Saturday, 5 November 2011

Latest Diagnosis of Ehlers Danlos / Benign Hypermobility Syndrome

This is the latest in my journey to find out what is happening to me and to explain the symptoms i am experiencing.
I went to see my Pain managment consultant in September 2011. I explained to him that i was experiencing constant dizziness , brain fog, severe pains in my hips, ankles , neck , hands , wrists , knees etc. I told him that my head felt too heavy for my neck and that i had to keep resting my head in my hands and keep mostretching it out to give me some type of relief.
He ask me to do a series of tests called the Beighton test .Helps to determine the degree of joint hypermobility. Currently it is used as part of the Brighton Criteria. For a positive Beighton Score, 4 or more points out of nine are required (i.e. two elbows and two hypermobile knees):

1. More than 10º hyperextension of the elbows

2. Passively touch the forearm with the thumb, while flexing the wrist.

3. Passive extension of the fingers or a 90º or more extension of the fifth finger (Gorling’s sign). This is used as a “Screen Test”.

4. Knees hyperextension greater than or equal to 10º (genu-recurvatum)

5. Touching the floor with the palms of the hands when reaching down without bending the knees. This is possible as a result of the hypermobility of the hips, and not of the spine as it is commonly believed.

I scored a total of 7 points and he told me that he believed i was suffering with Benign Hypermobility Syndrome / Ehlers Danlos syndrome type III. He then made me a referal to see a Rheumatologist who specialises in these syndromes.
I attended the appointment with the Rheumatologist a couple of weeks ago where she asked me to do the Beighton test again, ( again i scored 7 points ) This time the Rheumatologist looked at scarring in various places on my body to see how they had healed ( papyraceous scarring ). She made comment of how my eye lids had drooped. She even tested how flexible my ears were.
She told me that i had BHS / EDS type III and that she would arrange for me to see a specialist Physiotherapist to help me with strengthening my muscles. She told me that it explains why i have cranial instability in my neck which explains the pains i am having in that area. I am now taking 400mg of Tramadol a day to help with the pain and 40mg baclofen before bed.
I am awaiting now to see a Cardiologist to see if he can test me for POTS as this may explain the dizziness. My Journey continues.

Major Criteria

· A Beighton score of 4/9 or greater (either currently or historically)

· Arthralgia for longer than 3 months in 4 or more joints

Minor Criteria

· A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)

· Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.

· Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.

· Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).

· Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactily [positive Steinberg/wrist signs]).

· Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.

· Eye signs: drooping eyelids or myopia or antimongoloid slant.

· Varicose veins or hernia or uterine/rectal prolapse.

The Joint Hypermobility Syndrome (JHS) is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

JHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1986 and the Villefranche 1998 criteria respectively).

Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.


judy said...

I don't know where you live but if its anywhere in or near London you should try to get a referral to Professor Matthias who is the country's only Neurologist who specialises in EDS. Myself, my son and my daughter all have EDS type 3 and he sees my daughter for her POTS. Unfortunately because my son is under 18 he can't be seen by Professor Matthias but as soon as he gets to that age he will be able to get a refferal to him.

judy said...

I meant to also say he works at The Royal National Hospital for Neurology at Queen's Square near Great Ormond Street.

Maria said...

Sad to say that I don't have a knowledge about this disease. It's my first time hearing about it and I'm getting curious. A visit for a neurologist will surely answer your concern and its medications.

caroline knightley said...

Hi there and its nice to know not alone in this little strange annoying world which outside do not understand the suffering. On beta blockers 160mg rapid pulse. Some how i dont think i can get my head around everything,
I have bhms/eds 111, CFS (severe sleep deperavation)? , IBS, GI, cervical spinal cord root compression to which cannot be operated as 90% paralys or Death and this is very scary as any of you suffering will understand .
I am in process waiting outside rails putting on stairs as for some reason i lose balance i am clumsy trip and i am having hand rail on inside staircase, i have a trolley and chair for work top food prep to help as cannot stand long .
I am on 450mg lyrica.
Anyone else like me??? Please i want to know and not feel alone as my hubby and daughters good but i just seem alone,
Do anyone get DLA or ESA with this condition(s) i am 43 from UK leeds.

caroline knightley said...

Ps i have seen rheumo, physios, all discharged me, i seen Neuro surgeon & neurologist and both say far too high risk to operate! Is this mad or what am scared , i was refered brain specialist and now back to pain management again! Its annoying x please talk to me

tinhatpixie said...

Ouch, reading this has made me think I need the tests done. Although GP's have done the simple ones. My Aunt has been diagnosed with EDS. She has told family they need to be checked. I've shrugged it off. For over 3 years I've had pain in right shoulder, getting worse and spreading into left side. I'm in worse pain when I sleep, 500mg of tramadol don't help with pain at night. I've not slept through the night in over a year. My ankles hurt and seem to slip out of joint when I walk. I've had to fall out of bed and try and stand because I can't stand normally. I've had other symptoms but brushed them aside, you end up feeling like a hyperchondriac. The GP's I've seen have been no help. I've had countless blood tests, and ex rays. Physio caused more pain, and them said she was treating an in known entity to go back to GP. Advanced physio therapist sent me packing, and the last GP I saw told me to go to yoga. After 3 years I am at my wits end and wasted most of the time on tramadol, which they tell me is bad for my health. That doesn't stop them writing the prescription every 3 weeks. I don't have flexible joints, or elasticity in my skin. My eyes apparently don't have blue scelera tint. They say there maybe arthritis dotted about from wear and tear of a 41 year old. They also say the physical job I do is not good, even though there is less pain when I'm active. Surely yoga is being physical and uses stretching and excercise.I understand everyone is different, from reading the above the signs are there. Any suggestions would be gratefully received. I'm not a grumpy so and so who complains for nothing, I'm done in from 3 years of pain and sleepless nights. Thanks a lot. Tania. Please email at

tinhatpixie said...

PS. Had nerve conductivity tests all OK, neurologist told me to get well soon in typical hospital letter. Chiropractor was amazing 1 year ago, she spoke more sense than any one else. That's what you get for going private. Back to her for advice this week.