The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis (A Rant)

challenges in the medical journey ahead

Please note that the following is based on my own personal experiences as a patient living with autoimmune encephalitis. Everyone’s journey is different; some may disagree with the views presented.

The world that doctors live in…

The classic story arc of autoimmune encephalitis goes something like this: a patient presents to the hospital emergency room with an abrupt onset of psychotic features that include hallucinations, paranoia, anxiety, and strange behavioural changes.

They’re evaluated by the psychiatry service, medicated with psychotropic medication, and monitored to ensure they don’t become a danger to themself or others.

But the patient’s symptoms persist; then, seizure-like activity is witnessed by someone on their medical team. Physical weakness, loss of balance, autonomic dysfunction, and other physical abnormalities quickly follow.

The neurology service is called in. An EEG, lumbar puncture, comprehensive blood panel, and an MRI are ordered and come back with several abnormalities.

The diagnosis? Presumptive autoimmune encephalitis (AE), made definitive a couple weeks later when serum and spinal fluid results are reported positive for antibodies against the NMDA receptor (or another recently discovered autoantibody).

Steroids and IVIG are promptly administered, further deterioration is halted, and the patient begins to make a turnaround. Within weeks, family members start to recognize their loved one again. The patient has some months of rehab work ahead of them, but the disease has been arrested—modern medicine has done its job!

At the 6-month post-discharge follow-up, healing is judged to be progressing smoothly; 1 year later, the AE patient has recovered and made a full (or nearly full) return to their pre-illness activities. No further complications. Case closed.

….Versus the world that patients live in.

Oh what a neat, tidy, and ultimately naïve narrative.

It’s the idealized version of the patient journey—the one that, in fact, most (but not all!) AE survivors fall short of.  Myself being one of them.

More saliently, it’s the story that poorly-informed physicians—often neurologists—tend to stick to. No ifs, ands, or buts.  In their eyes, anything that colours outside of these lines can’t be related to autoimmune encephalitis.

…..Okay, that’s probably an unfair characterization and overgeneralization. Forgive me for being salty right now; not all doctors are narrow-minded. I’m just exasperated after speaking with 5 neurologists too many in the past month, continuing to have ongoing medical chores, running on fumes, and feeling mostly like crap for the past 8 months. It’s both heart-warming and soul-crushing to think back to this time last year, November 2019, the first time I felt like I had finally gotten my neuropsychiatric symptoms into remission after a 5 year journey of misdiagnosis (which culminated in being hospitalized).

One whole year after recovering from Rituximab and IVIG, I didn’t think I’d be back here. In some ways, 2020 has been like the sped-up version of my 2010s: a time that began with unbridled dreams, aspirations, and excitement (excellent health as I began adulthood); a sudden lurch that threw everything up in the air (medical challenges and uncertainty); and a bumpy landing where all sense of progress ground to a standstill (poor health). In 2020, I started a new chapter of life in a new country (USA); I relapsed; and the subsequent months have gone sideways. The 2010s DID conclude with some medical miracles and a remarkable recovery—if I look through an optimistic lens, I can see how things may be shaping up for a similar outcome again.

But right now I’m just bitter.

This time last year, I knew relapsing was a possibility; however, I was comforted by the belief that I had a concrete diagnosis after spending 5 years without one. I thought I was done with falling through the medical cracks. As far as I knew, autoimmune encephalitis is a very treatable condition, we know what medications work for me, and the field continues to rapidly progress. And I managed to get my full health back, after progressive worsening that started in 2014.

So maybe you can imagine my anguish from realizing I’m in the midst of reliving parts of that 5 year nightmare once again. Instead of accessible medical care and treatment after my relapse, I’ve been met with medical gaslighting and new barriers—made all the more heavy by avoidable medical trauma of my past.

light faded in the distance

Here’s a story that amalgamates parts of my own healthcare experience together with common experiences I hear from other AE survivors in patient support groups:

What happens when you relapse

1. Some doctors dismiss the possibility that you are relapsing.

One day, you face the fact that some vague symptoms have crept up on you the past couple weeks. Maybe your balance seems a bit off. Maybe you’re slurring your words a bit. Maybe your emotions are uncharacteristically erratic. Maybe you’re having bizarre memory lapses and spells of paranoia.

So you go back to your doctor, only to be given a flippant reminder that AE often leaves long-term sequelae and permanent deficits you have to learn to live with. There’s nothing more to be done. The inflammation can’t be there anymore. Go home and get on with your life.

But lo and behold: a couple weeks later, your health deterioration is undeniable. You’re forced to take a leave from work, and find yourself readmitted to the hospital after having a seizure.

Evidently, overcoming a return of AE symptoms is not simply a matter of pulling up your bootstraps and willing your way back to good health.  The immune system can be a temperamental beast; having it knocked out of balance once can certainly make it more susceptible to being knocked out of balance again. While not all symptom exacerbations point to a true relapse caused by immune dysregulation, there’s certainly reason why patients should remain closely monitored.

Research is limited, but a publication from Dalmau and Graus (2018) estimates that relapses occur at a rate of 12-35%. You learn later (just a little late) that some doctors know to be vigilant and closely monitor for returning symptoms, while others adhere to the misguided dogma that AE is rarely more than a monophasic illness.

2. There are no widely accepted protocols for what to do during a relapse.

Autoimmune encephalitis entered into the neurological mainstream in 2007, when Josep Dalmau at the University of Pennsylvania published his discovery of anti-NMDA receptor encephalitis. That makes the field of autoimmune encephalitides relatively young.

While AE experts have roughly come to agree on the medications and protocols for patients early in their disease course (particularly anti-NMDA receptor patients), there’s little out there to guide physicians dealing with patients who recover slowly, have ongoing symptoms, develop new symptoms, or end up in a full-blown relapse.

So when you consider how common initial treatment delays are due to under-recognition of the disease and lack of worldwide AE expertise, it’s not hard to imagine the reluctance and challenges faced by the average physician when confronted by a patient in the midst of a relapse.

3. Your response to medications may not remain consistent over time.

A logical place to begin re-treatment might be to use the medications that helped you recover the first time AE entered your life.

Thus begins the lovely road of IV medications all over again.  It’s a painful crawl down memory lane, but it’s a relief to know that at least your medical concerns are now being taken seriously. Further deterioration seems to have halted, and everyone’s hopeful that you’re back on the mend.

Fast forward a few months however, and it’s clear that your relapse recovery is swerving left in a new direction. This isn’t the familiar road you were expecting.

Later, you learn it’s not uncommon for medications to lose their effectiveness as time goes on. Sometimes, different methods of targeting the immune system are warranted.

more medical investigations
Back in the thick of the medical journey.

What happens when you talk to other AE patients

4. You realize that the presentation, diagnosis, and treatment protocols for AE are more heterogeneous than you were led to believe.

When you realize your recovery isn’t going as planned, you reach out to patient support communities to find out how others are coping after their AE diagnosis.

It’s a boon to find real human beings who lend colour to the lonely experience that is autoimmune encephalitis. It’s also shocking at times: you learn far more from fellow patients than you do from your own doctors. You’re far from the only one without a textbook case of AE.

There can be a range of severity levels, some are misdiagnosed for years before receiving immunotherapy, people have variable responses to the conventional treatment protocols, and relapses ain’t so rare afterall.

It becomes clear that the current state of AE medical literature is not wholly representative of what this disease looks like for the patients living with the condition.

5. You observe large differences in the style of treatment offered to patients.

What dose of insert-name-of-medication-here should be administered? At what point do you escalate to second and third-line treatments? Are there instances where therapies should be combined? How long should a patient be kept on immunosuppressants? When and how do doctors make the judgment call that indeed, this patient is in an AE relapse?

You see people from across the globe chime in to various online forums with their experiences. Turns out people are placed on medication regimens and provided with recovery guidance as variable as the manifestations of the disease itself.  Sure there’s many points of commonality, the basics of which appear in the medical journals; but it’s also unnerving to realize that the standard of care can differ from physician to physician, let alone state to state and country to country. This has drastic implications on a patient’s ongoing quality of life.

Some people are automatically provided with rehabilitation support in the form of occupational or physical therapists. Some are directed to get a neuropsychological examination. And some others might be left to their own devices after their discharge from the hospital, clueless that perhaps they could benefit tremendously from a speech language pathologist that provides cognitive therapy (i.e. me, up until a few months ago).

Most strikingly, you learn of several major U.S centres that have adopted the convention to keep patients on at least 2 years of immunotherapy after the acute phase of their illness. You notice a minority are even told by their docs that they’ll be on some form of an immunosuppressant for life.

Well, perhaps THAT partly explains why I relapsed, you think with some bitterness. Your original care team cut you loose from all meds and deemed you fully recovered, after only half a year of immunotherapy.

another doctors office
Another glum doctor’s office.

What happens when you get a second opinion

6. Seronegative patients battle the ongoing question of whether they really even had autoimmune encephalitis in the first place.

After you’ve spent a couple months at a plateau—and far from your healthy baseline—you seek out a second opinion.

But you end up worse off than where you began prior to the appointment. Rather than offering insight into how they treat AE patients and suggestions for how to adjust your current regimen, this doctor spends an inordinate amount of time hung up on the fact that you do not have an identified autoantibody, despite other abnormal biomarkers consistent with AE. Even though antibody negative AE is a legitimate diagnostic entity. Even though new antibodies are continually being discovered.

They’re skeptical about the recovery you describe the first time you were given immunotherapy a couple years ago, using your inadequate response to recent treatment and ongoing symptoms as ammunition for their assertion that you have a primary psychiatric disorder instead. Even though there are more and more case reports of antibody positive encephalitis patients who fail first and second line immunotherapies.

You realize you’ve just been medically gaslit. Join the crowded club.

7. The search for proper medical recognition and treatment can become just as exhausting as managing the symptoms of AE itself.

You pingpong back and forth between your longtime family doctor, psychiatrist, and neurologist. They’re sympathetic to your situation, but ultimately reluctant to be the one to step up and alter your current medical treatment. They’ve had little to no experience treating a patient like you. Maybe your body is just taking longer to respond this time, one of them tries to reassure you. Another shares your concern that you’re backsliding, and encourages you to continue to seek out further AE expertise and guidance.

So you spend more time in patient groups, gathering names of doctors that others suggest. You take a deeper dive into the medical literature. You read stories from other patients in similar situations as you, sharing your darkened reality of falling through the medical cracks. You follow up on every promising lead, and spend countless hours reaching out to medical institutions that are miles and miles away.

You search. And search. And search.

Your efforts sporadically yield a new piece to the puzzle, with key information and answers feeling so close yet so damn far away.

8. It may take a few “second” opinions, but your persistence finally pays off.

After dead-ends ranging from offices that never return your messages, to doctors transitioning out of clinical practice to focus on research, to doctors with new patient appointments booking too far away in the future, you manage to line up some appointments with a few of the top experts in the autoimmune encephalitis field.  There’s no shame in doctor shopping by this point; you can’t waste any more time putting all your eggs in one basket.

One doctor stands out, offering an astute assessment of your present situation and outlining a clear plan for future treatment. Why can’t all neurologists be as straightforward, compassionate, and solutions-focused as her?!

There’s no guarantees that the new protocol will work, but at least your sense of hope is renewed once again.

brightening light

So… now what?

Turning the narrative back to me: I started writing this post on a day I was frustrated, having spent large portions of the past weeks scraping by in survival mode.  It’s been a grueling time, with doors thudding shut and leaving echoes of the question “why do I even bother?”

I thought I simply needed to rant—which I ended up doing, to an extent. Part of me just wanted to curse away and throw a pity party, nevermind whether I should bother to post something like this at all. Let’s just say that these topics can get touchy and lead to arguments that cross lines into toxic territory.

But then I thought about the types of people who reach out to me. Oftentimes they’re stuck in a similar maze, feeling unsupported by their current medical team—or worse, by friends, family, and even other AE community members. I’m not a medical professional, but at least I can offer thoughts based on my own patient experiences.

And then I realized I’m tired of these pain points being swept under the rug. They’re shared by a significant number of AE patients, whose quality of life is worse off than it needs to be because a multitude of barriers exist between them and the medical attention they deserve. Throw in self-doubt, a lack of support, and discouraging remarks from those they’ve placed their trust in, and it’s easy to get lost in that “why bother?” mindset.

To those that are fighting similar battles right now, please carry on. Push forward and don’t lose sight of the light at the end of the tunnel, no matter how much it seems to be diminishing. It’s still there, waiting to brighten once you make it around the right corner.

I’m not sure exactly what’s ahead in my health saga, but I’m pleased to say that some new puzzle pieces finally came together this month.  Another doc, another approach, and better medical support have entered the picture.  And right now I think that’s enough to celebrate: getting to this point has been a hard-fought win.

Want to connect? Find me on FacebookInstagram, or Twitter.

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You may be interested in reading the following next:

Autoimmune Encephalitis Recovery, Part 1: A Look Behind the Scenes of Emotional Breakdown

Autoimmune Encephalitis Recovery, Part 2: The Day-to-Day Emotional Challenges

The 7 Ways To Piss Off Your Neurologist, As Told By An Encephalitis Survivor

 

8 Comments

  1. Lori Workizer
    November 28, 2020 / 11:19 pm

    Different disease but similar trajectory. We have in common a similar frustration: herding doctors when you feel like crap, while trying to manage a 40+ hour a week job, not to mention a life. It makes me cranky.

    • wherearemypillows
      Author
      November 29, 2020 / 12:17 am

      Dang—kudos to you for hanging on to a full-time job through a heavy medical situation. With the amount of energy that goes into advocating for healthcare in situations like ours, it’s no wonder you feel that way! Some weeks the medical fight leaves me more exhausted than when I was working full-time on my career. I wish doctors would understand the sheer amount of strength and determination it takes to manage our ailing bodies before brushing us off.

  2. November 29, 2020 / 8:00 pm

    Beautifully written and told. You have captured the most common situations patients and families face as they travel this difficult journey. I’m sure many will feel not so alone and validated by this article. Keep writing, we love your blog. #AEWarrior #StrongerTogether

    • wherearemypillows
      Author
      November 30, 2020 / 6:37 pm

      Thank you Tabitha, your comment means a lot! It’s so disheartening to go through these things, but the burden is made lighter with the support of AE community members like you! 🙂

  3. December 7, 2020 / 5:48 pm

    I really feel like you capture the stress and frustrations of AE and the fight to get good care. Thanks for sharing! Alyse

    • wherearemypillows
      Author
      December 18, 2020 / 8:36 pm

      Thanks Alyse, I hope you keep pushing for the right care and docs!

  4. Mary
    February 2, 2021 / 12:38 pm

    This is an amazing article. It is well written and captures a unique aspect of a unique situation. I am curious if you can write another article about how this may look/feel to the extended family or caregivers- how even though diagnosis is there- care is not always immediate and not always accessible.
    It would help so many struggling.
    XOXO
    Mary

    • wherearemypillows
      Author
      February 2, 2021 / 5:17 pm

      Hi Mary! Thank you for your kind words – I’m hugely passionate about advocacy in this space, and I’m glad to hear that my words are reaching others. That’s a great topic suggestion and I will note it down for a future post!

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