As soon as I saw the bit about many people with recurring CSF leaks also having connective tissue disorders, my heart sank. Sure enough, after reading a follow-up[0], it seems she has Ehlers-Danlos Syndrome. The experience I have of this is a bit off the beaten path: a kitten we adopted a few years ago ended up having the feline version of it... she was such a sweet, affectionate, energetic little girl, but we lost her to complications after only a year and seven months; she had very weak skin and slow healing, and the disease damaged her heart. (It's been a year and a half, and I still miss her dearly. She made a huge impact on my life in such a short time.)
EDS is uncommon in humans (and even less common in cats), and hits in varying levels of severity (there are a few different subtypes of the disease, caused by different genes). It affects the entire body, since there's connective tissue more or less everywhere, that depends on correct collagen packaging to function well. But it's rare enough that it's unlikely we'll find a cure, or even particularly effective treatment, for quite some time. I can't help but be impressed by people who manage to live full lives, while dealing with its effects for decades on end. I don't know if I'd be strong enough, in their position.
As a person with EDS, i would counter that it is way more common than realised. As its a systemic genetic driven inability to produce collagen correctly, the only cure will be when we can rewrite and organisms genetics wholesale, which we are not exactly close to.
Treatments are poor, the condition itself and its systemic effects are almost unstudied. Most doctors either dont know about it or their knowledge stops at 'flexible'. Some doctors (surgeons esp) may even decline to work with you if you disclose EDS.
CSF leak, either from a lumbar puncture, or from things like chiari malformation and other cervical abnormalities are a well known phenomenon within the community. A person complaining of persistent postural headaches usually gets csf leak as the first response from said community. It is however, incredibly difficult to get a doctor to be willing to diagnose it, to have it competently found, and then patched.
Just two weeks ago I found out my sibling has Ehlers-Danlos, and that it was the likely (undiagnosed) disease that killed my dad and grandpa each at a young age. I found out yesterday I have some sort of congenital (but hopefully benign) heart condition, the Dr thinks may be also related to the same.
Reading that in the article and then your comment feels very surreal. I understand Baader-Meinhof, but this timing is particularly strange.
>> In retrospect, I realized that many of the aspects of the puncture—the position (curled over the side of the bed), the needles (18g, very large), the type (cutting needles), and that it wasn’t done under guidance—all contributed to my leak being complex but to the fact that I obtained a spinal leak from the procedure.
>> The hospital’s advice to lay down that evening and then just carry on as normal also prevented self-healing after a procedure with all of those risk factors. I did not know that many of the things I did—like shaving my legs in the shower, bending in certain ways, lifting a jug of milk, etc. were all things that likely impeded my healing at a critical time.
Well I did get a lumbar puncture when I was a kid, hospitalized for mumps. I recall being upright, on a chair, hugging the chair's back seat, didn't hurt excessively. But reading the story, the difference was that after the puncture I laid flat in bed for a long time, further scared by the doctors that moving around will make me sick. Which, I guess it's true, this is the first time I hear of a CSF leak.
I also had a lumbar puncture as a child (Viral Meningitis was diagnosed by it) and mine was like the author's, lying on my side in a foetal position.
I don't remember it being painful, but in retrospect, my head felt like it wanted to explore from the Meningitis, so it could have just been the more "pressing" pain.
I do remember being told I could try and walk, but when I did, I felt a horrendous pain in my back, so didn't do that again.
I had what I'd guess were around a dozen spinal taps as a kid/teen. Had issues with back pain at the spot that took some years fully go away. One of the times I thought I was pretty much recovered around three or four days later and went off a small jump with a Razer scooter. Got one of the worst (and weirdest) headaches of my life and it lasted for like a week.
Can't imagine dealing with that for years.
They are infinitely less of a pain the thinner the needle is.
Scary story and I sympathize. I assume this is pretty rare though, which is probably why it's hard to treat in the first place (there haven't been that many patients to research better treatments on).
E.g. doesn't every mother getting an epidural while giving birth effectively have a lumbar puncture? And most of them turn out fine. Maybe the bed rest after labor helps.
The fact that CSF pressure is too high after patching makes sense, I guess the body ramps up CSF production to combat the leak. This probably also makes healing harder because there is constant increasing fluid pressure, which pushes fluid out, which makes any hole resist closing.
This suggests a better treatment might be actively tapering down the flow? E.g. instead of attempting to close the hole completely in one go, install some kind of controllable shunt and gradually reduce the flow over a longer time, then once reduced to nothing, close the hole?
I'm a doctor training in anaesthesia. The idea with epidurals is to introduce local anaesthetic outside the compartment where the spinal cord is, literally epi-- --durally.
To sample the CSF, or to give a spinal anaesthetic, the needle needs to be introduced into the subarachnoid (one of the membranes that surrounds the central nervous system) space, but at a level below where the spinal cord has terminated. This is why they're done in the lower back typically.
Bending over is a totally legitimate way to perform them, and doesn't really alter the complication rate, while making the procedure easier. Also, there isn't really any realistic option to perform them in real time with imaging guidance. The author is right in that larger cutting needles are associated with increased rates of PDPH.
Hi there, thank you for the comment. Yes, bending over is a legitimate way to perform them, but some leak experts theorize that it can contribute to additional issues (e.g. an arachnoid bleb, which needs surgical intervention as EBPs won't suffice for treatment).
> instead of attempting to close the hole completely in one go, install some kind of controllable shunt and gradually reduce the flow over a longer time, then once reduced to nothing, close the hole?
That would only work if they can find the hole. According to the article, it often doesn't show up on imaging, and even if they know roughly where it is (as in the case of a lumbar puncture gone wrong), it's still incredibly difficult to pinpoint, and patching it usually involves trial and error.
It wasn't completely clear from the article, but it sounds like often they don't ever really pinpoint the hole, even when they are able to successfully patch it. The patching process seems more like "spray and pray": do some patching (she had 22 patches done, I think I read), and once it seems like the leaking has stopped, they assume they got it. Not sure if this is true, but that's the impression I got.
For clarity, I had 4 rounds of targeted patching done, but I wanted to be clear that there were a total of 22 needles — it wasn't a high volume EBP, but smaller, directed patches with blood+fibrin.
Puncture leak sites rarely show on imaging, it's true. At Duke, Cedars, or other leak centers they have techniques for CTMs or DSMs that yield leak sites but that's usually for spontaneous CSF leak patients or post-surgical, not us puncture people. They're using a PCCT machine to find CSF venous fistulas at Mayo and at Duke, but again to my knowledge puncture leaks remain a needle in a haystack even on that quality of imaging.
Well either that, or "spray and pray" to fix unknown holes, but deliberately make another small, known, controlled hole that can relieve pressure and gradually be shrunk until it's shut.
I actually knew someone who had a valve like that installed because her CSF pressure kept changing. Periods of high pressure were the most dangerous and led to rapidly deteriorating mental function—and eventually death.
The valve never really worked well, and she didn’t live long enough to determine why.
Retired neurosurgical anesthesiologist (38 years) here. I performed 500-1,000 spinal taps.
>... deliberately make another small, known, controlled hole that can relieve pressure and gradually be shrunk until it's shut.
This would make things worse. The pressure has already been relieved by the original hole with subsequent loss of cerebrospinal fluid.
Post-spinal/dural puncture headache results from NOT ENOUGH CSF surrounding the spinal cord and brain as a result of its leaking out: thus, the brain sags and positions itself on the base of the skull rather than floating, with subsequent headache.
Pretty rare, versus pretty hard to diagnose though? I'd be more surprised if everyone wasn't leaking a little and a lot of people are leaking enough that it would be noticeable if they knew to recognize it.
Re: your question I think the needle goes to different places.
> One large difference between a spinal tap and an epidural is the area that the needle is inserted. With an epidural the needle does not go into the spinal cord, but the epidural space just around the spinal cord. Spinal taps however, require the needle to be inserted all the way into and through your spinal cord. [1]
which at least matches my experience of needing a lumbar puncture for them to extract spinal fluid for testing. I've had them before, twice, never an epidural though. One leaked, and they didn't waste a moment doing a blood patch in urgent care (America). This entire post is a horror story, I would never have known to ask.
This person lost ten years of life and the rest of it irrevocably changed because some idiot didn't know something this basic. I'm just stunned, despite my intimate familiarity (lost the last three years frankly), it's just literally on the wiki for spinal tap headache treatments that you need to take action if they last more than 24 hours! [2]
If they hadn't thought to do a blood patch when I went to urgent care (which I had the privilege to do!) I'd literally be sitting here with probably the same leak now 13 years later. Malpractice, sorry, this is horrifying. It's one thing when it's hard to guess, I had a slipping rib that's apparently super hard to identify for sure and took those three years to be sure enough that just doing some PT wouldn't cut it. Three years. I'm not getting younger... I hope I at least get to be close to as in shape as I was three years ago.
This piece hit hard. I also spend days in bed just... tolerating... sometimes. Learning to be present and accept things that I cannot do anything about. I really hope they're able to go on walks in the park again.
I am a retired neurosurgical anesthesiologist (38 years) and performed 500-1,000 spinal taps.
>Spinal taps however, require the needle to be inserted all the way into and through your spinal cord.
NO! Through the dura and into the subarachnoid space but NEVER into or through the spinal cord, which would likely result in permanent motor/sensory deficits and possibly paraplegia.
Her lumbar puncture was in 2017, so not quite. But the leak came back in late 2018, and it seems like she's been living with it since then, which is incredibly awful. Based on her other issues (MCAS and EDS), diagnosed since the first leak, it does seem like she'll be living with this (and possibly worse) for the rest of her life.
At any rate, wherever you are on your healing journey, best wishes for further recovery!
Thanks, I missed that it was 10 years of being a nomad first and then this stuff. It said it so many times I lost track of time timeline!
I can very much relate to the meditation aspects. Sometimes I know that if I go to the Urgent Care the best they'll do is have me lay down for some hours until I'm okay to leave. I can do that at home, the hard part is letting myself believe everything is fine and that the best that can be done is to be present.
> It turns out that I have both a connective tissue disorder [Ehlers-Danlos Syndrome (EDS)], and a mast cell disorder, both of which make my situation more complicated.
I got 1/3 into this and had a panic attack. Literally nothing gives me the willies like the thought of losing spinal fluid. Good on her that she's recovering.
Oh yeah. I've been worried about prions for ages. Especially having eaten beef in Britain in the 80s. The concern has somewhat faded, but I won't touch venison.
My mother in law woke up one day, couldn't stand. Everything was spinning. She said it was like the most intense carnival ride of her life, endless spinning. Closing her eyes didn't help. She began vomiting, and didn't stop. 12 hours later she went to the ER. They tested for everything they could, specifically to rule out stroke. The only thing that came up was a small bit of fluid buildup near her ears that appeared on an MRI (maybe it was in her ears, I can't remember, this was several years ago).
Doctors gave her steroids and antibiotics. After a week of feeling like being on a carnival ride (and endless puking), she could close her eyes without spins, and she went home. It took another week to be able to open her eyes without the spins and focus, but it was still very wobbly, like a seesaw back and forth. She went from being a marathon runner to essentially hobbled.
Every day was a tiny bit better. After a month she could watch TV. After 2 months she could walk without assistance. She wasn't fully better for 6 months.
You never know what life will bring. One day you wake up and you are on a carnival ride that never ends.
My wife had something similar - though slightly different symptoms (migraines, memory loss, paralysis, seizures). It was a bumpy 2 years, to say the least, until she was 80% functional.
Now, about 7 years from the first ocurrance, she's pretty much 100%. The best we've figured is an infection (she was sick as a dog beforehand) interacted weirdly with her (then undiagnosed) familial hypercholesterolemia and inherited predisposition for migraines.
My take away was not only how vulnerable we are, but also how useless modern medicine is in some situations. We went through a bunch of specialists - only one helped at all, but the side-effects of the drug he prescribed were almost worse than the symptoms. We ended up making the most progress by experimenting with diet, exercise and supplementation (which makes more sense now that we know about the FH).
> ... but also how useless modern medicine is in some situations.
Yeah. I've read enough online about people with uncommon illnesses to realize that our medical profession is exceedingly heavily optimized for common cases. That might sound weird, given that there are so many common cases (tens of thousands? more?), but that's why medical diagnosis is so difficult, and the doctors best at it so prized.
But doctors see enough patients who try to self-diagnose, and fail miserably at it, that they're inherently skeptical of anyone who suggests they're suffering from an oddball disease. That's a pretty dangerous bias to have. And on top of that, it seems like there are quite a few doctors who will go even farther into denial, and default to assuming a patient's pain isn't real, when their usual diagnostic tools fail to find a problem.
I'm not trying to be super negative on doctors; there are so many out there who do their best and genuinely want to help. But I'm not sure how we solve this problem.
> And on top of that, it seems like there are quite a few doctors who will go even farther into denial, and default to assuming a patient's pain isn't real, when their usual diagnostic tools fail to find a problem.
100%. I had a neurologist tell me (while my wife was in a hospital bed, unable to move) that the tests he had done proved that there was nothing wrong with her. I said "you mean the tests don't show any possible issues?" and he said "no, they prove that nothing's wrong. I don't know why she's choosing to act like this".
After that, we never went back to hospital no matter how bad her symptoms got - we just dealt with it at home, which was terrifying. At one point she almost drowned in the bath.
I have some sympathy for him (in hindsight - at the time I was full of white-hot rage). He can't know the sort of person my wife is, and how ludicrous the idea of her faking an illness is.
Might have been the mumps, particularly if encephalitis complication occured, "Symptoms include abdominal pain, nausea, and vomiting. The mumps virus also leads to permanent hearing loss in about 5 out of every 10,000 cases."
She checks for nausea and also ear-related stuff. Also, one symptom for mumps in me as a kid was nausea so bad I couldn't stand up, only fetal position with eyes closed helped somewhat. I suppose she didn't get tested for the mumps virus, right? Not that there's much to do, the support is largely palliative until the body fights the virus off.
My father had spinal meningitis and they took a spinal tap and fucked it up by sitting him upright too soon and he ended up leaking fluid.
Nearly killed him. Could barely move his head. Couldnt walk, could only crawl for a long time. He was unable to work for like 9 months or something. He ran a fever over 100 for like a year and a half. Got him to quit drinking though. Still gets dizzy and neasous easily.
I've yet to learn of or meet a patient with my facts who's gotten sealed and stayed sealed. For now, just hoping for medical advancements that can benefit those of us in that position.
I had a lumbar puncture about 5 years ago because I got meningitis. Thank God I didn't have any issues with CSF, this poor lady's life sounds like a nightmare. I feel terrible for her and I hope she gets better, it's not fair that she had to go through this.
I had a CSF leak caused by a bone spur on my spine poking a hole in my dura.long story short, spinal surgery to remove the spur and seal the dura , which involved fusing three thoracic vertebrae. The “positional headaches” were horrible(when I was leaking). I consider myself lucky that it was treated as successfully as it was. This was over five years ago.
Been there, Jodi. Probably been worse, much worse, but further along and I can tell you it's entirely possible to come back and do relatively normal things.
One of the advantages, I suppose, of a high-deductible PPO is not needing referrals. I can't remember the last time I saw a general practitioner and don't have a primary care provider. I've always gone straight to a specialist, but the bad news with spine problems is, even when a care provider has a pretty good idea of what is wrong, or even a completely clear idea, it still may be difficult and take many years to treat.
For reference, I experienced spinal disc degeneration in my mid-30s coming out of the Army. It was never particularly clear why, but probably just genetic. I've been abnormally susceptible to herniations since I was a teenager and my mother and two of my sisters are as well. I used to receive epidural cortisone shots every three months for years, but never had a CSF leak from that. I did have a CSF leak after a microdiscectomy, though, back in 2016. It set me on quite an odyssey. Like her, I was bedridden for a while, could eat nothing but saltine crackers and lost 20 pounds, told to drink a lot and try to self-heal. It appeared I did, but appearance can be deceiving.
Rather than straight busting back out and experiencing headaches and nausea again, I instead grew a cyst on my spinal cord composed of excess fluid pushed into a sac that did not quite burst, but grew to nearly grapefruit size. I couldn't lay or sit in particular ways, couldn't let anything touch it because of how badly it hurt. I needed a walker to be able to move. When I finally got an MRI, they were so worried they pencilled me in for emergency surgery only eight hours later. So I got the patch she's talking about this time, but much more directly since I had to be cut open. I also had a laminectomy during the same procedure.
That did end up being the end of the CSF leakage, but not quite the end of the spine problems. My discs had been so beat up at that point that I'd lost an entire inch of height and was living virtually bone on bone. The sciatica was so bad I felt like I was walking on electrical wires and constantly being shocked with every footfall. I eventually needed a bilevel interbody fusion where they remove the discs entirely and replace them with metal cages seeded with a bone graft sawed off from the pelvis, held in place with titanium rods and screws while the bone grows around it. Today, my lumbar spine is mostly one solid bone, a huge bone you can feel from outside. The resulting limitation in lumbar mobility also leads to hip and hamstring tightness. In middle school, I used to come in 2nd every year in the Presidential fitness contest's sit and reach competition. Today, I can't even sit upright, can't even get to 90 degrees if my legs are straight out in front of me.
Nonetheless, I did come back. It took a lot of patience, starting with physical therapy, eventually striking out on my own and working out very diligently. For a long time, this meant resistance bands and walking and not much else. But progress compounds. 8 years later, I've got a power rack, barbells, C2 rower, and I lift and either run or row at least 6 days a week, sometimes all 7. It doesn't always feel great. I'm still stiff and aching all the time. My joints swell and lock up. I'm not very strong considering how much lifting I do. I'm not very fast considering how much running I do. But 8 years ago, I didn't believe I would ever lift or run again. I still needed a walker. I couldn't pick up my cats. I couldn't put my own shoes on.
Patience, persistence, and hope. It may or may not happen for any particular person, but you can come back from this. There's a lot to be said for still being relatively young.
I'm in my mid-40s, so I appreciate the relatively in young ;) Thank you for sharing some of your story. Unfortunately, my case is different to you — with EDS, MCAS, and an alphabet of other issues that underpin this spinal CSF leak. I definitely have hope medical treatment can evolve, but as of now I'm waiting for that to happen while surviving the best I can. I'm pretty even-keeled about it all, and find joy where I can. It's just not a very mobile life. All the best to you!
"She added me to a private CSF leak group on Facebook with several thousand leak patients from around the world."
My late wife joined that group when it was 800 people in ~2011. Today we are closing in on 9,000 people. Back in the beginning a few people though that my late wife Karen started that group. She didn't. She was just a prolific poster there helping a lot of people as she learned about Leaks in her medical studies.
Part of Karen's saga, and mine, is published on the CSF Leak Foundation website here "When Pain is Relentless" :
A few years later Karen's saga also became part of the documentary Pain Warriors. Which can be watched for free on Amazon Prime, TubiTV and Youtube. It is also on iTunes.
Our goal is to raise awareness of CSF Leaks and the devastation they cause to families like mine. Karen took her own life due to the pain from CSF Leaks and being poisoned by the flourquinilone antibiotic Levaquin (NEVER Take that or Cipro et.al. which I can back up with FDA data, I want to focus on Leaks here).
The top doc in the world Dr Schievink [1] has estimated that just under 100% of people that have CSF Leaks have some type of under lying Connective Tissue Disease. Karen saw him and the docs that are mentioned in the parent article that were there at the time in the 2011-2013 time frame.
I had Karen's spin exhumed and it was sent to Duke to be studied. Much was learned from it to help those that came later. Sadly not nearly enough.
All of this is documented in what has become known as Karen's Journal.
Free to read on Karen's website. Any profit from the sale of physical books go directly to the Spinal Leak Foundation. [2] I also get nothing from the Pain Warriors Documentary. [3]
2: Sadly the certificate expired so please ignore the scary warning until I get that fixed. I also need to fix a link that I see is broken to the PDF that was used in the documentary. http://www.kpaddock.com/book
What we desperately need is better imaging technology. When it comes to finding Leaks with today's methods we are still in the Stone Knives and Bearskin era.
CSFluid is almost identical to water making it hard to differentiate unless the leak is large. It does not take a large leek to cause devastating headaches.
My deepest condolences. I will have a lumbar puncture done in a few hours and am facing a diagnosis that could be extremely grave, but everywhere I look there are stories of people who have had it harder. I will keep you and your wife in mind today, and I hope you find peace.
Thank you so much for your work on this. I had a bad CSF leak in 2020 and was bedridden for months until surgery with Dr. Schievink. Karen’s book was with me much of that time.
Unfortunately that also demonstrates why a lot of people - especially women - have developed a serious lack of trust in medical professionals.
Doctors often believe they are the second coming of Christ, and if there's something which doesn't neatly fit into their preconceived notions of what it should look like, they will reject the possibility that they might be missing something and just jump to the conclusion that the patient is faking it. Lying to patients is also extremely common - just ask any woman who had an IUD placed if it really was "just a pinch".
EDS is uncommon in humans (and even less common in cats), and hits in varying levels of severity (there are a few different subtypes of the disease, caused by different genes). It affects the entire body, since there's connective tissue more or less everywhere, that depends on correct collagen packaging to function well. But it's rare enough that it's unlikely we'll find a cure, or even particularly effective treatment, for quite some time. I can't help but be impressed by people who manage to live full lives, while dealing with its effects for decades on end. I don't know if I'd be strong enough, in their position.
[0] https://jodiettenberg.com/connecting-dots/