Hyperpots With Supine Hypotension

[~elizabeth~]

I finally had the results of autonomic tests back this week.

To summarise, my problem is intense facial flushing on sitting or lying down. The problem isn't MCAS, so please don't try to initiate any discussion on this, it was initially thought to be, but then dismissed by the immunologist after severe reactions to MCAS drugs, and later completely dismissed by the leading authority on mast cell disease in the UK. Tests for neuroendocrine tumours have all been negative (5-HIAA, metanephrines, chromagranin A and B )

The autonomic test results showed that I have supine NE levels right at the top of the normal range which 'rose significantly' on upright tilt (I don't have the exact figures as the news was given as a phone consultation, but to me this sounds like what would be hyperadrenergic state outside of the UK, where the term doesn't seem to be used). This was what we were expecting to be honest, but it appears that despite some high surges, my BP is still very low overall, particularly at night. Apparently the tachycardia continued throughout the night, ranging from 90-114 through sleeping hours. I also had unusually severe changes in skin colour/perfusion during my 1 hr TTT.

I guess this is consistent with my neurologist's opinion that the flushing disorder is erythromelalgia. I'm broadly with him that it's something in that area, but I think the picture is more complicated. My nose and ears flush immediately on lying down, I think that the problem is actually primarily a Raynaud's-type disorder, pale while standing but hyperemia on release of sympathetic tone/lowered NE levels. The flushing on the cheeks is different, it was worst while I was on clonidine (which would have been significantly worsening the supine hypotension, it also made me bradycardic) or other medications that lower BP, so I think it may be some reaction to low oxygen/perfusion during the night when it happens, as apparently apnea can cause facial flushing (sleep studies have been suggested, although my specialist doesn't think it is true apnea).

My specialist has prescribed midodrine, as he says he's found it's helped people with EM in the feet, and I have painful, red feet after walking too. I'm only on 2.5mg t.i,d, , absolutely no symptoms while the drug is active in my system, I feel much better, my legs lighter, warmer, much less agitated. Rhe problem is when it wears off. Almost exactly 3.5 hours after to the minute, I suddenly start shivering, HR starts to go up, my throat is suddenly immensely sore and my throat so tight I can't talk. On lying, the painful nose flushing problem are worse, my mouth/throat/lips are bone dry and I'm twisted in pain from muscle spasms when I wake up. The withdrawal symptoms are very like those I've experience in the past when prescribed tricyclics or SNRIs for the pain, so I'm pretty sure it's due to excessive sympathetic activity during the night.

I was told to stop if I experience any adverse side effects. However, these aren't side effects as such, it's just a worsening of the existing problems. I'm not sure how long to give the midodrine. I don't think it's going to be a helpful drug for me unless some way of managing the peripheral symptoms can be found. I haven't taken the midodrine later than 6 pm, and my BP hasn't been high later in the evening.

Has anyone else experienced these rebound problems after a dose of midodrine wears off? If so, is it something that lessens with time? If it's not, I want to be pursuing other treatment options for my excruciating face pain/flushing as soon as possible.

Last year I had a large, painful ulcer that stayed open for over 6 months, consistent with the kind that people suffering severe Raynaud's get, which left a huge scar when it finally healed when the weather warmed up. I've also had sores develop on my face, so I'm desperate I don't develop anything like this on my face.

[Chaos]

Wow Elizabeth, sounds like you've been thru the ringer. Glad you've been able to rule out several things and sounds like you are working with knowledgeable providers.

I've not had any kind of similar rebound reactions when my midodrine wears off. It seems like you are having pretty sever reactions when it wears off so you might want to let your doctor know about that. Since you are having good effects from the midodrine while it's working, it's too bad there's not something that would provide similar effects with better long term coverage.

Best wishes!

[yogini]

When you say supine hypotension, what is your supine BP? Do you feel better sitting/lying down other than the flushing?

[~elizabeth~]

Unfortunately I don't know, I don't have the full results yet, only what I was told over the phone. No, I usually feel better standing, much as I'd love to just curl up under a duvet, I am better up and moving. This time last year I was having to stand about 20 hours a day, could only tolerate sitting for about 30 mintues, I was having to get up during the night and walk around for 40 minutes to get the flushing/pain to go down. That was on clonidine, things have been better since stopping but every now and again the same thing happens again. Apart from the rise in HR and 'hyper' feeling on standing, it's almost like the opposite of POTS!

No one has ever seen anything like this, I'm seeing the leading autonomic specialist in the UK, he said I'm one of his most challenging cases. I guess my vasculature is so defective I need the excess of norepinephrine I get from standing to maintain normal sympathetic tone and BP.

[~elizabeth~]

I should add that I was put on the clonidine by a dermatologist long before we knew about the EDS/POTS etc. He eventually had me on several different BP lowering drugs simultaneously, which just made matters worse (but did mean that the orthostatic tachycardia got so bad that we spotted there must be an autonomic problem).

[SarahA33]

Hi Elizabeth,

Thought I'd chime in and offer what I could, Sorry to hear that you've had your share of struggles, but your amongst friends here. I experienced ulcer's last year that the dermatologist told me was hand,foot, and mouth disease/Impetigo that honestly made children cry when they looked at me. I was terrifying I ended up showing my hypertension specialist who knew immediately they were amlodipine burns (what he called them) and was successfully sent to and treated by an advanced wound healing specialist. The dermatologist who i saw originally was so wrong.. sooo wrong, as they were actually sores/ulcers caused by the Amlodipine (Norvasc) (Calcium Channel Blocker BP Med).

The main symptoms I had from Midodrine were the goosebumps/temperature control which made my already difficult thermoregulatory issues that much harder to deal with.

The supine hypertension I was getting also made my doctors nervous, but I was noticing benefits from the midodrine and i didnt want to stop. I rearranged my dosing schedule so that my last dose of midodrine as 4- 6 hours prior to laying down instead of the universal recommendatiom of 4. We also increased the clonidine by 1/2 of .01 Just thought of something... do you measure your vitals at that 3.5 peak when your coming off from the meds? I wonder what your heart rate and BP's usually are and if somehow you might be able to play around with the timing of certain med's to prevent it?

Hang in there

Sarah

[~elizabeth~]

Thanks Sarah, that's really interesting. My idiot dermatologist tried me on labetalol (on top of the clonidine), it instantly made things much worse, I had terrible muscle aching, Raynaud's plus it just worsened the flushing. I had permanent goosebumps before I started midodrine, I guess from the high NE. I don't think I have proper HyperPOTS, my neurologist didn't call it that, although the first thing he brought up was the substantial NE rise. Although my BP rises when I stand by 20-30mmHg, it gradually falls and the 24 hr BP monitor showed that overall it was too low.

Yes, I sat measuring my BP/pulse intermittently as it was wearing off a couple of evenings back. My pulse went up a bit but BP was stable, actually it was rather low both during the peak plasma level and after it wore off and the shivering etc started. I've taken it lying down, but no sign of hypertension then either. I'm only on 2.5mg t.i.d though.

The real problem is the vasospasm in my feet. Since starting the midodrine my feet have been much more painful. Not just the burning I get on the soles when walking (which usually wears off after about 30 minutes) but a dull ache in the plantar fascia region, plus red hot pins and needles sensations which is there pretty much all the time since starting. It seems to be making the non-flushing dysaesthesias in my face worse too.

Antihistamines do nothing at all for me, zantac actually triggers flushing. However, I've found following a strict low amine diet has helped a lot. I found this out by accident (having to omit a lot of high histamine/tyramine foods for urinary 5-HIAA/metanephrine test and then had the most horrendous flushing ever when I went back to normal diet). I don't have MCAS, but I do have low diamine oxidase levels, probably because because the high norepinephrine means my body is having difficulty metabolising dietary amines. My neurologist agrees with this, that what I have is a purely neurological amine intolerance. Betablockers also inhibit diamine oxidase, stopping propranolol has helped me widen my very restricted diet.