Earlier this month, the spinal CSF leak group misplaced a shiny gentle when Connie Rim handed away. Her Fb web page, known as My CSF Leak Story, was the place she detailed her lengthy battle attempting to get sealed and healed. Just a few days after she handed, her husband posted phrases from Connie about her dying, sharing it with the world.
Connie sustained her leak 5 years in the past throughout spinal surgical procedure, and tirelessly rallied her physique and thoughts in her makes an attempt to get care and discover her approach again to a spot of much less ache. Like me, she discovered solace in sharing this journey publicly. In her case, through movies that she posted to Fb and TikTok, detailing her many debilitating signs, explaining the procedures and exams she bought, and patiently answering questions.
When somebody is chronically unwell and takes a break from therapies, there’s inevitably a swell of individuals asking why. “Why aren’t you doing extra?”, as if it’s as much as you to not be higher. As if there’s full management of the lengthy tightrope you want to stroll on once you’re coping with a number of advanced circumstances. Regardless of her openness and uncooked, unflinching movies, individuals nonetheless requested Connie this query.
Maybe in response to this, or for different causes (I don’t know), in Might 2023 Connie posted an extended checklist of procedures and exams and docs she’s seen proper earlier than she headed out to Mayo for extra leak diagnostics and procedures: “This can’t be my life. This isn’t a life. I’ve been combating to get higher and advocating for myself as a lot as potential however in fact, I’m drained now. Nothing is as straightforward because it as soon as was,” she wrote then.
By this level, she had been to leak specialists and nonetheless her leak(s) weren’t sealed. She was in fixed ache. She reposted that checklist, up to date, a couple of days earlier than her dying.
She did get remedy after that Might 2023 publish, one which introduced her ache down a little bit bit. We may all really feel her pleasure and hope when it did. However that remedy failed, and when it failed it introduced a brand new, horrible set of signs. Since late January 2024, she skilled extreme stabbing ache from three to over twenty occasions day by day, at what she categorized as a degree 10 ache. To be in ache all day, after which layer upon {that a} new and protracted, paralyzing ache… I can’t think about.
By mid-Might, she was gone.
Studying between the traces of the message her husband posted, she hit a wall the place the excruciating ache she skilled second to second couldn’t be managed.
“Some days, I’ve been given 10-Quarter-hour of my treasured baseline ache, and for that. I’m grateful. However throughout the remaining 23 hours and 45 minutes, I used to be at all times crying, combating, and, at occasions, in a lot ache that I may do something however curl in a fetal place in darkness,” she wrote.
Her household and husband have been loving and type, she had plenty of help, and she or he had a group who rallied round her. I perceive that these wonderful issues will not be sufficient when you find yourself trapped in a physique that tortures you.
Research present that in a persistent state, ache can disrupt the communications between mind cells, resulting in a discount within the skill to course of feelings — particularly adverse feelings. Ache modifications your mind. I skilled a model of this myself not lengthy after my leak started: already in persistent ache for years however not but conscious of mast cell ailments, I ate a very excessive histamine meal of spaghetti and shared a glass of wine with my household. It was this meal throughout these preliminary leak days, earlier than I went to Duke for remedy, that tipped my physique its new, unruly state. After that meal, I had full-body burning and nerve ache 24/7. And it didn’t go away. It felt like I used to be being dipped in acid, with no respite from the torture.
It was a fellow affected person who urged me to consider MCAS, given my signs. I hadn’t heard of mast cell activation syndrome earlier than. With no docs to help me, I scrambled to get my mast cells underneath management and attempt to make it cease. Throughout these weeks of absolute hell, till I discovered over-the-counter medicines that labored for me, I begged a detailed buddy to assist me discover a approach out ceaselessly. (They declined, have been compassionate all through, and as a substitute tried to seek out me a grief therapist domestically. What helped on this case wasn’t remedy, it was discovering respite from the fire-burn throughout my physique because of antihistamines and a low-histamine food plan.)
And that was how I felt with only some weeks of torture.
Connie lived with that ache that for a few years. As she mentioned in her writing, “The ache determines my feelings, NOT the opposite approach round (as some individuals imagine).”
Her passing hit the leak world very onerous. Even those that didn’t know Connie personally usually watched her movies on TikTok or Fb, or interacted along with her on the teams. In my case, I used to be digital buddies along with her for years and we exchanged audio messages about methods, instructed jokes, tried to maintain ourselves sane on this Groundhog Day world we lived in when staying sealed felt like a pipe dream.
And but, regardless of the ache and the exhaustion and the procedures, Connie remained joyful the place she may, she appreciated what love she had in her life, particularly her deep and delightful love for her husband, and noticed humour the place she was in a position. She had tons of help. The ache decided her feelings, as she mentioned. She was a prisoner of her fixed agony.
I really feel indignant for her and her household, and for all of us. In that lengthy procedures checklist, it appears like she was failed so many occasions alongside the way in which. At the same time as lately as this 12 months, she offered to the ER and later shared a video explaining that the on name physician mentioned her signs have been psychosomatic. How are you going to take a look at that blisteringly lengthy checklist of therapies and suppose it’s psychosomatic?! The continuing nerve harm and tissue harm, and scar tissue that may entrap her nerves, all of it is smart trying on the lengthy checklist of what her physique had been by means of.
However no, she was instructed it was all in her head.
That’s a part of what leaves me rattled, for anybody with this situation or different invisible circumstances the place metrics for prognosis and remedy aren’t reduce and dry. I’m engaged on the slides with the US and different spinal CSF leak foundations for the annual consciousness week for this situation, known as leakweek (which begins June third). In it, we share the several types of diagnostics and notice that regular imaging doesn’t rule out a spinal CSF leak. Regular opening stress doesn’t rule out a spinal CSF leak. Regular something doesn’t rule it out; we’re trapped on this countless loop of being unable to ‘show’ quantitatively what we have now apart from signs, but usually we’re instructed we aren’t dependable narrators when sharing our signs.
And so we strive, we strive so onerous.
We current ourselves firmly however with out panic, however not TOO jokingly else we appear ‘not sick sufficient.’ We hold spreadsheets, we share information, we come armed with supporting research however not TOO many, else it looks as if we expect we all know an excessive amount of. Outdoors the few leak specialists on the market, who’re fortunately coaching docs usually, this dance continues. It’s exhausting to reside in ache and to lose your mobility and a lot extra, however add the suspicion and disbelief to the combo and it drags your soul right down to the bottom.
As a result of regardless of the advocacy, regardless of the training, the myths persist.
Outdoors the leak specialists, sufferers report that docs nonetheless suppose a lumbar puncture leak is “self-limiting” (i.e., that it goes away by itself — I want!). Many nonetheless doubt the existence of spontaneous intracranial hypotension (SIH), the place a leak can happen abruptly; this may be on account of a bone spur or calcified disc gnawing into the dura til it tears, or a coughing match, or lifting heavy weights, or one thing else being the final straw for a weak spot within the dura mater the affected person wasn’t conscious of. And a extra recently-discovered kind of leak, a CSF-venous fistula, might be elusive on present imaging varieties, although a more moderen photon counting CT machine is recognizing them extra often. Canada doesn’t have one, and the US solely has a couple of.
The brutality of this situation is difficult to precisely put into phrases. Not solely the ache itself however the uncertainty and uphill battle in getting care. It’s generally misdiagnosed and under-diagnosed. The psychological well being burden can be vital: a high quality of life research in spinal CSF leak sufferers from 2023 discovered that over half of the respondents (64.2%) endorsed suicidality, and 22.4% had demonstrated suicidal behaviour. A 2024 research about high quality of life in persistent publish puncture sufferers (like me) discovered substantial psychological well being challenges with despair, nervousness and stress skilled by 83%, 98%, and 88% of the respondents, respectively.
It’s like an enormous cosmic joke, “hey, there’s a situation that usually doesn’t present on imaging, the place specialised imaging additionally entails making a brand new gap within the dura mater to search for the unique leak, the place the overwhelming majority of medical college students don’t be taught specifics of SIH or long-term puncture leaks in in present med faculty curricula, and the place we will’t but determine why persistent sufferers have a very onerous time getting sealed and generally find yourself with new leaks after remedy.”
***
August 2024 marks 7 years of leaking for me.
In these very troublesome intervening years, I’ve discovered plenty of issues about myself, and about my physique. Concerning the illusions of security I used to have. About how life simply isn’t honest.
The half that hits hardest has at all times been that stability appears like an phantasm. It’s so tenuous. Moments the place I get respite from the ache are so few and much between, however with frequent anaphylaxis and extreme MCAS within the combine, it appears like the sting of that knife is so skinny. I usually joke that I’m in mattress now, however we’ll all be in mattress finally as we age. I simply bought there first. Beneath the humour is the data that Connie’s story is unfortunately not the exception. That there are different sufferers barely hanging on as they watch for science to evolve and be capable to convey them lasting aid.
Daily, my inbox is filled with sufferers who can’t get remedy for his or her spinal CSF leaks. Docs who snort at them for asking for atraumatic needles for a lumbar puncture, regardless of research displaying it makes a big distinction in decreasing the danger of a post-puncture spinal CSF leak. Households who say that their youngsters or siblings or spouses are faking it, that they’re “simply lazy”, that they simply “don’t wish to be higher”.
Present me a chronically unwell particular person, in fixed ache, who doesn’t wish to get higher?! In the event that they’re on the market, they’re a rarity. Each affected person I do know, and I do know many on account of my public platforms, is like Connie: advocating fiercely, organizing effectively, holding hope as greatest they will, and desperately eager to get effectively.
I haven’t gone again for remedy as a result of repairs don’t maintain for me. I’ve complicating components like MCAS and adhesive arachnoiditis that make outcomes extra dangerous and fewer healing than the typical affected person. However that that doesn’t imply I’ve given up. I’ve mentioned it earlier than: acceptance doesn’t imply you’ve given up. Like Connie, I’ve a lot to be pleased about. However even acceptance, even discovering pleasure once more, isn’t sufficient to eclipse unrelenting ache. Fortunately, I’m not within the type of ache Connie was in, and by sharing all of this I’m not attempting to make her dying about me. It’s extra that her passing feels prefer it may have been any of us on this tight knit group. I respect her alternative, and I’m additionally heartbroken that she was backed right into a nook by her ache and felt like she needed to make it.
This publish isn’t my normal, I do know. I’m indignant. I’m devastated. And it’s lit a hearth underneath me to proceed my advocacy work as a lot as my physique permits. Folks generally ask why I’m so concerned with the Spinal CSF Leak Basis within the US, because it’s unpaid and I’ve my very own enterprise to run. (I’m now the Vice-President of the Board). Advocacy is why. Connie is why. Connie, and different sufferers like her. Different sufferers like me. For these of us who’re persistent, the place repairs don’t maintain, who’re additionally ready and hoping. For the sufferers who can’t get to care as a result of they don’t have docs who know leaks of their areas. For individuals who want assets to assist their households to grasp.
In supporting the Basis, and dealing with sister nonprofit organizations in Canada and the UK, I can hopefully result in extra change than I can do as one author with a leak.
I’ve been which means to share all of those ideas since I discovered of Connie’s passing earlier this month, but it surely actually messed me up an excessive amount of to place phrases to paper. Immediately marks the start of duradash®, although, and I’m fundraising for spinal CSF leak analysis. So I assumed it was a very good day to share, and to attempt to mud myself off and hold persisting. What else can I do however attempt to transmute my rage and my grief about Connie’s dying and the unfairness of all of it into one thing that may transfer the needle on all of this mess?
In August 2017, I knew nothing about spinal CSF, nothing about leaks apart from those in water pipes, and nothing about needle varieties or gauge. I had by no means heard of the dura mater. Years later, there’s rather a lot I want I knew then, however all I can do is hold writing and sharing, elevating funds for the trigger, and hopefully assist another person get care before I may.
I’ll at all times be grateful to Connie for her fierce willpower and need to teach others it doesn’t matter what she was going by means of.
Since I sustained my leak 7 years in the past, there was plenty of progress and analysis and advances within the discipline.
Dropping Connie reveals us how we have now a lot farther to go. 💔
Should you’d wish to help me in elevating funds for spinal CSF leak analysis, please see my duradash® marketing campaign web page right here. I set a aim of $1000 however have already eclipsed it because of my Instagram group; I will probably be upping the aim to $2000 later this week. The two-week fundraiser will finish June eighth, 2024.
