bombsh3ll

What really clued me into CSF leaks was watching Dr Carroll's video within the past year & for the first time finding something that can be caused by a forceful Valsalva (my mode of onset), that causes these exact symptoms. Before then I had systematically worked my way through testing for all listed causes of POTS/OI, such as autoimmune, lyme, addisons etc which were all negative, but there was nothing on there that could be caused by a Valsalva. Once I learned that there actually was a recognized condition with these symptoms that valsalvas are known to cause, & that EDS which I have also been diagnosed with is a risk factor, I was pretty sure I was on the right track. EDS was one of the "secondary causes" listed which let to me seeing a geneticist, having features of it in myself & my family, but to me EDS alone cannot "cause" POTS as people are born with EDS & POTS is not congenital, EDS simply increases predisposition to actual secondary causes such as CSF leaks, craniocervical instability & chiari etc. I then printed off material from the CSF leak association website, highlighted the relevant symptoms, valsalva being listed as a cause, plus the connective tissue disorder, & asked to be referred to a neurologist that I knew had at least heard of CSF leaks. He ordered a MRI brain and whole spine with gadolinium contrast, supine. That was reported as negative by the NHS hospital & I was discharged yet again with no further investigation or treatment. The negative result really surprised me as my onset & symptoms were so classic, however I moved on to explore my next differential, craniocervical instability. I sent the same supine brain & spine MRI scan on a disc, plus a couple of upright MRIs to Dr P Bolognese (neurosurgeon) in New York who offers a remote radiological evaluation service for craniocervical instability. I had not disclosed my suspicion of CSF leak to him or that my illness was valsalva induced, and was just expecting either positive or negative for craniocervical instability. So I was blown away when he wrote back that I had no apparent instability but thought I had, guess what, a CSF leak! And he should know, because he causes them. Not by doing anything bad, but when you operate on the spines of EDS patients, a few duras get pierced. That was it for me, I found a UK specialist in CSF leaks & went private to get a diagnosis. He agreed my presentation was absolutely classic & should have been picked up 5 years ago when it first happened. It is a long journey still ahead with no guarantees, but finally I feel I am on the right track with a credible mechanism for how my illness was caused by the forceful valsalva. B xxx

Thanks, I definitely will do! I remember you posting about headache a while back - wasn't it you who was going to ask Dr Raj about the splitting orthostatic headaches? - could this be something to look into for you too? That can't be a coincidence! But you continue to have symptoms after the leak was fixed? Thanks lieze, I really hope things start to pick up for you also! B xxx

If there was such a pill nobody would be on here. What a B****. You are well rid. You mentioned paying an extra $100 per month for that service - personally I would stop that & spend it on private help. That could include someone to be with you whilst you do floor exercises or supervise/assist you bathing. I am not able to stand at a sink (or anywhere), but can sit/lie in the bath to wash, so maybe with someone there in case you faint it may be possible. B xxx

Spinal CSF Leaks have a troubling similarity to POTS – adapted from Ian Carroll’s 2017 presentation. CSF Leak POTS • Feel worse when upright • Feel worse when upright • Headache is prominent • Headache is prominent • Lightheadedness/dizziness • Lightheadedness/dizziness • Nausea/vomiting • Nausea/vomiting • Brain fog/cognitive impairment • Brain fog/cognitive impairment • Increased risk of suicide • Increased risk of suicide • Predominantly female reproductive age • Predominantly female peak onset 30-40s but can affect any age • Associated w connective tissue disorders • Associated w connective tissue disorders • Understood • Mysterious • Treatment can be effective • Treatment: “learning to cope”

This is for everyone whose cause of orthostatic intolerance/POTS is as yet unexplained, particularly if it was of sudden onset, associated with upright head/neck pain & nausea, and typical treatment for POTS has had no or minimal effect. Please also continue reading if you have or suspect a hereditary connective tissue disorder - these are present since birth, yet OI is not. You may have simply been told "people with EDS get POTS" with no explanation as to a possible mechanism, & worse, no further effort made to search for one. Perhaps you have read or been told that EDS patients have "stretchy veins", but wonder why the exact same veins the day before it started were not so stretchy you suddenly couldn't stand up, or why, if that is the problem, compression stockings have made no difference. It is also for everyone who has ever checked their vitals & found them to be completely normal, yet still feels awful upright. Yesterday, after five years of searching & racking my brains as to why, having been fit and healthy all my life, at the age of 34 a forceful Valsalva left me instantly unable to be upright without feeling like I am dying, I was diagnosed with intracranial hypotension (spinal CSF leak). I now have a long fight ahead of me for NHS funding for the recommended treatment for this, in my case a series of non-targeted high volume epidural blood patches, and treatment is not effective in every case, but the bottom line is there is a treatment. This is not a misdiagnosis of POTS as such, as I do technically meet the diagnostic criteria for POTS, rather it is a missed diagnosis of an underlying cause of secondary POTS. Valsalvas are not the only cause of a tear in the dura (the lining surrounding the brain, spinal cord and spinal fluid), although one has to wonder how many people with "viral onset" did not as postulated become "deconditioned" or develop rogue autoantibodies, but whilst unwell had a violent bout of coughing, sneezing or vomiting which may have put a rent in the dura; spinal CSF leaks can be caused by a minor trauma such as a fall, whiplash, concussion, car accident, lifting a heavy bag, a sudden twist or collision playing sports & many others. 2/3 of people with a spinal fluid leak do not recall a specific inciting event. Spontaneous leaks are more common in people with connective tissue disorders whose dura is thinner and weaker to begin with. Iatrogenic leaks can also occur for example after a spinal tap, or if the dura is accidentally punctured during an epidural anaesthetic (postpartum onset anyone?), however these are more likely to be diagnosed promptly. Obviously POTS is not a disease, simply a description of feeling very unwell upright & having an increased heart rate standing (also found to be universal in one study of CSF leak patients), so this will not be applicable to everyone, but is a recognized cause of this exact symptom complex, & has a treatment, so is too important not to consider if only to rule out. It is not widely known even among medical professionals, so do not assume your doctor has considered & discounted it (or even heard of it). The following resources are what helped me discover this diagnosis after five years of being harmed by inappropriate medication and unnecessary investigations (such as the endoscopy for intractable nausea - which completely abates supine unlike any gastrointestinal condition causing nausea), and being sent away to spend the rest of my life supine, syncopal & suffering: CSF leaks - what the POTS community should know, by Dr Ian Carroll on vimeo vimeo(dot)com/243145060 Dr Laurence Kinsella talks about POTS, headache & CSF leak https://journals.sagepub.com/doi/full/10.1177/2515816318773774 https://www.ncbi.nlm.nih.gov/pubmed/31118385 https://www.csfleak.info/ https://spinalcsfleak.org/ There is also a 2003 study by Mokri et al https://www.researchgate.net/publication/9051563_Orthostatic_headache_without_CSF_leak_in_postural_tachycardia_syndrome, which has probably contributed to the failure to suspect/investigate CSF leak in POTS patients, however this study included a whopping FOUR patients, of whom 3 cases had no spine MRI, 2 cases had no CT myelogram, none had digital subtraction myelogram, & 2 had documented temporary benefit from epidural blood patching. If this information helps just one other person, I want it to be out there & easier to find than it was for me. B xxx

I know it seems like research in this field is so patchy & they just go around & around, with promising leads picked up then dropped & picked up again ten years later, but IF the evidence continues to come out that some people have an autoimmune cause, I think treatments will start to emerge. In the UK it will probably be some years before they are made accessible for patients, but at least there would be a direction for many people to pursue who don't currently have one. What I can say that might provide a little bit of hope is that when I went to the NHNN in London, despite the neurologist there missing absolutely classic symptoms, inciting event, upright MRI signs & connective tissue disorder all pointing to the spinal CSF leak that I am now being evaluated for, I was asked if my blood could be taken for research looking for autoantibodies in the blood of patients diagnosed with POTS/OI. I agreed, although they would not disclose specifics of what was being tested & nor was I told whether participants would ever be contacted if they tested positive for anything. So there is clearly something going on along these lines in the UK at this time. B xxx

That is really interesting to read, thanks for sharing! I wonder if the rabbits were symptomatic or simply had an increased heart rate, if only bunnies could talk! Unless any of them fainted, poor things! My own cause is extremely unlikely to be autoimmune but I am always interested in the latest research, much appreciated. B xxx

@Wufflebear thanks for replying, I am so pleased you had a good effect from the blood patch!! I am really hopeful that this is my issue too, especially given my mode of onset & connective tissue disorder. I live in the UK so would not be able to visit Oregon anyway but I am sure it would be OK to say your doctor's name on here if you had a positive experience, in case it helps somebody else. If I get nowhere in the UK I may try to raise the money to go to Stanford & see Dr Carroll, or Dr Gray Leithe at Duke, but I am hoping it doesn't come to that. I had an MRI with contrast which was read as negative, & here a negative MRI is used to deny further investigation or treatment in the state funded system, despite picking up only 70-80% at best, but when I sent the same scan images to Dr Paolo Bolognese one of the world's top neurosurgeons in New York to look for craniocervical instability, he wrote back saying he thought I had a CSF leak, without me ever having mentioned it, or that my illness was valsalva induced, so I suspect it is not truly negative after all. As blood patching will likely be done blind with me as well, do you mind sharing what level(s) and what amount of blood you were patched? B xxx

Wow wufflebear thanks for coming back on and sharing that! CSF leak is what I am currently exploring - I do have mild EDS and my illness was caused by a forceful Valsalva. I am going to see a private specialist in CSF leaks this Saturday, hoping to ultimately access blood patching. I actually read your post above & thought tingling face - possible CSF leak because it is one of the listed symptoms (but not one I personally have). It can do. I had a clear inciting event as I tried to blow up a water bomb that I mistook for a balloon, but many people do not recall anything. It could be something trivial like a bout of coughing or sneezing or lifting a heavy bag in susceptible people. Connective tissue disorders are a risk factor but not every CSF leaker will have one, others will have an undiagnosed mild CTD. If you don't have splitting orthostatic headaches though it is probably less likely although again this is not universal. @Wufflebear I am particularly interested in whether the blood patch has improved your orthostatic intolerance? Even if I turn out not to have a leak, I bet there are a number of people on this site who do, so it is really worth spreading awareness. It is a tragedy when people end up near bedbound for years with no quality of life for the sake of a blood patch because the diagnosis is never considered. Although it took me 5 years to come across the diagnosis myself (thank you Dr Carroll) and pursue investigation, I am angry with the three separate neurologists I had previously sat in front of holding my head & nearly crying with pain, describing what I now know to be classic spinal leak symptoms, known precipitating event & connective tissue disorder, & nobody thought of it. B xxx

Lieze, I know you are in the US but here in the UK even the highest reading you mentioned above would not be treated with medication. Sporadic rises like that are not harmful, indeed they are normal, like during exercise in healthy people. Blood pressure varies throughout the day and is not meant to be the same all the time. Antihypertensive medication would definitely not be appropriate for someone whose lower end is 96/53. I would go by how you are feeling rather than the numbers. My blood pressure and heart rate are normal 99% of the time yet I feel awful upright. When I was on licorice root I developed iatrogenic hypertension and was in A&E with BP of 230/120, & I'm still here. Weightlifters get a spike like that when they lift heavy weights. B xxx

@edriscoll Thanks, I am glad you have ruled out a CSF leak. I just wanted to put it out there as although I have never come across another patient like me with a POTS diagnosis whose illness was brought on by a forceful valsalva (although since learning of CSF leaks I have encountered many with diagnosed leaks whose illness started this way), a lot of the things that have been reportedly associated with abrupt "POTS" onset such as car accidents, minor trauma, whiplash, concussion, spinal surgeries etc are also recognized to cause CSF leaks. Even more prominent are reports of postpartum "POTS" onset, when in fact the bearing down or inadvertent dural puncture during an epidural could plausibly have caused a CSF leak. Perhaps the largest pool of people whose underlying cause could be a CSF leak are those with "post infectious" onset - rather than deconditioning or antibodies it may be that a bout of violent coughing or vomiting during the illness in susceptible individuals could have torn the dura. I am so glad that you finally managed to get your Arterial Pulmonary Hypertension diagnosed & treated but sorry it took so long. I think advocating for yourself is so important & it is really worrying how some diagnostic labels, some well intentioned, others bogus can then act as a barrier to having underlying or concurrent treatable conditions investigated. B xxx

@Clueingforlooks do you have a gastroenterologist that you could discuss non-drug options with like fundoplication or the linx procedure if it is so severe & medications are not effective/tolerated? https://www.linxforlife.co.uk/about-linx.html?gclid=EAIaIQobChMIsu3bhZDp5AIVw7TtCh049Qo_EAAYASAAEgJ5oPD_BwE Before I became ill I was considering this. My omeprazole was (and still is) extremely effective but as I was young and had been on medication many years already, I would have preferred to not to have to take it for the rest of my life. It is no longer a consideration for me but it may be something you are interested in. PS I don't know if you are on any POTS medication but when I took fludrocortisone briefly that really aggravated my reflux (although I had been without my PPI for a couple of days at that time due to being in hospital). B xxx

Thanks for sharing your experience. How did you get on with the returns process, was it straightforward or difficult with hidden charges etc, did you get all your money back? B xxx

@edriscoll It is good to hear you had success with the bone mineral protein because I would definitely choose to supplement that too in addition to either my own bone and/or cadaveric. I know there are different types & some of the earlier ones were removed from the market, but some people have had problems with it overgrowing. ChronOs is reabsorbable though so I don't think it has those issues. Just thinking though about your sudden dysautonomia onset post-op, sorry if this is something you've already crossed off, I know how much all of us with chronic illness despise the unsolicited "have you considered...?" but what is top of my own differential diagnosis list right now is a spinal fluid leak (as I have mild EDS & my illness began immediately following a forceful valsalva, before which I hadn't even a whiff of orthostatic intolerance or splitting upright headaches). Surgery on the spine is a known risk factor for a dural tear causing a CSF leak, & they don't all heal up spontaneously without treatment, some people can be incapacitated for years, so I was just wondering if this might be relevant to you. Dr Ian Carroll from Stanford has some good videos on youtube if you are interested - he is an anaesthetist familiar with spinal fluid leaks & initially became interested in our population after reading a "medical mystery" case where a young woman with features of a connective tissue disorder & previous spinal surgery for scoliosis, was completely well then became immediately incapacitated with OI & upright headaches after a minor car accident. He felt it was a clear case of CSF leak & was disturbed when the authors called it by a different name (POTS) & deemed it untreatable. I am currently waiting to see a specialist in this field and hoping to access blood patching & fully rule this out before moving on to fusion surgery. Scans don't always pick it up especially where it has gone untreated for a long time, & often various different imaging types are needed. Again though sorry if you have previously explored this, just wanted to share it in case it may be another avenue for you. B xxx

No, I don't get that. Being upright is consistently awful, lying down is always lovely! B x

I don't have "flare ups", never have. In a way it sounds nice as I like the idea of it being less bad at other times rather than consistent. I don't tolerate heat well since I became ill, but I don't think that is what is being described here? @Pistol I hope you feel better soon. B xxx

I'm so sorry to hear that Derek, by all accounts it seems to be routine & the norm for a first application in the US, so please don't see it as anything other than just a part of the process. Keep up your determination & efforts, I wish you the very best with your next round. B xxx

POTS in teens is recognized to remit spontaneously more often than not. If you have the money to go there without it causing financial hardship then by all means, but I would not advocate anyone spending the kind of money this facility charges if it is going to compromise their own or family's financial security or lead to debts. There are other experiences online that you can read about this center, Ilana Jacqueline at letsfeelbetter and Jenna at parasympathy have both been there. I have personally spoken to a staff member there and corresponded with them directly about what is offered, and there is certainly no secret magic bullet that only they know about. As for my own illness we are now highly certain of a structural cause, either a spinal cerebrospinal fluid leak caused by a combination of weak dura and the very forceful valsalva that immediately precipitated my illness, or craniocervical instability. Both are treated with surgery, not biofeedback or counselling. B xxx

And thank you so much for taking the time to share it.! The surgeon I would be using typically harvests rib for the grafts but I would definitely ask about pelvic. They also use cadaveric donor bone and chronOs synthetic bone mineral protein either instead of, or as well as, bone grafts harvested from the patient so it seems to be tailored to individual needs. Having been unable to do weight bearing exercise for 5 years & being low BMI & largely housebound so not exposed to sunlight my own bones may be a little osteopenic, so I would need to consider the best option for the graft. I really appreciate your insight! B xxx

Your age & symptoms certainly don't scream Parkinson's (& could be a large number of other things) but there are things there that make it a possibility that you should definitely discuss with your neurologist, I am glad you have appointments lined up. More typical symptoms of PD would be slow shuffling gait, blank facial expression, "pill rolling" tremor & erectile problems in males. Cramps can of course be completely benign & occur in healthy people. I got them every now & again back when I was healthy, funnily enough not since developing dysautonomia. It is good that you got your blood electrolytes checked, but this only gives a snapshot at that time & doesn't rule out something being high or low in the interim. Quinine used to be prescribed for cramps but is now not used so much due to it causing renal problems. Other general home remedies for cramps would include tonic water & magnesium supplements (the latter are also great if you have constipation, but not so good if you are prone to diarrhoea). Good luck with your appointments, I know just how frustrating waiting can be. B xxx

It is brilliant, I wouldn't have thought it possible to capture what presyncope feels like in a painting, as images invariably tend to depict vertigo or spinning, which is not typically what is experienced when close to or just before fainting, but this one absolutely does! B xxx

Thank you so much for your insightful post and sharing your experiences, I really value hearing from someone who has had a fusion surgery. That is a lot of useful info that I will def consider when questioning my surgeon. That's awful, I'm so sorry. Is there any way the intubation/handling of your neck could have caused craniocervical instability? I have heard of others getting it after surgery (and recovering after craniocervical fusion). Alternatively is there any possibility the surgery could have caused a spinal CSF leak? That is my number 1 differential diagnosis just now. That is an excellent suggestion about trying something temporarily that would simulate the movement restriction post surgery. I did actually buy a hard cervical collar but sent it right back when it didn't help my symptoms (my particular issue requires upwards traction rather than just immobility of the vertebrae), but maybe I will get another just to see what living with a fused neck would be like. I also have the option of trying a halo brace, which is more invasive & costs a lot of money just for a couple of days, which is supposed to mimic the benefits of surgery by pulling your skull back up into place, but this does have some risks like further loosening the ligaments if you decide against surgery, and also is not always a good like for like trial as to whether the surgery will benefit a person. Was anything specific identified as to why your first fusion failed? Many thanks again for the helpful info! B xxx

My heart goes out to her dealing with all this at such a young age. I know kidney stones can be very painful (although I have never had one myself) & hope she manages to get over that quickly! I would have thought that would be one problem less likely to happen in someone with POTS given the volume of fluid we drink, so that is very unlucky! So the fainting post op didn't hurt the fusion? That is a relief to know. Firstly I am going to thoroughly pursue intracranial hypotension/spinal fluid leak as the cause of my illness given my mode of onset & splitting orthostatic headaches, but if I am satisfactorily cleared/treated & still unwell then I think I will pursue the surgery given the success others have had with this. Thank you so much for sharing experiences, I find it so helpful to hear from/about others who are dealing with similar. B xxx

Maybe take some of your home readings with you plus a well written handout like the ones from this site on POTS. That should help the discussion along & provide some info if they are not familiar with it. B xxx

How frustrating! I was quite familiar with the game of medical "hot potato" during my career, when multiple specialists would each argue that a complex patient didn't quite fall within their remit, or were only interested in addressing one piece of a larger puzzle, and now experience the same as a patient Yes, I just threw an opened but full sachet of trioral in the bin! I just hope it hasn't caused any lasting damage being like this for so long. People with diagnosed leaks are advised to lie flat in order to prevent excess traction on the delicate structures in the absence of a fluid cushion, but people with POTS are advised to force themselves upright as much as possible, & I have been doing the latter for 5+ years (without it becoming one ounce more comfortable or tolerable). That's true right across the board sadly. When I was at medical school there was a saying that 80% of the diagnosis comes from the history taking, the other 20% from examination & investigations. I think that has been forgotten now. The guy I am going to see later this month though, Dr Simon Ellis, wrote the UK CSF leak association booklet in which it states MRI misses a proportion, so I am hopeful he will be worth the money! I spoke to another lady here (also diagnosed with POTS) with classic upright headaches of sudden onset & borderline HR changes whose imaging was all reported negative & he patched after collaborating with Dr Gray at Duke in the US, & she is now upright & working again. Hopefully in time the arena of synthetic/cadaver/animal derived tissues will develop so you may have more options for repairing the thinned area of bone. B xxx