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http://mastcelldisorders.wallack.us/yabb/YaBB.pl Welcome! >> Introduce Yourself/Share Your Story >> Possible link between Ehlers Danlos Syndrome... is there one? http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1295541609 Message started by tburks on 01/20/11 at 04:40:09

Title: Possible link between Ehlers Danlos Syndrome... is there one?
Post by tburks on 01/20/11 at 04:40:09

My name is Terri, and I am 40 years old. I was recently diagnosed this year, by my own researching, then confirmed by a geneticist, with Ehlers Danlos Syndrome.

There is a lot of common symptomology with Mastocytosis and EDS. EDS is a connective tissue (as far as they know so far) disorder which has fragile weak tissue throughout the body. THere is also hypermobility of joints, causing us to be very "bendable".

I had 2 spinal surgeries, 5 days apart, in August of 2010.

About a month ago, I started "flushing" when drinking 3 sips of alcohol. After giving birth both times (6 and 11 years ago), I broke out in painful hives for 6-8 weeks. In the summer I always get some rashes.

Last week, I went in for an iron infusion due to low iron levels. An hour into it, I went into anaphalaxis with hives, swelling, and sharp stabbing pains in my abdomen that felt like a knife stabbing me everytime I took a breath. This stabbing feeling went in my abdomen, but then went to the middle of my back, down to the sacrum area. It was so painful to breathe.

I am researching mastocytosis and the possibility of having this condition as well. I am trying to educate myself, but feel somewhat anxious about this considering it is immediately life threatening.

Does anyone else here have Ehlers Danlos? I have the Classical type with hypermobility.

I live in the Kansas City area and am trying to find a doctor who will help me. Anyone else close to me?

Thank you!

Terri

Here is a link to give you general info on EDS.

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by tburks on 01/20/11 at 04:40:46

Here is a link to give you general info on EDS.

http://en.wikipedia.org/wiki/Ehlers%E2%80%93Danlos_syndrome

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Starflower on 01/20/11 at 16:19:30

Hi Terri,

Welcome to the group!

If you have any kind of mast cell disorder... whether that's systemic mastocytosis or MCAD... Ehlers-Danlos will make it worse. (I was just writing about this earlier today on the other website ;) ). There are two reasons for this. The first is because your blood vessels are weaker than normal... that means you're doing more clotting. Thrombin... one part of the coagulation cascade... is a known mast cell degranulator. The other problem with Ehlers-Danlos is that your body is constantly working extra hard to keep your blood pressure at a high enough level. That can wear down your adrenal glands... which is a bad thing when you have a mast cell disorder, because adrenaline is natural epinephrine. It keeps the activity of your mast cells in check.

Physical traumas like surgery and childbirth can also bring a mast cell disorder out of hiding! Stress is a big trigger for degranulation.

Unfortunately, you're a long way from any specialists. There are two things you could do right now, however. The first is to get some basic tests, starting with a baseline tryptase. It's just a blood test... you can do it any time. The second thing is to treat any symptoms you might be having... if antihistamines help, that's a positive sign that you're dealing with a mast cell disorder. If the signs keep pointing in that direction, you want to see if you can get to a real mast cell specialist. You could to the Mayo Clinic or Brigham & Women's in Boston.

If you do the tryptase test and it's less than 20, don't get discouraged. Some people have too many mast cells... tryptase is a great indicator for that. Some people have mast cells that trigger too easily... that's called MCAD, and it's much more difficult to diagnose (there are also lots of different types, so you end up needing a lot of different tests).

One thing at a time though! First... ask for that tryptase test 8-) Even better... if you ever go into anaphylaxis, as to have your blood drawn at least one hour after it starts, but no later than three hours after... test that for tryptase. If it's really anaphylaxis, the number should rise from your normal baseline.

Best wishes to you! Keep us updated and let us know if you have any questions.

Heather

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Beez on 09/14/11 at 02:45:07

Hi, I live in Australia and have EDS Hypermobility type, and like you have noticed mast cell like stuff (episodic GI/migraine/asthma/rashes/CFS/ POTS). Have two other friends & self who think we have type of systemic mastocytosis with the EDS. Trailing antihistamines and H2 blockers to try and settle some of the storm that comes. How r u travelling, any joy in getting help??
Bee :)

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by texan1960 on 09/16/11 at 07:23:31

Interesting line of topic because my uro wants me to go visit a physiatrist and get tilt-table tested etc for autonomic nervous system disorder...still have the guys name in my wallet, just haven't worked up the courage.

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by DeborahW, Founder on 09/21/11 at 15:53:15

Hello and welcome! As always everyone is welcome to discuss a variety of topics. drdiana - a big welcome to you as well. Although you actually have been with us since June, it is nice to hear from you on the forum. I see that you are a therapeutic optometrist -- that sounds interesting. So, you specialize in eyesight, right? How does that differ from a regular optometrist?

In general, I would like to remind everyone not to jump to conclusions in regard to people's user names. We have a number of doctors on this forum -- Ph.Ds, MDs, DDSs, etc. We want to be VERY CAREFUL when referring to doctors that we do not lead those new to the forum astray with all that is implied with the word, doctor. Sadly, there are only a select number of true mast cell doctors in the world, but it is a welcome thought that others may be investigating and researching the field!

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Traceyr on 09/30/11 at 06:32:28

I too have EDS3, POTS, Dsyautonomia, and just learning about the Mast Cell world. So many things collide with me, I dont know what is what. Im trying to learn all I can with help from this forum and others. I have contacted Dr Castells office awaiting paperwork for specific tests she may need.

I normally do not have some of the responses some of you mention. I usually do not have itching or hives. I have flushing, fatigue, brain fog, anxiety, sensory sensitivies, and sometimes digestion prob. But all of those are POTS issues due to EDS.... so trying to learn what is causing what. Its been a 7 year journey to get this far....Im thankful for all of you! Tracey

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Lisa on 10/01/11 at 05:32:16

Tracey, it's very important that you don't close your mind to the possibilities. This is what doctors try to do when working with us for if they close their minds and settle on a diagnosis, they aren't open for other options then. When you talk about your symptoms being due to the EDS, this is probably not so. Not all of us have itching or angioedema or hives. I almost NEVER get any of this!! I do have POTs and I have flushing, pressure changes, abdominal pain, brain fog, sensory sensitivies chronic diarrhea and reflux and syncope...along with the anaphylaxis. All of this is proven to be mast cell driven in me. And the doctors at Harvard also feel that the genetic defect of the mastocytosis is hiding behind my aortic aneurysm defect and is what allowed the aneurysm to form! (I'm not saying the masto cuased the aneurysm - there is a hereditary defect I have for the aneurysm, but they feel that the combo is what made it happen.)

So, this is why I say, what's the common denominator here - is it three different diseases or is there one common connection here? I'm voting for the one.

I'm awfully glad you're trying to see Dr. Castells. You are most definitely going in the right direction!!

Lisa

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Lisa on 10/16/11 at 23:50:01

Hi Issie, welcome to the group!

Yep, I´ve had this same kind of comment run past me too!! If your doctors speak with an allergist that doctor will say that allergies are NOT PSYCHOSOMATIC!! It´s impossible!! THERE IS DISEASE BEHIND IT THEN!!

This was a very pointed question I posed to several of my doctors in the process of trying to find answers. What could possibly make me allergic to things when I am totally IgE negative for allergies????

Well, mast cell disorders is one. Carcinoid syndrome is another. There isn´t much left.

Within the mast cell disorders you have histaminosis, hereditary angioedema, chronic urticaria, and MCAS. That´s about it in a nutshell!! I´m unaware of what else there could be.

As to the standard masto tests ruling out a mast cell disorder, it is now recognized that depending upon the form of the mast cell disorder, those tests return inconclusive. Not all forms of mast cell disorders will elevate tryptase or the other mediators. Dr.Cem Akin, a recognized authority, discovered by testing IA patients that 1/3 of them are in truth mastocytosis patients! They show neither elevated tryptase, nor histamines nor prostaglandins and don´t have any pathological evidence supporting a mast cell disorder suspicion. However, he found the genetic defect for SM on their mast cells and this revealed the truth about their disease. They are in truth, mastocytosis patients, but due to the type of genetic defect, their form of disease shows this total lack of evidence!!! This finding totally rocked the entire masto research world!!!!

Your doctors at the Mayo should know this, especially since the Mayo enjoys such a reputation.

Issie, if your doctors are needing information, please send me a PM with your email and I will send you information on the various forms of mast cell disorders that will help explain to them these issues. If you print it out and highlight the information as to the different findings, it will help them see what I´m saying here.

I hope this helps.

Lisa

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by DeborahW, Founder on 10/17/11 at 01:30:28

Issie,

You could have a condition similar to mine in that I test negative to everything but go into fullblown anaphylaxis. It is called Idiopathic Anaphylaxis (and may or may not be the same thing as Mast Cell Activation Syndrome). I have not gone into anaphylaxis for years, though, because I am now on the right preventative meds (histamine blockers) as well as knowing what to avoid so that I don't trigger. I wear a medical ID bracelet that says, "Anaphylaxis. No Benedryl." (I react to Benedryl....)

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Anaphylaxing on 10/26/11 at 11:16:58

My internist commented that I'm hyperflexible and I realized he might be implying EDS III.....I'm being worked up for masto/MCAD and POTS. I'm wondering if any of you have found treatments or strategies that have improved your symptoms? I've had my symptoms since anaphylaxis during a CT which was done for some tingling in my hands and feet.

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Anaphylaxing on 10/26/11 at 11:17:19

thanks!

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by issie on 10/29/11 at 06:49:50

Thank you for the welcome and information that you provided. I think that despite Mayo supposedly knowing allot about mast cell activation disorders - the doc that I'm seeing is just learning about the connection with POTS and Ehlers Danlos. He has started a file with articles I've taken to him and is trying to learn what he can about the problem. He called MN to talk to a doc there to learn more. He is in AZ. He seems to really want to learn about our issues and seems to be researching on our behalf. I'm to see him in a week. He is aware of full blown mystosis but seems hesitant about mast cell activation disorder. He wants concrete proof of the disorder. Sometimes that's just not possible with our issues. And, I'm afraid to not take anti-histimines. He wants me to be off of them and catch a flare. Well I have issues while on meds and don't want to find out what being off of them would create. So, he's not going to get his concrete proof. I'm okay with probabilities - he's not. I don't think science can catch everything and don't, at this point, believe that allergy testing is reliable.

Lisa, Could you post the info you have so that all of us can benefit from it? I'd love to be able to take it with me to my next visit - especially, since the doctor seems eager to take any handouts I give to him.

Issie

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Lisa on 10/29/11 at 06:55:49

Issie,

I have several PDFs, and the best way for your doctors to get these is for you to send me your email address so that I can send the PDFs directly to you. You can then either print it out or send it to your doctors through their email.

As to the doctors at the Mayo knowing about MCAS, the more I hear from patients talking about the Mayo in Minnisota, and in Florida, they are not up on this new diagnosis, so if you don't fall into the WHO criteria for SM then you won't get anywhere.

Lisa

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by issie on 10/29/11 at 07:54:39

We'll see what happens when I visit the doc in AZ - Mayo. There are several of us going to him that have POTS, EDS and MCAD. Since he has more than one of us with similiar issues - maybe - he'll pursue some answers.

Issie

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Anaphylaxing on 10/31/11 at 10:15:52

I agree from personal experience with Lisa's comments about Mayo. MCAS/MCAD is not on the radar and treatment without BMB is not attempted.

I have ?EDS III, MCAD vs indolent systemic and POTS am in MN getting checked an no one has brought up possible links or having seen this before...

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by Lisa on 10/31/11 at 23:41:30

What is it with the Mayo Clinic????? It seems that almost everybody I´ve spoken with who has been there in the past 4 years of talking to patients have had trouble!! If you are not a classic case of SM they won´t catch you!!! I think their strong point are other diseases.

All I can say if, if you are going around in circles with your doctors and they can´t find you to be a classic cast of Systemic mastocytosis, then you need to face up to this fact and go to those authorities who are KNOWN to be INTERESTED and KNOWLEDGEABLE about MCAS!! Within the States and Canada, that is basicly only 3 known doctors that I´m aware of, Dr. Castells, Dr. Schwartz and Dr. Afrin. In Europe I know of only two, Dr Escribano in Spain and Dr. Molderings in Germany.

Lisa

Title: Re: Possible link between Ehlers Danlos Syndrome... is there one?
Post by issie on 11/09/11 at 07:09:48

Got back from my Mayo visit. He had read most all the information that I sent to him from what was e-mailed to me and also had looked at some of our conversations in regard to what is being done for us. He corrected me in my statement about the immediate testing and not wanting to do it because of needing to take the antihistamines. He said to go ahead and take the antihistamines - it won't interfere with the test. He wants to catch me in a flare and see what the mast cell levels go up to from my baseline. He said if its a significant rise than we would say its mast cell related and go from there. Until we know that these flares are increasing mast cells in the blood - because they are usually in the bone marrow - we can't say for sure that its a mast cell activation disorder. That makes sense to me. So, the next bad one - I'll be off to the hospital for the blood draw and the 24 hour urine test.

Because I don't want to take the pneumonia vaccine challenge to check out my immune system response. He is going to just check my IGG and other levels like that every 6 months for awhile and see what is happening there. If it's still dropping, going up etc. Sounds like a good plan to me.

I also was able to talk with him about POTS and the research that is being done in regard to the different subsets. Us with HyperPOTS, very possibly have low NO (nitric oxide) this can also affect mast cell issues. Seems to be a connection there. Trying to get the pieces put together.

He has a pile of information from me and is reading it. So, feel very hopeful. He is at the AZ - Mayo. His name is Dr. Lewis - in the allergy dept.