all platforms
    • “People with #ALS #MND often mention stress as a possible trigger of their disease. Jane Parkin Kullmann (C40) discusses results of her study using the online ALS Quest questionnaire and reports no effect of stress and anxiety, but resilience higher in people with ALS” @mndresearch
  • During the time that I first noticed symptoms, especially hindsight from diagnosis, my job was extremely stressful. I was responsible for 270 college students in the dorm at a Junior College. I was so stressed that it affected me mentally, emotionally, and physically. More than once I confessed that the stress of the job felt like it was killing me. I do not feel that this is the cause of my diagnosis, but I do feel that it was a contributing factor.
• Preserving identity: The Speak:Unique voice banking pilot (C36)
  • “Background: Dysarthria in MND can be one of the most difficult symptoms to accept and manage. As speech becomes increasingly unintelligible, many rely on augmentative and alternative communication (AAC). Voice output communication aids (VOCAs), while facilitating communication, cannot preserve the identity of the individual, due to impersonal generic synthetic voices. The inability to accept an alternative voice has been cited as reason for AAC abandonment in people with MND (pwMND) (1). Preservation of identity through voice banking and personalised synthetic voices is increasing, however the process can be exhausting, time consuming and inaccessible where speech is impaired.
    Discussion and Conclusions: There is a powerful association of voice with identity among pwMND and a strong desire to preserve this. Short recordings conducted in a home environment increases accessibility of voice banking, including to those with dysarthria.” Platform Communications, page 27
  • Social Media Engagement: 300+ across all platforms
    • “I see this and think of ⁦@deb_gaudet⁩ who would switch her android voice to British when she wanted to sound impressive. She was so cool. Thus slide from Rewaj.” @alsadvocacy
  • Side note: Dysarthria is forever associated with Bruce Virgo in my book, you’ll have to ask him to tell you the story. Also, this reminds me of Kipling Jackson’s stories in his book: ALS: A Respiratory Perspective.
  • Loosing my voice is terrifying, so I’ve made a point to participate in every voice banking opportunity that I come across. I’m currently participating in the following programs:
    •  Precision Medicine Program through ALSTDI where I submit a voice recording and ALSFRS monthly.
    • Project Revoice through the ALS Association where I submit recorded phrases and a voice is generated through Lyrebird
    • Google Voice Project collaboration with ALSTDI using Webhound to record phrases to generate my voice. This was emailed to me, I cannot find a link to the actual project.
• Patient Reported Outcomes Measures (PROMs) in Amyotrophic Lateral Sclerosis (ALS) (C48)
  • “Discussion and conclusions: With this study, we want to show physical health assessments as reported by patients are strongly correlated with physical health assessments as recorded by healthcare providers.” Platform Communications, page 38
  • Social Media Engagement from others:
    • “Re PROMs… I trust data I enter much more than data that someone in the doc’s office enters on my behalf… but you have to ask the right questions. The problem w PROMs is often assumed to be the “P”, but I think it’s more likely to be the “OM”s.” @alsadvocacy
    • “Hardiman asks an interesting Q about differences in PROMS depending on the setting (clinic or home) where the P fills out the OMS.” @alsadvocacy
  • Read my blog post titled Bullsh*t for my experience with misreported information in the clinical setting. Honest mistake made for a good laugh, but I would have caught the error because of its absurdity.
• Understanding quality of life in ALS/MND by modelling patient-reported outcomes (C49)
  • “Background: Given our current inability to cure ALS/MND or to halt its progression, optimisation of the patient’s quality of life (QoL) remains a key focus of care. Many physical and psychological factors have been reported to influence QoL in ALS/MND, but less is known about how these inter-relate and where interventions should be targeted.” Platform Communications, page 38
  • Social Media Engagement: 140+ across all platforms
    • “Young: Novel model shows that the two most important contributors to QOL in ALS are 1) functional status and 2) anxiety. We need to pay more attention to measuring and targeting anxiety, which is often ignored. The model demands multi-disciplinary care.” @shellikeriii
    • “Prof Caroline Young emphasises that full MDT care is required to address full validated model of QOL and factors influencing it in ALS/MND” @dierdremurray6a
    • “A comprehensive reminder from Dr Carolyn Young @TheWaltonCentre of the many aspects of patient, their condition and their experience that contribute to quality of life in #MND/ALS – and why a comprehensive MDT is required to provide #MND care for each of those needs” @helen_ashcroft
  • Dr. Heitzman and I have open, honest, and frank conversations about my disease, its progression, and my feelings towards both of those topics. We often discuss whether the symptom or issue that I’m experience – if at all – would be best addressed with medication or change in behavior.
    • Example: I requested an increase in my antidepressant because my emotions had become too much to process and I felt like a zombie.
    • Example: Dr. Heitzman ordered routine blood work and found that my Vitamin D level was very low. He told me to add in a supplement and get myself outside in the sunshine, which would also help with the first example.
    • Example: I was at the dentist and they placed the weighted vest on me before taking x-rays and immediately I felt a sense of contentment and relief. I was given a weighted blanket for Christmas and it has helped me to sleep better as well as relieve general anxiety during the day.
    • Most issues that are behavior in nature are addressed by the staff of the multidisciplinary clinic that I attend quarterly.
• Jumping between the cracks: Attempting to navigate the world of care in motor neurone disease, a qualitative study (C50)
  • “Discussion and conclusions: Navigating the complex and varied world of MND care is challenging for patients and carers and no two patients have the same experience. This has both benefits in flexibilty and patient-centred care and drawbacks, particularly for those facing the most barriers to accessing care. Effective care coordination requires professionals to be accessible and committed to the patient throughout the disease course.” Platform Communications, page 40
  • “Patients identified their primary caregiver as their key advocate but most identified a “lynch pin”, a professional who was most important in coordinating their care. This role was played by many different professionals from different backgrounds. The key value of the lynch pin was their accessibility, offering regular and reliable contact and whom the patient felt had sufficient knowledge and interest to address their problems.” Platform Communications, page 40
  • Social Media Engagement: 300+ across all platforms
    • “Sheliah aka “The General” is my linch-pin and I love her for it” @sunnyberasmus
    • “@DrEstherHobson explains that complex problems require complex care. Specialist and community based services both have strengths and weaknesses. We must work together!” @dierdremurray6a
    • “The benefits of specialist MDT MND care are clearly evident but how do we overcome the challenges & embrace the benefits of clinic vs community based care to provide these services? Food for thought” @jodiallenslt
  • I was aware that the multidisciplinary clinic approach was a relatively new innovation, but it shocked me that it was unavailable in some areas. I attribute my slow progression, my understanding of my choices and options for care, and overall sanity to my team at Texas Neurology. My lynch pin is someone who knows me well, takes time to argue and discuss options with me, and spearheads all of my care from the therapists, my insurance company, and my longterm disability company.
  • When discussing clinic with others, especially newbies to the multidisciplinary clinic, I try to remind the person that they are one of many patients in the clinic so it is absolutely imperative that they advocate for themselves, utilize every minute spent with each profession, and prepare for each clinic with questions, self-assessments, and outside research. Clinic is an opportunity to seize, not just one to endure. Also, I have different people attend clinic with me each time. My husband comes for the important conversations – i.e. babies, Radicava, Advanced Directive, etc. – but it helps me to learn and process this disease when I have others there to shed light, ask questions, and give feedback on their experience at clinic.
• An online mindfulness intervention for people with ALS (C51)
  • “Background: Improving psychological well-being of people with ALS is a crucial task for health professionals working in this field. Although there is relatively minimal research on psychological interventions for ALS, preliminary evidence suggests that mindfulness-based interventions
    could be effective in promoting quality of life (QOL), reducing depression and anxiety. However, most of these interventions are based on meditation practices, requiring substantial time and effort. The approach used
    in this study was instead based on the work of Ellen Langer, whose framework is based on a social cognitive psychological construct. In the Langerian approach, mindfulness is defined as a flexible state of mind resulting from the simple act of actively noticing new things, which puts people in the present and utilizes the current context as the primary anchor for perceptions and decisions.” Platform Communications, page 40
  • Social Media Engagement from others:
    • “Great to see our friends in Dublin are also working hard to give the experiences carers a voice” @drestherhobson
    • “Excellent talk by @carneysile highlighting ALS caregiver burden and the impact of different coping mechanisms” @RoisinTCDNeuro
  • This is where trying new things is crucial to maintaining sanity through this disease. I find great support and sense of community from my social media platforms, where others enjoy the setting of a support group. In order to be successful in self-preservation you must acknowledge what brings peace and eliminate what attracts anxiety.
• Apathy subtypes, quality of life, wellbeing and caregiver burden in motor neurone disease (C53)
  • “Background: Apathy is the most common behaviour change in motor neurone disease… Initiation apathy (lack of motivation for self-generation of thoughts/actions) has been observed as characteristic in MND (3,4). However, the practical impact of these apathy subtypes in relation to wellbeing, quality of life and caregiver burden for people with MND and their families has not been explored.
    Discussion and conclusions: Presence of Initiation apathy was related to lower wellbeing and quality of life in people living with MND and their families. Specifically, Initiation apathy was most influential in relation to wellbeing and burden of family members of people with MND…” Platform Communications, page 41
  • Social Media Engagement: 270+ across all platforms
    • “further proving: ALS effects the entire family, not just the person diagnosed. not fair for anyone.” @sunnyberasmus
    • “I almost couldn’t be bothered, but glad I did. Check out – Apathy subtypes, quality of life, wellbeing and caregiver burden in motor neurone disease (C53)” @bvirgo1 – study participant
    • “Apathy subtypes – initiation apathy sig impact on mood with greater likelihood of depression” @simpsonresearch
    • “@ratkorad discussing the different types of apathy experienced by people with #MND & the effect on them & their caregivers. Assessment scale at http://das.psy.ed.ac.uk  now recommended in clinical guidelines @SchoolofPPLS” @euanscentre
    • “It’s more than just the physical disability. Initiation apathy (lack of motivation to self generate thought or action) also negatively impacts on the wellbeing and burden of carers and families living with MND. More food for thought from Dr Ratko Radakovic” @jodiallenslt
  • It’s not just me, Sunny Brous Erasmus, that has this disease; we all have it.
• The ALS-FTD spectrum: Diagnosis and management (C64)
  • “Overall around 20% of patients presenting with non familial ALS will develop frank FTD while a much higher proportion (around 50%) have behavioural
    and/or cognitive changes. Most prominent among these changes is apathy which is independent of depression but stereotypical behaviours, loss of empathy and craving of sweet food are also evident. These features are known to cause considerable caregiver burden and stress.” Platform Communications, page 51 
  • Social Media Engagement from others:
    • “Citation classic by Neary & Snowden #JNNP recognising overlap between #FTD & #ALS #MND Overlap now being dissected by John Hodges” @JNNP_BMJ
    • “MND FTD Future research needs to look at ecological effects on life decisions. ‘Occupational Therapists are definitely important’ as are SLT and support for carers in the care management of patients with MND FTD” @simpsonresearch
  • When I was first diagnosed the idea of cognitive impairments scared me, as most of the women on my maternal side have died from some form of dementia or were on their way there when they passed. Then, I went through a sick humor phase that welcomed the idea of losing my mind while losing everything else. Now, I’m not entirely confident in my true feelings on this topic. It’s one that I find intriguing, and in the right mental state enjoy exploring the pros and cons of this side effect. Though more times than not, it’s a topic that fast tracks depression and sends me into a funk that is difficult to recover from.
  • I hate the idea that I would cause burden or stress on my caregiver, but this isn’t the first time I’ve read about the cravings of sweet food. On my maternal side, I come from a long line of old, healthy women that eventually presented some form of dementia. FTD is not something that is foreign to me, and it’s not something I’ve thoroughly explored within myself. I do really, really like ice cream and Dr. Pepper, so interpret that anyway you seem fit.
• Cognition and behaviour does decline across the ALS disease course after controlling for attrition (C65)
  • “Discussion and conclusions: Neuropsychological functioning declines over the course of ALS. This decline is driven by advancing disease stage, older age of onset, fewer years of education, and the presence of the C9orf72 mutation. Neuropsychological functioning should be monitored early and continuously” Platform Communications, page 52
  • My notes above apply here as well.
• Clinical correlates of memory decline in motor neuron disease (C66)
  • “Background: By now it is well known that up to 50% of MND patients can present with behavioral or cognitive impairment. The latter has primarily been associated with executive dysfunction and consequently with impairment in several cognitive domains.” Platform Communications, page 52
  • Social Media Engagements from others
    • “We are reminded again of the importance of early cognitive screening of people with MND” @janeyfl1
  • With everything that is going on regarding my body and it’s fight against this disease, I don’t foresee memory decline as a major issue for me but I can see where others would struggle greatly with this issue.
• Unravelling psychosis in motor neurone disease: A study of clinical features, cognition, and survival (C67)
  • “Discussion and conclusions: Psychotic symptoms are common in MND with major consequences for mental well-being, although they do not impact survival. These symptoms are more likely to be florid in younger patients
    and cognitive impairment is a frequent feature” Platform Communications, page 53
  • Well, wouldn’t it make sense that psychosis accompanies this beast of a disease? Misery does love company, right?
• ALS onset in old people: Clinical features and prognosis (C78)
  • “Background: Amyotrophic lateral sclerosis (ALS) has a similar incidence in different geographical regions, but is age related and more frequent in men. ALS has a peak incidence between 65-75 years being less common in people aged 80 years or older, possibly due to underdiagnosis” Platform Communications, page 62
  • And then there’s me: 27 at diagnosis, a female, and no common denominators with most of my companions.
• An examination of riluzole effect on transitions from stage to stage in the PRO-ACT cohort (C82)
  • “Background: Riluzole is proven to prolong survival in ALS in randomized controlled and observational studies. A recent re-analysis of riluzole trial data suggests that the drug prolongs later stages of ALS.” Platform Communications, page 65
  • At diagnosis I was asked if I wanted a prescription for the only known drug to slow the progression of ALS. My question was why would anyone say no? Dr. Philip informed me that it is a very expensive medication and for some it makes more sense to take the money and go on one last experience with loved ones. I agreed to the prescription just to see how expensive it was going to be. When I dropped it off at the pharmacy, I cried tears of relief that this disease wasn’t so prominent that the pharmacy kept the medication in stock. The follow day I returned to the pharmacy to pick up the prescription, it was $10.

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Sunday, December 9, 2018

• Wishes for hastened death in ALS: How should we as clinicians respond? (C93)
  • “How to respond to a wish to hasten death (WTHD) is a harrowing question for most ALS clinicians.” Platform Communications, page 74
    • “Acknowledge the frequency. Data show that a majority of ALS patients can imagine asking for hastened death in the future, and up to 20% of ALS patients die through medical aid in dying in jurisdictions that allow it.” Platform Communications, page 74
  • Social Media Engagement: 100+ across all platforms
    • “as a pro-choice advocate in every aspect of my life, this conversation is very important to me. It’s my body, it’s my decision, it’s my right to make the decision on how I want to end my life. 🌻 ” @sunnyberasmus
    • “Ashley Wywias I never knew much about assisted suicide until I watched a documentary called “How to Die in Oregon.” If a person has a terminal illness, in my opinion, they have the right to choose. Death with dignity. It’s your body, it’s your illness, it should be your choice.”
    • “Cindy Sloan Sims Your choice. Without a doubt.”
    • “Rob Ryan Rodriguez As difficult as the conversation is, a person with ALS should be able to choose and receive the assistance needed to follow through.💜😘”
    • “Melinda Baker Shaw We should all be able to make decision for ourselves.”
    • “Brenda Harcrow Maxwell Stroud It should be an individual’s choice. No doubt about it!!”
    • “Even though the chances of dying from choking are low, people wishing for hastened death (medical assistance in dying) choose this option out of this exact fear. We need to address this” @shellikeriii
    • “Final day of the MND symposium Currently hearing about how to support patients wishing for hastened death Beliefs and norms influence our attitudes towards the dying process Spiritually found to be a protective factor Acknowledging talking listening” @simpsonresearch
    • “It’s wrong to assume that people with depression cannot make a decision about assisted suicide’. (C93)” @LucyP_MNDAssoc
    • “Great positive talks at the #alssymp about assistive dying. Really important topic we often shy away from in als mnd care” @estherhobson
    • “Prof Gian Borasio of University of Lausanne starts the End of Life session at #alssymp by outlining important lessons that should be followed by all #ALS #MND clinicians.” @mndresearch
    • “Three plenary speakers, Professors Borasio (Switzerland), Johnston (Canada) and Ogino (Japan) shared their views on and experience with wishes for hastened death. They are now all meeting at the podium for further discussion on this topic.” @mndresearch
    • “There are important spiritual and religious aspects to all end-of-life decisions that I have found in the past that ALS organizations and many HCPs are ill-equipped to address.” @alsadvocacy
    • “Why not let people roll the dice more w access to investigational drugs where informative data can be gathered, even outside the clinical trial. Thus should also be part of those death-hastening conversations.” @alsadvocacy
  • This entire session is one I reference in conversation when asked about my experience in Scotland. This is a hot topic in every culture, across all diseases, and in all countries. It was interesting to hear the topic presented from different cultures, from different mindsets when discussing physician roles in this disease. My firm belief, and motive behind my decision of being Pro-Choice, is that the decision for hastened death has nothing to do with the government. I don’t consult my legislators for any other medical decision, why should this be different? This is a decision between myself, my family, and my care team.
• MAiD in Canada: Legalization of medical assistance in dying has changed the discussion of WTHD in ALS (C94)
  •  “Background: ALS is a degenerative motor neuron disease characterized by progressive paralysis leading inexorably to disability and death due to respiratory failure. Effective and ethical clinical communications between persons living with ALS, their families/caregivers and their healthcare providers (HCPs) are essential for clinical management of ALS, particularly around options at the end of life (EOL). Medical Assistance in Dying
    (MAiD) became a legal EOL option in Canada in 2016 providing further challenges to EOL discussion. EOL communication is uniquely complex, prone to conflict, and one of the most clinically challenging components of palliative care. Prior studies have shown that anticipatory suffering drives the wish to hasten death, whether through MAiD or other means. Accordingly, there remains a need for an improved evidence base to develop communication
    tools about EOL planning.” Platform Communications, page 74
  • Social Media Engagement: 75+ across all platforms
    • “Sound familiar? As a researcher and active caregiver in ALS, I can speak from personal experience that these are the top two reasons my mother considered assisted suicide” @shellikeriii
    • “Dr Wendy Johnston: Media reports (newspapers) on medical assistance in dying and ALS seem to go hand-in-hand. With legalization in Canada, reports on both were exponentially increased.” @shellikeriii
    • “A talk from Canada on medical assistance in dying there. Very important for countries to learn from each other’s experiences. Most people learn about this subject through the media, just another example of how critical the messages from the media are (C94)” @LucyP_MNDAssoc
  • I learned in this session that it is legal in Canada for ALS patients to be organ donors as long as the donor discloses that they had ALS. I’ve been an organ donor since I was 15 in drivers education, so this news was very, very exciting to me.
• Wishes for hastened death in ALS in Japan: The need for culture-sensitive answers (C95)
  •  “According to our research (3,4), families often want TV used for their
    relatives, but would not want TV if they themselves were affected… As physicians, we have a professional duty to support our patients in finding value in life. Despite our inability to grant it, having the courage to discuss a patient’s desire for a hastened death can help them find a path forward.” Platform Communications, page 75
  • Social Media Engagement:
    • “Very interesting slide from Dr Ogino (Japan)on the definition of hastened death from country to country…. is there also different meanings/definitions from clinician to clinician?? I suspect so..” @hanratty86
    • “Last and best comment about end of life choices from @sunnyberasmus Choice should be there for people to have some control over their death #alssymp Great discussion.” @drestherhobson
  • This session brought to life a difference in cultural views when making decisions pertaining to end of life. We learned about the implications on the family when hastened death is utilized by a person with ALS. We explored the purpose of the Hippocratic Oath, and is “doing no harm” defined by a physician assisting in death or letting someone suffer while they have the ability to put an end to the suffering.
• Measuring rate of decline in pulmonary function in ALS: Results from the ALS Nutrition/NIPPV Study Group (C103)
  • “Background: Objective measurements of respiratory muscle function are used to determine the timing of noninvasive ventilation (NIV) and prognosis.” Platform Communications, page 80
  • Social Media Engagement:
    • “Even negative results help us learn and inspire clinicians to re-examine how they care for ALS/MND patients.” @julietmpro
    • “#alssymp disappointing that less that half of patients managed to adhere to NIV. There is much work to be done to help people get the most out of NIV. Our @OfficialNIHR study should help and Dave O’Brien will tell us more at 11.30″ @drestherhobson
    • “Dr Jackson: 1) FVC may not be the most ideal measure to track early resp insufficiencies- MEP shows the greatest decline in this cohort. 2) Early NIV (FVC<85) does not appear to slow rate of decline. Intervention timing is key.” @shellikeriii
    • “”Compliance” is another of those words that isn’t very patient-centric. Think about it. If you are handed an intervention that makes you feel worse (enough to stop it), you are labeled “non-compliant.” We blame the patient and not the intervention.” @alsadvocacy
  • I made the choice to forgo NIV for years because I wanted to remain eligible for clinical trial research. Once I started on Radicava I was automatically disqualified from clinical trials, so at that point we – my neurologist and myself – made the decision to order NIV. Honestly I’m horrible at getting my hours in daily, but I do find benefit from using the NIV when I slow down long enough to wear it.
• Slow vital capacity and functional decay in ALS (C104)
  • “Background: Functionality in amyotrophic lateral sclerosis (ALS) is usually assessed by the revised ALS functional rating scale (ALSFRS-R). Respiratory function is a critical predictor of survival in amyotrophic lateral sclerosis
    (ALS). The role of respiratory function as predictor of functional decay in ALS is not fully established.” Platform Communications, page 81
• Enhancing the efficacy of non-invasive ventilation for patients with motor neurone disease: A systematic review and multicentre survey (C105)
  • “Background: Non-invasive ventilation (NIV) is known to prolong both survival and quality of life in motor neurone disease (MND) however, usage and effectiveness must be optimised in order to gain maximum benefit. While
    guidelines exist, the process of initiation and management is known to vary between centres, and there can be uncertainty regarding best practice.” Platform Communications, page 82
  • Social Media Engagement:
    • “Dave coping with tech problems: shows how getting your tech working is important both at conferences and when using #NIV” @drestherhobson
    • “Anyone who has ever visited an ALS support group website knows a lot about this. Slide from O’Brien.” @alsadvocacy
    • “Need to consider different types of NIV machines and modes based on individual symptoms. We may need to switch between modes as nature of symptoms change.” @shellikeriii
    • “Interesting presentation on enhancing the efficacy of NIV Systematic review indicated MDT care & effective coordination are optimising factors, suggestion of need for more specialist physio & nurse roles in the UK. Work with MDTs ongoing with aim to produce a toolkit” @simpsonresearch
• Impact of a combined expiratory and inspiratory respiratory strength training program in ALS: Results of a randomized sham controlled trial (C106)
  • “Objectives: Determine the impact of an in home combined expiratory and inspiratory respiratory strength training (RST) program on pulmonary, swallow and cough function in individuals with early stage ALS.” Platform Communications, page 82
  • Social Media Engagement:
    • “Plowman. And even in a strength training program, when you have ALS, you never want in the placebo group.” @alsadvocacy
    • “Plowman: Bulbar onset case study showing improvement in MEP and peak cough flow with inspiration+ expiratory muscle strength training” @shellikeriii
    • “Ot appears to me that Plowman is showing us a training program that has far superior efficacy to any drug on the market for ALS.” @alsadvocacy
    • “As always a great talk from Prof Plowman. Respiratory training that can be done at home looks like a promising way to not just sustain but improve breathing, cough and swallow” @drestherhobson
    • “Plowman: With resp strength training, improvements were noted in MEP/MIP, cough peak flow, and swallow function. No change in FVC was noted. Suggests that training can improve respiratory reserve, airway clearance, and oral intake.” @shellikeriii
    • “A paradigm shift in ALS? @emplowman presents her most recent paper on proactive management of swallowing and cough via inspiratory and expiratory muscle strength training. What a great advocate for therapy & putting our patients back in control of their disease” @jodiallenslt
    • “Mild to moderate intensity respiratory strength training is feasible and safe in early affected pwMND. Dr Plowman reports has also used successfully in pwPD” @simpsonresearch
    • “A shift in the way we approach treatment for #cough and swallow. A great talk by @emplowman on combined inspiratory and expiratory muscle strength training in #ALS A very exciting area!” @char_massey
  • Connecting with Dr. Plowman to learn more about the techniques used in this study. Strengthening my respiratory muscles is high on my priority list, especially when the exercises can be completed while maintaining my crazy schedule.
• Survival and outcomes of 122 patients with ALS/MND using tracheostomy ventilation (TV) (C107)
  • “In deciding on whether to transition to TV, or to start TV in event of emergency ARF, clinicians need to know expected survival and outcomes of TV users with ALS/MND.” Platform Communications, page 83
  • Social Media Engagement: 275+ across all platforms
    • ““Patients must indicate in advance directives if TV withdrawal is desired, if they could no longer communicate. “🗣🗣🗣 I DO NOT WANT TO BE TRACHED ” @sunnyberasmus
    • “Another of my favourite speakers that stimulated our NIV work:Pamela Cazzolli reminds us pts may deteriorate suddenly so if they don’t have their NIV with them or don’t know to use it they may die or need tracheostomy. Need to educate staff and patients” @drestherhobson
  • I do not want to be trached. I do not want to be trached. Ever.
• Energy metabolism defects in ALS (C108)
  • “Background: Beyond motor symptoms, patients with ALS suffer from major defects in energy metabolism…support the view that nutritional intervention could be useful to slow down disease progression, and a proof of concept has been provided in patients. Further studies are warranted to identify pathways that could be pharmacologically targeted to counteract weight loss and hopefully slow down disease progression and improve quality of life of ALS patients.” Platform Communications, page 84
  • Social Media Engagement: 120+ across all platforms
    • “(C108) @mndassoc funded Luc Dupuis @ldupuis_Inserm is talking about defects in energy metabolism control in MND and how these could be targeted with drugs to counter weight loss and perhaps slow down disease progression and improve quality-of-life” @mndresearch
    • “Ben and Jerry’s Therapy” @alsadvocacy
    • “bring me all the ice cream” @sunnyberasmus
    • “Dr Dupuis: Half to 2/3rds of ALS patients may present with presymptomatic hypermetabolism (increased energy expenditure) which is not explained by a decline in food intake.” @shellikeriii
    • “Kevin Talbot & Matthew Kiernan @JNNP_BMJ open the #alssymp joint closing session and introduce L. Dupuis (France) to present on energy metabolism defects in #MND #ALS – a hot topic – weight loss/energy homeostasis often precedes motor symptoms by many years.” @cebirks
    • “Weight loss starts years before #ALS #MND diagnosis and is associated with disrupted energy homeostasis, which could be targetted by adapted nutrition. Prof Luc Dupuis @ldupuis_Inserm provided an excellent overview of energy homeostasis in MND in the closing session of #alssymp.” @mndresearch
  • I tell everyone that electrolytes are my go-to for energy, endurance, and efficiency. I drink bottled water with electrolytes when I don’t have access to my Spark and Rehydrate from Advocare. Gatorade helps too in a pinch but has lots of sugar. My dietician told me at my first appointment that he wouldn’t sweat over 5 pounds either way, but anything more we would need to discuss how to add more calories to my diet. I get busy and often forget to slow down and eat, or eat well, so when I start feeling the fatigue, I eat ice cream or drink Ensure to feel better. I attend clinic quarterly, so I always pay extra attention to my eating a few weeks before clinic to avoid getting in “trouble” for me weight.
• Fitness, exercise and ALS (C109)
  • “Background: Despite the well-established health benefits of physical activity (PA), a high activity lifestyle may also be associated with an elevated risk of ALS/MND. Reports of an increased incidence of ALS/MND in professional sportsmen have stimulated research in this field” Platform Communications, page 84
  • Social Media Engagement: 200+ across all platforms
    • “my take away is that i should have been less active and eaten more” @sunnyberasmus
    • “Brilliant talk by Professor Dame Pamela Shaw from @neuroshef about the fitness, exercise and ALS” @kingsmnd
    • “What a privilege to have Prof Dame Pam Shaw of @sheffielduni delivering the final talk of the 29th #alssymp. Increased physical activity might only be a risk factor in people with specific genetic variations, and studying gene-environment interactions is paramount.” @mndresearch
    • “Another hot topic – Prof Dame Pam Shaw (UK) on fitness, exercise and #ALS #MND – many anecdotes but what do we know? Concludes by suggesting that physical activity may only be a risk factor for people who may have certain genes – more research needed” @cebirks
  • I’ve recently started having PT and OT come to my house for home appointments. This addition of stretching, potentially adding in strength training but not now, and ideas for adding in exercises to my current routine has been beneficial. I sleep better, have become more in-tune with my body’s movements, and feel better overall. I’m aware of the limit my body needs to meet for increased benefit, without exceeding that limit and putting myself in risk. I had forgotten how much my body craved physical activity, so these additions have been welcomed.
• Patient Impact Award
  • Neale Daniher & the late Ian Davis @FightMND as winners of the @PrizeForLife Shay Risoni Patient Impact Award
• Poster Prize
  • Clinical Poster Prize winner, Harry McDonough
  • Biomedical Poster Prize winner, Fleur Garton and Yuri Ciervo
• Invitation to Perth 2019
  • I have to be in Perth. I’m doing everything I can do to earn my attendance to this conference. Scotland was a game changer for my efforts, sparked my determination to be involved in changing the future of this disease. I will be in Perth, it’s happening.
• Late breaking news
  • “ICYMI: @Cytokinetics presented new data at the recent @mndassoc #ALSsymp, incl. patient baseline characteristics & demographics from FORTITUDE-ALS, the Phase 2 clinical trial of reldesemtiv in patients w/ #ALS. Learn more: http://bit.ly/2rygzr4 .

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In conclusion, this experience was one of a lifetime. We had the opportunity to interact with the future of ALS in the form of scientists, therapists, and people with ALS. Each of the patient advocates has their own story, their own takeaways from this symposium. Additional voices from pALS would be greatly beneficial to the enhancement of this event. Having the opportunity to share this experience with my caregiver, in my case my sister, was impactful in a way that could only be achieved through attendance.

My sister had the following input:

• It was very interesting to see all the different aspects of mnd /als, the different clinical trials they were doing or had done and the outcomes of them all
• I didn’t think I would understand a lot of the conference but some of the things that were discussed made complete sense to me
• I would suggest that the conference discuss more and be more open about the “end of life” part of the disease
• I personally agree with Cathy when she said more people doing the presentations need to thank the people personally affected with the disease rather than the team that helped them

Acknowledgements: I’d like to thank all ALS and non-ALS participants who completed any research that contributed to the presentations of this conference. Thank you to the other patient fellows for investing in the success of this experience, for being vulnerable enough to share with the world. And last but certainly not least, Thank you to our fearless leader, Cathy Collet, for getting us there, organized, and engaged in the event.