Eds And Mcad

[juliegee]

Hi All,

I've recently joined another support group for folks with mast cell disorders, both mastocytosis and mast cell activation disorder. I've been so struck by the vast number of folks I've run into who have some sort of mast cell disorder AND a connective tissue disorder like EDS, Marfans, etc. Most of them also have POTS. I'm being evaluated for a connective tissue disorder too, currently Loeys-Dietz syndrome (described by my geneticist as in between EDS & Marfans.) Such an odd coincidence. I can't help but think that the two are connected somehow. Maybe the errant mast cells cause leaky veins (leading to huge drops in BP/anaphylaxis and subsequently high HR to counteract) and loose bones and muscles? Wonder if researchers have noticed this trend?

I notice so many of you have EDS and possible mast cell issues. Very strange!

Julie

[iheartcats]

I tried to PM you, but you're full!

I had a question related to allergies/MCAD (which I'm checking into) so guess I'll throw it here if you don't mind.

Have you ever used a Beta Blocker? My EP wants me on one as my tachy is so bad, but I don't know if my allergy meds would still work. If you aren't on any, how do you keep your heart rate under control? Mine is crazy lately.

[juliegee]

Hi Cat Lady,

Have you taken a beta blocker before with good results? They are contraindicated for MCAD. I know you don't know for sure that's what you are dealing with, but since you have so many symptoms, you certainly need to be careful.

I've taken beta blockers with mixed results. The last few times, my allergy symptoms went beserk- daily anaphylaxis AND my epi-pen didn't work (I needed another one!) because of the beta blockers.

My HR is controlled because I control my MCAD. My antihistamines prevent my blood vessels from leaking out, which in turn causes low blood volume, low BP, and the subsequent high HR to counteract all of this.

Make sure your EP understands that MCAD may be causing your POTS. If that's true, beta blockers would NOT be indicated. Calcium channel blockers are somewhat safer, but a MCAD regimen would be the best. Let me know what you decide.

Julie

[flop]

Hi Julie,

it is odd how these things seem to go together. My GP dismissed the POTS/EDS connection and then when I tried to talk to him about allergy issues contributing last week he just said "weird people get weird diagnoses" and wouldn't discuss it further.

I know that there must be a connection between all my symptoms, I refuse to believe that I am just mega unlucky and have several unrelated problems.

Flop

[mkoven]

I think Nazli McDonnell has found an association between allergic-type reactions and eds.

[juliegee]

I think Nazli McDonnell has found an association between allergic-type reactions and eds.

Do you have any research to share or know a place where I can find it? It totally agree with Flop, it is NO coincidence that I have met dozens of folks with connective tissue disorders/allergy-mast cell issues/autonomic abnormalities. I hope somebody is taking notice or i will jump up and down until they do

Julie

[Maxine]

Julie,

This makes a lot of sense, and I think there must be some kind of connection with EDS, ANS dysfunction, and allergic---or autoimmune responces.

I don't know if I have any major allergies, but I'm so sensitive to many things. Do any of you have problems with IV tubing? Is it super weird for me to have a reaction to the plastic? When I had my loop recorder removed my BP went way up to 168/100 when I went to recovery. They have IV fluids, but a really slow drip. As you know from previous posts that I have sensitivities to local anesthetic, so carbocaine was used. I didn't think this caused my problem, as I didn't have a reaction to the carbocaine when my tooth was extracted. However, more was used to remove the loop recorder.

Tylenol is the only thing I use for pain because I react adversely to different drugs more often then not. I was surprised I could handle the ceftin antibiotic I took over the holidays. I also notice a continuous rash on my mid to lower back about 6 inches long around the spine area. My eyes water a lot, and I notice I can't tolerate eye shadow anymore, especially anything metallic.

As far as "allergies" go, it's hard for me to tell, as I don't have the obvious symptoms like rash, runny nose ect. But I know allergies manifest all kinds of different symptoms.

My anti-tpo levels are really high @ 1,600----this is autoimmune thyroid hashemotos.

It's just so hard to believe I would have so many things going on that are NOT connected.

Maxine :0)

[mkoven]

Not sure what she's publsihed yet. In a relatively recent issue of the ednf newsletter, there was a summary of some of her recent findings of things that occur in eds patients--and allergic reactions werethere. Maybe you could see if they have back issues on their site?

[jjb]

My GP dismissed the POTS/EDS connection and then when I tried to talk to him about allergy issues contributing last week he just said "weird people get weird diagnoses" and wouldn't discuss it further.

I know that there must be a connection between all my symptoms, I refuse to believe that I am just mega unlucky and have several unrelated problems.

Flop

Seriously??? Are you still w/ the GP?

I was examined by Nazli McDonnell last year as part of the NIH study. It seemed clear to me that dysautnomia was something that commonly occurred w/ EDS (same w/ chiari).

In fact as part of the EDS study, they test for POTS.

[flop]

Jen,

I had suggested EDS to my GP after finding out about the connection on here - he thought I was just imagining stuff.

I then saw Prof Mathias (neurologist) in London and he was asking me about scars, stretchy skin, gymnastics, bendy joints etc. He actually asked me "do you know why I am asking you this?" my reply was "you're wondering if I've got Ehlers-Danlos syndrome aren't you?". He said he knew that I was one of those patients who reads everything they can about their illness to try and work out how to take care of themselves.

After that apt I saw my local cardiologist who thought the EDS/POTS connection was a load of rubbish too.

After having testing with Prof Mathias and being seen by Prof Grahame (rheumatologist) I was confirmed to have POTS and officially diagnosed with EDS. Once it was in a letter from the consultants my GP has just accepted it (but now gives him reason to dismiss my MCAD suspicions). My cardiologist who was sceptical now is checking all of his POTS patients and sending them to rheumatology and genetics clinics!

In the UK you can't just choose your doctors, mostly you are given a GP at the practice closest to your home and if you need a specialist it is your GP that chooses but it is almost always the one at the nearest hospital. For example I wanted to see an immunologist for my allergies but the GP said he couldn't refer me as there aren't any near us. So he referred my to a local dermatologist for patch testing - the derm said I didn't need patch testing and referred me to an immunologist at a teaching hospital 100 miles away. Crazy system!

Flop

[jjb]

Ah .. sorry about your crazy system.

I recently saw Dr Peter Novak and he was telling me about some dys & eds docs o/ in england.

When I see him again, I can ask him who he was referring to.

Here is a teensy bit about POTs & EDS from Dr Nazli & Franomano:

Postural Orthostatic Tachycardia is an age dependent manifestation of Ehlers-Danlos Syndromes. C. Slemenda1, B.F. Griswold2, L. Sloper2, C.A. Francomano3, N.B. McDonnell2 1) LI, NIA/NIH, Baltimore, MD; 2) LCI, NIA/NIH, Baltimore, MD; 3) GBMC, Baltimore, MD.

Postural Orthostatic Tachycardia (POTS), defined as a heart rate increase greater than thirty beats per minute from supine to standing, has been reported to be associated with joint hypermobility. We studied the prevalence of POTS among 61 consecutive patients with hypermobile and classical forms of Ehlers Danlos syndrome seen at the National Institutes of Health. Supine, sitting, and standing heart rate measurements were obtained for each subject with five minutes of rest between each position. Thirty eight percent (23/61) of the subjects met criteria for POTS. The condition was significantly more common (p<0.001) in patients under the age of 25, with 72% of such patients being affected, as compared to 13.9% of persons over the age of 25. The presence of POTS was associated with a reduction in quality of life, including inability to maintain gainful employment or attend school. The etiology and natural history of POTS in this cohort is not well understood and merits further investigation.

[flop]

Thanks for the info.

I suspect that Prof Novak was referring to Profs Mathias and Grahame, they are the big names in the UK for ANS probs and hypermobility respectively and are working together on the two conditions.

Flop

[juliegee]

jjb-

You're quite right about the connective tissue and autonomic connection being well known, at least among ANS doctors. BUT, I was commenting on how many people I have met with those conditions AND mastocytosis or Mast Cell Activation Disorder. I suspect that researchers should be looking at that connection.

I think there's something about the weakened connective tissue that's allowing the blood vessels to leak out causing low blood volume- seen in both autonomic disorders AND allergic reactions.

Mkoven, I tried to research Nazli and did find very general information (no specific studies) connecting the dots between allergy symptoms and connective tissue disorders. I hope SOMEBODY besides us is noticing this!

Julie