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
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