Treatment suggestions for Orthostatic Intolerance (POTS or NMH)

[Jonathan Edwards]

I think looking at your recent lab work, done with fasting, is a good place to start to understand OI with ME. Are you usually low in sodium and potassium? How about calcium?
.

I think there may be a confusion here. Low serum sodium has nothing to do with low blood volume so is unlikely to have any relevance to OI. I cannot see that potassium or calcium are going to be relevant either, are they?

 

[ahimsa]

The problem for me is that if I see a list of disinformation I feel obliged to say so. I realise that patients are posting with good intentions but if the stuff is bogus science then surely saying so needs to be mixed in with the list in order for it to convey real information?

That's a fair point. After all, so many doctors recommended exercise for ME patients. And it was repeated in many official documents.

The problem for me, as a patient, is how to tell what is misinformation? Especially when this information is not from fringe sources but comes from what appears to be the majority of mainstream doctors who study orthostatic intolerance, at least in the USA?

I do not have any medical expertise. I cannot read original medical research and understand / debate those points. And even if I tried to do so I'm aware that "a little knowledge" can be a dangerous thing.

I rely on mainstream doctors and organizations that provide recommendations on how to treat orthostatic intolerance. And then I rely on consultations with my own doctors (primary care physician, an MD in Internal Medicine, and my cardiologist).

That's all I have. I do my best but I'm sure I fail a lot of the time.

I support skepticism and looking into things! I support reviewing the medical research (or setting up new studies!) to look for evidence on whether extra salt/water actually helps patients. But I can't do that kind of mental work.

So, in the meantime, I'm going to follow what doctors who study various types of orthostatic intolerance have suggested and what has helped me. Maybe extra salt and water is merely placebo but it has had no side effects for me and doesn't cost anything.

 

[Trish]

I think it is important that any thread where treatments are discussed needs to be open to discussion of the science behind that treatment. If we simply list resources that promote that treatment we do a disservice to members who want to know whether the treatment has scientific basis.

@Jonathan Edwards, do you have any information or ideas on what causes OI in ME patients, given that you say it's unlikely to be related to low blood volume?

I, for example, developed two distinct phenomena that made standing for more than a few minutes problematic right from the start of my ME.

One was a light headedness /near fainting on standing suddenly or standing for long.

The other, completely different, was a feeling that my leg muscles had run out of energy and were going to collapse if I didn't sit down soon.

Neither of these could be explained by being bed bound or deconditioned, since for the first decade I was relatively mildly affected and still working, yet these symptoms were there from the start.

I only came across the term 'Orthostatic Intolerance' relatively recently, and have no idea whether it applies to either version of my inability to stand.

Note, I am not asking for personal medical diagnosis or advice, simply trying to understand what is meant by OI, and what might be causing it.

 

[Jonathan Edwards]

The problem for me, as a patient, is how to tell what is misinformation? Especially when this information is not from fringe sources but comes from what appears to be the majority of mainstream doctors who study orthostatic intolerance, at least in the USA?

I fully understand your problem and that is why I think having a site like Science for ME where we can discuss these things is so useful.

So let me give a bit more detail of my reasons.

Unfortunately 'the majority of mainstream doctors studying orthostatic intolerance' may not be quite what it seems. In general doctors interested in the rigorous science of blood pressure control do not get involved in orthostatic intolerance - perhaps because there are much bigger clinical needs with hypertension and suchlike. At least in the UK only doctors who are not rigorous physiologists go into this field. I say that just as a matter of what I have observed. So in reality, I regard any physician who majors in OI as 'fringe'. It would be different if there were some good studies using properly blinded controlled trials and if there are I would be interested to know what they show, but I have not seen evidence of them.

There is also the problem, as I see it, that in the USA virtually all clinical practice is for profit. Prior to joining in ME websites I had little information on what went on in US practice as compared to the UK. Having 'listened in' for four years it seems to me very clear that a lot of unproven treatments are offered to patients as 'mainstream' I the USA. In the UK doctors are mostly paid to do whatever work comes along and have no incentive to see more patients. So these treatments are not considered mainstream in the UK. The downside here is that there is not enough funding even to give people the mainstream treatments, but that is another issue.

The simplest thing patients can do if they want to know if a treatment is actually based on any reliable evidence is to ask the physician for references to the relevant studies. I realise that judging quality in studies is not simple, but if there aren't even any published studies then it is easy to conclude there is no reliable evidence. I quite agree that as a patient one has to follow the advice of the doctor taking responsibility for one's care. I would not want to put that in doubt. But it is possible to query the grounds for treatments.

 

[Jonathan Edwards]

I only came across the term 'Orthostatic Intolerance' relatively recently, and have no idea whether it applies to either version of my inability to stand.

I think this is very pertinent. I suspect the real problem is that the term has no very clearly defined meaning but is often used n the assumption that it means reduced brain perfusion. That might apply to one of your symptoms and to the other. From what I hear of PWME talking I suspect the big problem is the second one and that is probably nothing to do with cardiac output or blood volume.

 

[voner]

I think this is very pertinent. I suspect the real problem is that the term has no very clearly defined meaning but is often used n the assumption that it means reduced brain perfusion. That might apply to one of your symptoms and to the other. From what I hear of PWME talking I suspect the big problem is the second one and that is probably nothing to do with cardiac output or blood volume.

What an potentially useful thread! I have assumed that the term “orthostatic intolerance“ was commonly used because some ME/CFS patients fall a bit short of the “30 bpm increase upon rising" threshold, but still clearly have POTS-like issues, at least that is what I heard Dr. Bateman say once.

@Jonathan Edwards, do you also also hold little regard for physicians who specialize in “dysautonomia“?

Also, Jonathan, would you care to expand on the brain perfusion in regards to symptoms subject? I would love to hear more.

 

[Dial It In]

I am pretty doubtful that orthostatic intolerance in ME/CFS is a cardiovascular problem anyway.

My understanding is that the research is pointing towards a neurotransmitter issue, possibly caused by autoantibodies.

 

[ahimsa]

I think this is very pertinent. I suspect the real problem is that the term has no very clearly defined meaning but is often used n the assumption that it means reduced brain perfusion. That might apply to one of your symptoms and to the other. From what I hear of PWME talking I suspect the big problem is the second one and that is probably nothing to do with cardiac output or blood volume.

That's strange. I know that autonomic specialists have used some different terms and definitions. But I've never seen Orthostatic Intolerance defined as "reduced brain perfusion." Do you have a source for that definition?

For example, here's how the Johns Hopkins patient handout defines OI:

The medical term “orthostatic” is defined as relating to, or caused by, an upright or standing posture. Orthostatic intolerance, then, is an umbrella term for several conditions in which symptoms are made worse by upright posture and improve with recumbency. This document provides further information about neurally mediated hypotension (NMH) and postural tachycardia syndrome (POTS), two common forms of chronic orthostatic intolerance. Hypotension is the medical term for low blood pressure (BP), and tachycardia is the medical term for an increased heart rate (HR)

And here's a medscape article with a lot more detail by Dr. Julian Stewart:

https://emedicine.medscape.com/article/902155-overview

A search on pubmed brings up many documents. Here's one that mentions salt and water as treatment:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3896077/

Supportive therapy focuses on decreasing symptomatic OH and syncope. Such therapy would include physical countermaneuvers, including compression garments and dietary changes (increased salt, rapid water drinking). Supportive drug therapy often aims to increase blood volume by promoting salt and water retention (fludrocortisone) or by increasing red blood cell mass (recombinant erythropoietin) [55]. Defects in erythropoietin may occur as part of the denervation in autonomic failure [63]. Short-acting pressor drugs such as midodrine or drugs that enhance autonomic activity (atomoxetine, yohimbine or pyridostigmine) are also used [55,64].
Rapid water ingestion of approximately 16 ounces deserves special mention. Studies in adults have demonstrated that intake of water free of solute can increase BP and improve sympathetic vasoconstriction after a sufficient time has elapsed for the water to reach the small intestine, approximately 20 min [65]. The therapeutic effect of water encompasses all OI, including OH, POTS and VVS [66] and can be successfully used to prevent blood phobic VVS. Effects last for several hours. The mechanism is dependent on osmolarity and may depend on TRPV4 C-fiber receptors within the portal system [67]. This is a very important, simple and effective palliation that is not often considered by clinicians.

Now maybe all these documents are like the PACE trial, full of errors and flaws. I don't have the ability to assess all these documents. It's up to someone else to point out the errors.

 

[Dial It In]

I have assumed that the term “orthostatic intolerance“ was commonly used because some ME/CFS patients fall a bit short of the “30 bpm increase upon rising" threshold, but still clearly have POTS-like issues, at least that is what I heard Dr. Bateman say once.

Unless there is a logical reason, the test should be percentage increase on standing, not a blanket 30 beats per minute.
If your resting heart rate is 45 beats per minute, it would require a 66% increase to meet the threshold!!!

 

[MErmaid]

I think there may be a confusion here. Low serum sodium has nothing to do with low blood volume so is unlikely to have any relevance to OI. I cannot see that potassium or calcium are going to be relevant either, are they?

Yes, for me those values are relevant. Since we are having a discussion, I assumed it would not harm anyone to look at their lab work.

I must be confused, who said OI in ME has anything to do with low blood volume? Please define low volume.

 

[Jonathan Edwards]

@Jonathan Edwards, do you also also hold little regard for physicians who specialize in “dysautonomia“?

In forty years in medicine I am not aware of having met patients having 'dysautonomia' in any useful sense. I have come across people with autonomic problems associated with peripheral neuropathy but usually the other aspects are more troublesome. I am aware that there are well defined syndromes that include specific autonomic problems like Shy-Drager syndrome but as far as I know they are very rare. I would expect there to be maybe a physician per ten million people specialising in such rare autonomic disorders and I guess there are probably half a dozen in the UK at most. But I get the impression that there are a lot more physicians who describe themselves as specialists in dysautonomia in places like the US. I am not quite sure what they deal with and my experience with colleagues who dabble in this area is that they may not really know either. Dysautonomia is a very convenient term for explaining pretty much whatever you like and that raises my suspicions.

 

[Jonathan Edwards]

Also, Jonathan, would you care to expand on the brain perfusion in regards to symptoms subject? I would love to hear more.

As far as I am aware most physicians assume that 'orthostatic intolerance' implies symptoms due to inadequate blood flow to the brain. In orthostatic hypotension this is very definitely the assumption. If the systolic pressure falls below about 90 in most people insufficient blood is pumped to the brain with the result that about five seconds after standing the person feels light headed and after maybe another ten seconds they feel faint and may lose clear vision and have to sit down. At least that is the usual version in normal people who have been sitting a long time after drinking alcohol etc. In disease the time scale may change. The assumption remains that the intolerance of standing is due to not enough blood getting to the brain.

In POTS I suspect the assumption is the same. An increase in heart rate of 30bpm is not in itself distressing or troubling. Normal people playing tennis or swimming are likely to get a 80-110 rise in heart rate - in fact often much more. So presumably if the tachycardia is a problem it is because it is thought to be a reflection of a baroreceptor reflex signalling low venous return to the heart, which is normally a sign of relatively low blood volume. There might well be symptoms from associated adrenaline output rather than actual failure of brain perfusion but the adrenaline rush would be there to signal a potential inability of the heart to pump out enough blood to keep the brain fully perfused.

So my reading of the Johns Hopkins handout (from ahimsa) is that OI may cover various situations where standing is associated with symptoms indicating potential failure of brain perfusion.

 

[Jonathan Edwards]

Yes, for me those values are relevant. Since we are having a discussion, I assumed it would not harm anyone to look at their lab work.

I must be confused, who said OI in ME has anything to do with low blood volume? Please define low volume.

Changes in levels of sodium or potassium may be relevant to feeling ill but we are discussing orthostatic intolerance and I do not see their relevance to that. I think some people think that a low serum sodium goes with a low blood volume or in simple terms 'not enough salty fluid' but that is not the case. In most cases low sodium is associated with too much fluid.

As indicated above the general assumption behind the term OI is that the heart is unable to pump enough blood to the brain. The commonest reason for that is a low blood volume relative to the context. That gets complicated to explain so there is no single definition of low blood volume relevant here. In haemorrhagic shock you cannot stand up because there is literally an abnormally low amount of blood. But in most faints the problem is that there is not enough blood to fill a dilated vascular tree with the result that not enough blood is being returned to the heart for it to pump out enough to keep the brain full.

As I indicated, I am sceptical that OI in ME has anything to do with either situation, but I am pretty sure that all the physicians who deal with OI think it does - which is why they think heart rate matters. I suspect heart rate may be irrelevant.

 

[Jonathan Edwards]

A search on pubmed brings up many documents. Here's one that mentions salt and water as treatment:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3896077/

That review clearly assumes that the problem is relatively low blood volume. All the measures are directed at getting more blood to the heart. Compression stockings would make sense if blood was pooling in the veins but it would be no use to the common immediate form of OI that occurs before blood has had time to accumulate in veins.

The way the text is written is rather unconvincing - things like 'such therapy would include' suggest someone is in 'belief mode' rather than evidence mode. There is no reason to think salt ingestion over a long period would fool the cardiovascular regulatory mechanisms. It would just be peed out. And it is generally agreed that increasing slat intake is bad for you.

The bit about rapid water drinking seems very odd. It does at least agree with my suggestion that drinking fluid ought to do as much as IV fluid. But the kidneys will pee out the fluid within half an hour.

 

[Jonathan Edwards]

Something I had not thought of mentioned in the review cited by ahimsa is that in autonomic failure you do not get POTS because the autonomic system does not kick in to raise heart rate. In a normal person it does and it seems that 30bpm increase can occur in normal people. It all seems a muddle to me.

 

[ahimsa]

@Jonathan Edwards - I appreciate you sharing your thoughts.

I thought I was sharing helpful information. But in the future I will refrain from posting any links to medical information. I will leave that to people who have enough mental/physical energy to debate all the medical issues and support any claims made by doctors.

I thought the links I posted had been accepted by the medical community but I guess I was wrong. And I don't want to be accused of misleading people with bad information. So I'd rather not post at all.

I hope this makes sense and does not come across the wrong way. I'm just too exhausted to continue this discussion but I wanted to explain why without just leaving the thread and not responding. No hard feelings to anyone!

I am curious about one thing that was mentioned:

As far as I am aware most physicians assume that 'orthostatic intolerance' implies symptoms due to inadequate blood flow to the brain.

Do you have a source for this definition of orthostatic intolerance? (which I'll abbreviate to OI in this post)

I'm not quite sure what you mean when you say "as far as I am aware" and "implies"? How were you made aware? Where did you hear or read this definition of OI? I asked you for your definition of OI earlier in this thread but did not get an answer.

As I've said many times, I'm just a patient, not an expert! But every definition of OI that I've read is more of a "black box" type of definition (listing what happens from the outside) and not a "white box" definition (listing the exact mechanism behind why these problems happens).

There are different theories for causes of OI, different subsets of OI, even different terms (Chronic OI is one I've seen), and lots of complicated details that I don't remember. But I have never read this very narrow definition for OI = inadequate blood to the brain. I've read that blood flow to the brain can be an issue for people with OI. But I've never seen it listed as a definition.

I hope my question makes sense. Can you respond with a link, or some other reference, that shows a definition for OI that you would accept as correct? Or maybe you reject the term OI and you use a different term? At any rate, at least if I knew what terms and definitions you use then that would help me to see where you're coming from.

I won't be contributing to any further discussion but I thought that having a shared definition for OI (or whatever term you prefer to use) would help no matter who ends up continuing this conversation.

 

[Jonathan Edwards]

I thought I was sharing helpful information. But in the future I will refrain from posting any links to medical information. I will leave that to people who have enough mental/physical energy to debate all the medical issues and support any claims made by doctors.

It seems to me that you are doing fine and asking exactly the right questions. I also think it is helpful to discuss this information. But I think what is good about a forum is that nobody takes anything as fact without a good reason. People often assume that because something is in a medical textbook it must be true but that simply is not so. A lot of doctors, and perhaps particularly those who like to write textbooks and guidelines are pretty muddled about what is actually going on. And very often getting a better grasp of things just requires a little common sense scepticism.

So it would be a pity if you stopped taking part in the discussion.

Do you have a source for this definition of orthostatic intolerance? (inadequate blood flow to the brain)

As I said, I am unclear if there is any clear definition of OI. In medicine, as in all science terms are often used in different ways in different contexts and the idea that there is one single definition is often misleading. Also doctors, like other people, pick up words and use them without necessarily thinking very hard. Nevertheless, I am pretty sure that doctors assume that is what OI is about. The second review you quoted above at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3896077/
clearly assumes that thesis the basis, although I realise that it may not be spelled out.

I usually say 'as far as I am aware' because I am always happy to be told I am wrong and sometimes I have misunderstood things.

But every definition of OI that I've read is more of a "black box" type of definition (listing what happens from the outside) and not a "white box" definition (listing the exact mechanism behind why these problems happens).

And I think that is quite interesting, because it relates to where things do not seem to quite add up to me.

Let me start from first principles, as a lecturer to medical students might do. OI is about feeling awful when standing (I presume that is agreed). It is not about walking or using muscles, just about the difference between being flat/sitting and standing, as in a tilt table test. The only things that change much during that shift are the fluid parts of the body - blood and the fluid in the balance organs. Problems with balance organs produce vertigo, so we are not dealing with that.

The only problem that anyone I know can think of with blood shifting on standing is that there is not enough getting to the top (brain). And we are all familiar with feeling faint and we know that is due to not enough blood getting to the brain via arteries. The only reason for not getting enough arterial blood to the brain is a low systolic blood pressure (you need to pump at least a foot so you need enough pressure).

This all makes sense for orthostatic hypotension (=low pressure) with the symptom of feeling faint on standing.

But the review you quoted says something strange about POTS. It says that the pressure does not fall, just that the heart rate goes up. Moreover, to be POTS the rise in heart rate must be associated with symptoms. But what symptoms? He does not say. If the pressure is OK there should be no faintness. The rapid heart beat might seem disconcerting but in itself it is something we are all happy about when we exercise. It should not be something that requires medical treatment. So what is it that people with POTS complain of? I realise that I do not know.

Moreover, lots of patients on the forum say that PWME in fact tend not to have POTS but rather other forms of OI such as neurally mediated hypotension. That would make sense because people would feel faint. So I would interested to know anyone on the forum does actually have POTS in the technical sense and what the symptom is because I do not understand why there should be any symptoms of concern.

 

[OverTheHills]

I have had a tilt table test which confirmed I had POTS about 3/4 years ago.
I did not have low blood pressure during the test.
I would not describe my symptoms as faintness, but mental confusion and a feeling of weakness/heaviness all over ( e.g. in arms, legs, trunk, etc). Also a bit sweaty sometimes which I assume was adrenaline.

I requested this test because I had been prescribed a beta blocker for another problem (hyperthyroidism, now resolved/in remission). I found that a low dose of propanolol improved my problems with being upright markedly. Every time I tried to stop the propanolol symptoms would worsen, every time I restarted they improved. Since beta blockers are a common treatment for pOTS I requested the test as confirmation (stopping the medication for the test)

As a keen hiker and excerciser before ME I can confirm that a 30 BPM increase in heart rate was fine before i got sick and felt absolutely nothing like the POTS feeling.

 

[voner]

I have POTS "in the technical sense". As far as, “what symptoms does it cause that are problematic?” I personally can't say. some of the symtoms could be driven by POTS, but might not be. i’ve never been able to sort it out and I’ve never run across a medical provider who had a clue, even ones very experienced ones with POTS treatment.

my experience with talking with clinicians who treat ME/CFS and participating in forums for years have given me the impression that the autonomic system dysfunctions in ME/CFS patients are all over the map and not limited to POTS/ orthostatic intolerance, thus I usually use the term “Dysautonomia”.

for a education on Dysautonomia, I suggest going to the website for, “dysautonomia international”.

http://www.dysautonomiainternational.org/

 

[ahimsa]

So it would be a pity if you stopped taking part in the discussion.

I appreciate that, but pretty much anything I post now is going to based on documents that I already referred to earlier. And I'm not sure how that will further the discussion.

You said:

I strongly suspect that none of these advice documents are based on any reliable evidence.

Over the years I rubbed shoulders quite a lot with doctors with a special interest in 'autonomic dysfunction' and pretty much without exception they were the ones who did not really understand the physiology. There may be doctors out there who do but I strongly suspect there isn't actually any reliable evidence for these things.

If the information that I posted earlier is not reliable then anything I say is going to be even more unreliable! It will be based on my limited understanding of that information.

On the other hand, if the information posted earlier is reliable, then it's much better to get the information directly from Dr. Peter Rowe, Dr. Julian Stewart, Dr. Blair Grubb, Dysautonomia International, or whatever other doctors / organizations that I referenced above.

I can't see what value I bring to this discussion. Plus it's simply exhausting to try to put these thoughts into words (takes much longer to write these posts than you probably realize).

That is why I was just posting links earlier and not trying to explain or defend the information in them.

Maybe this example will help. I could post links from the EPA about climate change. I trust that the EPA is a reasonably reliable source. But if someone said that they don't trust the EPA at all, and they asked me to defend the EPA's work, asked me to prove that climate change is valid, then I'd bow out of the discussion.

I hope that makes sense.