Slides
Transcript
Thank you for the introduction. Growing up, I had the privilege of thinking that medical problems would have simple solutions. I never broke a limb myself, but for friends who did, it was a straightforward fix. I had significant stomach and GI issues when I was younger, which in my early 20s I learned was celiac disease. Again, a fairly straightforward fix, albeit a tough one to adjust to at first.
I thought this all the way into my late 30s, lucky as I was to have gotten through life mostly unscathed. I spent a decade traveling, writing, eating my way around the world, blissfully unaware that I was a “canary in a coal mine genetically”, as a doctor later called me. Hypermobility, paradoxical reactions to medications, and a talent for tearing ligaments were really my only clues about what was to come.
I sustained my leak in 2017. I underwent a lumbar puncture without image guidance and with 18-gauge cutting needles, which took multiple attempts. As I said last year, I’m very small, and I wish I knew then what I know now about needle size and type. The local anesthetic didn’t work. Again, now I know that this is common with patients who have a heritable connective tissue disorder, but it made the procedure very painful.
From my initial frantic reading, it seemed like the solution for that leak would be straightforward too, with an epidural blood patch. But it wasn’t so simple for me. I was denied patching at the facility that did the LP and was told that post-puncture dural headache (PDPH) was self-limiting. It’s been 7 years, so I can safely say that’s just not the case for everyone. As a writer, too, I cringe at the reductive use of the word “headache” to describe a spinal CSF leak pursuant to a puncture. It’s so minimizing of the chaos that one feels that goes far beyond a simple headache. It’s more like someone is hanging you from the back of your neck on a meat hook, with all of the downstream symptoms and pain that would follow.
I sustained my leak in the US while housesitting for a friend, but I was living in Oaxaca in Mexico, at the time, so I had no care coverage or doctors in my home country of Canada. Fortunately, I was able to self-refer to a leak center in the States, and I had four rounds of blood and fibrin glue patching – dorsal and circumferential each. Though I wore my lucky llama shirt each time, my patching process too ended up being not so simple. During the fourth round, I went into anaphylaxis – the suspect being the fibrin glue – and I ended up needing epinephrine on the table. That patch did seal me, though, for eight glorious months, and I had RIH, rebound intracranial hypertension, that was treated with methazolamide.
In late 2018, I sat on the ground, reached for something with my left hand – I’m a lefty – and I felt lancing at my left lumbar, followed soon by a descent into the telltale leak symptoms, including the positional aspect. I went from being up all day, every day, to being flat again for 22 hours a day, and my mental state descended in tandem with my CSF volume.
So, if patching sealed me once, the question often is why haven’t I gone for treatment again? This is what I’ve been asked to speak to today. Though I was offered treatment when I re-leaked, as with my initial leak, the facts that I have make this decision denser than it appears. The heuristic for me isn’t “Will this work or won’t it?” it’s “Can I live with myself mentally and physically if this procedure lowers my baseline or treatment makes me worse?”
My answer, as you might guess given the topic of this presentation, has thus far been no. Of course, I want to be sealed and healed, and of course, I want to be well. And while it’s not a guarantee that I would worsen, I would be foolish to think that, with my facts – what I call my “onion of catch-22s” – that my odds would be the general odds. Despite the hard work, the dedication, research from all the physicians here or listening in today, it’s just not simple for patients like me who are affected by comorbidities that can impact healing, that can impact leak recurrence, and that can impact treatment tolerance.
What am I talking about with this Onion of Catch-22s?
Number one: As with many puncture leaks, my leak site just hasn’t been visible on imaging. There were also multiple punctures with large gauge cutting needles, without guidance. Do I have more than one leak? I don’t know. Surgery would be exploratory, and my successful patching has been multi-level.
Number two: A geneticist diagnosed me with a heritable connective tissue disorder, which comes with delayed wound healing and impaired tissue repair.
Number three: Lab testing confirmed a mast cell disorder that causes frequent anaphylaxis for me since that 4th round of patching — and that’s without medical procedures — as well as many other debilitating symptoms. I am mostly stable, but I’ve had hospital visits to that end. I know I can’t get fibrin glue anymore, but how else will this disorder affect my treatment? Will my body reject a graft if I get one in surgery? How will dyes, if any, in permanent stiches affect healing? Could stitches themselves cause new microleaks? These are all questions I don’t have answers to, and there’s insufficient data for us in the leak world to really answer them right now. But given my reaction to dyes and to metals and many other triggers, it may very well be an issue for me.
Number four: My imaging shows adhesive arachnoiditis, nerve clumping, and adhesions in my spine – a condition that further treatment or invasive imaging may worsen.
Number five: Outcomes with patients who have my facts are just not very reassuring. Do I know everybody with AA, a connective tissue disorder, MCAS, and an LP leak? No, of course not. But the upside of having a public-facing business is that you hear from anybody who recognizes themselves in your facts. And what I’ve heard over the last seven years is just not comforting in weighing my decision that does impact my math.
Number six: I had RIH following my last seal, and that was after only a few months of leaking, so I do worry about maintaining closure and managing my CSF pressure if I can get sealed, especially with all these other facts that I’ve listed today.
And number seven: There are other factors too beyond the ambit of this talk – like the cost of returning to the US for treatment, who could come with me and take care of me when I do, I have no specialists in Canada. It took years to even get a PCP here, and as an example, when I was sealed last time, my family had to drive to the States to get methazolamide because I could not get a script here. So, these factors add to the intricacy of my decision.
And that decision is a hard sell for people outside the leak world. When you’re sick, there’s just so much pressure on you to get well. People tell you to keep fighting, to keep pushing, to be a warrior – much like my former colleagues telling me that I would ruin my life if I quit my law job to travel the world. That pressure from others is often caused by them being forced to confront their life choices – of them fearing, them not getting well if they were in my shoes. Part of it comes from caring, no doubt, but I think a lot of it comes from this binary view society has about the choices that we make in life – that I’m either ruining my life and my career, or I’m not. That I’m either giving up on getting better, or I’m sealed. But that’s not life. Life is nuance. Real life is complex, and real life for me is hoping for a future where I can be sealed with these facts without a significant risk of worsening my baseline – something that, for now, really feels less assured than the potential to get worse.
The second question I get after the why is the how. How have I lived with this choice, and how do I actually seem okay? My contentment is confusing to people, and I get it, because from the outside looking in, my life is something most people pity me for. And for a very long time, I pitied myself too. Every second of every day requires this exhausting calculus of uptime moments. It’s like the Spoon Theory that Leah spoke to, but in teeny tiny increments. I’m in pain except when I’m flat, and there are new griefs and moments that I have to miss out on all the time. I can’t make it to this conference and speak with all of you in person because my mobility is just so limited. I can’t even tie my own shoes.
But overall, I really do feel a deep sense of peace despite the pain. And that may sound delusional to you, but chronic illness has taught me that the nuance I spoke to earlier – it’s also the place where the joy lives. It’s taught me that I can’t just be happy when things are going well in life. It’s taught me how to hold two birds but in one hand. The staggering pain, the grief for what I’ve lost and keep losing, and the beautiful, awe-soaked moments at a level of granularity much like my uptime that I never contemplated before. It’s taught me that there really is beauty in the small.
It wasn’t instinctive, though. It took me work to get to this place, and it’s still a choice that I get to make every day. From those early days of deep despair, what changed for me was interacting with life in three main ways.
Number one: A return to the ethos that I’d abandoned when I sustained my leak, which is curiosity. I’ve always been curious. My friends call me Jodi-pedia because I love to learn things. I knew very little about my conditions at the outset, and I didn’t get a chance to research my LP beforehand. But I can now. This means gathering my notes, writing about my conditions, learning from and connecting with other patients or people in my reader community who’ve walked a path like mine. I’ve made a monstrous spreadsheet to track my symptoms and variables for the last seven years to look for patterns. Another patient told me about PubCrawler searches – still very 1990s GeoCities look and feel – along with Google Alerts, and those have helped me keep up with research for all of my conditions. I’ve written to scientists who’ve published, asking questions, and same to leak experts. And thank you to those in this room who’ve given me the time and the patience to answer my questions. To me, more information makes things less fearsome, and data demystifies things and empowers me to make informed decisions out of facts and not out of fear.
Number two: I became my own lab rat – my n=1. I threw a ton of spaghetti against the wall during these last seven years, and I’ve tracked how it worked for me. I do hope to write more about what I’ve tried, but it includes supplements and minerals, injectable and oral peptides, red light therapy, mast cell stabilizers, and much more. And the spaghetti that stuck on the wall really helped me stabilize my mast cells and really helped me lower my pain, which is crucial because I’m not in pain when I’m flat despite having the arachnoiditis on my imaging. A big part of my consideration and my hesitation to proceed is losing that grace. If my AA worsens and lying flat becomes painful, like it has for other patients I’ve spoken with the condition, then there would just be no escaping the pain – whether I’m upright or I’m flat.
Number three: I decided that if I can’t address my leak, I can work on my mental state instead. I tried different types of therapy to see what resonated for me, and EMDR is the one that’s worked best for my brain. Alongside meditation, humor, and spiritual exploration, it’s taught me how to process my present and taught me the skill of reframing my fear and my grief and my resentment over and over during this tragedy. This has required a fundamental shift in my relationship to myself, but also in how I react to circumstances that are beyond my control. And it really is a skill. Please don’t buy the toxic sludge that it’s just “easy “ to reckon with dark times if you want it badly enough, because that is a lie. People outside the leak world say to me often, “I could not do what you’re doing, Jodi. I couldn’t live the way you live.” But they can. I’m not special here. It’s a process, it’s work, it’s tiring, and there are many days where I just want to hibernate and rest, and I do. But we’re all more adaptable than we think.
My turning point in terms of my own joy really was turning inward – this rewarding process of continuing to stay curious, continuing to keep celebrating the small, continuing to keep finding peace. And my acceptance of this, which, as I said last year, does not mean giving up on getting better, nor does it mean minimizing my pain. What it does mean is an opening of space. It frees me from clogging my mindset with anger or resentment. It allows me to look to solutions, to progress, and to hope.
I’m hoping for medicine to evolve to safely handle my “onion of catch-22s”. And it’s not an indictment on the field, I want to be very clear – because all of the physicians are working very hard to do just that. So much progress has happened since I sustained my leak, and I know people like me are the outliers to the norm. We aren’t the simple patients. We aren’t even the majority of patients. And despite not being a majority, I was asked to speak to my path here today, which is an opportunity I’m extremely grateful for.
My case isn’t everyone’s case, but for me, a lasting pause has been the safest to protect my hard-earned stability, to protect my quality of life, and to navigate my comorbidities while celebrating what I can. Even though, for now, I’m still leaking.
Thank you.