The Drama of EDS - The TV Freakshow

EDS is becoming the subject of TV dramas as the condition is becoming more well known. You would expect that this would be applauded by sufferers and their families as a much needed awareness achievement. However the storylines used by show writers using the condition to boost their ratings are failing to raise any awareness of what living with the condition is really like. Instead EDS is frustratingly becoming the freak show storyline causing more confusion and increasing ignorance surrounding the condition.

Last night's episode of House sparked a flurry of complaints and discussion on social network sites just weeks before May becomes EDS Awareness month in many American States.
While support organisations work on the finishing touches of awareness strategies and campaigns for more funding for essential research into what can be a fatal condition, they are sidelined into dealing with the aftermath of another poorly researched storyline causing upset and anger amongst sufferers.
The episode focussed on a woman with a severe anxiety disorder that culminated into an extreme state of hoarding. The character's house was filled to the ceilings with years of personal belongings, rotten food and even a dead cat in the freezer.
The hoarding caused the woman and her husband to become ill from a disease caused by raccoon droppings. The husband quickly recovered while the wife was suddenly struck down with a heart attack. A few scenes later and House's medical team discover the woman had suffered three miscarriages which sparked the anxiety and hoarding.
'You have Ehlers Danlos Syndrome which explains the symptoms and probably caused the miscarriages. We can treat the symptoms as they occur'.

Most people with EDS watching the show did a double take as her diagnosis was so quickly achieved wondering if they had missed a few scenes while making a coffee.
'Did I miss something?' One EDS sufferer asked her husband...'No you heard it right' was his reply.

I watched the show knowing the expected diagnosis so looked for small clues but other than the heart involvement there were no clues that an EDS diagnosis was to come. Even then heart attacks are rare in people with EDS and are not a known symptom or complication of the condition.
In the Vascular type of EDS patients are at risk of aneurysm in the brain and heart and it is this rupturing of the heart vessels that leads to death, not heart attacks.
The only symptom that connects to EDS is anxiety. Anxiety disorders are very common in EDS but feature more in the Hypermobile type, not the Vascular type. As the woman had no joint or skin involvement the only possible way that EDS was believable is that some patients with Vascular EDS can be affected with no joint or skin involvement and the condition is only discovered following death from an aneurysm. It seems totally implausible that a patient could present with no joint or skin symptoms, no signs of aneurysm but then quickly and easily reach a diagnosis of EDS because of repeated miscarriages and anxiety.

The main concern of EDS sufferers watching the show is that this story line has only fuelled the already present attitude amongst some doctors that EDS patients have a tendency to have mental health problems.

A recent episode of Casualty, a medical drama by the BBC (UK) included a very small scene of a patient with EDS appearing in the A&E department with a dislocated shoulder. The Dr quickly pushed the joint back into place WITHOUT pain relief or any warning and then told another DR that the patient dislocates his joints ON PURPOSE in order to obtain morphine.
This storyline also fuelled outrage and upset amongst the online EDS community with the BBC receiving many complaints.
A lot of people with EDS refuse to go to hospital following a dislocation having received terrible treatment and attitude from Drs who do not understand the condition in the past. Some patients have been accused of dislocating their joints on purpose for attention as drs refuse to believe that joints can dislocate so easily without force repeatedly. Others have been refused pain medication for a dislocation as drs believe that the patient does not need it if they can relocate their joints easily.

I myself have avoided attending hospital for dislocations because of previous treatment. Having been sent to A&E directly from a hydrotherapy session that left me with drop foot and numbness in my left leg the consultant dr on duty asked me 'What leg do you THINK is numb'. Set back for a few seconds by his intended meaning I explained that I was sent by the physio and that I had not come of my own accord. But with a past history of depression the Consultant jumped to the conclusion that I was either attention seeking or that it was in my head.
My physio discovered a few days later that I had suffered from a build up of trapped nerves in my spine that had bulged during the hydro session but later released themselves of their own accord, not uncommon in EDS patients.
I have only attended A&E once since then following a fall and was lucky to be seen by a different Dr who was over cautious and kept me in for observation. Luckily for me this turned out to be a blessing as monitoring showed erratic blood pressure and heart rate which led to the first suspicion of POTS.

Another show which instigated anger was another BBC show, shown just weeks before the episode of 'Casualty' which also received complaints. Some of these complaints were addressed and received promises to look into any misrepresentation of the condition in order to improve further mention of EDS on the BBC.
The show 'Freak like me' followed a young girl who 'loved clicking her body'. The girl, undiagnosed, would click all her joints, including her hips and shoulders, many times a day. The show addressed this as a 'freaky habit' with no mention of the medical danger of this behaviour. Many EDS sufferers who saw the show wrote to the BBC asking them to inform the girl that she should look into EDS due to the obvious extreme hypermobility of her joints and other signs that she may have the condition. What upset EDSers is that the actions she took to 'click' her joints were similar to that they needed to do to relocate dislocated and subluxated joints. They were not only worried that the girl could be causing long term damage to her body but that the BBC had not looked into the medical risks of such behaviour which could have made them aware of EDS in order to give a medical warning to viewers.

It seems that EDS is becoming the condition to use if show writers want an interesting storyline with a freakish twist but they are failing to show even the basic symptoms of recurring dislocations, chronic pain and mobility problems. Instead they are using the condition to highlight drug misuse, mental illness and freakish habits.

The only hope is that writers of Coronation Street, a UK soap opera, uses the character of Izzy, a wheelchair user with EDS, to highlight some of the effects of this chronic and disabling condition. Izzy is played by actress Cherylee Housten who also suffers from EDS who, when joining the show, sparked interest and hope from the EDS community that her character would have the condition involved in some of her storylines. However, so far, there has been no mention of 'Ehlers Danlos Syndrome', only 'a degenerative disease' and a few small mentions of being able to stand and walk 'which makes people think I'm faking being in a wheelchair'.

We know that EDS is rare and there are many more interesting conditions out there to boost ratings but all the EDS community ask is that if writers insist on using the condition in their storyline that they stop using it as a basis for freaky and offensive storylines that are only adding to the misunderstanding and potentially damaging attitude amongst Drs. Instead show EDS sufferers the respect and admiration they deserve living with a painful, life changing and sometimes fatal condition.

How to have a good GP appointment

For most people a visit to the gp is a quick 5-10min job to have a sore throat or upset stomach checked out.
For people with Ehlers Danlos and other multi-system conditions it’s a mammoth task that needs preparation and the ability to keep it together under pressure better than a politician on a pre-election live TV debate!

Quite often you don’t just have one thing you need to see the gp about, there’s a list. You have a new pain or symptom that needs attention, you need a referral to a new specialist or refferred back to a previous one because an old problem has reamerged. You need one of your medications looked at because its interacting with another and causing problems. You need your ribs checking because you dislocated them badly sneezing, oh and that reminds you, can you please have the flu jab you made the appointment for in the first place!

Its no surprise that your gp is stressed and flustered by the end and you are almost in tears.
You are half way home and realise you forgot to ask for a months supply of pain medication and your gp has forgotten you wanted referring to another specialist so you have to chase him up about it in a few months time when you realise the referral hasn’t been done.

GP appointments don’t have to be like this. Your gp is your front line guy, the one in charge of your medical care and the guy you HAVE to have on your side right from the start.
Your gp and you should be a team working together not at loggerheads fighting and dreading every meeting.
Quite often I hear about EDSers having gps who started out ok but then they start having problems and eventually they have to move on and find another:

“He used to be really patient with me and very understanding. He would really listen to my concerns and always refer me to the specialists I needed. Now he rolls his eyes when I walk in his office and I’m having a hard time getting him to re-evaluate my pain medication”

I myself am having similar problems. My gp was very patient with me and never questioned my requests for referrals or treatments. The past few appointments however have had me in tears and him not making any changes to my medications. Iv even been told I can’t be referred to an essential specialist for my GI issues and have to stay within my local PCT when he said previously he would refer me to the specialist once my rheumatologist gave him the nod.

I understand where I have gone wrong though and caused my own problems. I’ve learnt through my own mistakes what needs to be done to ensure the best chance of having a good gp appointment.
I have now found a great female dr in the same practice which works even better for me as I can talk about the more sensitive female issues of the condition and be physically examined easily rather than the previous dr going on what symptoms I am describing alone.

So how do you give yourself more chance of a good appointment?

Here are a few tips…

1) Prepare, Prepare, Prepare.!! It’s almost like what teachers would say to you getting ready for a big exam. ‘The more you prepare and revise the easy the exam is. And the same is true for dr appointments.
If you just turn up to the appointment wanting to try new pain medication but don’t have any ideas which ones your gp has to work out which ones might be suitable for your condition, and that’s when he’s figured out if its for muscular pain, neurological pain, joint pain etc. If you have gone to EDS forums or asked other EDSers what pain meds are popular for EDS, work well and have little side effects you can suggest these to your gp who then has just a couple to choose from rather than hundreds for you to try.
Another example…you are having a lot of stomach problems and you know that this is related to EDS and you need reffereing to a gastroentologist to get it checked out. You just turn up to the appointment and say ‘I need reffereing to a GI dr’. Your gp has no idea if any of the GI specialists in the local area have even heard of EDS never mind know how to treat the GI issues. And that’s if your gp even understands this side of EDS. If you look into this yourself before the appointment you can find out what GI issues can occur in EDS, why they do, what causes them and what type of GI dr you need(functional GI, Neuro GI, etc) what tests you require if you gp can refer you straight for these (colonoscopy, slow transit, bariem xray etc).

2) Book yourself a double appointment if you have more than one (or 2 simple) issues to discuss. A single appointment is usually between 5mins and at most 7mins. Giving you both at least 10mins gives you more time so you can discuss things in full and not be rushed.

3) Write a list of the things you want to discuss. You can either hand this to your gp at the appointment or refer to it yourself. You can write notes next to the items on the list if you need to such as side effects of the medication you want reviewin, hospital details if you are asking for a referral, brief history of what happened and symptoms if you are wanting a injury looked at.
At the end of the appointment you can go down the list quickly to make sure you have covered everything.

4) Take a notebook and pen to write things down. When you have a lot of things to ask your dr he will have a lot of things to say in return. Its most likely by the time you get home you may have forgotten the correct dosage for your new medication or the name of the condition he mentioned they wanted you tested for because of your new symptoms. You can also ask your gp to write down names of over the counter medicatoin they recommend or website links etc.

5)Always take some information leaflets on EDS/HMS You can hand them one and say ‘I just wanted to give you this in case you wanted to look into anything specific on EDS or want to pass some support info on to someone else’
It’s a sneaky way of making sure they have information on your condition in case they know less than they let on. Support organisations tend to provide much better information on EDS than medical journals in terms of living with the condition so it will also provide your gp with information should they be asked to provide evidence for your disability benefit assessments.

6)If you don’t already have it, get Dr Brad Tinkle’s book ‘Joint Hypermobility Handbook’ A guide for the issues & management of Ehlers Danlos Syndrome hyper mobility type and the hypermobilility syndrome’ It covers pretty much every part of the body affected by EDS and is laid out simply and straight to the point. I take this book to all my appointments so that if I need to I can give my dr the relevant page/paragraph to quickly skim over. It also provides web links for further reading.

7)Don’t be afraid to challenge your dr. but don’t over challenge! If they are dismissing your concerns or putting an issue down to something else eg loss of appetite or digestive system problems down to depression or weight concerns, don’t confirm their lack of understanding by submitting to them.
Stand your ground if you believe your EDS is causing issues and needs looking into.
Researching any new issues before your appointment and being prepared will really help you here, especially if you can provide some printouts from the internet or a relevant page from Tinkle’s book.

‘Im sorry but I know this isn’t depression. EDS does cause problems with appetite and eating. Here in Dr Tinkle’s book its says about early satiety’.

If they still don’t take it seriously and still put it down to something other than EDS don’t get into an argument. You need a good relationship with your dr. Drs don’t often like it when the patient knows more than them so you have to handle it correctly. Something like..

‘OK,I know this isn’t depression. It is EDS causing this. But I don’t want to ruin our good relationship we have built up over it. Can we come to an agreement that if things haven’t improved in 2 weeks/a month we look at it again?’

You might face the issue of a stubborn dr like many of us have at times but if you are politely assertive rather than angry and argumentative you have much better chance of winning.
I’v had 2 appointments recently with different drs where I’v had to really stand my ground and fight. 1 eventually gave in after 20mins of telling me I was wrong, my body wasn’t doing what I was saying it was and that my medication hadn’t stopped working because of interaction from another. He was so assertive that he had ME convinced I was wrong! But I stood my ground calmly without it turning into a argument or battle of wills. I just kept saying ‘but how come I am experiencing this symptom if my body is supposedly not doing that?’, ‘can you explain again how my medication can still be working if I am experiencing this?’ etc.I asked them genuinely not ‘well explain this to me then clever clogs!’
Eventually he couldn’t explain it and had to quickly research my medication and discovered I was actually right (by this point I was convinced he was right and I was wrong!).

The other appointment I had where I had to challenge a dr was when my gp refused to believe my loss of appetite was related to my EDS. He believed it was to do with depression and being worried about weight. He just kept saying ‘Why are you telling me you are worried you can’t eat, just eat. Its not hard, put something in your mouth and swallow it’. If you are depressed you loose your appetite but you just have to force yourself, you are going to make yourself ill if you don’t eat’.
I didn’t win that one because I ended up in tears.
Keep your cool, keep collected but be prepared to loose some battles. You just have to go back and try again another time.

DO NOT PUT YOURSELF AT RISK HARM

If you believe something is seriously wrong and needs urgent attention and your gp is not taking it seriously see if you can get an appointment with another gp at the surgery. Otherwise you can go to a NHS walk-in clinic and ask to be seen by the on duty dr or go to A&E department.

8) Be honest with your gp. If you don’t want to be referred for physic say so. If you are unhappy with how your last appointment went, tell him. Your gp will appreciate your honesty.

9) Always be prepared for the appointment not to go how you expect. What will you say if your gp says he won’t refer you to the specialist you want to see? What are the other options of medication if he says you can’t have the one you wanted because it interacts with others you are on?

10)Drs are people too and have bad days like the rest of us. Sometimes that means you can be in the firing line and this can result in a disastrous appointment through no fault of your own.

For example my last appointment with my gp went something like this…

I enter the room, he’s sat at his desk not waiting at his door like usual. He’s red in the face and not smiling like normal. it’s a few days after I was admitted to hospital overnight after a faint and concerns from an on-duty Consultant of possible POTS(postural orthostatic tachycardia syndrome) resulting from my EDS.
I have my discharge letter which asks my gp to do regular monitoring of my heart rate and blood pressure and recommends a referral to a vascular specialist if I have another fall for Tilt Table testing (standard POTS test). It also says Iv been referred for an endoscopy due to concerns for my loss of appetite and swallowing difficulties. The on-duty dr tells me to see my gp as soon as I can to discuss his recommendations and ask again for a referral to the GI specialist who specialises in EDS.

My gp asks what I want. I tell him I was admitted to hospital after a fall and Iv come with the discharge note to discuss the recommendations. He has a quick read and says ‘I don’t understand, what do you want me to do?’

I tell him that the dr thinks I may have POTS and wants my blood pressure and heart rate monitored regularly and a referral to the vascular specialist If I faint again.

He replies ‘whats POTS, Iv never heard of that, is that just fainting or something? I’m not going to do your bp and heart rate, what do I need to do that for you fainted cos you’re not eating. Just stop being silly and eat. Of course you will pass out if you don’t eat.’
I am just staring at him in shock as this is totally out of character.

He continues ‘Are you worried about your weight is that why you’re not eating?

Im crying at this point so he shoves a box of tissues towards me and rolls his eyes.

That was one of the worst appointments Iv had and it was with a gp who I’v had nearly 3 years of brilliant support from. It just goes to show that drs can have really awful days too and unfortunately we sometimes get caught up in the firing line.

You have to just accept it was a bad appointment, cut your losses and go home.
Be polite as you leave. Don’t get angry or react to his bad attitude. Thank him for his time, smile and hold it together until you are out of his door.
Try not to let it affect you and especially don’t give up any of the concerns or requests you had.

Simply make another appointment at a later date if you feel you just caught him on a bad day or think about trying a different gp at the same surgery if you can.
Sometimes we can only go so far with a dr before we have to move on.

GP appointments for people with EDS can be quite an ordeal, they can be like doing a round of ‘Weakest Link’ with Anne Robinson or they can be like popping round a friends house for a cuppa and catch-up.
Whatever type of appointments you have remember your gp is crucial to your medical care so preparing well for your appointment and working as a team, not against them is how to have a good appointment.

cx

GI Joe!...the unrelentless gastric EDS soldier

Gastroenterology is a word that many with EDS become fluent in rolling off their tongues. Secondary to joint instability, the digestive system can be the most problematic system in the EDS body for some.
It is now becoming more accepted amongst the sceptic medics that EDS, or Hypermobility Syndrome is more than just 'being bendy' because of this increased awareness of the severity of GI complications in EDS patients.

The GI system, or digestive tract, is one long tube made up of lots of rings of muscle. You put food in your mouth, chew then swallow. The food enters this tube of muscular rings where it is squeezed down the tube by the muscles contracting in turn. It is kind of like when you are trying to squeeze the last blob of toothpaste out of a tube.
In an EDS affected digestive tract the muscles loose their tightness as the collagen breaks down. When they do squeeze they don't do so enough to push the food along. The food then is then simple passed through the system too slowly. In extreme cases the system may not be able to move, or function enough to tolerate solid foods.

When food reaches the stomach it can take an EDS stomach longer to do its job or it can not work as well by thinking it is already full. This causes mixed signals so that the person thinks they are full or is unable to eat decent amounts of food. It can also take the stomach longer to empty. The top of the stomach might not close properly making the contents come back up into the oesophagus or the stomach (reflus) or in extreme cases, fails completely and is unable to digest solid food at all. In this case it is sometimes necessary for the person to have a PEG or GI tube fitted so they can be fed directly into their stomach.

When the food finally leaves the stomach it enters the small and large intestine (or bowel). Here it is passed along using the same muscular method. This time squeezing the food along while digesting absorbs nutrients and water and turns the food into stools (faeces) ready to be eliminated from the body.
In someone with EDS that affects this part of the system, the muscle weakness can cause slow transit, constipation, ibs, cramps, pain, and in severe cases the bowel can become unable to move the waste along at all. Finally the rectum and anal muscles can be affected which can cause incontinence or inability to expel stools.
In the severe end of the lower GI symptoms the results can lead to the person requiring irrigation treatment (inserting catheters into the rectum to fill the bowel with water to flush the lower GI system. When the top part of the lower GI system becomes affected the irrigation is sometimes done through a tube that is surgically inserted through the upper adomen wall). If irrigation fails then the bowel is removed and a stoma (colostomy/illiostomy) is placed. This is in rare circumstances when the bowel no longer functions at all.
Usually if a person is affected badly in their lower GI system they may require very large doses of regular laxatives.

Other GI issues not caused by muscle weakness in EDS can include fool allergies an intolerances, hiatus hernia (where the stomach comes up into the chest cavity), pelvic organ prolapse (where parts of the pelvic organs including the bowel collapse into the pelvic floor), rectum prolapse (part of the bowel falls out of the body through the anus), severe and chronic acid reflux, constant nausea and vomiting.

The majority of people with EDS who have GI problems will only have the milder end of symptoms that can be managed with diet or medication. However it is becoming more recognised now that the GI problems that were once thought to be unrelated are caused by EDS or the Autonomic Dysfunction that EDS patients also tend to have.

I have always have lower GI problems since birth. I suffered from slow transit and a number of impacted bowels as a child.
I have been using irrigation treatment for over a year now which is no longer really effective. My bowel has started to collapse in areas which causes immense pain and blockages that make the irrigation even less effective.

I was very lucky to meet a neurogastoentologist who has taken an interest in EDS patients. He believed at the time that the upper abdomen tube to irrigate through could be an option before bowel removal however it has taken nearly 6 months to convince my GP to refer me to him. My lower GI problems are much worse now but I hope that this is still an option by the time I finally get to his clinic.

I also have upper GI problems now. Mainly I have no appetite now. When I do force myself to eat I can only manage very small amounts and the food takes its time getting down into my stomach. I sometimes have a hard time swallowing and can feel and hear 'clunking' in my oesophagus when I have to gently squeeze and rub my neck to encourage the food down.

I had an endoscopy last week to check for anything none-EDS. I am very scared of my upper GI system failing in the way my lower system hasso I was hoping they would find something non-EDS that is easy to treat. However all they found was some inflammation in my stomach which could be caused by a bacteria they also found. Neither of which would cause my symptoms.
It looks likely that my upper GI symptoms are EDS, particually muscle weakness.

I have a couple of friends with EDS who have serious GI problems. One has the hypermobile form of the condition like me, the other has the vascular. Both have feeding tubes and have had their bowel removed.
I have lots of other eds friends who have GI problems but none have got to needing irrigation treatment or it failing like me.
It does worry me that I may be coming close to the end of the line with options in terms of what can be done for my GI problems.

This issue of my blog was intended to raise awareness of just SOME of the GI issues that can happen with EDS. I hope that it has helped others understand just how serious this part of the condition can get and encourage those with EDS who are experiencing GI problems to look into this area and ensure that your Dr understands that your GI issues could be due to your EDS and should not be ignored.

End of 2010, where I'm at.

Its been quite a year bendy-wise.

I thought I'd do a quick recap just to see how far I'v come in just one year.



Jan-Mar
*Xrays and Mri of spine show curvature of spine and degeneration of SI joints :(
*Assessed for my 1st wheelchair - sad times.
*Start investingations for Crohns Disease - come back clear - Yey!
*GI dr and EDS dr discover my Gastointestinal system is now affected by EDS.
*Reffered to endocronologist for suspected thyroid damage from medication
*Begin physio and Occupational Therapy at Leeds.

April-June
*Recieve my wheelchair. Amazed how much freedom I now have. :)
*Getting physio back at hartlepool. knees and hips getting weaker. Walking less.
*GI nurse and EDS dr agree lower GI system failing. :(
Start wearing ankle splints and hip belt to help walking.
*Using wheelchair indoors at least 3 times a week now. Standing becoming difficult.
*Pain med review no.8

July-Sep
*Pain becomes much worse.
*Pain meds review no.9. Introduce Neuropathic pain medication
*Start working with new physio who is keen to learn about EDS
*Try accupuncture for pain which triggers major pain flare
*Reffered for hydrotherapy
*Stomach gets symptomatic. Begin part-liquid diet.

Oct-Dec
*Begin Hydrotherapy with great success. Pain reduced and core stability improved.
*Major hip dislocation trigging escalating hip and pelvis instability
*Hip continues to dislocate and subluxate evenutally causing impingement and nerve damage.
*Referred to Orthopaedic surgean for both hips.
*Walking deteriates to point of needng chair outdoors full time and indoors most days. Unable to self-propel outdoors and sometimes indoors. Refferd for powerchair assessment.
*Begin fainting and admitted overnight after paramedics called after bad fall. Blood pressure very low and heart rate topping 143bpm when standing and 110 sitting. Dr on duty suspects Postual Orthostatic Tachychardia Syndrome. Rheumy agrees continueing readings and symptoms suggest autonomic dysfunction and requests further tests with possible reffereal for POTS testing.
*Rheumy requests refferal to Prof Aziz in london for GI isssues
*Endoscopy booked for 5th January for upper GI issues.
*Reffered to pain clinic.

Looking back its clear in which direction my EDS is heading for now. However I have found that even though my condition may have become worse over the past few months, my abiltiy to cope with it and the amount it affects my ability to get on with my life is very differnt to what it was at the begining of the year.

I wish all my blog readers and fellow EDSers a wonderful and healthy 2011!

cx

New Rheumy, New hope?

I recently saw my new EDS rheumy(rheumatologist)
For people with EDS, the rheumy is the top of the food chain when it comes to our long list of drs and medical professionals we see regularly.
He is the guy who is the EDS expert, the one who understands what EDS is, how it works and how it affects our different body systems...that's when we are lucky enough to finally find a rheumy who specialises in EDS!

The EDS rheumy is the wedding planner of our medical care. He coordinates all the other drs, decides what should and shouldn't happen and pulls all the other drs' tests, work and ideas together to get a full picture of what is going on in the EDS body. He then rolls out orders to the GP (family dr) to refer the eds patient to new specialists if needed, order more tests, start new or change current treatments and keep him up to date with what is going on. He will also contact other specialists working with the eds patient either directly or through the GP to discuss current treatment with the aim to either stop proposed treatment/surgery (an orthopaedic surgeon may think ligament tightening surgery will stop a joint dislocating but a EDS specialist may suspect that it will not with this patient), suggest treatment or avenues of investigation the other specialist hasn't thought of, or simply just to keep uptodate or create a new network with the specialist for future patients.

My previous EDS rheumy was one of the country's (and world's) leading EDS/HMS experts. Professor Howard Bird has made some of the most important discoveries and moved the research and treatment of eds/hms forward massively since he began taking a special interest in the condition in his early career.
His retirement shook the eds/hms community and the proposed closure of the specialist clinic that he had set up in Leeds, UK was feared.
I had been lucky enough to have been a patient of Prof Bird for 1 year before his retirement. Although I had received my diagnosis in 2007 I hadn't received any real treatment so was finally refereed to Prof Bird in December 2009 where I received a thorough assessment. It is here I found out how bad my body was affected, how much my joints had deteriorated due to lack of treatment and how I had developed scoliosis (curvature of the spine) and osteoarthritis of the SI joints (where the spine meets the pelvis.
It was also Prof Bird who noticed the obvious EDS characteristics in my Mum and due to taking such a detailed family history we discovered that both my parents had the EDS gene. My mother very affected, my dad only mildly. My mum has since gone on to receive treatment for pain and physio and is now waiting her initial appointment with my new rheumy.

So back to my new eds rheumy.

I had been given his details before my final appointment with Prof Bird by a fellow member of the Hypermobility Association (HMSA) who recommended him highly. I knew that with Prof Bird's upcoming retirement I would have to find a new rheumy experienced in EDS.
I was told Dr S. was a great dr and Prof B agreed that he would be the best rheumy for me.

My 1st appointment couldn't have gone better. Dr S. and his staff were very welcoming and pleasent and it didnt take long for me to relax and laugh at an unfortunate gramatical error on a recent discharge note with him.

We covered my current EDS issues, mainly my ongoing gastointestinal deteriation and
a new set of symptoms that some drs are suspecting could be indicative of Postural Orthostatic Tachychardia Syndrome (POTS), a form of Dysautonomia that can occur in EDS (see links at bottom for info on POTS)

I was in with Dr S for over an hour! I had to apologise profusely to the lady following me but she didnt mind 'at least he's thorough' she said. That he certainly is.

I then had lots of bloods taken.

I received a copy of the letter Dr S wrote to my gp within days.

He has requested a referral to Prof Aziz in London for my GI issues with a referral to a local GI consultant with knowledge of EDS for long term monitoring.
He has also requested that I have a number of tests done before I see my endocrinologist at the end of January. These are to look for other causes of my dropping blood pressure and raised heart rate that suggest POTS just in case.
Finally Dr S has requested copies of all correspondence from physio, orthopedics, pain clinic, endocrinology, past GI investigation and recent xrays, mri (hips and spine) and an endoscopy I am due to have next week. (To see if there is anything non-EDS causing my upper GI symptoms).


I will not be having regular follow-up appointments with Dr S as I already spend at least 1 day a week at hospital with appointments. Plus Dr S is trying to set up a eds/hms clinic and it is not financially viable to offer regular follow-up appointments to all patients.Instead Dr S said I can phone up and ask for an appointment if I have any serious issues or I can arrange a phone appointment.

I am looking forward to seeing the eds/hms clinic taking off with Dr S as it is very much needed in the North East of England.
Dr S admits he may not have the knowdlege and expertise of Prof Bird, after all he is only young and just starting his work with eds/hms patients, but he is very open and the most important thing is that unlike some drs, he will take the patient's suggestions and concerns when deciding on appropriate progression of treatment.

I am really looking forward to working with my new rheumy for the coming future and hope that as one of his patients that he will learn something from me.

links for further info :
Postural Orthostatic Tachychardia Syndrome (POTS) - http://www.dinet.org/pots_an_overview.htm

Deck the halls and all that stuff!

Hi everyone,

First thank you all to everyone who has contacted me to let me know that my blog is helping others to understand EDS/HMS and also offering comfort to fellow bendies.
Its hard sometimes to come on and write about the month I've had but now that I know that it is helping others I have more reason.

So how have things been since October?
Still waiting to be rehoused and currently living in my extremely bare flat having packed everything ready for my proposed move a few weeks ago. Still here and possibly for another few months but loving having very little to tidy or trip over!

I enjoyed a wonderful birthday, my favourite present was the entire works of Shakespeare found in an old 2nd hand book shop from my boyfriend.

So on to the health stuff...

As predicted the winter is hitting hard and its been an interesting few weeks trying to keep up with my EDS.

My physio became very concerned about my hips after they started to subluxate (partially dislocate) repeatedly during the day. An x-ray has shown 'some structural abnormality' but until the official report comes back from the radiographer they won't tell me what exactly is 'abnormal'. There are a few hip issues that are more common in EDS due to the increased friction from the movement in the joint or from the recurrent dislocations/injuries.
I see a specialist physiotherapist in a few days who needs to assess my hips and pelvis so they can refer me to an Orthopaedic surgeon who will then decide if surgery is needed.
The pain in my hips has become very bad and touching my upper pain threshold so I have finally be referred to the pain clinic.

Last week I passed out and fell badly in my kitchen. It was the first time I used my falls alarm and now realise just how essential it is. The nurses got to me quickly and I was sent to hospital for xrays and pain relief. I expected to be sent home that night but my heart and blood pressure weren't behaving and I was kept in overnight.

My heart rate and blood pressure were monitored through the night and in the morning I was told that I could possible have POTS (Postural Orthostatic Tachycardia Syndrome). This is a condition that basically means your body has become allergic to gravity. It affects the Autonomic system which controls heart rate, blood pressure, temperature control and other essential systems. There are various reasons people develop POTS including as a result of a primary condition, in my case EDS.
If the falls continue I will have to be tested for POTS.

Hearing this was not especially great as I know how bad POTS can get and also how it can cause more complications in EDS.

For more information on POTS see the links at the end of the blog.

Generally I feel really rubbish and think this has to be the worst I've been so far with EDS. However I have been through so much worse in the past and know that whatever happens it's how you approach the challenges that determine how they affect you. As usual I am staying positive (although a little harder than usual), and focusing my energy on just keeping safe and warm over the next few weeks.

I hope that you are all having a great snowy week. Please comment and continue to share your EDS stories. If there is anything you would like me to discuss in a future blog please let me know.

Hugs and prayers to everyone

Cx

More info:

What is POTS? - http://www.dinet.org/ -POTS place. info on POTS and other types of Dysautomia.

POTsgirl's video - http://www.youtube.com/watch?v=OvENfW6scZk - great video explaining POTS simply and to the point.

Time for a catch-up

It has been quite some time since I last blogged about living a bendy life. Alot of things have happened in the few months that have gone by. Some very positive, some not so positive.

I am in the process of moving to a new home and the usual stress involved with such a huge expereince is having quite an effect. So I thought I would use this as an excuse to come and let you all know whats been going on in the past few months and what the future might have in store for me.

First of all some non-bendy news

I have been on the council housing list for some time slowly climbing my way up to getting my own little wheelchair friendly bungalow. It has taken 7 months to get to the point where I can say that I may have somewhere before Christmas. However this week it's all gone a bit pear shaped.

The housing people have decided that you can either be a full-time wheelchair user or you only use your chair outside so have no need for an accessible home. I use my wheelchair indoors alot now as my mobilty and condition continue to deteriate. However I can still stand and I can still walk around my flat, on some days very well, so am not classed as a full-time chair user. As a result they have decided that I am no longer a priorty for a wheelchiar friendly home. I have been dropped right down to the bottom of the housing list and told it would be at least a year before I am at the top again.

Thankfully God has been working his wonderful ways as usual and an extremely kind property manager has offered to put me at the top of his waiting list for a private rent bungalow in a village just outside of Hartlepool.
I love the village and spend alot of time there at my boyfriend's home so it is perfect. However because life is never that simple it has caused a few issues...

The village comes under Durham County Council so I have to give all the equipment I have from social services back (which includes a bath lift, special perching stools for getting a wash/working in the kitchen, and my 'falls alarm'). I then need to refer myself to Durham social services to be assessed by them who will then decide if I can have similar equipment from them. This could take anything from a couple of months to a year to happen so it means going back to basics in terms of managing day-to-day and also looking for some 2nd hand equipment I can get hold of myself.

Obviously that is a big pain but nothing serious. However there is a chance I may have to give my amazing wheelchair back also and be reassessed by Durham Wheelchair Service. I have been extremely lucky to get the chair and without it I am very limited in where I can go and what I can do. I can't imagine my life without my wheelchair and it's not till now that I realise just how much it has changed my life. I feel quite humbled to realise just how grateful I have been to have it and how lucky I am compared to alot of people with EDS who are still fighting to get wheelchairs.

Like any big moving of home I'm dealing with all the issues of bills to be paid, bonds to be found and the financial and other aspects that are involved. The stress of moving home is said to be at the top of the list and like anyone else I am starting to feel it.
For me, and for other people with EDS, stress works a bit differently.
I'v had a couple of tears like anyone would when it feels like you can't quite climb that huge mountain in front of you (until you have a cuppa and some chocolate and you are ready to take on the world!). However its more than a short temper and a few tears that I am experienceing.

Most people with EDS have cfs/me (chronic fatigue syndrome) and/or fibromyalgia. see the end of this article for links to some great info on what these are
People with CFS/ME and/or fibromylagia experience different effects from stress where stress causes physical symptoms. Usually this is a flare-up of their usual cfs/me and/or fibromyalgia.
For me its the fatigue that is being triggered and as a result I am in that place where it seems I see more of my bed than I do my friends.
Thankfully it's been over 6months since I've had this level of cfs/me flare and again I am reminded of how lucky I have been the past 6 months.
I am in a much better place to manage the exhuastion as I now have a wonderful partner who seems to know better than I do what I need and how to help.

Right now I know that the next few months are going to be tough but I'm ready to take them on and have the right support and mindset to be able to look forward to the challenge.

Now for the bendy stuff...

It's been quite a year not only for my Ehlers Danlos but also for the hypermobility community as a whole.
It has finally been agreed by the amazing drs who specialise in Ehlers Danlos and Hypermobilty syndrome that they are in fact the same condition. Before now it was often the case that someone diagnosed with Ehlers Danlos Syndrome would manage a little better at getting the right treatment and support and have their condition taking seriously by medical professionals. However someone with a diagnosis of Hypermobility Syndrome would often be told that its not a serious condition, 'you are just bendy' and would have to fight, often unsuccesfully, to get even the basic pain treatment.
Now that Hypermobilty Syndrome is recognised as the same condition (but just a difference in label) as Ehlers Danlos Syndrome, it means that hopefully the condition will continue to get recognised and treated effectively.

As a result of this huge milestone I can now officially say I have Ehlers Danlos Syndrome. It might seem that its just a label and means nothing but already I am seeing a difference in how I am being treated by my drs and physiotherapists.

Treatment has moved in a new direction in the past few months for me. Physio is now focussing on pain relief as it seems that building up the muscle around my joints isn't working. My hips have now started to 'turn themselves off' and are rolling inwards alot. This is affecting my walking and standing alot. I am currently having hydrotherapy with my physio. Its doesnt seem to be having any major impact on my muscles or joints in terms of strength or condition but being in the warm pool is helping with the pain.

I have also been trying new pain medication with the focus on neuropathic pain. This is pain caused by recurrent injury damaging the nerves making them constantly fire off pain signals. It's very annoying being in pain when you know that its simply the nerves getting mixed up and there's actually no injury.
Pain medication for this tends to be the same as that for epilepsy. It works by dampening the nervous system. Due to my sensitivity to certain medications and interactions with other treatments it's proving quite difficult to find something that works. I am simply going to keep my fingers crossed that the right combination of medication is there waiting to be discovered.

I recently attended the Hypermobility Syndrome Association residential. This was a chance to meet up with other bendy people and to listen to talks by the wonderful drs who dedicate so much of their time and career on helping us.
One dr, Dr Aziz, specialises in the gastrointestinal problems that occur in people with EDS. It was quite an eye-opener to discover just how serious this part of the condition can be. My GI issues have been quite bad this last year resulting in my bowel giving up and not functioning. As a result I now have to use an irrigation treatment to keep my insides functioning.
It was a bit of a relief to hear that this can be the eventual treatment for some people with EDS so I am not alone. I had chance to speak to Dr Aziz after his talk and he suggested that in the future I may need to adapt this treatment by having a tube through my tummy as the upper bowel stops functioning. He also said that it sounds like there is alot going on inside me that needs looking at properly so I will be getting reffered to his specialist clniic in London. Again all fingers are crossed that its not as bad as it first appears.

The amazing Prof. Bird has retired from Leeds Hospital where he led an amazing team of drs, physios and other staff in managing the care and treatment of alot of people with EDS in the UK. I was lucky enough to be under his care for the past year and it is becuase of his knowledge, expertise and understading that I have moved forward so much with treatment.
I am now awaiting my first appointment with a new rheumatologist in Newcastle who will take over where Prof. Bird left.

As the wnd of the year slowly creeps up on us I am already enjoying wrapping up in layers of thermals and working out when to get my flu jab. I know that winter can be a tough time of year for me and my EDS becuase of the extra issues of temperature control, lower immuninty to colds and viruses and the cfs/me flares that come with burning more energy trying to keep warm. I know from last winter what to expect and already am preparing. I have dug out my thickest socks and hinting for lots of 'warm' birthday gifts.

It has been the usual rollercoaster that you ride with EDS this past few months and while some of the dips might have seemed impossible to get over at the time, my strentgh to cope and my optimism for my future continues to grow.
I know that life with EDS will always be intresting. It will be fun at times and heartbreaking at others but whatever the next 6months brings...

I amd ready!!!



cx

Links:

Simple guide to CFS/ME http://chronicfatigue.about.com/od/whatischronicfatigue/a/understandCFS.htm

Simple guide to Fibromyalgia http://chronicfatigue.about.com/od/whatisfibromyalgia/a/understandfibro.htm

Social Services and Care Support

I have just found out from my social worker today that a care plan has been approved for me to provide me with a carer to support me with personal and social tasks.

Social services have approved the following support for me:

1 hour 3 times a week for a carer to come and give me a bath and get me into bed.
2 hours twice a week for a carer to take me to do my shopping or go to appointments.
4 hours once a month for social stuff eg going for a night out, going for a girly shopping spree with my mum or friends etc.
1 hour once a week for a carer to clean my flat and do my ironing.

There is also a contingency fund so that if my condition deteriates at times and i am stuck in bed they will send a carer to get me out of bed, dressed (if possible) and give me a meal and then come back in the evening to get me back into bed.

I am so happy i can't believe how much support they have said i will get. At first it was only going to be 2 hours twice a week to go shopping and give me a bath. I really hope that getting all of this support will make a big difference. I especially feel hopeful that it will get me back out there and getting a social life back. Iv lost alot of friends over the past year due to my condition deteriating and that is the one thing that i have found hardest of all. I have one friend who visits me regulaly and takes me out in my wheelchair but as he has a milder form of the same condition, he is finding it harder to push me. I also hate my friends having to push my wheelchair or having to rely on friends to go to the bar to get drinks, or help me find a disabled loo, carry my shopping etc. To be honest, I hardly see anyone now so I don't even have the chance to ask them to do these things for me.
It will be great if I can get out more and start spending time with friends again knowing that I won't have to ask them to do anything for me other than just enjoy our time together.

My social worker has spoken to the care agency and they have carer in mind who they said should really suit me. I don't know anything about her yet other than she is young which is what I wanted. That way when we go shopping or out on a night out it will just look like I am with a friend rather than a carer or an older relative.

I feel like this is a massive breakthrough in my region as I don't think there is anyone else with hms/eds who gets care support , or at least to this level. I'm hoping that with them approving this amount of support for me it will make it easier for others with hms/eds to get better support in the future.

People with hms/eds find it extremey difficult to get this type of support due to the lack of understanding of the severity of the condition. It makes it even harder to get support becuase the condition can fluctuate so much within such a small amount of time. This often means that when people with the condition do ask for help, it is refused becuase it is assumed that they can function much better than they can. It is also hard for service providers to provide care on the basis that the support is helpful becuase it PREVENTS deteriation and injury rather than it being as a result of deteriation or injury.

Of the few hundred people I know with hms/eds from the www.hypermobility.org.uk forum and facebook, only a very small handful recieve any support with care. Those who do either have another condition that has justified they need for support or they are at the extreme end of the spectrum being completely dependant on others for even very basic tasks. They often also have children with the condition so the support they recieve is often more support for them to be able to care for their family.

I know of a few people in my town alone, and more in the surrounding area who would benefit from some kind of support from social services, either in terms of care support, or simply with loan of equipment such as bath seats, electronice raising beds etc. I feel positive that with the local social services accepting and approving this level of support for me, that it will mean that others in the area will find it much easier to get the support they need too.

Big step forward for recognition of hms/eds in the care system today!!

eds/hms videos

here are a couple of videos that look at eds/hms. Not up to posting a long blog today due to a naff day pain wise but very pleased to say i passed my driving theory exam today Woohoo!!

here are the links to the videos. enjoy!

http://www.youtube.com/watch?v=PunQu-bId1M
http://www.ehlersdanlosnetwork.org/mysterydiagnosis.html

My bendy story

Like everyone with hms/eds, I was born with the condition. There were obvious signs something was wrong very early on but due to the common lack of knowledge of the condition i did not get diagnosed until I was 27years old, by this time the condition had become quite bad.

Although There didn't appear to be any problems when I was born, I did have hms/eds symptoms as an infant. I did not walk until I was 4 years old, which is common with hms/eds, but this was put down to me developing slowly.
When I did start to walk I would become tired easily and it took my parents a few years of hard work to get me to walk normally.
I complained of pain in my legs from an early age but drs put it down to growing pains (again very common for people with hms/eds). At age 12 I started to get alot of pains in my chest. This was the start of my ribs dislocating (yes ribs can and do dislocate in people with hms/eds although alot of drs will swear blind they do not, even when they see it themselves). Again, after various hospital visits and lots of tests, it was put down to growing pains.

I injured myself very easily and was covered in bruises and sprained joints as child but it wasn't until I was 20yrs old that I myself started to think something wasn't right. I started having problems with back pain which I visited my gp for on a few occasions but this was dismissed as being 'psychological' rather than a physical issue. I also started having more and more problems with my digestive system which was put down to IBS as a result of 'stress'.

By the time I went to uni at the age of 22 I was struggling more and more with poor energy levels and widespread pain. I studied performing arts and a weekly dance class would leave me stuck in bed exhausted and usually with at least one injury. My 'IBS' became much worse and I was eventually seen by a specialist who tested me for Crohns disease (which runs in the family). However no crohns showed up so again it was diagnosed as IBS. In my 2nd year at uni I spent a month bedridden due to the back pain and stomach pain become so severe that I could not move. Eventually after lots of rest things improved and I was back to my normal self.

At age 25 things started to deteriorate and my symptoms became more and more frequent. I was finding walking tiring and was becoming more and more fatigued. Even after a good nights sleep I would need to go to bed for a few hours in the afternoon. My back pain become more severe again and the pain episodes became more frequent. Again I asked my gp for help but once again it was put down to depression, which was becoming the constant cause of my problems according to drs.

When I was 27 I was becoming less able to stand and walk. I started to use a walking stick to help me stay on my feet longer and even started hiring electric wheelchairs from Shopmobility (a wheelchair hire company) in order to go shopping. I noticed at this point that my toes were curling under each other. I thought that maybe this was the reason I was finding walking harder so I decided to visit a private chiropodist. I was fed up with my gp at this point who was equally fed up with me.
The chiropodist I saw was the first person to mention the word 'hypermobility'. I had never heard of this before so didn't think much of it when she told me the bones in my feet and ankles were very hypermobile. She gave me some orthotic supports to wear in my shoes which would lift the flat feet and support my feet. I went home and decided to look into hypermobility rather than go to my gp as she had advised. I was gobsmacked when I came across hypermobiltiy syndrome as I was like someone had written all my problems down and given it a name.

I couldn't believe how much the condition fitted my history or problems. I was amazed how I had had so many obvious signs of the condition but no one had picked up on it.
I didn't dare go to my gp and ask for a referral to a rheumatologist as advised by the information I had found because by this point the word 'hypochondriac' was beginning to appear on my medical notes. Alot of people with hms/eds are labelled as hypochondriacs or attention seekers by gps and drs who refuse to take the person's concerns seriously. When tests continue to come back normal and more common conditions are ruled out, gps often put the symptoms of rare conditions like hms/eds down to psychological issues.

I carried on as I was using my walking stick and spending more and more time in bed and less and less time doing things. After about 6months of finding out about hypermobility syndrome, my back pain kicked off big time. The pain level was so high that I was spending more and more time unable to move and stuck in bed crying with the pain. I was trying everything I could including heat wraps, hot baths, massage, over the counter pain relief and even psycological techniques but nothing worked. My hips and knees were now becoming very wobbly and were starting to dislocate. One night my knee dislocated badly while in bed and it took me 30 minutes before it eventually went back into position. The next day I went to Boots the chemist and asked the pharmacy for the strongest pain relief they had. She asked what problems I was having and she said that I needed to ask my gp to refer me to a rheumatologist to examine me for hypermobility syndrome. After another week I finally bit the bullet and made an appointment with my gp.

I took the letter the chiropodist had written for me and told him all about the problems I had had and showed him information on hypermobiltiy syndrome. He was not impressed at all. He told me that I had no right to assume I knew my body better than he did and that I had to stop trying to seek attention and to stop making things up. I couldn't believe how horrible he was. I had gone to him with loads of information and the possible key to solving the mystery of years of endless symptoms. I burst out crying as I thought, 'this is it, this is the end, I'm never going to find the answer'. Crying obviously reinforced his opinion that my problems were down to depression but he told me he would refer me to a specialist just to prove to me there was nothing wrong with me.

The day I saw the rheumatoglist was the happiest day I had had in a long time but also, just like with my gp, became a day of having any hope of help crushed.
The specialist examined me testing the movement in every joint. At one point my hip dislocated as he moved it which made him panic. Eventually after 20mins of prodding and poking he confirmed that yes, I did have Hypermobility Syndrome. I couldn't believe it! I was so happy I had finally been told it wasn't in my head.
But then it turned sour. The specialist told me that the condition doesn't really cause any real problems, that it just means you are very bendy and that it can actually be a good thing if you are a dancer or sports person. He said that the level of pain I said I had couldn't be caused by the condition and so he was doubtful that my pain was a bad as I said it was. He also said that I shouldn't need a walking stick and that i just need to exercise more and spend less time in bed. The exhaustion, he said, was also not related. I was devastated. I had found a forum on the Internet for people with hms/eds and had read that many people had experienced the exact same thing. They finally get a diagnosis but are told that its not that big a deal and to just get over it and get on with your life.
I was offered no treatment. None of the recommended things I had read that would help such as physio, pain medication, splints etc.

I went home very confused. I was so happy I had finally got a diagnosis but had no idea what I was going to do about it. I decided to change gps that day as I had succeeded in proving to my gp that he was wrong and did not look forward to having to see him again.

My condition continued to deteriorate over the following year and eventually I was refereed to physiotherapy after a very bad dislocation of my shoulder. At last I was going to get help. However I was seen by a trainee therapist who, after just 3 sessions, told me that physio won't make any difference and I was discharged.
My new gp was more sympathetic than my previous one and by this time had put me on codiene to help with the pain. He also refereed me to be assessed for a wheelchair and also refereed me to a specialist nurse to help manage the digestive system problems I was having by that point.

After a further year of my gp trying to help me, I discovered there was a dr in Leeds called Professor Bird who specialised in Hypermobility Syndrome. He had a specialist clinic of physiotherapists, occupational therapists and other staff who he had trained to treat and understand hms/eds at a high level.

I asked my gp to refer me to see this amazing dr who had developed a great reputation on the online hms/eds forum. My gp agreed.

6months later I had my first appointment with Prof. Bird. He examined me and took a very detailed medical history of myself and all of my family. He confirmed I had Hypermobility syndrome and that most of my joints were affected. He also told my mum who was with me that she too had the condition due to her medical history and from what he could see of her joints. He also said that it sounded like my father also had the condition and this is why, by this point, the condition had become very serious for myself.

After taking xrays and blood tests he refereed me to see his physiotherapists and occupational therapists.

I went home extremely happy that at last the pain and exhaustion I had were genuine and that I had been right all along to fight for someone to take me seriously. However I was also very upset that it had taken so long and as a result my joints were now in serious condition.

Prof. Bird advised my gp to put me on a high dose of tramadol and amytriptaline for pain relief. He also asked that I would be refered to see various other specialists to look into the related problems I had.
A few months later Prof. Bird contacted my gp to say that the xrays I had done showed that I had already developed osteoarthritis in my lower spine and that the Sacralilliac joints (where the spine joins the pelvis) were degenerating. Although this can be a result of hms/eds, because of the family history of crohns disease he wants to look into this again as this type of degeneration of these joints is often a result of crohns diesease.

My physiotherapy and occupational therapy assesments confirmed that my joints had become very unstable and weak as a result of recieving no treatment for so long. I was refered back to my local physio department under a senior therapist with the advice that I should recieve treatment indefinatly on a weekly basis. My new therapist is amazing and understands the condition so well.
I was given various splints for my wrists and hands by the occupational therpaist and advised to ask my social services to provide me with various aids such as a bath seat that lowers me into the bath, as well as equipment to lift me up to sitting position in bed.

I am due to see professor bird again in about 6 weeks time. I am hoping that he will have more information on my test results and has decided whether to be tested again for crohns disease.
Prof. Bird is due to retire in the summer so I am hoping to be reffered to another rheumatologist in Newcastle who also specialises in hms/eds.

I now have a social worker who is in the process of setting up a care plan for me in order for me to recieve support from a carer a few times a week in order to help me bathe, shop, and make meals.
I am also waiting for a new wheelchair that has been made for me by the nhs. The new chair is much lighter than my current one so I should be able to push myself. Its also designed for much more frequent use including for inside my home as my mobility has become less and less.

I am also having driving lessons in an adapted car so that I can remain independant.

My story shows just how quickly and rapidly hms/eds can deteriate when no treatment is offered. It shows how much damage can be done by ignoring the problems and why it is essential for gps and drs to gain more understanding of connective tissue disorders.
I am luckier than some as I am now receiving very good support and treatment. Even though it was a long hard fight, some people are still fighting.
I have no idea what the future holds but I do know that I am unlikely to be able to walk properly again and that my condition will continue to deteriate rather than improve. I have learnt alot in the past year about how treat my pain effectively with medications, TENS, and heat treatment. I have also developed more understanding of the importance of pacing myself strictly so that I use the little energy I have effectively. I have learnt not to feel guilty or upset when I am unable to get out of bed and to feel ok about not being able to do alot of things that I used to be able to do.
I feel much happier in myself and while my quality of life has reduced a great deal, I no longer suffer from depression and feel very positive about life.

I hope that in the future more drs gain understanding of the condition and it my dream that one day, people with hms/eds recieve a diagnosis and appropriate treatment very early on so that no one has to become as disabled as I am.
It is also my dream that one day a cure will be discovered so that people with hms/eds can at least be prevented from deteriating further.

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.