~elizabeth~

Sorry, 400 mcg a day (so .4mg) (My usual waking bp is around 85/45 too). Having done some reading, I think Clonidine is helping the erythromelaglia not because of its effects on alpha receptors as a constrictor, but because of the nature of sympathetically-maintained neuropathic pain. It seems that damaged sensory C fibres have alpha receptors which can be excited by norepinephrine, so presumably its the central sympatholytic effect of the clonidine that is inhibiting the neuropathic pain process if I've understood correctly. It has certainly helped reduce the generalised dysaesthesia sensations in my face, even though it doesn't help so much with bad flares of flushing/burning set off by heat/posture/hormonal triggers, so it must be having some effect on sensory nerves in addition to autonomic ones that control vasoconstriction. Increasing the dose of clonidine has had more effect on unpleasant dysaesthesias than taking Pregabalin did, even at a high dose. However, I've also read that cognitive impairment associated with dysautonomia can be related to low levels of acetylcholine. I know Clonidine must be a powerfully anticholingergic given the severity of dry mouth/eye, gastroparesis etc side effects. Looking up common symptoms of anticholinergic drugs I find all the things I've been experiencing recently, including eye focussing problems, double vision, brain fog/confusion etc. That said, I'm sure it isn't the whole problem, I certainly had some degree of parasympathetic system failure and intermittent brain foggy symptoms before starting clonidine, so it's possible the underlying problem is just getting worse, and that the clonidine and other drugs like pregabalin are just exaggerating pre-existing symptoms. I don't think taking anything that upregulates acetylcholine would be a viable option, when I tried pilocarpine for my dry mouth/eyes I had terrible pain/swelling in my mouth and tongue, which I now realise must have been oral erythromelalgia as there are muscarinic receptors on blood vessels which dilate when stimulated by ACh or drugs such as pilocarpine. I'd guess that a drug like Mestinon would do just the same.

Eating (esp. large meals) raises core body temperature and this tends to rise through the day (then fall later in the evening), so if your autonomic system is is poor at regulating temperature, eating can be enough to trigger flushing (or sweating) in response. I know in my case this is the cause, nothing to do with mast cell issues or eating histamene containing foods etc. If your veins have a tendency to be dilated (e.g. due to EDS) then it only takes subtle changes in core or ambient temperature to push them into overdrive. I think mast cell issues are over estimated, heat regulation, either secondary to loss of sweat glands that accompanies autonomic neuropathy, effect of raised NE on hypothalamus or other neurological issues are probably behind a lot of autonomic-related flushing.

I have been taking clonidine for about 18 months. I was put on it by a dermatologist, back in the happy days when I was misdiagnosed with self-limiting 'vascular rosacea', since upgraded as erythromelalgia secondary to autonomic neuropathy. I take between 200 and 400 mg a day, this is the only drug that helps with my facial flushing/burning. I had noticed that I had POTS before I knew anything about autonomic problems EDS, and before started clonidine, and I have found it helps with reducing heart rate. About 4-5 months after starting clonidine, I started to feel dizzy and fell over a couple of times, and this began to get worse in the early months of this year, I started to feel very weak with balance issues. Salt/fluid loading did initially help with this, but then things got worse again when I was put on a very high dose of pregabalin for unbearable erythromelalgia pain. Over the course of time, I've just got weaker and weaker, with increasing brain fog, and now worse balance and vision problems. I'm gradually decreasing the pregabalin, as my first thought was that the symptoms were side effects of this, but things haven't improved. My second thought is that the clonidine may not be helping. Although the main benefit of the drug for me is the effect it has on vascular tone as an alpha 2 agonist, I wonder if its other qualities of lowering circulating norepinephrine levels might be worsening my orthostatic problems, impeding sympathetic messaging in eyes etc. Equally, it could all just be down to worsening neurological function secondary to the EDS. It's a bit academic, as I can't stop taking the clonidine due to my horrific face problem.

Ditto Kelly, I have terrible hot/cold/numb sensations in my feet if I wear them, I've tried different strengths. Even the strong ones do nothing to make me feel better or raise my BP (despite having significant signs of pooling in my legs).

Hi, I have a question about Midodrine. I read on the list of side effects that midodrine can cause facial flushing. I thought that this drug was supposed to cause peripheral vasoconstriction, so how is it that it can cause vasodilation in the face? Surely it should prevent this, rather than worsen it? The reason I ask is that I have facial erythromelalgia, and am worried that I won't be able to take midodrine as any thing that makes the face flush causes me severe pain. I've tried salt/fluid loading and fludrocortisone, but they aren't helping to raise my BP or make me feel any better, plus they give me peripheral oedema if I go any higher.

Presumably increased cardiac output/flow is what happens when excessive vasoconstriction is uncontrolled over the long term, leading eventually to this form of heart failure. I'd guess that EDS or dysautonomia could also be added to the list of conditions that underlie this: High output cardiac failure http://qjmed.oxfordjournals.org/content/102/4/235.full I'm still waiting for the result of my echocardiogram, but I'm feeling increasingly worried that this might be my problem.

All I could find was this: Clinical and Physiological Effects of an Acute -1Adrenergic Agonist and a -1 Adrenergic Antagonist inChronic Orthostatic Intolerance

Sorry, I don't understand the last comment. I have EDS, is that known to be associated with high-flow POTS? I don't know anyone else with EDS who has facial erythromelalgia. They don't do QSART tests in the UK, I'm waiting for results of autonomic testing done using these techniques: Circulatory autonomic testing I'm more interested in how this might affect possible treatment options as I've either failed to tolerate them in the past, or fear they'll make my face worse as most seem to include 'flushing' as a side effect.

Probably more that they are both caused by the inability of the autonomic system control blood flow / constriction of veins.

Someone just posted an article on another forum about high/low/normal flow POTS. Only last week I saw a neurosurgeon about my neck problems but ended up talking about my autonomic problems. He said my facial erythromelalgia was probably due to peripheral vasodilation combined with increased cardiac output. Reading about high-flow POTS, this is exactly what he was describing. I can't find any information about high flow POTS and what the implications might be for treatment. I'm very worried as I think most of the usual POTS medications might make my erythromelalgia worse (which has been the case with SSRIs, SNRI, betablockers, alphablockers and drugs like pseudephedrine). Clonidine is the only thing that has helped a bit, but is making my blood pressure go too low at the dose I need to take it to help my face.

I think they are all related to a lack of oxygen in the blood due to low BP. The arm burning could be a variant of coat hanger pain, which mainly happens in shoulders but can cause burning pain in arms (particularly when raised) or calves. I reached to switch off a light this week and one simple movement triggered terrible pain in my shoulders, back and arm. I'm having awful problems with the 'floor swimming', again I think it's due to reduced blood flow to the brain affecting vision and balance nerves. I've been having terrible problems with double vision at the same time. When I checked my BP I found it has dropped significantly since I last measured (85/45 etc) it so I'm sure the onset of these problems is linked to that. More on coat hanger pain http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1296807/?page=1

I have been experiencing similar numbnesses since my problems started 2 years ago, including my lips and cheek. When I started taking clonidine a year ago, I had a lot of odd sensations in my fingers and toes which I blamed on the drug, particularly first thing in the morning.. Then I started losing the sensation in my outer fingers on both hands when I sat down. When my whole arm went numb a few weeks back, I had a brain/cervical mri performed. Unfortunately this showed that I have degenerative disc changes in my neck, and a prolapsed disc with an osteophytic arthritis growth. Earlier this year I was diagnosed as having EDS III, which I hadn't really looked into at all since then. I'm still a bit in shock about all of this to be honest. It seems that most people with EDS have all sorts of numbness issues and no one seems to be able to decide if they are down to entrapment, vascular issues or just nerve damage that seems to go along with the condition. It's funny, not that many people on here seem to have a diagnosis of EDS with their autonomic issues, but in the UK most people with POTS do seem to turn out to be hypermobile, perhaps because the doctors who diagnose POTS seem to have a good awareness of the connection between the two conditions.

Facial erythromelalgia, which also affects my eyes.

I am interested in mestinon as I have severe dry mouth and eyes as well as POTs and other autonomic problems. Recently I've been suffering severe muscle weakness and eyelid drooping/double vision which I suspect are connected. In the past I have tried pilocarpine for dry mouth but found it gave me intense mouth burning, similar to the erythromelalgia pain I get in my face. Given that mestinon is also a parasympathomimetic agent, does this mean it would also cause the same oral burning and worsen the facial erythromelalgia?

Earlier this year I was diagnosed with suspected POTS and facial erythromelalgia after seeing Professor Mathias. I'm currently awaiting the results of autonomic testing, I'm hoping that I can soon start some treatment for my problems but I'm worried I won't be able to tolerate some of the medications because they might worsen my facial erythromelalgia. I notice that flushing is given as a side effect for drugs like midodrine and mestinon. Has anyone else with erythromelalgia any experience of how these drugs might affect the condition? Initially I'd hoped that drugs that help with vasoconstriction might also help calm my face, but having heard they cause flushing as a side effect in others I'm worried I'll be extremely limited on treatment options for my POTs/low BP. Having read that vasoactive peptides might be involved in the mechanisms of erythromelalgia, I wondered if Octreotide might be a more useful drug to help treat my POTS and also the EM. Does anyone here have any experience of how this drug might affect facial flushing. I've only heard of one doctor who prescribes this drug in the UK, Dr Glyn Thomas, does anyone have any experience of seeing him? I take 200mcg clonidine for my flushing but it isn't really an adequate treatment, and I've tried every sort of neuropathic and conventional painkiller without any relief. My skin feels as if it had been scalded, and the skin peels off just like a real burn. The problem is much worse when I'm lying down, the only thing that relieves it is walking for about an hour which I've been told is due merely the effect of gravity reducing the blood pooling in my face.

Re Wilson's: The American Thyroid Association (ATA) describes Wilson's syndrome as at odds with established knowledge of thyroid function. The ATA described the diagnostic criteria for Wilson's syndrome as imprecise and non-specific, and found a lack of any scientific evidence supporting Wilson's claims. The ATA further raised concern that the proposed treatments were potentially harmful.[2] Florida State Medical Board members described Wilson's syndrome as a "phony syndrome" and a scam during disciplinary action against Wilson,[3][4] while Quackwatch has stated it is "bogus diagnosis".[5] http://en.wikipedia.org/wiki/Wilson's_temperature_syndrome

I wouldn't want to say that it's not possible to have a condition that closely mimics thyroid disease with normal hormone levels, but autonomic problems can also account for all these symptoms, including the poor thermostatic control. Knowing what I do about POTS, all my unresolved 'thyroid' problems fall entirely within this spectrum I just wish I'd found out about it 20 years ago. As far is I know, very few endos think T3 is a good idea, it seems very controversial and having tried it I'm not sure I'm convinced either, my POTs type symptoms just got worse over the time I was taking it. The Endo who prescribed it for me believes that any effect it has is largely due to the brain not converting T4 as efficiently as other tissues, but that apart from this sole possible cognitive benefit, it doesn't help with any of the other problems people complain about with thyroid disease, probably because they might be caused by autonomic failure or additional auto-immune activity.

I've only just discovered that the problems I've been suffering for over 20 years are probably due to POTs, which I didn't know existed until someone recognised the symptoms I was describing on the erythromelalgia board. I've had symptoms of autonomic problems since my teens (very low body temperature, almost complete inability to sweat, tinnitus, allodynia and chronic neural ear pain, numb extremities, periods of uncontrollable diarrhoea followed by extreme constipation, racing/irregular pulse, disturbed menstrual cycles, virtually every symptom on the list). Until recently I'd assumed it was all thyroid-related. I also have a diagnosis of undifferentiated connective tissue disease. Last year I had a really bad attack of all of these symptoms. My right leg went completely numb, purple with muscle claudification, had breathing problems, non-stop diarrhoea and terrible tachycardia with angina pain. At the same time my face started flushing/burning with change of posture from vertical to horizontal. This for me is the real problem, as the scalding pain is unbearable. After going to various dermatologists and trying all the usual rosacea treatments, we've now ended back at square one. Alphablockers didn't do much, betablockers actively made ALL symptoms worse, high-dose gabapentin and morphine have made no impact on the pain. The only thing that stops it is standing up for as long as possible, whereupon the problems shift to the legs. Once I lie down again, the burning and redness gradually shift back to the face. The only thing that seemed to normalise it temporarily was when I was on a drip for a few hours over this weekend, having gone up the hospital as I just couldn't stand the symptoms any more. I'm due to see Professor Mathias at St Mary's hospital (the UK expert on these things) but I'm not sure if he'll be able to help with the face flushing problem as it's not a standard POTs symptom. I'm fairly sure it isn't a mast cell problem, as anti-histamenes have zero effect on it. I have a feeling that it will improve if I can sort out the circulation. I've tried taking salt/fluids but they caused really bad diarrhoea which probably just made things worse. At the moment, I can't really stay upright for more than about an hour without feeling really dizzy/faint; I can only walk with compression stockings otherwise the foot/leg sensations are really bad, and my heart rate goes up by at least 30bpm when I stand up along with diastolic BP, the drip over the weekend made no difference to this at all.

The symptoms of thyroid disease are virtually the same as POTS, it's very difficult to distinguish between them. In theory thyroid-related symptoms should resolve with hormone replacement, but for many they don't, I'd guess because it has already caused some autonomic nerve damage. I don't see how anyone could be confused as to whether they have autoimmune thyroiditis or not. If you have tested positive for anti-thyroid antibodies, you have Hashimoto's thyroiditis, doesn't matter what your TSH or T4 readings are. I think Thyroid antibodies in your system are a bad idea, they might not only destroy the thyroid but might attach to other tissues with thyrotropin receptors, such as the eye. In my opinion the only way to get rid of them is to take thyroxine and get your TSH as low as possible without completely suppressing it. I think that most doctors don't understand any of this, they only look at hormone levels without the least bit of understanding of the autoimmune and neurological elements of thyroid disease. I think Armour might not be a very good idea at all, and I'd be extremely cautious about taking any form of T3 with tachycardia problem, it might make it worse and could cause quite bad angina pain. I think that even endos who believe that T3 is beneficial don't recommend Armour, as the proportion of T4 to T3 are wrong. Also, as far as i know armour is essentially pig thyrotropin, the substance which your bodies makes anti-bodies against so I think it can reactivate thyroid antibody activity. If you need to suppress your thyroid completely, I think it's probably better to take a measured amount of synthetic T3 and leave armour well alone. I was taking 5mcg of T3 as a supplement to 100mcg T4 for what I thought were thyroid problems but which I now realise were due to POTS. It worked to some extent, overstimulating the metabolism probably does help the brain fog/energy stuff but possibly has been making the tachycardia/POTs issues worse.

I've been taking piriton for years when I had feelings of being highly adrenalised, it was the only thing that stopped the violent diarrhoea/shivering fits/racing pulse. I now find out that piriton/chlorphenamine inhibits norepinephrine uptake in addition to having a sedative effect, maybe benadryl has similar properties. I've never found any other AHs had the slightest effect on me at all, so I guess the benefit I've been having from piriton was largely from the SNRI effect.