Wednesday, 13 April 2011

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.

April Showers and Springtime Sun

We are a quarter of the way through 2011 and for people with EDS a lot can happen in 3 months.

Last time I posted things weren’t great and I was in abit of limbo-land waiting to see new drs and move on with new areas of my condition. Since then there have been some new issues to deal with as well as increased hope in other areas.

My GI issues have continued to be a focal point in my dr’s concerns but thankfully haven’t changed a huge amount since the beginning of the year.
I had tried a new drug that is being used in women with chronic dysmotility of the GI system but had to stop taking it after one month due to complications with it. It seems my body always finds a way to dash the hopes of me and my drs when we hear of a new wonder-drug that we think will be the answer.
My GI dr at my local hospital has had to admit defeat and can no longer treat me due to his lack of knowledge of EDS and the eventual failure of medication based treatment being an option.
I have now been referred to a specialist further afield who has a particular interest in severe dysmotility and unusual GI conditions. His treatment ideas are on the ‘radical’ side involving pacemakers for the stomach and other surgeries that wouldn’t normally be used for the problem he is treating but things have got to the point of having to try anything that might help.
My stomach is struggling to digest food properly and absorb nutrients so I am starting to experience symptoms of malnutrition mainly a lot of hair loss and sudden energy slumps. I am hoping that we can find a way to get things working again quickly before I have to start buying a lot of hats!

I am very lucky to have been referred to an orthopaedic surgeon who knows and understands EDS and what that means in terms of what we can realistically acheive. I was referred for my recurring hip subluxations/dislocations and increased pain in my hips and pelvis. At my first appointment he was very honest admitting that EDS can only be helped so much with surgery and that he would not be able to get me walking again. Finding out that eventually I will be unable to stand due to the effect the EDS is having on my knees and back was a shock but knowing this now means I can finally let go of the hope that I will one day wake up miraculously healed and be running and dancing down the high-street.

We are focussing on surgery that will make my hips much more stable in order to reduce the pain and make sitting in my wheelchair much more comfortable for long periods.

I am also halfway down the very long road of POTS investigations. POTS (postural orthostatic tachycardia syndrome) was first suspected in November following a fall I had. I am lucky that 3 out of the 5 drs I see regularly suspect I have POTS now as this has meant bypassing some of the unnecessary testing and loop-holes needed to get a referral to a cardiologist for the all-important Tilt Table Test which is the primary tool for diagnosis POTS. I still hope and pray that I don’t have POTS and the symptoms I have are down to a medication or drinking too much tea but it’s becoming harder for me to not prepare for a POTS diagnosis.

My tachycardia (fast heart rate) and low blood pressure is becoming more and more frequent and I now have to try to manage other possible POTS symptoms which only aggravate my EDS. Fatigue and ironically insomnia are worse than ever. I seem to only manage an hour or two out of bed at a time without ending up exhausted and feeling like I have ran the London Marathon. Nausea and dizziness are constant and never go away now and I have developed headaches that at times are very painful. Hopefully I will get a diagnosis (or more preferred a “You don’t have POTS”) soon and treatment that can get things back under control.

I am also now just waiting for the appointment letter for my powerchair (electric wheelchair) assessment which will bring a huge amount of freedom and opportunity to my life again.

Although I’m spending more time living life from my ever-loving bed, I am still hopeful as ever that if a cure isn’t found during my lifetime that at least my Drs can work together to find a combination of treatments for the different EDS problems that will give me back some of my life. While I wait I continue to be positive using my time to help raise awareness of EDS and support others who are just finding their feet in the overwhelming world that is Ehlers Danlos Syndrome.