bombsh3ll

5 minutes ago, GasconAlex said:

the treatment helped, but never touched the POTS. I think I always had this but was never symptomatic, again a history of a connective tissue disorder but I have it mildly (my boys are showing much more in the way of symptoms)

Sorry your boys are dealing with this too. I think watching your children suffer must be harder than having it yourself. When you say treatment never touched the POTS, are you referring to numerical heart rate changes or how unwell you feel upright? I am only concerned with the latter (& my HR is normal anyway). 

How frustrating that you have started to leak again, still at least you should be able to obtain treatment more quickly now that the surgical findings were proven last time.  How can you tell it is from a different site without it showing on imaging?

For me, nothing leaks from my nose, I believe a spinal leak is likely. You are right most of the literature doesn't describe cranial leaks as causing the splitting orthostatic headaches, but I believe textbook descriptions, particularly concerning rarer conditions, originate from an era where patients' symptoms were not listened to much, or discounted if they didn't fit an expected pattern. 

I think a similar thing happens with spinal leaks in that the lightheadedness & fainting is often not described in medical literature, yet having reached out on CSF leak forums to people with proven leaks, it appears to be fairly common. My guess is if you go to a headache clinic, syncopal complaints are ignored, & if you go to a syncope clinic, headaches are ignored, because they only look at their own specialty not the whole picture. Also in my case nobody listened to the fact that I was completely normal prior to a forceful valsalva. 

I hope you can either reseal spontaneously or get a successful repair more quickly this time. Again thanks for the info. Whilst I don't yet know conclusively if I have a leak at this point, I would put money on it that others on here do, & may not realize, particularly if they were fine until some trauma like me or childbirth with epidural etc. then suddenly couldn't be upright. 

B xxx

On 5/27/2019 at 8:50 PM, jayut said:

Fresh off the presses. Dated May 22, 2019.

https://www.ncbi.nlm.nih.gov/m/pubmed/31118385/

On 5/27/2019 at 1:47 PM, GasconAlex said:

Imaging of patients with CSF leaks often initially is read as normal, and MRI is not an adequate evaluation in the high clinical suspicion of a leak, Dr. Carroll said. “It is a good place to start, because if you see a leak on your MRI, maybe you do not have to get a CT myelogram,” he said. “But if you have a clinical suspicion of a leak … you should pursue that in the face of your radiologist telling you that there is nothing.”

Thanks for the shares guys.

I've come back around to this after further research & consulting with another couple of specialists.

Alex I know you've had successful treatment but what about you Jayut, have you pursued this line further?

My MRI contrast was normal but I am not letting go now without further imaging & preferably a couple of blood patches, given I have such classic symptoms, a clear precipitating event known to cause spinal leaks, before which I was completely normal, and a connective tissue disorder. 

B xxx

Bending over exacerbates the searing pain in my head, & coming back up again worsens the lightheadedness. 

I sit on the floor to pick things up, or get my kids to do it (as it is usually them who have dropped things).

B xxx

Very similar to me! (except I can not remain standing that long & my diastolic tends to go up a bit more)

Your HR increase is just about borderline for POTS - going from 69 lying to 97 standing is 28bpm. 

Typically the threshold for a POTS diagnosis is 30bpm but I have also read of 30 being used for a tilt test, and 27 or 28bpm being the cut off for an active stand test which is what you did (as the latter uses the leg & trunk muscles). For adolescents an increase of 40bpm is quoted. 

The cut off is very arbitrary though, & whilst meeting it can be helpful in terms of a "diagnosis" for disability or other practical purposes, it is not particularly helpful to apply it rigidly in determining if/how to treat a patient, which I personally believe should be based on alleviating symptoms rather than obtaining a certain heart rate or blood pressure. 

Also, POTS in itself is not a disease as such, it is a description of feeling unwell upright and having an increased heart rate. There is always something causing it which should be looked for.

B xxx

Midodrine will drop your heart rate reflexively. I do sometimes have a resting HR in the 50's naturally, but it was in the 40's when I tried midodrine.

B xxx

You don't even need to have an abnormal HR either to have symptoms unfortunately. My BP and HR are usually perfectly normal yet I am unable to stand for severe presyncope, & very lightheaded sitting up too. I met criteria for POTS on testing but mostly not in day to day life. 

What even a lot of doctors fail to understand is that cardiac output is not equal to, nor can even be approximated, by measuring these variables. 

Cardiac output, ie the amount of blood pumped per minute, is stroke volume (amount pumped per beat) multiplied by heart rate. 

The equation is CO = SV x HR

So stroke volume needs to be known (and is only measurable with invasive/complex tests) in order to measure cardiac output, which is actually far more important than blood pressure. 

You can have a normal BP with a low cardiac output since MAP (mean arterial pressure) is the product of cardiac output x vascular resistance. 

So two people can have the exact same blood pressure, but one may have a good cardiac output and low vascular resistance - this could be an athlete with a large blood volume, and another can have a miserably low cardiac output and high vascular resistance ie the sympathetic nervous system is frantically trying to compensate by making all the blood vessels constrict, hence, the overall BP as measured at the arm looks "perfect" but the tissues including brain are not receiving an adequate blood supply. 

Now, I just wish someone had the solution!

B xxx

I just had a look at this as obviously anything that potentially helps blood back upstairs or even maintains muscle tone in non upright folks could help. 

Whilst I am not convinced it would be hugely beneficial, I cannot see any way for it to be harmful other than financially or in terms of disappointment. 

There are a number of similar devices around which do the same thing, so you may be able to get one of those for less, or alternatively see if you could buy one second hand. It looks like the sort of thing people would get as an unwanted gift or fad which they only used a few times, so there are likely some on ebay or similar. 

I know it is something different to this but I have a seated pedaller called gym mate which also gets the blood pumping & can be used seated/reclined or even lying on a floor mat. It cost much less than the hummingbird & also provides cardiovascular exercise rather than passive vibrating. 

B x

12 minutes ago, DizzyGirls said:

one of my daughter's neurosurgeons hand picked the anesthesiology team because of her POTS.

That is fortunate & probably made the world of difference. I am lucky in that regard as the hospital in Barcelona have many patients with our issues.

I have tried both of those meds (and a number of others) but they didn't help me. My BP and HR are generally textbook normal, so wouldn't benefit from being manipulated. I believe I have low stroke volume as a result of hypovolaemia, hence low cardiac output. That's more difficult to prove, and to treat. Or, it could just be that the inflow/outflow to the brain is compressed as a result of spinal fluid leak or cranial settling. 

Did she ever faint post op, & was the fusion OK? I am guessing soft landings & possibly the brace are important there!

B xxx

Thanks. I would be in the hospital the first 10 days or so then a disabled adapted apartment with nurses on site for a couple of weeks after that. It is not so much being alone that bothers me, more the impact of landing on the fusion if I was still a fainting risk, although of course having someone with me would be helpful. 

The standard advice of "lie down when you feel faint" doesn't really help me, as I would spend my entire life supine, so what little I do, I do presyncopal.  

I will speak to the surgeon about this of course, but if there was medication to stop fainting nobody would be having the surgery, so I am not sure what his advice will be.

B xxx

On 8/27/2019 at 5:44 PM, aelizabeth3300 said:

I'm terrified to exercise with my heart rate and exercise intolerance as they are right now. Light cardio gets my heart rate above 200 easily even on a beta blocker. My muscles have also been losing strength at an alarming rate despite regular activity. I don't know what to do.

If heart rate zones are an issue for you - they are for me just the other way around, I would be unconscious long before my HR got into the recommended zone - you can go by the RPE (rating of perceived exertion).

Yes, this protocol was developed for people who are significantly limited in terms of orthostatic ability, I would think those who are able to walk with no problems or work out upright are not really the group it is aimed at. Of course healthy people or those with mild symptoms that don't limit their daily life can benefit from exercise too, they just don't specifically require a recumbent plan to do so.

 B xxx

I was not able to progress through the full thing but found it helpful to customize to my abilities & equipment that I have. For me the heart rate zones were an issue as it seems to start from the assumption of a high resting heart rate (therefore a relatively low level of exertion would take you into the required zone), which wasn't the case for me, however you can use perceived exertion instead. 

I just thought it would be helpful to let anyone know who might be looking for it as it is much discussed & until now remained a big mystery to anyone who didn't get a copy through their provider. When I got mine it specifically forbade sharing, but now that it has been released to the public I can see the sheets are exactly the same as what I received, so it comes raw & not already individualized. 

Good luck with your PT.

B xxx

A kind of sad but sweet thing happened with my family regarding this the other week - 

My son has heard both my parents (separately, they have been divorced for many years) trivialize & dismiss my illness. He had a nosebleed one morning, didn't lose a lot of blood but the sight of it made him feel really faint, & he went pale & had to lie down quickly. He didn't actually pass out but came close, it really gave me a scare at the time as it is FAR worse seeing your kid go through it, but by the time we got to the hospital he was absolutely fine. 

I later heard him telling my dad how horrible fainting felt & "that's how mum feels all the time, so that's why she sits down all day". My dad has been noticeably less insensitive since. 

B xxx

17 hours ago, Pistol said:

I read an article stating that flight can improve POTS symptoms b/c the cabin is under-pressurized, therefore causes a different homeostatic environment

Thanks, I would be really interested to read about that, do you have a link to it or know the author? It was such a miracle, I would have thought the opposite would occur with flying generally being considered dehydrating! There has to be something, especially given it happened twice with me & several times to the other person I know about.

B xxx

I honestly think that people close to us minimize our suffering as a coping mechanism for them, because it is easier to believe their loved one is lazy/weak/selfish etc than to accept that person has a life destroying illness. 

It is about prioritizing your own safety and wellbeing above their psychological discomfort. 

B xxx 

Anyone interested in the Levine protocol who hasn't been able to access it through their healthcare provider - it is now publicly available from the following website:

https://sites.google.com/site/patientcarejs/levine-protocol

B xxx

I actually had the opposite experience with a long flight, although granted I was not staying at a high ground altitude for several days. 

Earlier on in my illness when I was still well enough to travel, I went on an 8 hour flight. The 24 hours after that, I was restored to completely normal health. The same happened following the return journey, so it wasn't a fluke. I have never experienced remission any other time.  

I have heard of one other patient with POTS who experienced the same thing.

My theory is that the mild hypoxia led to reduced renal blood flow (a recognized physiological response), which induced production of renin, & consequently aldosterone, transiently restoring my blood volume to normal or close to normal. 

B xxx

19 hours ago, KiminOrlando said:

saw CCI and thought he could fuse only C2 & 3, but said he only goes in once and if he thought I needed more he would do it then or he wouldn't do the surgery. At that point it became a risk of not being able to drive. I'm not married and no real public transportation where I live. Not being able to drive would be catastrophic to my quality of life. Also, I have an autoimmune component to this little drama,

I totally get why you made that decision. Single level fusions in EDS often end up exacerbating problems above & below & further surgeries later on are common. Also that level is too low to have any bearing on any brainstem pathology. 

13 hours ago, DizzyGirls said:

You asked about fainting?  I have a story.....  The first time riding in a care without the brace, we went to SF to see her neurosurgeon.  On the way there, we were in a car accident.  I have never been so scared in all my life.

That must have been really scary, I am glad her fusion was ok. Frustrating that the PT didn't listen post-op. The team in Barcelona where I would have surgery have a lot of dysautonomia patients so hopefully they would be prepared. How did you deal with fainting in daily life whilst it was healing? That is what worries me, if it didn't work I could knock it all out of place before it healed if I went down & hit my head or neck. 

B xxx 

13 hours ago, KiminOrlando said:

For me, I have a slight Chiari, but they decided it wasn't impacting CSF and wasn't worth fixing.

That's smart - many people have had innocent Chiaris operated on but didn't get better without coexisting instability being addressed - Drs Bolognese & Henderson term these the "complex Chiaris" where there is CCI as well, & I read a paper about the Chiari being what gets them in to see the neurosurgeon, but isn't actually the problem, then the CCI is discovered (if they are lucky). 

There have also been reports of patients with the appearance of a Chiari which is actually the result of a spinal fluid leak & the brain is sinking downwards. In these cases, when the leak is found & sealed, the "Chiari" has resolved. I do not have one, but my cerebellar tonsils are in the foramen magnum on upright scan. 

13 hours ago, KiminOrlando said:

I would have had the zipper scar, but my hair would cover it, so it wasn't a big deal.

I could probably cover it too but frankly I wouldn't care if my head looked like a second backside if it got me better . 

13 hours ago, KiminOrlando said:

My neuro is Dr. Sunil Patel out of the Medical University of South Carolina. I think he publishes on PubMed if you want to read his stuff. He supposedly specializes in this.

I think I have come across some of his material, he sounds good. What level did he suggest fusing you to?

B xxx

It is an expected response unfortunately that alpha blockers lower blood pressure and are unlikely to be tolerated by a patient with dysautonomia. 

I would see if your urologist has any other suggestions that do not interfere with BP.

B xxx

16 hours ago, Pistol said:

What does your physician tell you about these concerns?

The neurosurgeon that I have seen was very honest about both the potential for, and degree of, improvement being impossible to predict in advance of surgery, however he only offers surgery where he believes it has the potential to improve someone's quality of life. He does however specialize in disorders of the craniocervical junction, particularly among patients with connective tissue disorders (ie atraumatic acquired instability) and the majority of those he performs this surgery on have symptoms of cervicomedullary syndrome - predominantly dysautonomia, some have additional neurological symptoms. 

We did not discuss my concerns about continuing to faint with a fusion if the surgery was not successful at my appointment, as I have not yet decided on the surgery but would definitely be raising this beforehand if I did. Obviously if there was effective medical treatment people would not be having surgery in the first place, so it must be an issue for those in whom the surgery doesn't work.

Some patients have much more marked radiological instability than I do, and also have seizures and/or breathing problems, so in their case the decision is more clear cut, it is a difficult one! I certainly intend to pursue exclusion of a spinal CSF leak to the nth degree before deciding on this route, as treatment of this is easier, less expensive & doesn't result in any long term loss of movement, & also my mode of illness onset (Valsalva) fits far more with this - although EDS is a risk factor for both spinal CSF leaks and CCI, and there are certainly patients who have experienced both. 

B xxx  

I do not believe it is currently considered a standard treatment unless you have been diagnosed with an autoimmune disease, however research is increasingly focusing on an autoimmune basis for POTS, with a number of antibodies being implicated in at least some cases.

Although I personally have reason to suspect a structural cause in my own case & have no features suggestive of autoimmune disease, I would still give IVIG a try myself if it were offered, as some patients have really benefited & at this stage the science isn't at the point of being able to predict in advance which patients will or won't

Has your specialist suggested it & if so did they indicate why in your particular case?

B xxx

13 hours ago, DizzyGirls said:

She is fused from C3-T4. 

Thanks for replying, it is great to hear the experiences of someone who has actually dealt with this. I can understand her not wanting the upper segment done as this is where most of the neck's range of movement lies. Can I ask what benefits she gets from wearing the collar? The adjustable positioning being helpful makes sense - I think there is a common perception even among some surgeons that there is one optimal position for the neck to be in, which cannot really be the case as like other joints it is designed to move. 

One thing that concerns me about the fusion surgery is if I had it & it doesn't work & I am still fainting afterwards, what about the risk of damaging/breaking it, especially in the early months when the bones are fusing? Was syncope/seizures and protecting the fusion an issue for your daughter & if so how did you manage this?

Regarding the level of fusion though, what I have observed from reading published cases & speaking directly to a number of patients it has helped, is that only fusions skull downwards (ie occipitocervical) to various levels appears to cure or improve orthostatic intolerance/syncope, presumably as this is the level of the brainstem. I have not seen anyone recover whose fusion began lower than the skull (although obviously it can needed & helpful for other instability related problems pain/neuro etc).

I hope your daughter manages to find some relief from her vertigo.

B xxx

3 minutes ago, KiminOrlando said:

No. I was given a regular neck collar that made things worse because it was too tall and kept my head in a position that put pressure on my brain stem. My muscles started getting weaker and I realized it was a bad thing to wear it.

I tried the popular Aspen Vista which is adjustable, and it didn't help me either - I understand collars are only really helpful for those with rotational and/or horizontal instability, not vertical. 

B xxx

21 hours ago, KiminOrlando said:

I used all this to decide that it probably wasn't a good risk/reward situation for me.

PS Kim were you ever offered a trial of invasive traction (halo brace or Gardner Wells tongs) to help give an idea of whether surgery would benefit you?

B xxx

20 hours ago, KiminOrlando said:

I was given this option and declined surgery for this reason. I can still drive but would lose it with surgery. There are people on here that had surgery with no relief of symptoms. Recovery/rehab was brutal. I used all this to decide that it probably wasn't a good risk/reward situation for me.

Thanks Kim that is useful to hear. I cannot drive anyway due to syncope/presyncope, but can totally understand that being a concern if you can. 

18 hours ago, toomanyproblems said:

IIRC your onset of symptoms was related to a specific incident (blowing up a ballon?), and that subsequent to extreme valsalva during childbirth that you felt was involved. Why would those incidents be related to cranial settling, assuming that would cause a more prolonged course? 

Those are my questions exactly! - I still feel that a spinal CSF leak is the likelier culprit (as valsalvas are a recognized cause of leaks, & EDS is another risk factor) which is why I am continuing to pursue this avenue first although my MRI contrast was negative. Childbirth x3 didn't bring it on for me, it was just blowing up that dratted balloon, but it was a slightly different type of expiratory straining which is hard to describe as opposed to birthing (where the uterine contractions do most of the job).  The searing orthostatic headaches which most people on here don't seem to have also point towards this, as do the numbers of people on forums with diagnosed leaks who have/had lightheadedness, fainting & haemodynamic instability as part of their symptom complex. Many of them have been diagnosed with POTS and like me have had no response to POTS treatments. Stanford's Dr Carroll's youtube video on this is also very informative. 

I also do not see how that could have caused cranial settling, but there remains the possibility that it tipped something that was there but asymptomatic at that time into becoming symptomatic - or perhaps I would have started fainting soon afterwards anyway, maybe it was always on the cards.

The cranial settling is the only positive finding I have been diagnosed with to date (after very many tests) that is known to cause dysautonomia (via cervicomedullary syndrome) & is one for which there exists a treatment that has been shown to cure or substantially improve it in SOME patients (but by no means all). So for me, if I can eliminate a CSF leak & am left with only one diagnosis that is known to cause it, which has a treatment, I would probably go for it than sit there & do nothing when there is something that could potentially help. 

The symptoms of CSF leak and craniocervical instability are remarkably similar, & I really believe one of these two differentials could be it for me. 

B xxx