What is POTS?

POTS stands for postural orthostatic tachycardia syndrome. In this condition, patients complain when they stand up for a prolonged period of time, they feel very uncomfortable with dizziness, palpitations and tremulousness and therefore they either have to sit or lie down or they risk collapsing. When you examine them, the heart rate can be found to be excessively fast. 

As doctors, sometimes when we can’t explain what is going on, we just take what the patient tells us, give it a fancy technical name and make it a condition. This patient says her heart rate goes up excessively when she is in an upright posture. Let’s call it Postural Orthostatic Tachycardia Syndrome – that’s not really a diagnosis – it’s just a medical jargon filled term for what the patient has just told us. It tells us nothing more than that – but this is the term that we have ended up sticking with! In fact this term does patients a disservice. Because of the name of the condition, many clinicians have incorrectly assumed that it is only a condition that is manifest when the patient is upright or standing up. This is incorrect. I have over a thousand patients with POTS and I have spent a lot of time listening to their stories. All of them say ‘ I feel rubbish all the time – I just feel rubbisher when I am upright’. 

 

What do they mean when they say they feel rubbish all the time?

They are always tired, they have bad brain fog, they have issues with lack of refreshing sleep, they have horrendous gut issues, they have chest pain and breathlessness, they have headaches and they even have bladder symptoms.  Unfortunately, the term POTS does not capture all these other symptoms and therefore I prefer the term dysautonomia which means a disequilibrium between the flight and fight system and the rest and digest system. In essence these patients spend a lot more time in flight and fight mode and very little time in rest and digest mode and therefore they are always simultaneously tired and wired. This is a far more appropriate and accurate name for this condition.

 

How do patients develop POTS? 

Increasingly we are seeing that they often inherit a genetic vulnerability such as Ehler’s Danlos syndrome/joint hypermobility syndrome and then at some point in their lives they get hit by some kind of infection, this vulnerability is unmasked and the patient starts noticing these symptoms. In essence, people are born with a genie in their lamp and then an infection comes along and the genie is unleashed and they then can’t get the genie back in the lamp. The most common infection that I have come across as a trigger for dysautonomias is Glandular fever, However there are other infections that can also trigger dysautonomias including coronaviruses and it is therefore not at all surprising that so many people have developed this condition called LongCOVID which has almost identical symptoms to a dysautonomia like POTS and I would argue that perhaps POTS and Long COVID are indeed the same condition.  

 

Are POTS AND LongCOVID the same condition?

Why do I say this?

  1. Only 10% of patients with COVID develop Long COVID – why? If it were just something about the virus then surely everyone who got COVID would be expected to get long COVID. There must be something about that 10% which makes them more vulnerable.
  2. The severity of COVID does not have a bearing on whether you get Long COVID or not. Well if it was just about the virus then logic would dictate that the more severe the illness, the greater the chance of having Long COVID. We do not see this. Again makes you think that the virus simply flicks the switch in those people who possess that switch.
  3. When you talk to many Long COVID sufferers, they will admit to having some dysautonomic symptoms albeit mildly even before they caught COVID. Many times they had just assumed that those symptoms were normal for them but when they get hit by the infection then they find that all those symptoms which were very mild get so much worse.

 

So it is highly likely that a majority of patients with Long COVID have POTS and the problem is that because the definition of the term POTS is so narrow, patients with Long COVID will be managed as they have a completely separate condition rather than being managed as a post-viral dysautonomia, the way POTS is managed. There are too many doctors who are interested in treating conditions rather than treating patients. This means that many patients with LongCOVID may miss out on lots of helpful treatments which we use in POTS, and just be asked to pace whilst we wait for some fancy American pharmaceutical company to produce a mega-expensive and potentially harmful new drug specifically for Long COVID. I have hundreds of patients with Long COVID and I can categorically say that many of them feel better when they are managed in the same way as I manage my POTS patients.

 

In terms of optimal management for POTS patients, i use 4 approaches:

  1. Lifestyle management
  2. Physiotherapy
  3. Medications
  4. Patient advocacy where the doctor who is interested in the patient helps the patient maintain their identity by advocating for them to access modifications at school and work.

 

You will find a lot more details about these 4 pillars of treatment on other videos on my channel. To be honest, these measures make a difference but they don’t transform patients. I usually see like a 40-50% improvement but patients still remain enfeebled.

Today I wanted to talk about an intervention that in my experience can be transformative for some patients and in my -opinion should be offered more widely than it is.

Today I am going to talk to you about the benefit of regular  intravenous saline infusions in POTS and potentially even in many patients with Long COVID. 

One of the most consistent symptoms in POTS is that patients generally feel worse when they are upright. The one thing that happens when we are upright is that gravity will pull blood towards it and therefore it is more difficult for the blood to get up to the brain which is the furthest organ from the ground. We therefore rely on our legs muscles and the blood vessels in our legs to squeeze and help push the blood upwards. In patients with POTS this does not happen as well and therefore blood pools in the legs and therefore less blood is available for circulation. We also find that this same phenomenon happens when it is warm because when it is warm our blood vessels open up and therefore the leg vessels open up and this encourages more pooling. Similarly many patients will feel worse after a big carbohydrate rich meal because the carbs need more blood to go to the gut and the blood tends to pool in the gut – a phenomenon known as splanchnic pooling. We also know that because of this reduction in circulating volume, the heart has to work with less blood and therefore over a period of time the heart can actually become smaller which means that the heart now is pushing less blood out with each beat  and has to beat faster to get the same amount of blood around. In addition, the legs muscles start getting deconditioning which continues to propagate this vicious cycle. We also know that patients with POTS tend to run low on the hormones that are produced to by the kidneys to help retain water and so not only can’t they circulate it, they even have difficulty holding onto it which is why many patients with POTS will say that they are constantly urinating and many have to undergo investigation for Diabetes insipidus. 

If we can therefore increase the circulating volume, then patients do feel better. The easiest way to do so (at least theoretically) is to ask the patient to drink more and this is why the first recommendation is to ask patients to substantially increase their fluid input to at least 3L of water daily and cut down on diuretics such as soda etc and carb rich meals. Because extra water does not stay in the blood vessels, we usually ask patients to take more salt and electrolytes as these encourage fluid retention in the blood vessels. However despite these measures, patients see only a mild/at most modest benefit. The reasons are many fold.

  1. It is requires a lot of discipline to make sure you are constantly hydrating
  2. The frequent urination is inconvenient,  bothersome and tiring
  3. Many patients struggle with increasing salt intake and electrolytes because these can be unpalatable
  4. Patients with POTS suffer from gut issues so they feel nauseous anyway and get easily bloated and also may have impaired digestion and this may have an impact on absorption
  5. Finally when the water finally gets into the blood vessels, they have difficulty holding onto it

So in some ways, if one could bypass the gut and in some way deliver the fluid with the right concentration of salt directly into the blood vessels and bypass the gut altogether then that would be expected to have quicker and more dramatic effects.

This is where the idea of giving intravenous saline comes in. The problem is that the patient still struggles to hold onto the water for a prolonged period of time therefore you would expect the patient to feel better when they get the fluid and then you would expect them to deteriorate after a few days and this is why intravenous saline infusions have to be given repeatedly.

Is there any evidence that this works?

Well there is an interesting paper by a very prominent physician called Blair Grubb from Toledo which was published in the Journal of interventional cardiac electrophysiology in 2017 called ‘Effects of intravenous saline infusions in patients with medication – refractory postural tachycardia syndrome’. 

They took 57 patients who were already medicated (at least 3 different types) for POTS but were still struggling. They recorded measures of quality of life before initiating intravenous saline infusions regularly (1L every week) via peripheral cannula (or in small numbers through PORTS) and they followed these patients up over the next 3-12 months to see if they reported an improvement in quality of life.

The results showed that only 4 patients of the 57 did not feel that they benefitted. All the rest reported a benefit and the benefit was seen in all domains across the quality of life assessments.  Most patients reported an immediate improvement in symptoms that lasted upto 3 days.  Many patients subsequently found that because they felt so much better they were able to use that improvement to do more physiotherapy, get more conditioned and many were then able to discontinue the IV fluids altogether. More importantly there were no major adverse events from the IV saline. 

This was clearly very encouraging even though this study was a non-blinded observational study rather than a randomised placebo controlled trial which most doctors pay more attention to. Despite these encouraging data, as yet I am unaware of anyone who has done a randomised controlled trial and this is probably because there is no real money to be made from IV saline (which is cheap as chips).

On the basis of this study, one would think that this is a simple and safe intervention to offer those patients who continue to struggle despite lifestyle physio and medications and I have many such patients and so I was keen to explore this option for my patients. Unfortunately I found that it proved far more difficult to convince the NHS gurus that this was worth trying for several reasons.

 

  1. Many doctors don’t know anything about POTS
  2. Many who do know about it dont believe in it
  3. Those who believe in it, fail to understand why just telling the patient to drink more is not an adequate enough intervention
  4. As it is not dangerous, it does not seem to be important enough to address
  5. Many feel that that benefit is simply due to a placebo effect (even though you have to ask whether that really matters – if someone says i don’t enjoy my quality of life and after delivering a cheap, safe intervention, the same person says i feel so much better!)
  6. Finally there were no easy mechanisms within a cash strapped, space-starved, staff-depleted NHS to provide a service.

 

Despite all these challenges, i was keen to see if i could access fluids for some of my patients and my breakthrough came when one of my patients wrote to Mr RIshi Sunak, who was then Chancellor of the Exchequer and her local MP and Mr Sunak wrote back requesting that on humanitarian grounds she be offered IV fluids because she was so incredibly debilitated. Happily my hospital agreed and we started offering some of our patients regular IV saline infusions via peripheral cannula (we do not put ports in because there is a much higher risk of blood clots and infections with something that remains in the patient). I am delighted to say that the vast majority of my patients (about 30 or so) have found this simple intervention to be transformative.  They come once a week, sit in our day case unit and receive IV saline 2L over 4 hours via a peripheral cannula. They then go home and engage with physiotherapy and conditioning work and come back and do it all over again in a week.We have not been able to offer it to more simply because of a shortage of resources but i am hoping that soon we will be able to add to the evidence base and fund more resources.

I wanted to share some feedback from the patients in their own words.

Dear Dr Gupta,

I thought I would write to you now we are a couple of months into my IV treatment.

I can’t believe how much of a difference this treatment is making. I admit I was sceptical at first but having run out of options in my treatment I had no alternative but to give this a try.

To start with I didn’t see much difference but then after a couple of weeks my wife commented that I looked different immediately after receiving treatment, that I looked well and my complexion was more refreshed, my skin was less pale and I had more of a glow about me.

I am able to get more done than I have in the last 6 years on the day of the infusion, I can bare to stand up for longer where usually I would be rushing for a chair or my mobility scooter.

This would last a day but that day is time I can spend with my family instead of being left behind as I was unable to participate.

I noticed a longer effect if I wear my compression socks that I purchased from Amazon (the ones you suggest are way out of my price bracket unfortunately). Ever since I did this I’m able to extend these effects from my treatment into the following day.

Although you only get 1-2 days of lesser symptoms from this treatment, it has made such a difference to me. I very much hope that funding will continue so that I am able to have more of a life instead of being confined to my home. 

 

Dear Dr Gupta,

 H has now finished her once a week, over 4 weeks course of infusions. I have to say I did not expect the infusions to make such a difference to H, but they have!

This therapy has given us glimpses of our daughter back, that we haven’t seen for over 8 years. She has struggled not only with Ehlers Danlos Syndrome, but also with Chronic Fatigue, Brain fog, Auditory delay, Headaches and Difficulty processing speech from others and giving a related answer.  

Therapy has given her so many benefits.  She has not had a headache since starting treatment. For 3 days a week she is animated, chatty and can process conversations correctly.  Her energy levels have increased vastly for 3 days a week she can do hobbies, sit downstairs ( she is normally bed bound) and has now been for days out. This is huge for H and us as a family.

 If H were to continue and possibly have infusions twice a week, she may benefit so much. It would give her more family and friends time. Less time being fatigued and in a state of all consuming brain fog, which makes life so hard for her. She would not feel isolated, in H’s words she feels,”Normal” for those few days. To a disabled person that word is HUGE! To a parent it’s a life line we thought she would never have. Thankyou for this opportunity you have given to H.

 

So as you can see – these are incredibly heartwarming stories and it is a shame that this is a service that is not offered more widely to carefully selected patients. Although one of the reasons is that there are no mechanisms in place to offer this service within existing NHS services, a bigger reason is the attitude of doctors. Doctors these days think, what will happen to me if i try to help this patient when they should be thinking ‘what will happen to this patient if i don’t help them’. My own feeling is that a doctor who is not prepared to put him or herself out of his/her comfort zone, for the sake of the patient then that doctor is not deserving of their title.

I hope this video/blog will empower patients who suffer from dysautonomia, POTS and long COVID to access the care that they truly deserve.

https://youtu.be/retGCkEuE5A

P.S We now have a new website that I have started for patients with POTS. This has lots of free resources you can access. The website is www.potsspecialist.com. If you get a minute to check it out please do and let me know how we can make it better and more useful.