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Tuesday, June 25, 2024

Losing Connie


Earlier this month, the spinal CSF leak community lost a bright light when Connie Rim passed away. Her Facebook page, called My CSF Leak Story, was where she detailed her long fight trying to get sealed and healed. A few days after she passed, her husband posted words from Connie about her death, sharing it with the world.

Connie sustained her leak 5 years ago during spinal surgery, and tirelessly rallied her body and mind in her attempts to get care and find her way back to a place of less pain. Like me, she found solace in sharing this journey publicly. In her case, via videos that she posted to Facebook and TikTok, detailing her many debilitating symptoms, explaining the procedures and tests she got, and patiently answering questions.

When someone is chronically ill and takes a break from treatments, there is inevitably a swell of people asking why. “Why aren’t you doing more?”, as if it’s up to you to not be better. As if there’s full control of the long tightrope you need to walk on when you’re dealing with multiple complex conditions. Despite her openness and raw, unflinching videos, people still asked Connie this question.

Perhaps in response to this, or for other reasons (I don’t know), in May 2023 Connie posted a long list of procedures and tests and doctors she’s seen right before she headed out to Mayo for more leak diagnostics and procedures: “This cannot be my life. This is not a life. I’ve been fighting to get better and advocating for myself as much as possible but in truth, I’m tired now. Nothing is as easy as it once was,” she wrote then.

By this point, she had been to leak specialists and still her leak(s) were not sealed. She was in constant pain. She reposted that list, updated, a few days before her death.

She did get treatment after that May 2023 post, one that brought her pain down a little bit. We could all feel her joy and hope when it did. But that treatment failed, and when it failed it brought a new, terrible set of symptoms. Since late January 2024, she experienced severe stabbing pain from three to over twenty times daily, at what she classified as a level 10 pain. To be in pain all day, and then layer upon that a new and persistent, paralyzing pain… I can’t imagine.

By mid-May, she was gone.

Reading between the lines of the message her husband posted, she hit a wall where the excruciating pain she experienced moment to moment couldn’t be managed.

Some days, I’ve been given 10-15 minutes of my precious baseline pain, and for that. I’m grateful. But during the remaining 23 hours and 45 minutes, I was always crying, fighting, and, at times, in so much pain that I could do anything but curl in a fetal position in darkness,” she wrote.

Her family and husband were loving and kind, she had a lot of support, and she had a community who rallied around her. I understand that those amazing things may not be enough when you are trapped in a body that tortures you.

Studies show that in a chronic state, pain can disrupt the communications between brain cells, leading to a reduction in the ability to process emotions — especially negative emotions. Pain changes your brain. I experienced a version of this myself not long after my leak began: already in chronic pain for years  but not yet aware of mast cell diseases, I ate a particularly high histamine meal of spaghetti and shared a glass of wine with my family. It was this meal during those initial leak days, before I went to Duke for treatment, that tipped my body its new, unruly state. After that meal, I had full-body burning and nerve pain 24/7. And it didn’t go away. It felt like I was being dipped in acid, with no respite from the torture.

It was a fellow patient who urged me to think about MCAS, given my symptoms. I hadn’t heard of mast cell activation syndrome before. With no doctors to support me, I scrambled to get my mast cells under control and try to make it stop. During those weeks of absolute hell, until I found over-the-counter medications that worked for me, I begged a close friend to help me find a way out forever. (They declined, were compassionate throughout, and instead tried to find me a grief therapist locally. What helped in this case wasn’t therapy, it was finding respite from the fire-burn all over my body thanks to antihistamines and a low-histamine diet.)

And that was how I felt with only a few weeks of torture.

Connie lived with that pain that for many years. As she said in her writing, “The pain determines my emotions, NOT the other way around (as some people believe).”

Her passing hit the leak world very hard. Even those who did not know Connie personally often watched her videos on TikTok or Facebook, or interacted with her on the groups. In my case, I was virtual friends with her for years and we exchanged audio messages about strategies, told jokes, tried to keep ourselves sane in this Groundhog Day world we lived in when staying sealed felt like a pipe dream.

And yet, despite the pain and the exhaustion and the procedures, Connie remained joyful where she could, she appreciated what love she had in her life, especially her deep and beautiful love for her husband, and saw humour where she was able. She had tons of support. The pain determined her emotions, as she said. She was a prisoner of her constant agony.

I feel angry for her and her family, and for all of us. In looking at that long procedures list, it feels like she was failed so many times along the way. Even as recently as this year, she presented to the ER and later shared a video explaining that the on call doctor said her symptoms were psychosomatic. How can you look at that blisteringly long list of treatments and think it’s psychosomatic?! The ongoing nerve damage and tissue damage, and scar tissue that can entrap her nerves, it all makes sense looking at the long list of what her body had been through.

But no, she was told it was all in her head.

That’s part of what leaves me rattled, for anyone with this condition or other invisible conditions where metrics for diagnosis and treatment are not cut and dry. I’m working on the slides with the US and other spinal CSF leak foundations for the annual awareness week for this condition, called leakweek (which starts June 3rd). In it, we share the different types of diagnostics and note that normal imaging does not rule out a spinal CSF leak. Normal opening pressure does not rule out a spinal CSF leak. Normal anything does not rule it out; we are trapped in this endless loop of being unable to ‘prove’ quantitatively what we have other than symptoms, yet often we are told we aren’t reliable narrators when sharing our symptoms.

And so we try, we try so hard.

We present ourselves firmly but without panic, but not TOO jokingly else we seem ‘not sick enough.’ We keep spreadsheets, we share data, we come armed with supporting studies but not TOO many, else it seems like we think we know too much. Outside the few leak experts out there, who are thankfully training doctors often, this dance continues. It’s exhausting to live in pain and to lose your mobility and so much more, but add the suspicion and disbelief to the mix and it drags your soul down to the ground.

Because despite the advocacy, despite the education, the myths persist.

Outside the leak experts, patients report that doctors still think a lumbar puncture leak is “self-limiting” (i.e., that it goes away on its own — I wish!). Many still doubt the existence of spontaneous intracranial hypotension (SIH), where a leak can occur suddenly; this can be due to a bone spur or calcified disc gnawing into the dura til it tears, or a coughing fit, or lifting heavy weights, or something else being the last straw for a weakness in the dura mater the patient wasn’t aware of. And a more recently-discovered type of leak, a CSF-venous fistula, can be elusive on existing imaging types, though a newer photon counting CT machine is spotting them more frequently. Canada doesn’t have one, and the US only has a few.

The brutality of this condition is hard to accurately put into words. Not only the pain itself but the uncertainty and uphill battle in getting care. It’s commonly misdiagnosed and under-diagnosed. The mental health burden is also significant: a quality of life study in spinal CSF leak patients from 2023 found that over half of the respondents (64.2%) endorsed suicidality, and 22.4% had demonstrated suicidal behaviour. A 2024 study about quality of life in chronic post puncture patients (like me) found substantial mental health challenges with depression, anxiety and stress experienced by 83%, 98%, and 88% of the respondents, respectively.

It’s like a big cosmic joke, “hey, there’s a condition that often does not show on imaging, where specialized imaging also involves making a new hole in the dura mater to look for the original leak, where the vast majority of medical students don’t learn specifics of SIH or long-term puncture leaks in in current med school curricula, and where we can’t yet figure out why chronic patients have a really hard time getting sealed and sometimes end up with new leaks after treatment.”

***

August 2024 marks 7 years of leaking for me.

In these very difficult intervening years, I’ve learned a lot of things about myself, and about my body. About the illusions of safety I used to have. About how life just isn’t fair.

The part that hits hardest has always been that stability feels like an illusion. It’s so tenuous. Moments where I get respite from the pain are so few and far between, but with frequent anaphylaxis and severe MCAS in the mix, it feels like the edge of that knife is so thin. I often joke that I’m in bed now, but we will all be in bed eventually as we age. I just got there first. Underneath the humour is the knowledge that Connie’s story is sadly not the exception. That there are other patients barely hanging on as they wait for science to evolve and be able to bring them lasting relief.

Every day, my inbox is full of patients who can’t get treatment for their spinal CSF leaks. Doctors who laugh at them for asking for atraumatic needles for a lumbar puncture, despite studies showing it makes a significant difference in lowering the risk of a post-puncture spinal CSF leak. Families who say that their kids or siblings or spouses are faking it, that they’re “just lazy”, that they just “don’t want to be better”.

Show me a chronically ill person, in constant pain, who doesn’t want to get better?! If they’re out there, they are a rarity. Every patient I know, and I know many due to my public platforms, is like Connie: advocating fiercely, organizing efficiently, keeping hope as best they can, and desperately wanting to get well.

I haven’t gone back for treatment because repairs don’t hold for me. I have complicating factors like MCAS and adhesive arachnoiditis that make outcomes more risky and less curative than the average patient. But that that doesn’t mean I’ve given up. I’ve said it before: acceptance does not mean you’ve given up. Like Connie, I have much to be grateful for. But even acceptance, even finding joy again, isn’t enough to eclipse unrelenting pain. Thankfully, I am not in the kind of pain Connie was in, and by sharing all of this I am not trying to make her death about me. It’s more that her passing feels like it could have been any of us in this tight knit community. I respect her choice, and I am also heartbroken that she was backed into a corner by her pain and felt like she had to make it.

This post isn’t my usual, I know. I’m angry. I’m devastated. And it’s lit a fire under me to continue my advocacy work as much as my body allows. People sometimes ask why I’m so involved with the Spinal CSF Leak Foundation in the US, since it’s unpaid and I have my own business to run. (I am now the Vice-President of the Board). Advocacy is why. Connie is why. Connie, and other patients like her. Other patients like me. For those of us who are chronic, where repairs don’t hold, who are also waiting and hoping. For the patients who can’t get to care because they don’t have doctors who know leaks in their areas. For those who need resources to help their families to understand.

In supporting the Foundation, and working with sister nonprofit organizations in Canada and the UK, I can hopefully bring about more change than I can do as one writer with a leak.

I’ve been meaning to share all of these thoughts since I learned of Connie’s passing earlier this month, but it honestly messed me up too much to put words to paper. Today marks the beginning of duradash®, though, and I’m fundraising for spinal CSF leak research. So I thought it was a good day to share, and to try and dust myself off and keep persisting. What else can I do but try and transmute my rage and my grief about Connie’s death and the unfairness of it all into something that can move the needle on all of this mess?

In August 2017, I knew nothing about spinal CSF, nothing about leaks other than the ones in water pipes, and nothing about needle types or gauge. I had never heard of the dura mater. Years later, there’s a lot I wish I knew then, but all I can do is keep writing and sharing, raising funds for the cause, and hopefully help someone else get care sooner than I could.

I will always be thankful to Connie for her fierce determination and desire to educate others no matter what she was going through.

Since I sustained my leak 7 years ago, there has been a lot of progress and research and advances in the field.

Losing Connie shows us how we have so much farther to go. 💔

Jodi ettenbergJodi ettenberg

If you’d like to support me in raising funds for spinal CSF leak research, please see my duradash® campaign page here. I set a goal of $1000 but have already eclipsed it thanks to my Instagram community; I will be upping the goal to $2000 later this week. The 2-week fundraiser will end June 8th, 2024.

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