October is Dysautonomia Awareness Month. I’m not going to write a post for every awareness month or day that exists but I think it is particularly important to raise awareness of Dysautonomia within the ME community. This is because nearly everyone with ME has Orthostatic Intolerance and around a 1/3 of people with me (pwme) have POTS which is a form of dysautonomia and yet others have NMH. Now that is all very well to say, but if you’re reading this with no prior awareness you probably don’t know what these terms mean or why I think you should be aware of them! Here is a brief explanation, adjusting definitions from Google:
Dysautonomia (or autonomic dysfunction, autonomic neuropathy) is an umbrella term for various conditions in which the autonomic nervous system (ANS) does not work correctly. An easy way to think of this is that something is going wrong with some of the processes that are meant to happen automatically, without conscious thought, like heart rate and blood pressure.
Orthostatic intolerance (OI) is the development of symptoms when standing upright which are relieved when reclining. There are many types of orthostatic intolerance. OI can be a subcategory of dysautonomia, a disorder of the autonomic nervous system occurring when an individual stands up. I’ve read that 97% pwme have this, although I don’t know how credible that figure is.
Postural tachycardia syndrome (POTS, also known as postural orthostatic tachycardia syndrome) is a condition in which a change from the supine position to an upright position causes an abnormally large increase in heart rate, called tachycardia. There are also many other symptoms which overlap with ME whether or not you have POTS as well.
Neurally Mediated Hypotension (NMH) (also called Neurally Mediated Syncope (NMS), previously called Neurocardiogenic Syncope (NCS)), is a chronic condition characterized by peripheral vasodilation (blood pooling in the extremities), a decrease in blood pressure, and a slow or lowered heart rate upon standing.
I’ve chronicled my own recent diagnostic journey with POTS on this blog (see this link). The reason that I’m evangelical about raising awareness of dysautonomia within the ME community is that the doctor who diagnosed my POTS in December 2014 said that when it goes together with CFS/ME/Fibromyalgia they usually had the same trigger, which he thought in my case was glandular fever. I had glandular fever in January 1998. Potentially I had undiagnosed POTS from 1998 until 2014.
The POTS symptoms had been lumped into ME in my mind (and my doctors’) in a category of “badly understood and almost no treatment”. I even knew that people with ME got POTS but I had the mistaken idea that it involved fainting a lot (only 1/3 people with POTS ever faint) so when I saw other people online writing about it I dismissed it as not relevant to me. In hindsight this is very frustrating as there are treatments and lifestyle adjustments to manage POTS. It is generally better understood than other aspects of ME. Nancy Klimas reckons that the POTS subgroup has the best prognosis.
Starting from the assumption that this maybe something your doctor has missed, how can you recognise POTS, get it officially recognised and then get it managed?
The Gold Standard diagnostic procedure for POTS is the Tilt Table Test. You get strapped to a tilting bed and they measure your heart rate and blood pressure lying down and then they tilt the bed up and take the readings for a while. When I did it my heart rate went to 137bpm pretty quickly, my legs felt heavy like concrete (I get this a lot in everyday life) and then my legs gave way but I was strapped to the table so that was OK.
You can do a stand in for this at home or in your GP’s office. It is called the Poor Man’s Tilt Table Test. Use this form (which also has some instructions). If you have something that can measure pulse and blood pressure that is ideal, otherwise start by just measuring your pulse by hand. In POTS you’d be looking for an increase in heart rate of 30bpm or more or 120bpm+ while standing. If you’re measuring your blood pressure as well, it usually stays about the same in POTS, but if it does anything weird it would be worth noting down and taking the results to your doctor in case it’s one of the other dysautonomias.
It is worth noting that POTS isn’t just the heart rate increase it also comes with symptoms
“patients can experience lightheadedness, intolerance of exercise, fatigue, visual blurring, weakness, imbalance, headaches, shakiness, clamminess, anxiety, shortness of breath, and the same type of mental fogginess that those with NMH describe…
Symptoms of NMH and POTS usually are triggered in the following settings:
with quiet upright posture (such as standing in line, standing in a shower, or even sitting at a desk for long periods),
after being in a warm environment (such as in hot summer weather, a hot crowded room, a hot shower or bath),
immediately after exercise,
after emotionally stressful events (seeing blood or gory scenes, being scared or anxious).
in some people, after eating, when blood flow shifts to the intestines during digestion.
if fluid and salt intake are inadequate” Peter C. Rowe
If you recognise your experience and have the heart rate increase it would be worth taking this information to your GP. You may need to be prepared for them either not knowing that POTS exists or thinking that in your case it would all be down to deconditioning (even if it was deconditioning it would still now need to be treated). It is probably worth printing out information and having it with you in case they look at you blankly. For example this page from POTS UK is written for GPs (but note that it recommends graded exercise as one of the management strategies, fortunately my GP knew that wasn’t appropriate for me).
Before I went to my GP I kept a diary for a couple of weeks of my pulse and symptoms standing up. This was useful because in the appointment my pulse was high (120 sitting) but didn’t increase by 30bpm, which I could show was unusual, and this got me to the cardiologist. This paper out last month shows that ME “good” and bad days interfere with POTS so a diary record can come in handy. When the cardiologist saw me it was 120 sitting and 150 standing so my GP was right to take my record seriously (he simply checked by hand, no fancy devices). He was prepared to treat me straight away from that but I did the tilt table and other tests later to confirm.
Treatment and Management
If it is relevant, hopefully your GP will refer you to a specialist. This will probably be a cardiologist (this is a list of UK doctors with an interest in POTS). In the months of waiting you might want to try some of the lifestyle management techniques. Talk these through with your GP:
increasing salt and trying non-sugary electrolyte balancing drinks (not if you get high blood pressure)
drinking more, staying hydrated (the salt and liquid combined helps to increase blood volume)
compression or support tights/stocks/stockings to constrict blood vessels from the outside
lying down exercise like pilates, yoga, swimming, air cycling (if your ME can take it, be very cautious)
standing briefly but frequently if deconditioning may have been an issue
experimenting with diet and noticing symptoms eg try smaller, frequent meals, less carbs, less sugar, caffeine?
sitting and lying down as much as you need to, adapting standing tasks to sitting
fidgetting while standing or sitting upright
elevating legs while sitting if you get sitting symptoms (foot stool)
When you get to the specialist even if they think it is POTS they will probably do tests for other things to be on the safe side. They are likely to prescribe you medication which is a bit of trial and error but some people get very good results (I’ve read stories of people returning to work with the right medication tweak, not something you really hear with ME in general). I’ve been prescribed Bisoprolol (a beta blocker which is heart specific and slows heart rate) and I noticed a wider range of symptoms ease than I’d expected, within a few hours. Unfortunately the effect seemed to wear off after a few months and getting an appointment is taking ages. I know there are several different avenues to try such as medication that constricts blood vessels, or increases fluid retention. I’m not an expert on these but a good source of information is this guide by Peter Rowe (page 14 onwards). Getting “concrete legs” is a major problem in my life so I expect vasoconstriction is a good bet.
Discovering POTS hasn’t led me to a miracle cure or ended frustration with the medical system. It is hard to get specialist appointments and GPs don’t have the expertise to adjust the medication. I saw on Facebook that people are getting follow up appointment letters now for December 2016. There needs to be better medical education on this so that more primary care doctors recognise it and feel able to manage it.
Why the Overlap?
Science Bit alert!
The overlap between ME and POTS is more than a little confusing and I’ve written about that elsewhere. Something that came up last week out of the Rituximab research may explain it a little though (further research required):
“We provide evidence that 29.5% of patients with CFS had elevated antibodies against one or more M acetylcholine and ß adrenergic receptors which are potential biomarkers for response to B-cell depleting therapy. The association of autoantibodies with immune markers suggests that they activate B and T cells expressing ß adrenergic and M acetylcholine receptors. Dysregulation of acetylcholine and adrenergic signalling could also explain various clinical symptoms of CFS“. Rituximab research
“Epinephrine (adrenaline) reacts with both α- and β-adrenoreceptors, causing vasoconstriction and vasodilation, respectively. Although α receptors are less sensitive to epinephrine, when activated, they override the vasodilation mediated by β-adrenoreceptors because there are more peripheral α1 receptors than β-adrenoreceptors. The result is that high levels of circulating epinephrine cause vasoconstriction. At lower levels of circulating epinephrine, β-adrenoreceptor stimulation dominates, producing vasodilation followed by decrease of peripheral vascular resistance.” https://en.wikipedia.org/wiki/Adrenergic_receptor
I think this means that these autoantibodies dysregulate adrenergic stuff which leads to inappropriate blood vessel constriction and dilation like you see in dysautonomia. It is promising for those of us with these symptoms, because we may be Rituximab responders.
Last year similar results of autoantibodies directed toward the β2-adrenergic receptor (β2AR) and M3 muscarinic receptor (M3R) were found in POTS patients (not looking at ME/CFS):
“POTS occurs frequently, but not exclusively, in younger females and its onset is occasionally preceded by or associated with a viral-like illness. It is more than a minor annoyance for most patients and leads to significant life changes and limitations in normal life. Our present study has produced data supporting the idea that production of autoantibodies, circulating proteins that normally fight such infections, have instead interacted with critical site(s) on specialized cell membrane proteins which alter their normal cell function.
These autoantibodies interfere with the system which controls the ability of blood vessels to constrict, which is needed to prevent a drop of blood pressure as a person stands. In POTS patients, this inadequate response to standing leads to a generalized increase of activity in the body’s sympathetic nerve system, which frequently normalizes the blood pressure. This increased nerve activity, however, increases the heart rate which is a prominent symptom in POTS.
We have also discovered a second group of autoantibodies in some POTS patients which directly increase the heart rate.
The combination of these two autoantibodies appears to cause the abnormal heart rate response observed in all 14 POTS patients we have tested to date for these autoantibodies… These autoantibodies may explain why beta blockers aren’t always effective in treating the tachycardia seen in POTS, since beta blockers fail to completely block autoantibody activity on their protein receptor and they fail to alter the partial blockade of the autoantibodies on the arteriole blood vessels that initiate the orthostatic problem.” Autoimmune Basis for Postural Tachycardia Syndrome
I urge everyone with ME to do a quick pulse checking version of the tilt table test at home, to see if POTS may be relevant. It will only take a few minutes and may open a range of new ways to feel better. Please let me what you find out, either way.
It would also be great if you could share this message with everyone you know who has ME. Too many people have undiagnosed POTS and other Dysautonomias.