Is ME due to Ehlers-Danlos Syndrome "stretchy veins"

[Sharon Lang]

There was a patient I knew who had Chiari malformation and ME/CFS. He had surgery where they basically carved out the back of his skull. So you could push on the back of his brain. Not good. It did nothing to improve his condition. He remained disabled and in pain. I would forget ever getting this done. He was desperate and talked a brain surgeon into doing it.

The key to most patients with ME/CFS is the EDS, not the Chiari's. The doctor mentioned that 12% of the patients with Chiari were EDS. I'll bet the percentage is a lot higher. But there is no accurate of assessing what is EDS and what is not. Simply asking a patient to bend back his thumb or touch his toes is not a good assessment.

Chiari patients need both a decompression and a fusion if they have coexisting EDS. Chiari institute in great neck y found the correlation S to why some patients did not do well after the decompression alone. My guess is this person you knew had both and needed the fusion as well.

 

[Sharon Lang]

Rosner has just doing the decompression. Both are needed in EDS patients to stabilize the cervical cranial instability and top the progression....

 

[Allyson]

If anybody can step forward with a a more widely shared anatomical abnormality than "connective tissue disorder," for patients with some kind of "chronically fatiguing illness," please step forward and be heard. Obviously, there are post-polio syndrome, true Lyme disease, and other sources than can cause the main symptoms we all have. But those can be ruled out. Here we have to consider that the most obvious anatomical manifestation is probably the most likely cause of CFS. It's basic logic.

when I phoned my gentics clinic for an appointment I asked if everyone who is hypermobile has EDS; she said no, there are several different connective tissue disorders.

Not sure it that throws any light on it Mish Mash.


Also someone wrote this in a PM so I ansewr it here with permission::

i don't have a definate diagnosis yet but if i was a betting girl my money would be with you! seeing doc who specialises in chronic health issues...) in july (booked in jan - this was the earliest i could get in) so am going to ask her and try and get a referral.

My answer -

Several people have had trouble getting referred by thier GPs - you just need any GP referral; so I suggest putting your foot down in a nice way; even my lovely GP was hesitant about the genetics referral and suggested Iphone them first.

I did they were lovely and most interested to see me so she happily wrote the referral.

THIS is our right to be referred o relevant specialists for THEIR opinions. GPs can't know eveything in this complex field. It is only when we see the clinics at large public hospitals that are affiliated with universities that we get counted and - incidentally the best treatment - from people up with the latest research.

There is no need to wait 3 months to get the referral - any GP can and should refer you. there is a further to see most specialistsafter you have the referral.

　

This is what I and many others went through to t get an ME/cfs referral - 20 years of "you don't need it, you look fine to me".

　

And that adds to the problem of slow diagnoses. so no-one knows we are ill so no-one does any research.

　

Finally 2 specialists diagnosed me with it after I read enough books and looked enough on the internet and said to my doc - Look, I am SURE I have this disease and asked for a referral to a specific doctor for their opinion.

　

I took the number and name with me so all she had to do was write a brief note and fax it to them.

I do suggest you do this as it saves them time and effotr; also they know there is someone who specialises in that field so it is a valid option.

 

[Allyson]

It's at least testable, for lack of a better word. I say if you have chronic fatigue, OI, gut issues and severe pain. Ask your doctor. You may have the vascular type and then you need to know,as this is life threatening.

According to EDNF now ALL types need to be tested as not only the vacular form can be life - threatening as formerly believed. ( Makes sense as so few were diagnosed who whould know how many died??)

When I rang to make my genetics cliinc appt they asked me a few questions then immediately booked me in for a cardiac ultrasound even before seeing me and I am not vascular type but type 3 .

 

[Allyson]

It is not a spectrum. The test is called beighton abound just like FMS , you need so many to get the diagnosis for the HYOERMOBILE type. The Beighton Score is a simple validated system used to quantify joint laxity and hypermobility. If you think you may have hypermobility, you can check your Beighton Score ...

I unedersrtand the ypes are bing re- classified and there may be 10 types now Sharon;
My point about the hypermobility term is that you do NOT heed to be hypermobile at all to have EDS
SOme people are very stiff and still have it..

PS I kindly note you seem to share my dyspraxia of bad typing - that is an EDS symptom too - really !

 

[Sharon Lang]

Allyson,

How do you feel in the morning after lying down (and hopefully sleeping) all night.

I'm a bit pressed to understand how EDS could cause ME as ME has a history of epidemic type outbreaks. That is unless EDS does run in epidemics or some thing could cause it in a group of people in the same area at the same time?

It's not a causation issue. There are overlapping symptoms. Some people may have both. Some may have been misdiagnoses.

 

[Sharon Lang]

According to EDNF now ALL types need to be tested as not only the vacular form can be life - threatening as formerly believed. ( Makes sense as so few were diagnosed who whould know how many died??)

When I rang to make my genetics cliinc appt they asked me a few questions then immediately booked me in for a cardiac ultrasound even before seeing me and I am not vascular type but type 3 .

I am type, 1 and 11 as well as 111. A rheumatologist can also diagnose hyper mobility type. Bring literature on Beighton scale..

 

[Sharon Lang]

I unedersrtand the ypes are bing re- classified and there may be 10 types now Sharon;
My point about the hypermobility term is that you do NOT heed to be hypermobile at all to have EDS
SOme people are very stiff and still have it..

PS I kindly note you seem to share my dyspraxia of bad typing - that is an EDS symptom too - really !

Yes, I agree with you. I have grand mal seizures and that affects the neurological issues as well....

 

[Sharon Lang]

According to EDNF now ALL types need to be tested as not only the vacular form can be life - threatening as formerly believed. ( Makes sense as so few were diagnosed who whould know how many died??)

When I rang to make my genetics cliinc appt they asked me a few questions then immediately booked me in for a cardiac ultrasound even before seeing me and I am not vascular type but type 3 .[/quote


Exactly. Claire francomano in md is who diagnosed me...

 

[Sharon Lang]

My specialist thinks it may all be due in my case to " stretchy veins " in the Ehlers Danlos (EDS) spectrum
so that when i stand up i get inadequate blood to the brain
(yesterday my BP dropped 20 points in a minute when i stood up - from 125 to 105 systolic but then returned to 125 within 5 mins) so adrenaline must have kicked in quickly
this instant adrenaline release happening constantly may be the cause of many ofl our problems
(possibly leading to low Cerebral spinal fluid levels too which exacerbates things further)
maybe a high protein diet helps as it helps build collagen to strengthen the veins
IM Vit B12 increases blood volume so may help because of that
certainly since he suggested this i have been observing my position in relation to symptoms and noticing a definite correlation
eg. resting sitting up does me no good so it is not the resting that helps but the lying down
yes i am ill after being upright- no exertion needed
and i am better after a day or two lying down
and it would explain why we have an energy boost sometimes for no apparent reason...eg in the evenings or early mornig.. after lying down for 6 hours or so all day or night

not wanting to be over simplistic here but would love to know if anyone has researched this aspect of the illness or if anyone else has observed this correlation between posture and symptoms

years of constant adrenaline boom crashes - and resulting in blood sugar boom crashes too - would take its toll on anyone.
It should be easy to test and even predict crashes according to your position... and yes I know for sure that a certain amount of time spent upright ...even sitting up in a chair doing nothing... will cause me to crash, while after about 2 3 days off lying flat my symptoms will improve or even disappear.
Lyng down is the first thig we all do for a crash, and in fact most of us feel we would die if we coujld not lie down but we never mention it in list of effective treatments
so it may not be fatigue we suffer at all
rather need to be horizontal plus acing muscles and joints, headache, swollen tissues of feet and calves due to fluid estravastation and accumilation there...all of which is summed upp erroneoulsy as fatigue. Fatigue is cured by rest and selppe. ME isn't.


I would love to hear everyone’s thoughts on this theory that comes from top ortodox medical sources please.

at a recent EDS conference it seems the list of symptoms closely overlaps ours.so they may be the same disease for at least some of us…and wiki is not reliable on EDS..

cheers
Ally

Early diagnosis is key in preventing the exacerbation of the afflicted body's progression towards premature deterioration and ultimate enhancement of the patient's lifespan and overall quality of life.

Although all types of Ehlers-Danlos syndrome affect the joints and many also affect the skin, features vary by type. An unusually large range of joint movement (hypermobility) occurs with most forms of EDS, particularly the hypermobility type.

Infants with hypermobile joints often appear to have weak muscle tone, which can delay the development of motor skills such as sitting, standing, and walking. The loose joints are unstable and prone to dislocation, chronic pain, and early-onset arthritis. Dislocations involving both hips are a characteristic finding in infants with the arthrochalasia type of Ehlers-Danlos syndrome.

Many people with Ehlers-Danlos syndrome have soft, velvety skin that is highly elastic (stretchy) and fragile. Affected individuals tend to bruise easily, and some types of the condition also cause abnormal scarring. People with the classic form of Ehlers-Danlos syndrome experience wounds that split open with little bleeding and leave scars that widen over time to create characteristic shallow "cigarette paper" scars. The dermatosparaxis type of the disorder is characterized by skin that sags and wrinkles. Extra (redundant) folds of skin may be present as affected children get older.

Some forms of Ehlers-Danlos syndrome, notably the vascular and kyphoscoliosis types, can involve serious and potentially life-threatening complications. Blood vessels can tear (rupture) unpredictably, causing internal bleeding, stroke, and shock. The vascular type of Ehlers-Danlos syndrome is also associated with an increased risk of organ rupture, including tearing of the intestine and rupture of the uterus (womb) during pregnancy. People with the kyphoscoliosis form of Ehlers-Danlos syndrome experience severe, progressive curvature of the spine that can interfere with breathing.

See Ehlers Danlos Syndrome -Morphopedics for more great information regarding description, anatomy, incidence/prevalence, clinical presenation of all types, potential etiologies, diagnostic tests, and conservative treatments.
Ehlers Danlos Syndrome: Signs and Symptoms


Btw, I have low b12 am not a vegetarian...
Totally agree that wiki is not comprehensive enough...

 

[Sharon Lang]

justy,
hypermobility is classic symtom of EDS ; but i do not have it - hypermobility all. There is a spectrum - some people have all the syumpotms and signs some have a few; i have none except varicose viens and history of feint and migraine in adolescence. But needing to sit with your legs up is interesting; i used to be like that but after 3 years of forced exercise i have deteriorated; but if you crash do you not need to lie flat?
I am chasing up the symptom overlap now; there was a good list circulating on Me cfs forums a while back for ME and my speciallist has just been to and EDS conference and showed me his list of their symptoms and they seemed so similar - such that he thinks it is the same disease - but i do not have hard copy. They were geneticists at te conference apparently looking at EDS in kids but my OI specialist says he is seeing hundreds of patients with similar symptoms...and they are a huge range of symptoms including almost all possible gut issues.

It may well be a risk factor, but many people with ME don't have EDS, so the direct link isn't there.

I respectfully disagree. The evidence shows,there is not necessarily a cause but a link.

 

[Sharon Lang]

https://www.edsers.com/

Totally agree that wiki is not comprehensive enough...[/quote]

 

[Sharon Lang]

EDS and POTS could well lead to the symptoms of ME. It's definitely something you should investigate, and it could lead to useful treatments for you (there are drugs like midodrine, and specific exercises which can be helpful for POTS and EDS too). Good luck with it.

http://www.ednf.org/images/2011conference/Handouts/EDNF_2011_Rowe_Fatigue.pptxSymptoms Of Orthostatic Intolerance: Lightheadedness, Dyspnea, Syncope, Chest Discomfort, Diminished concentration, Palpitations, Headache, Tremulousness, Blurred vision, Anxiety, Fatigue, Nausea, Exercise intolerance, Nocturia Working hypothesis: Connective tissue laxity in blood vessels allows increased vascular compliance, promotes excessive pooling during upright posture, leading to diminished blood return to the heart, and thus to OI symptoms (Rowe PC, et al. J Pediatr 1999;135:494-9) Chronic Fatigue in EDS [Rowe] (http://bit.ly/HQDNwk)

 

[Sharon Lang]

I respectfully disagree. The evidence shows,there is not necessarily a cause but a link.

Symptoms Of Orthostatic Intolerance: Lightheadedness, Dyspnea, Syncope, Chest Discomfort, Diminished concentration, Palpitations, Headache, Tremulousness, Blurred vision, Anxiety, Fatigue, Nausea, Exercise intolerance, Nocturia
Working hypothesis: Connective tissue laxity in blood vessels allows increased vascular compliance, promotes excessive pooling during upright posture, leading to diminished blood return to the heart, and thus to OI symptoms (Rowe PC, et al. J Pediatr 1999;135:494-9)
Chronic Fatigue in EDS [Rowe] (http://bit.ly/HQDNwk)
• 273 patients with EDS

 

[Allyson]

My specialist is going to find an EDS specialist for me he has one other patient with EDS so he is going to ask her who it is. I'm glad he didn't wave it off.

Several people have had trouble getting referred by thier GPs - you just need any GP referral; so if anyone as troubleI suggest putting your foot down in a nice way; even my lovely GP was hesitant about the genetics referral and suggested I rang them first.

　

I did they were lovely and most interested to see me so she happily wrote the referral.

　

THIS is our right to be referred o relevant specialists for THEIR opinions. GPs can't know eveything in this complex field. It is only when we see the clinics at large public hospitals that are affiliated with universities that we get counted and from people up with the latest research.

　

There is no need to wait 3 months to get the referral - any GP can and should refer you. there is a further to see most specialistsafter you have the referral.

　

This is what I and many others went through to t get an ME/cfs referral - 20 years of "you don't need it, you look fine to me".

　

And that adds to the problem of slow diagnoses. so no-one knows we are ill so no-one does any research....viscious circle.

　

Finally 2 specialists diagnosed me with it after I read enough books and looked enough on the internet and said to my doc - Look, I am SURE I have this disease and asked for a referral to a specific doctor for their opinion.

　

I took the number and name with me so all she had to do was write a brief note and fax it to them.

I do suggest you do this as it saves them time and effort; also they know there is someone who specialises in that field so it is a valid option.

 

[MishMash]

One of the popular treatments nowadays for fibro is Ketamine infusions. Ketamine is a anesthesia which was found to have brain rebooting properties. This was first discovered in severe depression patients, who felt instant relief from symptoms. It was later tried out on certain pain patients. The relief from RSD pain was almost instantaneous, and sometimes long lasting.

One of the doctors heavily involved in this treatment is Dr. Kirkpatrick in Tampa, Florida. He puts up videos of interviews of all his patients-- before and after. I thought this particular one was quite interesting, because the patient himself is a FM sufferer, and also a medical doctor.

Please take note of how the patient sits. He always tucks his legs up underneath him, as a normal sitting posture. Very strange for guys to do this. This tends to be a chick thing. Also, when he is not sitting on his legs, his feet are stretched back wrapped behind the chair.

To me, this patient clearly has obvious blood-pooling in his lower extremities. Which is probably linked to some kind of connective tissue disorder. It is pretty striking seeing his behavior. More anecdotal evidence, for me anyway, that ME/FM and EDS are inextricably bound. Incidentally, he underwent the Kettamine infusions and claims to have benefitted.

 

[Allyson]

Interesting thanks Mishmash and especially the fact aht he says he is a doctore and they alll ued to roll their eyes before seeing a FM patient as they knw this would be whiny helpless person
This is sad as he is evidently recently trained so this is STILL going on.


My specialist said he "used to be skeptical" but after he saw enough people saying the same thing he started to believe..
How many wil it take til they all believe?

Also the fact aht he was extremely active prior to getting sick - I wal like that tooa nd I know many others were too and I would never sit around wathcing tv or chatting to friends. I now think that is due to a dislike of stasis and a need to keep moving to get the blood moving.
in other works you choose a very active lifestyle for a long as you cna ... then at some point it catches up and you crash out - maybe that i what they used to call burn out??.

so sorry for my bad typing dyspraxia - i do edit but it is still bad; my posiition does not help.

 

[Allyson]

I always feel great just after a general anasthetic - I bet it works as a total muscle relaxant so effects will be short term

Massage does the same to a lesser extent.

 

[Allyson]

Yes, I have ME and EDs III.

EDs was diagnosed by an autonomic specialist in 2006 by a physical exam and history. This diagnosis was confirmed by two other doctors. ME was also diagnosed by several doctors, one being one of the top specialists in ME.
......
Some of the ME doctors and researchers do have good theories about how this can happen. It has been explained to me by my doctor but it is complex, I only understood part of it, and have forgotten most of it!

So I can just say that treating ME has, for me and some others here, reduced OI greatly.

Sushi

thanks for that Sushi.. that is hte think I like aboutthe EDS theroy - I can cleary understand it and remeberit, it explains most of my symptms to my satifaction - which nothing else does, and On that priciple I can now predict wwhen i am going to crash and learn how to aoid/limit it.

Peripherally related I see a major sport wear company here - Skins - has stared prooting compression clothinsg ans asying it is helping athletic performance - presumably on the same priciples - iincreased blood supply or imporved cirulation - same thing is said to help jetlag that we gt so badly and prevent the DVTs some are getting now from Flying - high altitude - depressurisation , dehydration, heat and sitting upright for many hours while not moving -the perfect recipe for a crash!

 

[Sushi]

Peripherally related I see a major sport wear company here - Skins - has stared prooting compression clothinsg ans asying it is helping athletic performance - presumably on the same priciples - iincreased blood supply or imporved cirulation - same thing is said to help jetlag that we gt so badly and prevent the DVTs some are getting now from Flying - high altitude - depressurisation , dehydration, heat and sitting upright for many hours while not moving -the perfect recipe for a crash!

I checked with a representative with Skins (it is international) and they sent me the compression levels for their clothing. It is fairly low and probably not the best choice for the type of compression we need. They said that all their garments had this same compression.

Here is what they wrote:

-Ankles: 20 +/- 3 mmHg
-Calves: 18 +/- 3 mmHg
-Lower Thigh: 15 +/- 2.5 mmHg
-Upper Thigh: 9 +/- 2 mmHg

Too bad, as their tights look very comfortable.

Best.
Sushi