It’s an EDS thing!
Hi, my name is Lawrence Jordan, I’m 24 years old and was diagnosed with EDS in 2017, and PoTS in 2018.
As a baby I had 36 ear infections aged 1-2 so, at two years of age, I had grommets fitted due to “floppy” Eustachian tubes. I couldn’t sit unaided or walk so they assumed it was my balance due to the ear problems. Aged three I started to walk but kept falling over as my knees gave way, and mum said I would just fall headfirst from standing without even putting my hands out. One day my left eye just turned into a squint and, due to the fact I was falling over constantly, I was rushed to hospital for a brain scan as they suspected a brain tumour. The eye was caused by a loose muscle behind the eye and I had to have a squint correction operation.
Under a paediatric team they quickly diagnosed clumsy dyspraxia and joint hypermobility, as well as deficit of attention and motor perception (DAMP). Mum said these diagnoses came as a huge relief to her as we were constantly at the hospital with various dislocated joints needing to be put back into place, splinted or casted, and the doctors were starting to question whether my “injuries” were signs of physical abuse! My Nan, as an ex nurse, could vouch for the fact that ‘we were just putting on his coat when his wrist/arm popped out’. They still said these injuries were not normal.
The journey to starting school began and on the first day I remember a girl in the playground holding my hand to guide me out of the way of a delivery van and again, my elbow clicked and wouldn’t go back in. The poor girl cried more than me as she thought she had broken my arm! Mum met me at the hospital in a sling! This was to be the first of many incidents. After a few years at mainstream school they couldn’t cope with the dislocations and falls – one which ended in a concussion – and said I couldn’t go out in the playground for my own safety.
Being statemented, I was supposed to have been provided with a Bambach chair from occupational therapy (which I never did receive) as I kept falling off the school ones. I should also have had daily physio, which I got once a week if I was lucky! I had no friends as I was in my ‘own world’. I still couldn’t write, let alone join the letters. I felt very isolated and was called a ‘retard’.
I was finally moved to a special needs school where the teachers didn’t mind that I couldn’t write, and I got my physio and Bambach chair!
Operations and referrals
After many years of dislocations – mainly on my knees, wrists, elbows and ankles – numerous splints and plaster casts, and the paediatricians being puzzled about the severity of the hypermobility, I was sent from Maidstone, where I was living, to Tunbridge Wells Hospital. Now an adolescent, it was decided that they would need to operate on my knees because they just slipped out and my walking was also really difficult. They would operate when the growth plates had closed when I reached 18 as the dislocations had worn away my ball and socket joint. They started on my right knee in 2014.
I had a medial patellofemoral ligament (MPFL) reconstruction using some of my own hamstring to pin the patella in place but within six weeks it dislocated again. Suspecting more than hypermobility now, the consultant sent a referral to University College London Hospital and six months later I received a letter stating they couldn’t accept the referral from my senior orthopaedic consultant at Tunbridge Wells as it must from a rheumatologist (which I didn’t have).
I was told the NHS waiting list for a rheumatologist was a minimum of eight months. In 2018 my Nan decided to pay £250 privately for a rheumatologist in Maidstone who looked at my medical history and asked me to summarise what I needed him to write in the referral letter. He also said it could still take up to a year to be seen at UCLH so advised us to pay privately in London to see a colleague who would get things moving a lot quicker. Thanks to my Nan again – as mum has a very limited income with only carers’ allowance – I went to see Dr Alan Hakim at St Johns Clinic, who was incredibly helpful. But he was sorry – we had been sent to the wrong place, he hadn’t worked with UCLH for years. I thought ‘not another waste of time, I’m still not going to get into there’, but I was wrong, Dr Hakim did help with getting seen by the right doctors and gave me a full examination – highly suspected EDS. On handing over the envelope of money at the end of an hour long appointment Dr Hakim said “I’m so sorry you’ve been messed around for so many years” and refused to take the money, for which we will be eternally grateful.
He referred me to the rheumatologist at the Royal National Orthopaedic Hospital at Stanmore for an official diagnosis of EDS. The consultant quickly offered me a three week stay at the rehabilitation unit due to the severity of my condition and referred me directly to Mr Patel at UCLH who offered me a more stable operation on my knee (this time using donated ligaments). Although not 100% assured of success, it was worth a try as my mobility is severely restricted and extremely painful. The surgery was to try to improve stability but wouldn’t necessarily give pain relief.
Three months before my next operation in October 2018 we moved from Maidstone to East Sussex to be close to Battle station giving easier access to London for all the ongoing appointments. To my delight, in our local GP surgery is a musculoskeletal expert who had patients with EDS when he worked at Guy’s Hospital. It was so refreshing and assuring. Dr Meilak said “I’m so sorry you have this nasty, complex and painful condition and I will do everything I can to support you”, and he does, in every way and I never want to move now I have a GP who truly understands.
The operation in 2018 was a right knee medial patellofemoral ligament reconstruction and tibial tubercle transfer. This was when PoTS was diagnosed, following complications with my heart during and after surgery. My recovery was slow but at least that explained the constant cold sweats and always being cold and feeling like I might pass out sometimes when I get up! I thought it may be an EDS thing – turns out it was!
Four weeks after surgery I went back for my post- op check. The wound wasn’t healing well and I hated the bumpy ambulance journeys. It turned out my shin was broken. Mr Patel couldn’t believe I was holding a normal conversation with him and not screaming in agony – I said “it’s an EDS thing, I’m used to pain!”
I needed emergency surgery to have the screws in my shin taken out and a plate fitted. Four weeks later after my follow-up, again there were complications, this time with my body rejecting the plate as it was discovered the bone was very brittle and a third operation would be needed to remove the plate. I just felt sorry for Mr Patel! He’d tried so hard to give me a better quality of life and is a wonderful consultant, it was just my body being awkward.
The wound really wasn’t healing well and opened up in several places, so weeks later I was hospitalised and on IV antibiotics for an infection which went on for months. In November last year I had a fourth operation to investigate the rupturing scar, clean it out and have a biopsy done. Today, two years after the first operation, it still hasn’t healed properly and I am waiting for another referral to a rheumatologist as it’s been over a year since I was seen by one.
My knee stabilised for a short while but in the last three months has since suffered two nasty subluxations. I can’t face having the other knee done now but have no regrets at giving it a try thinking it might have improved my mobility and quality of life.
The stress of benefits claims
Just before we moved in October 2018, I had my PIP assessment. I’d been on higher rate disability living allowance (DLA) with both mobility and care components indefinitely since I was a child. Having gone to a special needs school, all assessments were dealt with there by the doctor who visited, but on reaching adulthood things became very challenging as I tried to work part-time (9 hours a week) at my local cinema. After a few months of dislocations, I was considered a liability and advised by my then GP to give it up as I was doing more harm to my joints than good. I was tired all the time as I was constantly in plaster casts so couldn’t have physio. He told me to apply for ESA, which we did. Having been denied and appealed it went to tribunal in 2016. After 10 minutes with the very understanding judge and doctor, he said “I don’t know why you’re here, this is overwhelming medical evidence” and I was awarded support rate ESA.
When it came to the PIP process I assumed they would see I had already been through a tribunal and given higher rate ESA, so it would be straight forward using the same “overwhelming” medical evidence, but I was only awarded six points for daily living and four points for mobility so no PIP was awarded.
After the mandatory reconsideration we went through 18 agonising months living on just my ESA as mum’s carers’ allowance stopped, as well as my DLA. Waiting for the tribunal date, Mum had to deal with everything as the stress of the paperwork, and not being believed, really exasperated my PoTS symptoms – and my writing is illegible! Unfortunately, mum couldn’t cope with the stress either and suffered a nervous breakdown. When the community nurse came to change my dressing she was so concerned that we couldn’t afford to put the heating on or buy food, she helped get us brilliant support from social services and the community nursing teams, as well as my GP and our local church.
Financially, without relying on the kindness of family and friends, we would be in dire straits. We were so overwhelmed and happy with the support, but what about those who aren’t so lucky and don’t have family to help? Thanks to all the agencies involved with my care during this time I now have a P.A for nine hours a week, which my social worker arranged, allowing mum to go out and me to spend time in different company, which I thoroughly enjoy.
I still couldn’t understand why an appeal for PIP was disregarded when my GP wrote a letter for them as supplementary information stating “I have supported an appeal as the patient continues to be severely affected with multiple joint dysfunction and arthralgia, requiring extensive medication in order to manage the levels of pain. I am of the opinion that my patient is indeed severely affected by his connective tissue disorder and deserves and qualifies for the highest level of financial assistance”.
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My consultant also wrote a letter – which was disregarded – stating “Lawrence has a background of EDS characterised by frequent dislocations and subluxations of his joints, predominantly effecting his wrists and knees as well as elsewhere and causing him significant patellofemoral instability. I would be grateful for assistance with regards to his care because obviously he lacks a significant amount of mobility for the foreseeable future, and he is going to need ongoing operations in the future as time progresses. He will remain at high risk for dislocations due to his underlying connective tissue disorder of Ehlers-Danlos syndrome, and this is associated with chronic pain throughout his body”. A third letter – a report from social services who carried out an independent assessment – was very long and would probably take up half a magazine!
I wasn’t able to attend the tribunal this time, in January 2020, as I was post-op and unable to travel, so mum represented me and was questioned for over an hour, then called in after half an hour waiting outside for the verdict. I was awarded both the daily living component and mobility component at the standard rate. When mum questioned why the lower rate for mobility, the judge said they couldn’t use any of the new evidence which was sent in over the 18 months that we waited for the tribunal, and it would need to be used in the next application for PIP in 2022. My mum said it is not a new condition, it is all to do with EDS which was already diagnosed. PoTS is part of the condition. The judge said ‘well, you’d be better off taking the lower rate as another appeal would take another 18 months anyway, which will take you to nearly 2022!”
The freedom of wheels
The PIP benefit I thought was there for people like myself who are unable to work and look after themselves due to their disability and limited mobility. I would love more than anything to not require it, but I have no other choice and need it to survive. It would have been nice to have access to a car large enough for a wheelchair hoist so I could go out without my mum struggling to get my wheelchair in and out of the car – she broke her back three years ago and it was discovered that she has osteoporosis – I worry that it’s too heavy for her and I’m incapable of helping her lift it myself.
So, in July this year our friend Natasha said she was going to set up a GoFundMe page to raise money for a lightweight portable scooter that can be easily broken down and lifted into the car. After a lot of thought (as mum was worried people would think we were begging) it was set up and my Nan posted it on her Facebook page. Within three days we had received £1,500 in donations from family and friends, and even friends of friends. We were totally overwhelmed and humbled by the amazing generosity.
Sadly, we are still waiting to pick it up as Covid has slowed down production, but I’m so excited and can’t wait to have the freedom to independently ride along the promenade with mum or my PA, whatever the weather! I just can’t wait for the day I get my scooter!
Learning to manage
Throughout my journey to diagnosis, with managing pain and trying different forms of pain relief for my physical symptoms, the anguish of 18 months of not being believed by the DWP and the outrageous lies and lack of understanding in their report was like mental torture. Not to have a voice was almost too much to bear and I dread going through it again.
Thanks to Stanmore’s techniques I have learnt to manage my day with some physio in the morning, then I like to watch a DVD or maybe play cards. After lunch I do my mindful colouring as I enjoy cutting out the pictures and making up scrapbooks for family and friends’ birthdays (very therapeutic) then early dinner and a film in the evening. The best part of the day though, going to bed listening to an opera/ cast recording on my headphones!