To some extent, we’re all trying to achieve the same goals.
Earlier diagnosis. Better treatment. Better outcomes.
But we’re not on the same page with respect to how to get there. Patients, caregivers, clinicians, scientists, nonprofits, obscure blog writers… when it comes to autoimmune encephalitis (AE), these stakeholders are conceptualizing the roadmap very differently.
Which isn’t a bad thing per se.
Except that these stakeholders aren’t talking to one another. Not in meaningful ways at least. Sure, we brush past one another with the vague understanding that we’re all in this together; but I’m not seeing an engaged dialog or sustained collaboration amongst different groups. (If you’re seeing something I’m not, please let me know.) What I’m seeing instead is a series of disjointed, uncoordinated, and sometimes redundant efforts to solve problems that don’t add up to a cohesive whole.
Don’t get me wrong though: effort is better than no effort. You have to start somewhere; some, like Australia’s Monif Group, have recently started a Community Engagement Program in an attempt to bridge the disconnect between encephalitis researchers and patients. And the field is moving forward, as evidenced by things like the strengthening of patient/caregiver communities, the growing attention encephalitis is receiving from the medical community, and the increase in encephalitis/autoimmune neurology-focused conferences. These things should not be understated.
I just think they should be happening quicker. And I don’t mean that in some pouty or vague and aspirational way; I mean it in realistic terms, with the current resources we already have. The stakeholders exist in formalized ways today, they care, and they are positioned to make a difference; but now these disparate groups need to come together, cooperate, and move forward informed by perspectives from across the table.
We don’t need to reinvent the wheel
At the spring 2022 Treat AIE Networking Conference there was discussion about establishing patient registries and coordinating efforts to share information amongst researchers within and amongst different European countries.
Nearly 10 years ago, 5 German clinicians came together to plant the seeds of what would eventually become the GErman NEtwork for REsearch on AuToimmune Encephalitis—or GENERATE, for short. Their membership has now grown to 117 clinical centres who have not only collected information on 1800+ patients but have also published some excellent papers. You can see a sampling of what they’re up to here.
Now, Italian doctors are in the infancy of setting up something similar: the Network Italiano Neurologia Autoimmune, or NINA. You know what was smart? The conference program included a presentation from a member of GENERATE and a member of NINA. They shared experiences, challenges, lessons learned, and future goals. There were questions, answers, and open discussion. They learned from one another. Actionable tips were exchanged. Future energy has been spared from having to reinvent the same wheel.
The lesson here: you can learn a lot from studying the people who have already solved their version of your problem. More on that later.
This group deserve major props for organizing the conference, encouraging greater research collaborations, and making a livestream accessible to an obscure blog writer the public. This is actually really important groundwork (and maybe I’m not appreciating how much time this in itself takes to set up).
But there was a call from one researcher, who investigates outcome measures, to go beyond the modified Rankin scale (mRS) and actually measure the things that matter to AE patients—like cognition. There was also a comment by a colleague that basic scientists can tend to forget that patients are more than just bags of blood cells and CSF. Statements like these, combined with my own bias, only make me wonder—would it not have been appropriate to have a handful of patient/caregiver representatives present at this meeting? For a conference meant to develop European AE treatment guidelines…. to serve patients?
Besides giving you a taste of what’s on the minds of leading European researchers in the field, I meandered down this road to illustrate a point: it’s a waste of time and energy to reinvent the same wheel. Not just for NINA above, but for wide pockets of the autoimmune encephalitis field.
Yes AE is a unique disease entity, yes it’s important to do rigorous studies, yes it’s a special snowflake with nuanced considerations—but why does it seem like we’re examining it in a vacuum? Why aren’t we looking harder at the things that work in other related diseases? Why do scientists and clinicians seem so slow to pick up on patterns in broader autoimmunity and neurology, and apply principles from those learnings in the absence of data specific to AE?
For starters, how about looping the patient voice not just into scientific discussions but into our personal care and treatment decisions?
Turns out that’s not just a nice sounding but impractical hope from a disgruntled patient. Turns out that’s what the multiple sclerosis field (and many others) has already learned works best.
The hard way.
The way future AE patients can avoid if clinicians start paying closer attention.
A glimpse into the multiple sclerosis world
I started poking around the multiple sclerosis literature earlier this year after the nerd in me ran out of encephalitis papers to read.
At first it was hard—a humbling reminder of how challenging it can be as a lay person to learn the scientific ropes of a new field (easy to forget when you’ve already spent years exclusively immersed in reading encephalitis papers). Then it became familiar. Then it became provocative, as I started recognizing parallels between the hunches I’ve developed as an AE patient and the facts spelled out in MS literature.
I’ve only scratched the surface of what’s out there—the depth and breadth of MS research is much greater than what you’ll find in AE, due to reasons including its discovery a couple centuries earlier (~1868 vs. ~2005 for NMDAr encephalitis), its higher profile, and greater research funding. So you may want to take what I say below with a few more grains of salt than usual.
But here’s my take: even with 150+ years of study, the multiple sclerosis field remains rife with unmet needs. Though considered rare, there are still an estimated 2.8 million worldwide who live with it. And unfortunately there’s no cure; it is generally understood to be a progressively disabling autoimmune illness that can rob those afflicted of their mobility, vision, bladder control, cognitive function, and a host of other things. It was just earlier this year—in 2022—that Epstein-Barr virus was all but proven as the cause.
And MS researchers are still attempting to find answers to the types of questions that are being asked in the AE world today. Ones that seem unlikely to have a rigorously tested, science-backed answer for decades.
That does not inspire confidence in how quickly we’ll unravel the big questions of AE. Oops, pessimism was not the tone I was trying to go for with this post. But what I wish to emphasize is the fact we don’t want AE to follow this trajectory. While the landscape of scientific innovation in the next 150 years is certain to look radically different from the past 150 years, we can’t just assume that our understanding across all diseases will dramatically accelerate. Resources are fragmented. The institutions one might expect to forge these new frontiers can be…. Fragile. Bogged down by politics. Inefficient.
I’ve learned so much from reading MS research that I could spin this post in a multitude of directions. But I’ve chosen to focus today’s post on the role of the patient voice, tying in with my earlier assertion that stakeholder groups need to improve their lines of communication with one another.
The TL;DR of my 7 year AE journey: sick in 2014; not diagnosed until 2019. Spent the past 3 years running through all standard first-line, second-line, and experimental treatments: steroids, IVIG, plasmapheresis, rituximab, and Actemra. Doctors dragged their feet until they eventually had to reach for cyclophosphamide, the chemotherapy grandaddy of them all. Which—knock on wood—seems to have finally extinguished the inflammation for good.
I’m not trying to be arrogant here but I spent ~80% of that whole journey miles ahead of my specialists. I had already identified AE as the likely diagnosis several months before I finally received the right medical attention via being hospitalized for the first time. But I had to wait to deteriorate to the point of catatonia first. I had already identified that I was in a genuine relapse, twice, before my doctors finally escalated treatment. But I had to wait until significant, career-damaging impairment first.
Other AE survivors share similar stories and unnecessarily protracted recoveries—sometimes permanent brain damage—thanks to the profoundly paternalistic dynamic of the average patient-doctor relationship in the AE world today.
Maybe this made a bit more sense in the pre-internet days. Physicians often acted as the gatekeepers to specialized medical knowledge, and rare disease patients had little means of connecting and comparing notes.
But this is an outdated model now. Especially in instances where the treating physician has never encountered the rare disease you’re presenting with. Patients and caregivers can gain access to many of the same resources that said physician might consult to inform their often rushed “expert” decisions—peer-reviewed journals, medical textbooks, and sometimes more!
Online forums now afford the opportunity to harvest hundreds of insights from those with the lived experience of the illness. Assuming judicious assessment of the quality of discussion (which, admittedly, patients/caregivers do not always exercise), surely that is worth something compared to the specialist’s background of n=0?! I’m thankful for the two neurologists responsible for getting me to where I am today, who have demonstrated the willingness to engage in collaborative patient discussion where others have not.
As for the 10 other neurologists in my history—well, let’s just say they’ve left me with an overall unfavourable view of their specialty. I respect that they have years of training and hands-on treating experience that I obviously don’t; but their broad failure to give any credence to the patient perspective is absurd.
MS in the 21st Century Initiative
Bringing this back to MS: I came across the MS in the 21st Century initiative, which began in 2011 “as a group of MS healthcare experts aiming to standardize care worldwide.” They’ve got some incredible movers and shakers on board, and I’m a fan of the papers I’ve read from them. And curiously enough, they state:
“Recognizing the importance of including the patient voice in our work, in 2016, we also welcomed MS patients to the group.
Through our joint research, two things quickly became apparent:
- There are considerable differences between what patients and healthcare professionals believe to be patients’ greatest unmet needs in MS care.
- In order for the two groups to better understand and support each other, communication in MS care needs to be improved.”
A+ move. Cool. Great. So what?
WELL, what might read as a little basic and unexciting to the casual reader is, in fact, a pretty big f*cking deal (!!! 🤯 ) to someone like me. Someone with an illness that is paternalistically managed by the average doctor, who seems to be oblivious to the fact that patients can provide valuable insights that improve the success of their healing efforts.
How long until this “MS in the 21st century” spirit bleeds over into the AE world? Who knows; but I can tell you that it’s sorely missing from the landscape today. And both patients and doctors are all the worse for it.
Here’s an example of something I’m afraid AE docs are going to take eons to explore: that the average AE patient is undertreated. That the threshold used for escalating treatments is way too high.
It’s one thing to be one vocal, disgruntled patient telling you this (I sat cringing the other day when I heard a message of caution at a neuroimmunology conference, warning colleagues against overtreating. I have personally talked to hundreds of AE patients and I don’t think I’ve come across a single one who felt they were overtreated).
It’s another thing when the number grows: I have dozens of AE friends with personal journeys that bear testament to the same conclusion.
It’s yet another thing when stories on the same theme are sprinkled across patient support groups on the daily.
It’s YET ANOTHER thing when similar ish autoimmune diseases, with a field of research more advanced, have steadily moved towards more aggressive treatment styles. Like…. MS!
Granted, MSologists aren’t a united bunch. They remain pretty variable in their approaches; you still have conservative doctors, aggressive doctors, and everyone in between. But the field has steadily shifted over the years, in part led be vocal proponents urging their colleagues to “flip the pyramid to treat MS” and rethink their treatment paradigms so patients are given the choice to start high-efficacy disease-modifying therapy (DMT) early.
Just one of many, many studies that illustrate the benefit is a paper comparing national Swedish and Danish MS registries on 4861 total patients between 2013 and 2016. The Swedish were more aggressive: 34.5% of Swedish patients initiated a highly effective DMT vs. only 7.6% of Danish patients. The Swedish strategy was associated with a 29% reduction in disability scores vs. the Danish strategy. The authors conclude that “escalation of treatment efficacy was inferior to using more efficacious DMT as initial treatment.” Yeah, no kidding—for one, just think of how the different national strategies translate into numbers of individuals remaining in the workforce vs. collecting disability payments.
Again, I recognize that the immune system f*cks up in a different way between MS and AE. They are distinct diseases. And it’s conceivable that evidence in the future could emphatically quash my assertion that current AE treatment standards are skewed towards undertreatment. But at the very least, the things I’ve told you here and in other posts illustrate that a) AE best treatment strategies are highly debatable b) patients should have a seat at the table of debating the details of what AE “undertreatment,” “best treatment,” and “overtreatment” entails.
That’s not happening today though. Instead, at the Treat AIE Networking conference, the European medical experts spent a couple hours debating whether or not there’s enough evidence to conclude that treating AE patients with immunotherapy, period, lessens patient disability. It’s not that I think we shouldn’t evaluate the quality of evidence for that; it’s just that the field needs to employ better strategies to pick up the pace of our understanding and improve recovery outcomes ASAP.
(On a separate note, there was also a lot of stimulating conference discussion that I found thought-provoking and enjoyable. I really do respect the commitment, determination, and sophistication of the researchers that are paving the path forward in the encephalitis field—I fully acknowledge I wouldn’t be in recovery without their efforts—but that’s a discussion for another day.)
Are you just going to talk the talk? Or are you going to walk the walk?| A personal update
I started writing this post last month, aiming to have it published by World MS Day on May 30.
But here I am now, 1 month late, feeling a mixture of frustration for neglected AE patients, ambition to make a difference, and exhilaration in recognizing that my brain is back to cranking out hundreds of words an hour again. Rather than say, 100 total, when I was actively relapsing. That’s a huge emotional win for me, especially given my previous work as a Communications Manager.
You might already know that it’s been a very long road for me to get to this point of healing—a pretty dumb and needless road, honestly—but what has been hitting harder in the past few weeks is the realization that I actually got here.
Well, I’m still working on getting all the way there. I’m not back to 100% baseline the way I was at the end of 2019; in fact, some days are still kinda sh*t. But on a really, really good day when Concerta is hitting all the right neurons, I’d say I can get to a pretty solid 80-85% for several hours. Which I can appreciate is far further along in recovery than some patients ever get to. Still, I can’t help but to be a tad bitter that I need a stimulant now in the first place, and that I do feel a sense of residual brain injury—preventable had doctors been on the ball—that hadn’t been there before my first relapse.
Whining aside: I got here. As the weeks wear on I feel more and more assured that the active inflammation is completely behind me, and that I actually managed to slay the AE beast that many still battle. I should write a different post on this; but with people asking me what’s up, I did want to provide that quick update here. It feels like a miracle. I have so many people to thank for their support, kindness, and encouragement.
That was a long way of saying that I’ve finally recovered enough capacity to not just talk the talk but to start walking the walk. You can probably read between the lines of my thinly veiled attempts to water down my critiques of the autoimmune neurology field. There’s… a lot of room for improvement. And we don’t need to be reinventing the wheel to improve patient care today.
What are you going to do now?
If you’re not in survival mode and have a few ounces of energy to spare, I urge you to carefully consider what elements of your patient/caregiver journey should have gone better (if any). Be specific. Who dropped the ball? Why? What could have been done to prevent that?
More importantly, can you play a role in effecting change for future patients/caregivers who will inevitably encounter the same pitfalls? The answer is yes—yes, you absolutely can. Like I touched on earlier, the patient (and caregiver) voice is waaaaay underrepresented in discussions that are shaping this field. Some of the things that might seem obvious to us are not landing in the minds of physicians/researchers in the way they should be. And knowing the things I do now and the number of needless, enduring obstacles on the paths of struggling AE patients/caregivers today, that really grinds my gears.
If there’s one benefit of me having been a 3x encephalitis piñata to so many medical professionals, it’s that I developed the passion for channelling my pent up aggression applying my personal background and professional skills towards endeavours that will improve the autoimmune neurology landscape for others.
But I’m going to need your help. I’ve been busy the past few weeks laying the groundwork for some exciting projects that I look forward to sharing with you, hopefully later this summer. The success of them will depend on wider community participation, and I hope you might consider becoming involved. Feel free to shoot me a message if you want some early info or to throw an idea into the hat.
Cheers to those who’ve already started the heavy-lifting, and to those gearing up to get up and off the sidelines 🥂
You may be interested in the following posts next:
Who ACTUALLY treats autoimmune encephalitis? | Part 1: My experience with doctor shopping, a psychiatrist, and a dozen neurologists
The 7 Stages of Receiving Medical Treatment, as Told by a Rare Disease Survivor
The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis (A Rant)
What happens when your autoimmune encephalitis treatments aren’t working?
World Encephalitis Day: Where are we in 2022?
The 7 Ways to Piss Off Your Neurologist, as Told by an Encephalitis Survivor
Relapsing… again. Autoimmune encephalitis round #3: Let’s go 🥊🥊🥊