Monday, 2 July 2012


Hi all

I think it is about time that I updated this blog........geez it's been a long time.

A slight update on me and how things have been going.

In February last year, I visited the most wonderful Specialist in the field of EDS.  He is based in London and despite being retired, he still persues his love of his job and carries on his work to help people with this condition (what a superstar he is!).

I was with him for nearly two hours and he was unbelievably thorough. He confirmed that I have EDSH - it was a choice between this and classic but he said that I did not have enough atrophic scarring. I have Marfan Habitus - long arm span, arachnodactyly (long spidery fingers), hand and wrist sign (check out this link ).

He said I have signs of autonomic dysfunction and I am awaiting a tilt table test. This causes me to get tachycardia upon standing, blood pooling in my legs (due to the collagen defects of my vessels) and feeling faint or fainting. The longer I stand the dizzier I get and the faster my heart gets. When I done my first blog, my heart rate would rise to around 120 when I stood up and would remain at that, but now my heart rate can get faster and faster, maybe even up to the 170's, until I sit down which will of course then return to normal. It also has an effect on my blood pressure which can be extremely labile. Some weeks it can be very high, some days it drops ridiculously low. I also get very narrow pulse pressures. I have also been getting hearing problems: pulsatile tinnitus, stapedial myoclonus, but whether these are related to the autonomic dysfunction is anyone's guess until I am thoroughly tested.

I have been getting orthostatic headaches - when I stand up, I am in agony with the head pain - luckily it subsides most of the time within 30 seconds to a minute. Last year, I HAD to lay down as this head pain would not go away if I was standing. Luckily this only lasted approximately 3 weeks. It is now intermittent.

Roll on the tilt table test, that's all I can say about the above issues!

As for my EDS pain, right now, I am pretty lucky. I am only having problems with my hands/wrists and left hip. The SI joint seems rather stable right now but the other day, I decided to be 'clever' and get myself a step machine (I need to work on those soft non existent calf muscles of mine!) but that has now had an impact on my knees once again and they are feeling rather fragile and unstable.

My son has also had a DX of EDSH - he also has a mitral valve prolapse along with this - luckily not too much regurgitation is happening so he only has to have yearly checks to keep an eye on it.

To people who are struggling with their docs, try to persevere or ask for a second opinion.

I'm off now until next time.....

Wednesday, 18 November 2009

The symptoms I get with Ehlers Danlos Syndrome

The thing is with EDS, it seems to me that not many regular doctors know much about it. They seem to have given me lame excuses for all my troubles. When my SI joint (sacroiliac joint at base of spine)started to sub lax every morning, I went to the docs as I was in agony. He told me it was because I didn't want to get out of bed in the morning, oh how angry that made me. I asked to be referred again to a rheumatologist and he did, and so I was diagnosed with EDS. She was lovely...and for the first time, I had met a doctor who gave me no strange looks or fobbed me off as if I was crazy.
There are varying symptoms with EDS, they all vary from person to person. There are now only 9 classified types of EDS - some have recently been removed from the list, why I don't know. It can be in a form that is mild or severe. The severe type is the vascular type where it affects your heart amongst other stuff.  It even affects teeth and bowel movements. Following is a short list of some of the stuff I get:

Sacroiliac subluxation (incomplete or partial dislocation)
Hip subluxation (have to pop it back in)
Knee subluxation (have to pop it back on!)
Plantar fasciitis (inflammation in ligaments in sole of feet)
Costochondritis (inflammation of the cartilage and bones in the chest wall)
Pains and swellings of joints
Deep bone pains
Chronic fatigue and tiredness
Eye floaters
Dental and gum problems
Numb, prickling and painful skin
Stretchy slack skin(mild)
Soft velvety skin and extreme easy bruising, poor wound healing and scarring
Nodules in arms and shins
Flat feet
Hypermobility (scored 9/9 and completely bendy), muscle weakness, clumsiness
Intermittent pins and needles in arms/hands and legs/feet
Pain on exercising (this leads to extreme pain the next day)
Lack of core stability muscles, leads to weak ankles, hips and knees
Epidurals have no effect on me and a trip to the dentist can either be agonising as the injections do not work and I have even had a  seizure when given one of these damn things (EDS related I do not know)
Dizziness on standing, or standing too long, legs give way
Pains all over some days
Sleep onset difficulties
Dry eyes and mouth
Coldness of extremities and really feel the cold
Loss of sensation in legs etc etc etc!

EDS is a connective tissue disorder;our bodies are made up of connective tissues and this is the reason why it can affect the body in so many ways. It is not so well known but a lot more studies are being done into it. I will soon be going for genetic counselling and testing and will be involved in research too.

I won't go into the list that my family has - need to go to bed sometime tonight!! I hope that it gives you an insight into the various problems that I have had from a very young age.

My life with Ehlers Danlos Syndrome

Creaking, cracking, subluxations, shuffling around, cannot get out of bed due to severe SI joint pain, kneecaps popping, swelling ankles, teeth problems, severe tiredness, easy I talking about my Grandma, hell no, I am talking about me!!

I am 40 years old and some days I feel 95. Some days I cannot get out of bed for a long while and when I do, I shuffle along like an old Grandma! I have to try to make very slow movements in order to try to wrench my body back in place.Over night my SI joint likes to play tricks with me and decides to sub lax. Sometimes I dread going to sleep. That's if I can get to sleep.

All my life I have had pain all over. Every time I see a doctor, they said the old chestnut 'it's growing pains'. Well I would have thought by now I would have stopped growing! Of course I have but am still suffering the same old pains that I have dealt with all my life. My family have them too - each with their own combination of bodily 'faults'.

Finally this year, I have been able to put a name to answer all my pain issues......Ehlers Danlos Syndrome.

I am not a depressive, or a malingerer after all lol. I have an explanation of my life long issue.