stickler6

First, I must say a big thank-you to our editor for not giving up and giving me the opportunity to get back into  writing stride again.   In my hey-day I was selling around 56 articles a year as well as one or two books. Recently, three years of ill health has taken its toll, so with renewed enthusiasm I am grabbing this opportunity with both hands.   I suffer from a genetic disorder called Stickler syndrome and run a support group for the condition. I am no stranger to hospitals, but there have been occasions when I have ended up being the expert! For my first article I am going to recall a trip to the London Bookfair, a familiar venue I am sure.

Imagine the scenario – I had looked forward to the event for weeks, and had arranged to meet some authors for lunch. I set off that morning in buoyant mood, a little knee pain, but nothing too dramatic.   Within ten minutes of arriving at the Fair I experienced an excruciating pain in my left calf and distinctly heard a ‘ping’ – to be honest I thought I had been shot. I crumbled to the floor, wondering why no one had acknowledged the fact that I had been shot?   Why was no one running? Didn’t they realise there was a gunman in the building? I gingerly placed my hand on my calf and was surprised to find no hole – just an extremely knotty muscle. I was helped up, and eventually managed to hobble to a telephone box and ring my husband who took me home. Thinking it was yet another painful episode of joint pain, I rested for several days. But the pain remained unbearable and when bruising ranging from purple to yellow appeared from knee to ankle I took myself to my local A & E Department. Two nurses came rushing out to the car with a wheelchair, taking me straight in to see the Casualty doctor, despite the fact the waiting area was full to capacity.stickler

 

I spent the next ten minutes explaining that I suffered from a genetic disorder called Stickler syndrome. The leaflet taken with me explain the condition was dumped unceremoniously on the desk, ‘to be read later.’

‘No, not Sickle Cell,’ I kept repeating.

‘Who said you have Stickler syndrome?’ the doctor asked as I just stopped myself from saying, ‘The man in the Moon’

Instead I smiled sweetly and said, ‘ my ophthalmic surgeon after I had suffered repeated retinal detachments.’ ‘But he deals with eyes,’ he said puzzled. ‘You are telling me you have a pain in your leg. Your leg is a long way from your eye, you know.’

After 20 minutes of trying to make him understand that Stickler syndrome is a connective tissue disorder that affects, amongst other areas, eyes, joints, hearing and palate he decided I MUST have a deep vein thrombosis (DVT).   I immediately queried this.

Of course being a mere patient I had no opinions, so I was shipped off to have a venogram which proved negative. I resisted the temptation to say, ‘I told you so’ and was wheeled back to Casualty where a senior nurse suggested to our Mr know-it-all doctor. That I was displaying the hallmarks of a ruptured calf muscle.

‘That’s a sports injury,’ said the doctor.stickler2

‘Athletes and footballers suffer from an injury like that. He looked at me.

‘Are you sure you were only walking?’ The idea of me being able to do anything more strenuous amused me, as a mental image of me trying to kick a football and falling flat on my face flashed before me.

 

I was finally discharged and told to walk as best I could – more easily said than done, I can tell you!

Being a good patient, I persevered, but two days later the leg was so painful that I couldn’t put any weight on it, so I went to see my GP who immediately sent me to our local physiotherapy department where a ruptured calf muscle WAS diagnosed. A course of treatment, which included deep heat, massage with oils, ultrasound and gentle stretching exercises began and life became more bearable.

Eight weeks later I had graduated from two elbow crutches to one, then to a stick and was discharged from physiotherapy. Two weeks later I awoke to find the muscle in the right leg was extremely sore. I rested for a week, but found it excruciatingly painful to put any weight on it, so took myself to my GP. He of course diagnosed a ruptured calf muscle, but explained he needed to rule out a DVT so I was shipped off to my local A & E Department.

Yes, you have guessed it, it was the same casualty doctor. ‘You again?’ he said with a half smile, half snigger stretching across his face. This time I did not bother to explain Stickler syndrome! I was taken to x-ray to have a venogram, but by now it was 4.30pm on a Friday, so it was decided to start me on blood-thinning injections and a venogram was arranged for the Monday.stickler3

Much to Mr know-it all doctor’s surprise the venogram was negative. ‘This is most odd, there is no evidence of a past or present blood clot,’ he kept repeating as he looked at the computer screen and scratched his head.

‘Tell me, are you sure you are in pain?’ he smirked, ‘Sometimes people can imagine severe pain when it is only a twinge, you know?’

 

I accept I am a writer with a vivid imagination, but I was breaking out into a cold sweat every time I put weight on the leg, and my eyes were smarting with pain. If this was imagination then I must surely be a suitable candidate for the next Booker Prize for Fiction.

Eventually he discharged me, stopped the blood-thinning injections and told me to return to my GP! No explanation was offered. Having ruled out a circulatory problem, my GP told me to rest and make an appointment to see him in a week’s time. Meanwhile he would make enquiries to find a suitable consultant to refer me to.stickler5

I returned a week later to find that my GP had experienced problems finding anyone who knew about Stickler syndrome. One medical body he contacted said they would make some enquiries and came back to him.   They rang back to say they could give him the name and number of a support organisation that might be able to suggest a suitable consultant – MY name and MY telephone number!

stickler4

 

 

 

 

About Wendy Hughes

Wendy turned to writing, in 1989, when ill-health and poor vision forced her into early medical retirement. Since then she has published 26 nonfiction books, and over 2000 articles. Her work has appeared in magazines as diverse as The Lady, Funeral Service Journal, On the Road, 3rd Stone, Celtic Connections, Best of British, and Guiding magazine. She has a column in an America/Welsh newspaper for ex-pats on old traditions and customs in Wales. Her books include many on her native Wales, Anglesey Past and Present, The Story of Brecknock, Brecon, a pictorial History of the Town, Carmarthen, a History and Celebration and Tales of Old Glamorgan, and a book on Walton on Thames in the Images of England series, a company history and two books on the charity Hope Romania. She has also co-authored two story/activity books for children. Her latest books are: Haunted Worthing published in October 2010, a new colour edition of The Story of Pembrokeshire published in March 2011, and Shipwrecks of Sussex in June 2011 and Not a Guide to Worthing in 2014. She is working on a book entitled A-Z of Curious Sussex which will be published in 2016 Wendy also works with clients to bring their work up to publishable standard and is currently working on an autobiography with a lady that was married to a very famous 1940’s travel writer. Wendy has spent many years campaigning and writing on behalf of people affected by Stickler Syndrome, a progressive genetic connective tissue disorder from which she herself suffers. She founded the Stickler Syndrome Support Group and raises awareness of the condition amongst the medical profession, and produces the group’s literature, and has written the only book on the condition, Stickler The Elusive Syndrome, and has also contributed to a DVD on the condition, Stickler syndrome: Learning the Facts. She has also writing three novels, Sanctimonious Sin, a three generation saga set in Wales at the turn of the century, Power That Heal set in the Neolithic period entitled Powers that Heal, and a semi biographical book entitled New Beginnings which deals with two generations coping with blindness and a genetic condition. She has also had a handful of short stories published, and in her spare time is working on several at the moment. She also gives talks on a variety of subjects including Writing and Placing Articles, Writing Local History, Writing as Therapy, Writing your first novel, etc, and runs workshops on the craft of writing – both fiction and non-fiction. She is a member of the Society of Women Writers and Journalists, and a member of the Society of Authors.