Hi all,
I've read that mast cell disorders can be associated with low or high BP.  

When I feel funny, my BP is normal/below normal while supine, then gets really high upon sitting (150/100 to 210/110) then will remain high on standing until it plummets (lowest caught 88/66).  I carry a diagnosis of orthostatic hypotension and autonomic neuropathy, but wondering if this sounds familiar.  Could it be related to mast cells degranulation?

Thanks for your responses.  
Lyn

Mast cell disorders are just one possible cause of autonomic dysfunction.  A few others that I can think of off the top of my head... collagen and/or vascular disorders (like Ehlers-Danlos), de-conditioning, PTSD, chronic stress, and "pure autonomic failure" (PAF).

Doozlygirl... do you have other symptoms (besides fluctuations in your blood pressure) that make you think you have a mast cell disorder?  Have you experimented with antihistamines and found them useful?

Heather

Thanks for your input.  

Joan, you reminded me that need to tie up one loose end with carcinoid.  Had the 5 HIAA but years ago, but not during symtoms.  :(

Star, Been ruling out endocrine and neurologic causes for years --porphyria,diabetes, Addison's, Cushings, Pheochromocytoma, Pituitary Adenomas, insulinoma etc.  PAF and MSA have been discussed, but since my symptoms come in episodes, doesn't seem to match.  I understand there is no real testing for those disorders, just monitoring of progressive symtoms, and after 5 years one of those labels may be assigned.   I've had facial flushing/neck blotching for decades, anaphylaxis and syncope from fragrance/church insense and can't go near the detergent ailse.  I have taken antihistamines in the past, even had a allergist prescribe gastrochrom years ago with success.  Resumed antihistamines and was amazed with a terrible headache was aborted with zantac.  :)  A Mast cell disorder really could explain many oddities in my health.  I have also been reading up on connective tissue involvement, as I had a cavernous hemangioma of my skull removed 11 years ago, and have a significant family history of multiple types of aneurysm.  

Ana, I've got that article.  Very interesting.  My TTT, QSART, TST pointed to OH due to AN and not POTS.   It is very time-consuming and difficult to unravel all these symptoms and see which ones trigger other symptoms.

Take care, Lyn      

What is AN?  I've got the others, but that one I couldn't figure out!

Ana,
Sorry I missed your questions the first time and hope you see this now. My first clue to autonomic dysfunction was my variable HR.  Before my TTT, QSART, TST, I thought for sure I had POTS, because I was regularly having postural HR increases over 30.  Three and a half weeks prior to my TTT, I had a UAE for fibroid to treat my 2 year HX metamenorrhagia.  Murphy's Law for me meant some odd complication was bound to happen.  I had a progressing orthostatic HA later determined to be caused by a slow CSF leak s/p epidural requiring a blood patch about 2 weeks after the UAE. I was likely hypovolemic and my neurologist believes the CSF leak triggered my autonomic dysfunction to come out of hiding.  I had Rad RNs document my orthostatic BPs/HR post my blood patch which lead my endo to order ASAP autonomic testing, and due to a cancellation, I got in the following week.  

During my TTT, my Valsalva ratio did show excessive drop of 37 in phase II early.  Upon standing, my BP dropped 28-34 mm that never recovered for the 10 minutes of standing before the test was aborted due to symptoms.   This landed me the orthostatic hypotension diagnosis.  My HR increase was modest, not more than 20.  My QSART and TST were both abnormal.   My report was read out as sudomotor and cardiovascular autonomic neuropathy involving the sympathetic and parasympathetic fibers.  

In the beginning, I trialed Zabeta, a betablocker.  Told to take 1/4 of pill for a day and taper up.  By the 3rd or 4th day, my typical resting 120 HR converted to HR in the 40s.  Now know that betablockers are mast cell degranulaters.  I hated being bradycardic. I was bedridden for months.  

My BP is high upon sitting, then once I stand will drop within the first minute or so.  I do get a exaggerated HR increase when I am flaring.  Hadn't considered that I could meet POTS 30 BPM criteria during flares.  Is there such a thing as situational POTS? Docs don't know what to do with variable BP.  Can't tolerate Verapamil either.  Now that I am thinking in terms of mast cell, I should get out my BP cuff again...

Sorry Joan, yeah, AN is Autonomic Neuropathy.  I get so tired of typing out my diagnosis.....      

Interesting point Joan. I have read both in various places and just know to stay away from them. Did a quick check on emedicine:

"Administration of beta-blockers is contraindicated in patients with systemic mastocytosis (systemic mast cell disease) who are undergoing surgery, because these agents may interfere with endogenous epinephrine and may precipitate anaphylaxis."

For what it's worth. I'd need to find some good articles I remember perusing at some point but don't have the energy now, maybe someone else has them?

Wow, Lyn, what a confusing and complicated situation. I'm glad you had all of the appropriate testing. But what has it added like you said? Who knows!

One thing that crossed my mind, was the TTT etc done soon after the blood patch UAE debacle? (SO sorry about all of that by the way!!!). Could it have thrown off some results? Even if it missed things and you are POTS or OH or whatever, I'm not sure it makes much of a treatment difference at this point it's more out of interest? Or, do you think they would offer you something different? If it would make a difference maybe rechecking would be worth it. You could try a poor man's TTT. Yes I agree having a BP cuff is a good idea. I have an OMRON one and like it. I try to check it at the same time each day and when I flare.

Have you tried fluid and or salt loading? I'm sure you have.

Whether the Beta blockers were mast cell aggravating for you, or whether you are more sensitive to them/aggravated your underlying hypotension so much I think doesn't make too much of a difference for you as you should probably avoid them.

As far as situational POTS, I know my orthostatic tachy gets SO MUCH worse when I have a fare. After trying Florinef, I flushed head to toe for weeks and my resting heart rate was 100-120 bpm with 150 + standing and severe tachy after meals for about 4-6 weeks. It was NOT FUN. I try to remember that when my current level of tachy annoys me. It seems once I get out of the flare I get back to this bizarre baseline where I still flush with eating and have constant orthostatic tachy but not as severe

Long post, oops!

Ana

Joan,
Been looking for references to betablockers and mast cell issues, outside of blocking epi pathways.  This is not the one I was looking for, but stumbled across this tonight.  It is from the DINET website under betablockers under the What Helps? Secton.  

Beta blockers should be used with caution, if at all, in those with mast-cell activation disorders (Shibao, Arzubiaga, Roberts, Raj, Black, Harris & Biaggioni, 2005). Beta blockers may trigger mast-cell activation.
In case you don't recognize them, Roberts, Raj and Bioggioni are leading physcians on autonomic dysfunction at Vandy in Nashville.

Will continue to find that reference I am looking for.  Thanks, Lyn    


Ana,
Can I ask for you to elaborate your thoughts that my blood patch/CSF leak may have had on my TTT?  That was 2 years ago, and I haven't once questioned it. I am so intrigued by your perspective.  My UAE was March 10, Blood patch around March 22, EP consult March 30, who ran 3 week Holter, EKG, and labs, the deferred and TTT, since it was already ordered by endo to be done the next week with a QSART and TST on April 5.  Is the blood patch or my likely hypovolemia the curious part?  

Fluid and salt loading - not as effective as I would have liked.  My BP is pretty typical during a flare, but essentially normal while laying, then really high at sitting and drops with standing.    Out side of a flare, it is either normal or borderline high (110/60 to to 139/89).  

Lyn