A TREATABLE CONDITION TOO IMPORTANT TO MISS

bombsh3ll

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This is for everyone with orthostatic intolerance/POTS, 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.

Likewise everyone who can identify with PEM aka feeling worse after activity than they did before.

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.

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 this will not be applicable to everyone, but is a recognized cause of an extremely familiar 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/

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
 

bombsh3ll

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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 • Female>male, peak onset 30-40s
• Associated w connective tissue disorders • Associated w connective tissue disorders
• Understood • Mysterious
• Treatment can be effective • Treatment: “learning to cope”
 
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bombsh3ll

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It ´s a wonderful day that day when you got this diagnosis ! Would you tell how doctors finally found the cause of your illness?
xxx

sadly they didn't - I came across it myself by watching Dr Carroll's video. I then presented material from the CSF leak association highlighting my symptoms, inciting event & connective tissue disorder to my GP & asked for a referral to investigate a possible leak. I had a contrast mri on the NHS that was read negative, but when I sent the same scan to Dr Bolognese to look for cci (without me mentioning csf leak or even that my illness was valsalva induced) he wrote back saying no cci but suggested Csf leak. I also wrote to Dr Carroll who agreed i t was ve r suspicious for a leak, then I saw Dr Ellis a private neurologist who specialises in leaks & he diagnosed me clinically yesterday.

B xxx
 
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bombsh3ll

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@bombsh3ll
Do you mean that the specialist does the diagnosis base on symptoms and not on imaging nor on the open pressure test ?
They go off a combination of these (personally I have not had a LP as it has poor sensitivity & has a higher chance of causing a leak in EDS patients, also they are being less commonly done now in the UK unless there is a suspicion of high pressure or other indication), plus also response to blood patching can help confirm the diagnosis.

B xxx
 
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but whilst unwell had a violent bout of coughing, sneezing or vomiting which may have put a rent in the dura;
This is a very interesting....remark.

Just feel like commenting that I had two severe gastrointestinal events...not food poisoning. Posssibly Norovirus, but have no way to know. Or were these Mast Cell eruption events?

And I was so violentingly ill. I would entirely believe I could have damaged something during these episodes. I got much much worse after the last one. I also hypothesized they generated Nitrous Oxide eruptions. That may have re-trapped, exacer-trapped or otherwise worsened my base condition...which HAD been mild. And is not mild any longer.
 

bombsh3ll

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https://www.livescience.com/49783-pilates-caused-brain-fluid-leak.html
Leaking Brain Fluid Traced to Pilates Injury

So glad you figured this out! Do you think this applies to both POTS and NMH?
Yes it probably does. The brain sinks down having lost its fluid cushion, and the brainstem gets squashed. It won't account for everyone but certainly worth a look if a patient checks a few of the boxes & isn't finding any other explanations.

It is certainly more easily & cheaply treated than craniocervical fusion & doesn't result in loss of neck movement, so should absolutely be excluded before pursuing this.

Some patients have had unnecessary chiari or fusion surgeries only to not get better and then discover a leak (not caused by the surgery, usually found lower down the spine) was the issue all along. I spoke to someone who was literally about to get a fusion then diagnosed with a leak instead, & recovered to normal or near normal function after blood patching. Scary!

B xxx
 

bombsh3ll

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@bombsh3ll
If I understand well, the blood patch must be injected close to the leak, so in my mind it means that the doc needs to know precisely where the leak is, am I right?
It is definitely an advantage if the leak site is known, such as if it is the result of either a deliberate or accidental (eg during epidural anaesthetic) lumbar puncture, however with spontaneous/minor trauma induced leaks like mine, the initial MRI contrast usually just shows features suggestive of intracranial hypotension such as brain sag & meningeal enhancement, but not the leak site itself. In order to locate the precise site, you would generally require more specialized & invasive tests ie various types of myelogram.

However as these are both very expensive & come with more risks than non-targeted blood patching (myelography involves deliberately puncturing the dura to inject various contrast agents into the spinal canal), the usual practice is to try large volume non-targeted blood patching at a low spinal level in the first instance, as this is effective in many cases since the blood disperses throughout the epidural space and has a low risk of complications.

If after a prescribed number of non-targeted blood patches there is no improvement (up to three have been recommended in my case), then more precise tests are ordered to try and locate the leak so that either a targeted blood patch, fibrin glue patch or dural repair surgery can be done.

I am hoping that I will be one of the lucky ones who improves with first line treatment, but trying not to get my hopes up too much!

Unfortunately some people with clear evidence of a leak & no/unsustained response to blood patching are never able to locate a precise site despite multiple types of imaging being used, so treatment in this field is not perfect, but I am at least grateful to have something to try now that is aimed at the underlying pathology.

B xxx
 

Sushi

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Unfortunately some people with clear evidence of a leak
What are the symptom-signs that might point to investigating a possible CFS leak? My acute phase of NMH came on suddenly after a week of unusually strenuous aerobic exercise, but I wasn’t aware of any injuries. Before that week I would just ‘gray-out’ if I stood up suddenly.
 

bertiedog

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I am very pleased for you that you now have some hope @bombsh3ll.

It reminds me that in 1979 I used to play loads of tennis and during that summer my male partner served a tennis ball right into the back of my head which nearly knocked me over it had such force. I remember seeing stars at the time but don't remember how I felt an hour or so after but I definitely remember being very shaken up by it and also some of the members who I used to play tennis with never forgot what happened to me especially as I couldn't rejoin the next year because everything had changed for me.

However I do know for sure that around October of the same year I developed a severe flu from which I couldn't seem to recover. A couple of weeks after I improved very slightly energy-wise I developed very severe vertigo attacks that would go on for days or weeks at a time interspersed with very nasty migraines. I would feel like death during these episodes and remember being housebound the following summer because of the horrendous episodes.

Needless to say I was never able to play tennis at the club again even when my energy improved somewhat because I would be so dizzy as I looked up to hit the ball when serving. It would happen every time I tried to serve I would practically fall over with dizziness..

Obviously something radical changed and all though my 30s and 40s I would get these severe vertigo attacks followed by migraine but I was able to do some part time teaching. To be honest during this time I became very nervous and almost agoraphobic because I had no control over when the episodes would start and they really terrified me as I would feel so out of control and ill.

The major energy issues didn't hit until the late 90s when I had to give up teaching completely because I was permanently dizzy and exhausted but to this day I don't know if that event played any part in me developing ME. I am also hyper mobile btw.

Pam
 

bombsh3ll

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everything had changed for me.
Yes, everything changed for me too immediately with that one event that sounds so silly! So many times people have acted like I must be making it up, how could that possibly cause such severe disability etc..

I suspect a lot of cases go undiagnosed, which is so sad when it is treatable in most cases.

B xxx
 

Gingergrrl

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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 am so happy for you @bombsh3ll that you have figured out the true cause of your suffering and this is amazing news!

Do you know if "Valsalva" is what is done in a spirometry or PFT (pulmonary function test)? I did several of those tests (in prior years) and you wear nose-clips and have to breathe out as forcefully as you can. It was very hard for me and it felt like my ears had popped. I never made the connection to "Valsalva" until Googling it right now.

In my case, I already had POTS and was very ill prior to doing the pulmonary testing so it was not the cause of my illness but it reinforces to me that I will never do it again.

I now have a long fight ahead of me for NHS funding for the recommended treatment for this
Is the NHS saying that they will not pay for the recommended treatment even though you have a proven CSF leak? This is horrible (if I am understanding correctly)!

If this information helps just one other person, I want it to be out there & easier to find than it was for me.
I feel exactly the same way and it is the reason why I keep sharing about my story (which is a different story) but I pray that it helps others both present and future to solve their cases since I can't be the only one with these obscure autoantibodies (and you can't be the only one with a CSF leak from the Valsalva).
 

bombsh3ll

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What are the symptom-signs that might point to investigating a possible CFS leak?
The symptoms (see above "Spinal CSF Leaks have a troubling similarity to POTS") are typically splitting orthostatic headaches & neck pain, lightheadedness/dizziness/presyncope, nausea/vomiting upright, blurred vision, cognitive impairment & generally feeling extremely unwell upright, that is relieved or eliminated by lying down.

The initial investigation - varies between centres - is typically a supine MRI brain and spine with contrast. This usually does not show the actual site of the leak, but there are certain characteristics which suggest the presence of intracranial hypotension (SEEPS mnemonic):
S = subdural fluid collections – may be small to large fluid collections or subdural hematomas
E = enhancement of meninges (layers around brain more obvious with contrast than normal)
E = engorgement of venous structures (veins swollen)
P = pituitary hyperemia (pituitary looks swollen)
S = sagging of brain – including, but not limited to cerebellar tonsillar herniation into spinal canal (acquired Chiari)

This initial test is estimated to be positive in 70-80% of cases.

A low opening pressure on lumbar puncture is also diagnostic, but this is generally a poor test as >50% of patients with leaks will show normal pressures. It can also cause a new leak in itself, so tends to be done less often now for the purpose of investigating low pressure (although remains useful in other diagnoses or if high pressure is suspected).

B xxx
 

bombsh3ll

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Do you know if "Valsalva" is what is done in a spirometry or PFT (pulmonary function test)? I did several of those tests (in prior years) and you wear nose-clips and have to breathe out as forcefully as you can.
Yes that is a Valsalva. What I would say is that I blew harder to try and inflate that water bomb than I ever did giving birth 3 times, and I also have diagnosed EDS so my connective tissues (including the dura) are weaker than the average person which predisposes to CSF leaks.

I also did a spirometry years ago & it was nowhere close to the force of trying to inflate that thing, as a spirometry tube is actually designed to be exhaled into, a water bomb is not ;) however if someone was very unlucky it could conceivably happen from that.

I am glad you too found something treatable. That is why I want to share, even if it only affects a small number of people.

I know it can make your heart sink hearing "i found my cause woo hoo" stories, but where it is a legitimate & treatable medical issue like your autoimmune condition, Jeff's CCI or this, not just I went vegan, did DNRS or dipped my big toe in a jar of honey, I wanted to make it available for anyone to whom it might be relevant to find.

Regarding NHS treatment, I live in Scotland where the NHS only automatically funds treatment in your own area, & there are no specialists that treat it where I live. You have to appeal for funding to see a specialist out of area, & it is likely to be refused if there is someone in your area who has never done the procedure before, but is willing to have a go.

B xxx
 
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bombsh3ll

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Is Dr Ellis who diagnosed you, a british doc?
Yes, he is a neurologist in Stoke. He is on the board of the CSF leak association and co-wrote a lot of its information articles. I found him very knowledgeable & up to date with the latest research. He also examined me properly & genuinely seemed to want to help.

Since becoming unwell, most of my experience with healthcare professionals has not just been fruitless but dehumanizing, & it is rare to find someone with that level of knowledge & compassion.

B xxx