Sunday, 25 April 2010

What is Hypermobility Syndrome/Ehlers Danlos Syndrome

Before I start blogging about living with Hypermobility Syndrome/Ehlers Danlos Syndrome (hms/eds) it makes sense to explain what the condition is about.
Alot of people have never heard of hms/eds and those who have, often don't understand what exactly it is, after all its a very complex condition that even most drs don't even understand.

(added 2010)---
Hms/Eds is pretty much the same condition. Previously Hypermobility Syndrome and Ehlers Danlos were thought to be different conditions (with Ehlers danlos being more severe). However Drs have now agreed they are in fact the same condition, or there is so little difference between them, that people with either 'diagnosis' need treating exactly the same. So they are now seen as the same condition. Ehlers Danlos:Hypermobility Type. This is why often a person who is diagnosed with hms often hears drs refer to their condition as eds.

So in this blog I will use the term hms/eds. (in later entries I will refer to it as simply EDS).
They are connective tissue disorders which basically mean there is something wrong with the person's connective tissue, Collagen. You are born with hms/eds, it is not something you suddenly get, although some people (like myself) have very few problems until they reach their 20s). It is an inherited condition which means at least one parent will usually also have the condition however it often becomes more severe each time it is passed onto the next generation.

Collagen is a protein based material that has the basic job of allowing body parts to move. Collagen is present in the joints, mainly the ligaments, tendons and muscles, organ tissue, and skin. Its job in the joints is to allow the joint to stretch and move but also stops it from moving too much. If our joints didnt have a 'stop' point then our joints would not be able to support us and basic movemnets would be impossible. Our joints would fall apart. This is basically what happens in hms/eds. In people without hms/eds, the collagen is made up of fibres that stretch like an elastic band. If you stretch an elastic band eventually it snaps back to its 'unstretched' state which stops the elastic band from snapping. In hms/eds the fibres are like chewing gum rather than an elastic band. If you stretch a piece of chewing gum you know that eventually it tears and you end up with sticky gum all over your fingers! This is what the collagen is like in people with hms/eds. The collagen stretches and stretches but does not snap back. This means the joints stretch and move but move and stretch past the point they are supposed to. They are very bendy and wobbly and don't snap back into place and eventually when a joint stretches past the point its supposed to, it dislocates. Ouch!!
In hms/eds the main symptom is joints that dislocate on a very regular basis, often many times in just one day.
When you have joints that dislocate easily and joints which are bendy and wobbly, they don't do a good job of keeping you in one piece. Your body becomes very weak and unstable. Just trying to stand up can be impossible as you may have knees and hips that don't lock and wobble all over until you end up in a pile on the floor. This makes walking pretty tough too.
If your spine is unstable it makes it difficult to sit up straight and also contributes to the problems with standing and walking.
In the upper body, bendy joints in the shoulders, elbows, wrists and knuckles make it difficult to lift things, push yourself up from a lying position, and use things like cutlery, a toothbrush, a pen and even the crutches and walking sticks that help you to stand and walk become difficult to use.

Because of the bendiness of the joints, a person with hms/eds will use their joints over a 1000 times more in one day than a regular person. This means their joints grind and rub against the ends of their bones many more times than someone else of the same age. As a result the joints and bones get worn down very quickly which results in a very early development of osteoarthritis. People with hms/eds usually develop osteoarthritis as a result of degeneration of the joints as well as inflamation of the joints often before the age of 30. They also can develop osteoperosis, where the bones become weak, very early on.

Becuase collagen is present in organ tissue as well as the joints, people with the condition can also have problems with their organs, in particular, organs that are designed to move. The bowel and intestines work by muscles contracting to push food and waste through them. Because the muscles in hms/eds are much laxer(more relaxed and stretchier), they don't contract as well so in the bowel and intestines, the muscles don't do a very good job of moving food and waste along. This means that people with the condition often have alot of problems with the intestines and bowel, in particular ibs, slow transit, incontinence and other similar problems.
The same goes for the bladder too.
In women with hms/eds, the womb has to contract much harder during her period in order to shed the unused lining (this is basically what a period is). This results in very painful and very heavy periods. It also makes it difficult to maintian a pregnancy and many women with hms/eds experience more miscarriges and problems conceiving.
The heart is another organ that can be affected due to the amount of movement it requires to work. In certain types of Ehlers danlos, the heart can have serious issues such as heart murmers, slow and irregluar heart rates. People with the vascular type of Ehlers Danlos are at great risk of anurysisms and heart failure due to the rupturing of the blood vessels. The same with the brain which can result in stokes.
The skin is another major body part that is affected by hms/eds. In particular it is often very stretchy and soft which makes it more fragile. This makes it prone to tearing very easily and very poor at healing. People with hms/eds often have alot of scars that look like they are from serious injuries when in fact they can be from something as simple as a scratch. The skin also bruises very easily, often from simple things like kneeling on the floor or getting a hug from someone.

Because the joints in hms/eds are very unstable, the muscles around the joints have to do all the work to keep them held together. This means that most of your energy gets used up by the muscles. This means that people with the condition suffer really badly from exhaustion and very poor energy levels. Something as simple as walking up the stairs can exhaust the person so much that they have to lie down for 30mins to recover. This means that alot of people with the condition are also diagnosed with CFS/ME. Having such poor energy levels makes it even harder to do basic tasks such as dressing, washing etc.

For some reason, people with hms/eds are much more prone to alot of other conditions. Some conditions occur for obvious reasons to do with the weakend collagen and energy issues, other conditions occur but don't seem to have an obvious reason why.
The related conditions that people with hms/eds tend to also have include:
Fibromyalgia, flat feet, raynauds syndrome, insommnia, curvature of the spine, kyphosis (a hump at the top of the spine), hernias, neuropathy, carpel tunnel syndrome, premature aging of the skin, strokes, heart murmurs, rheumatoid arthritis, automic dsyfunction (a dysfuntion of the body's ability maintain normal function of systems such as blood pressure) and much more.

Hms/Eds varies massivley in severity. Some people may have very few problems and lead normal lives, able to do the same as people without the condition. At the other end of the scale, some people are seriously affected by the condition resulting in severe disability. They may depend on a wheelchair, they may need help to dress, bathe, change position in bed etc. In some cases, a person with hms/eds may become bedridden for periods of time due to the severity of the condition. People who are effected badly may not be able to work and rely on the help of others to get through the day. And of course in the case of Vascular EDS, the condition can often be fatal.

Because hms/eds is caused by a defect in the collagen fibres, there is currently no cure. Treatment concentrates on keeping the joints as strong as possible to delay the onset of degeneration. Physiotherapy concentrates on building the muscle strength around the joints. Occupational therapy looks at aids that can help make tasks easier.

Pain is a major symptom so becomes the main focus for treatment. Pain in hms/eds is difficult to control becuase it can fluctuate easily. But in alot of cases, medications such as tramadol, codiene and even morphine are used to help manage the pain. It often does not take the pain away but only brings it down to a more mangeble level.
Sometimes surgery is required to stablise joints but this is done less and less now as drs are aware now that operating on a joint just makes the other joints more unstable. Steroid injections are used more and more now to stablies joints by reducing inflamation. Steroids are injected directly into the joint which is extremely painful and doesnt work for everyone.
Other treatment focuses on stablising the joints with splints and braces so a person with the condition may wear various splints on their wrists, elbows, knees and ankles. They may also wear splinted supports to stabalise the spine, hips and shoulders. And of course the related conditions need to be treated. So someone who suffers from the hms affecting their bladder may need to use catherters and incontinence products.

Becuase the joints of a person with hms/eds are so unstable and dislocate so easily, alot of the time they go back into the right position very easily so people with the condition often learn fix to dislocated joints themselves. Sometimes however they do need to go to hospital as a result of a bad dislocation. People who suffer from the effcts of severe hms/eds often spend alot of time in hospital reciving intensive physiotherapy and pain treatment. They often have many drs from different areas such as rheumatology, endochronology, gasteonterology, etc and can have a hosptial appointment every week sometimes more than once a week!

Hms/Eds is becoming more recognised as a seroius condition by drs. Many people with the condition spend years trying to get drs to take them seriously and to get a diagnosis. Even then treatment can be very difficult to get. This is still the case in alot of places and as a result many people with hms/eds have to travel alot to recieve treatment and care from specialists who have more knowledge of the condition. There are currently only around 4 hms/eds clinics in the UK!

One of the biggest problems people with hms/eds face is the lack of understanding from friends, family and collegues. A person with hms/eds may appear to be fine, especially if they can walk. Often a person with hms/eds may be able to do something one day but not the next such as pouring a kettle or walking up the stairs. So it becomes difficult for others to understand just how difficult the condition can be. Even those who use wheelchairs or crutches to walk can experince problems with people not understanding. This can make relationships and friendships difficult and often a person with hms/eds becomes isolated as friends slowly disapear. Often a person with hms/eds has alot of bad days where they are unable to get out of bed or to go out. This means that friends often only see them on a good day so they then think that this is how the person is all of the time. They often don't comprehend how bad the bad days are.

Hms/Eds is a very complex condition that can affect the person's life in many ways. The condition often affects social and work life and can even greatly affect quality of life. Becuase of the massive range of issues the condition causes, people with hms/eds often struggle with many things that others take for granted. The lack of understanding of the condition and due to how it can fluctuate on a daily basis leads to the person finding it difficult to maintain friendships and to recieve emotional support from others.

This blog is intended to allow people to understand the daily issues living with hms/eds causes. It also is intended to highlight the strength and determination people with the condition have to get on with their lives and the ongoing fight they have to recieve appropriate treatment, and more importantly, understanding.


  1. Carrie,

    You've certainly been through a lot, dear lady! And you've also give quite an education to people here. As one with HMS, whose been a member of the HMSA half a year and often carries Grahame/Keer's and Tinkle's books, you've taught me some new things. Do you have favorite resources you've learned much of this stuff from?

    Thanks for sharing your insights. I'll be praying for you and all that's going on.

    By the way, your name is very pretty.


  2. Thanks lindsay.
    I've used lots of different resources over the past 2 years to find info on hms/eds. The HMSA forum is a huge resource as you know. I also trowel though the many articles and medical publications online.
    Alot of what i know has come from researching, more researching and researching again! ITs very easy to take everything you read as face value but it's important to compare facts across many resources. Doing this you will discover what is actually fact and what is absolute tosh!.

    Carrie (p.s. Im lil_miffy on the HMSA forum)

  3. Hi,

    I've recently been diagnosed with HMS and have learnt so much from reading this blog. It's made me feel so much less like a hypochondriac, so thank you!

  4. I laid down tonite with my laptop, planning a little more research into the cycle of my life since i turned 20- waking up feeling like my limbs would drop off me if i got out of bed, or like my muscles were concrete under my skin they got so stiff, and nothing in between. and long accepting that i was on my own and not to even bother with excuses about what i wasnt doing. i happened to stumble across this HMS thing, and 6 hours later im still too excited to sleep. i finally have something to tell the drs, and they cant say this is my wrongdoing or in my head. this is it- im done feeling stupid explaining the bruises i didnt know i had and nodding again and again that 'i should try SSRI's'. i have something to say this time.

    so.... thank you :)

    ps. i started 3000mg evening primrose oil daily for my bruises & scaly skin, and its done me a thousand times more good than any NSAID. after 3 weeks on it my scales were gone, body relaxed more at night and blood pressure less insane. dont know why, tried it on a whim, was the best thing id done until today.

  5. Wow this blog is excellent i've finally got something i can show my friend/family that they can easily read and understand


  6. Hi, i'm a mother of a 10 year old girl with hms she was diagnosed when she was 4, and has quiet an extreeme case she see's all types of doctors and because there is no pain clinic in the north east she has to go to Great ormand street hospital, we have to do lot for her on a good day she even uses a laptop to do her work and on a bad day she can not even stand up and touching her to help her is very painfull. the medication she takes is silly.(ibrufin,codine,paracetamol and donperidom) we have had adaption put in the property for her and i can not even hold down a job because of the condition. Could you please give me some info on getting her the DLA as we have been fighting for it since she 2009 and still no luck but she did get low rate when she was 5 for night time care, but the condition is worse now and thet still say no!.


  7. Hi,
    Thanks a lot for the info. I was diagnosed with EDS hypemobility type three months ago, finally after going through physical & emotional pain for so long. I'm having lots of difficulties now with people at work and with my wife, family and friends.They don't seem to understand my condition. Some also have an impression about me without knowing the name of my condition!

    Thanks for your efforts and God bless


  8. hi there, ive just been diagnosed with hms/eds after being made to feel like it was constantly in my head by my GP! i have bladder problems whereby i get up 3/4 times a night to pass urine as my bladder constantly feels full, a partial uterine prolapse, fatigue and pain in my thoracic spine which at times is unbearable. i also have POTS, postural orthostatic tachycardia syndrome, which has caused the symptoms of fatigue to worsen. i am a nurse and i had researched alot of my symptoms and had to force my GP to refer me to a rheumatologist as he basically said it was all in my head and put me on anti-depressants. i saw the rheumatologist and in 20 minutes he had diagnosed me with hms/eds. i encourage anyone that thinks there GP is ignoring your symptoms to ask for a second opinion or change your GP.

    Heidi x

  9. Like the others who have commented, I just wanted to say thank you for the very informative post. I was just diagnosed with eds almost 2 months ago after nearly 10 years of pain. Even with an official diagnosis, my friends (but especially my mother) still don't seem to understand how much pain I'm in. For years I've been told I was lazy, making it all up, or just had growing pains. My doctors told me it was all depression or because I was overweight. I couldn't get them to understand that the weight gain was because many days it hurts to even lay in bed- much less get out and exercise. It's nice to find an article that has so much information in one place- as there doesn't seem to be much info out there. So thanks again! :)

  10. Hello,
    I am currently battling with this problem myself--GP who doesn't care. It was my podiatrist who suggested I might have EDS because I've been going to her so often lately with my mangled feet. I have very flat feet which causes a lot of pain and swelling in my ankles. I'm double-jointed all over the place, my thumbs and knees dislocate and sometimes my shoulders when I sleep. My back and hip go out regularly making it very painful to walk. My back is so bendy that I can pull my leg behind me and past my head. I also have mild scoliosis. I've felt constantly exhausted ever since high school, I get migraines, and have problems with IBS--something that my mom and sister do also have.
    I told my GP all of this and told him what my podiatrist had suggested about the EDS. He quickly shut me down based on my not having debilitating pain in my knees specifically, and the fact that there is no genetic connection with my family. However, my mom is adopted and we don't know anything about her background. I'm 27, I'm in pain, and I feel so limited in the jobs I can perform. My situation isn't nearly as bad as others that I have read (I can still function and go to work/school), but I've definitely begun to notice some challenges that I don't think the average 27-year-old has to face.

    Thank you for the detailed and thoughtful blog post.
    -Lindsay #2

  11. Thanks fo the great post.

    I have EDS too. It has completely affected my life to the point where I've lost my family. I'm not as severe a case as you are, but I'm enough of an EDS case that health professionals have classified me as one.

    EDS has been the driving force behind my life. I endured a violent and bitter childhood because of it, barely got through college, and couldn't work the long hours needed to survive in graduate school. I ended up relying on my secondary skills to get by in life, but that is still a struggle.

    One area where my EDS gives me an incredible physical advantage is playing violin and guitar (which I do professionally). My soft hands (bilateral swan neck deformities) and loose joints allow me to contort my body into various shapes that allow me to play things that people without the condition cannot do without physical pain. I can practice long hours or play long gigs without a hint of tendinitis or carpal tunnel, though I cannot get through a gig without having to sit down multiple times due to my weak erector joints. Nicolo Paganini likely had the condition too, and i play all of his music for both violin and guitar with utter ease because it actually fits my body!

    The other area where EDS has turned me into a fine professional is combative martial arts. I was bullied relentlessly at school for being Asian AND frail, and then my family beat me daily because they assumed that I was being willfully lazy when I was actually battling the condition (my family is in medicine but they refuse to believe that I have EDS). As a result of daily physical combat at home and at school/work, I now have an incredibly high pain tolerance and also have a comprehensive understanding of how to defend oneself against any kind of bully. Today I teach combative Chinese kung fu in my own business because I have trained my EDS body to act as a flexible shock absorber while my contortive EDS body also allows me to do any kind of move from virtually any style.

    One aspect of EDS that isn't talked about much is vulnerability to upper respiratory infection. I have had to quit several jobs because I kept getting so sick from them on a daily basis. In my martial arts business, I completely stopped that by acquiring an Austin Healthmate Pro air purifier that removes both particulates and odors. I went from constantly missing work from flu level illness to having only minor colds. Unfortunately, I can't take my Austin onto airplanes where I ALWAYS come down with major respiratory infections even though I'm only 38.