The month of September always prompts a trip down the memory lane / abyss of September 1997, for me. For, it was at this time, aged 24, when I first heard those three words – ‘You have scleroderma’.
Resulting in my life being turned upside down, forcing me to abandon most of my dreams, and to be grateful for defying the medical statistics and long term outlook for most scleroderma patients.
During the summer of 1997, I had started to feel very tired all of the time. This was quite out of character for me as I had an insatiable zest for life. Not only was I working as a medical representative for Bristol Myers Squibb, I was studying on a part time, distance learning basis for a postgraduate diploma in law, which would then lead me to pursue my childhood personal desire to be a barrister and professional advocate.
By early September 1997, my hands had become swollen and puffy with shiny tight skin across my fingers. I had difficulty moving my fingers as they were stiff, as well as, I was having difficulty in gripping and making a full fist. I also found it difficult to open my mouth wide and to swallow. I wrote more about my initial symptoms on Day 4 of my June Scleroderma Awareness Campaign 2016
As the summer season in 1997 started to change into autumn, not only did I feel exhausted, but I also noticed that I was starting to actually feel the cold. This was a totally new experience for me, as even in the winter, I would seldom be wearing thermal clothing, previously. I thought that the ‘thermal vest’ was something to look forward to, for when you reached late middle age!
It was during Autumn 1997 that my internal thermometer ‘tripped out’ and it still remains very demanding. It took a few more months before the color changes would become visible in my fingers, presenting classic Raynaud’s symptoms, causing me to have to wear several layers even in the summer, which are then ‘bulked up’ in the winter. I wrote more about this
Having observed the change in my hands, combined with the tiredness and swallowing difficulties, I booked an appointment to see my GP medical doctor. At first consultation, the stiff puffy fingers were thought to be a form of arthritis, however, the blood test for arthritis returned a negative result. Which was a huge relief at the time, although, I knew that something was not right. I was then required to give another blood test which was used for a specific autoantibody test, and bingo, this test returned a positive RNA Polymerase result, confirming a diffuse systemic sclerosis diagnosis.
The EULAR /ACR classification code is the current criteria used for confirming a scleroderma diagnosis. DAY 6
I was then referred to my local hospital Consultant medical doctor in Rheumatology, who advised me that I was looking at a 15month prognosis and to give up on my idea of being a barrister. I felt as though my whole world had ended and that the rug had been pulled out from under my feet. There was no way that I was giving up on my law studies, I had already paid a fortune in tuition fees, afterall!
After the blood tests, I was then required to take many different diagnostic tests to assess the level of internal damage, if any. See DAY 5
By February 1998, my scleroderma disease was so aggressive with my skin involvement, that I was unable to stretch my arms or legs, and it was itchy, swollen and painful. I returned to the hospital and commenced all of the different immunosuppressants and disease modifying agents available, with little improvement on the disease, but horrendous side effects and continual nausea.
In December 1998 I went to see Dame Prof Black at The Royal Free Hospital in London. Within 30 seconds of being in the consultation appointment, I knew that Dame Prof Black would do everything she could to help me, especially with me achieving my dream of being a barrister.
15months of intense chemo drips (cyclophosphamide) combined with mycophenolate mofetil stabalised my symptoms, stopping the scleroderma in its tracks, thankfully. Otherwise, I was scheduled to be having a stem cell transplant, as my name was on the waiting list.
By now, Prof Chris Denton had returned from his scleroderma studies in the US and I had the pleasure of becoming his patient. My local Consultant medical doctor, (the diagnosing doctor), would not entertain ‘shared care’.
At the end of 2004 I returned to my family home town of Southport, and I am delighted that my local Consultant medical doctor here, Dr Hannah Sykes, is extremely supportive and co-operative with me having shared care with the expertise of the scleroderma unit at The Royal Free Hospital and Prof Denton’s medical expertise. This co-operation from the medics most certainly makes for an improved patient experience, especially given that medical appointments / tests and the like, become the long term scleroderma patient’s full time job due to the multi disciplinary care required.
I wrote about the importance of expert specialist centres here
I wrote about the long term scleroderma patient’s annual check up here
As well as, I wrote about the Olympian effort and attitude needed to become a Scleroderma Olympian, here
To view the UK treatment and management of scleroderma guidelines click here
I know that I am a very lucky scleroderma patient by way of minimal internal organ damage, even though I have had my life and body hijacked by scleroderma. This has made me be dedicated to using the remainder of my life to improve the scleroderma, raynaud's, rare disease patient experience. Read more here
I take huge inspiration from the scleroderma global trail blazer and pioneer, Prof Denton, who I am eternally grateful to, for his medical expertise and helping me realise my dream of qualifying as a self employed barrister on 1st March 2004.
Although I am no longer physically able to continue with my 60 hour per week role, I have transferred my professional skills to the global scleroderma landscape- being a voice at all levels, including decision making body levels such as NHS England, the British Society for Rheumatology, RareDiseaseUK the European Rare Diseases Organisation and The European Medicines Agency, and my column at Scleroderma News.
I am also delighted to have joined the ProVIDE Law team in Liverpool, with my first article
focussing on 'patient best care/practice and clinical litigation'.
Clearly these roles are secondary to the priority of me attending to, and respecting my symptoms, as, I want to feel good.
I very much hope to develop this platform further, for advancing the scleroderma and rare disease patient plight, thereby improving the 4 main hallmarks of the rare disease patient:
- commitment to research investment
- access to innovative medicines
Scleroderma has made me have to tweak my original dreams, however, I feel truly honoured to be using my background qualifications and skills to being a scleroderma, rayanud’s and rare disease patient on the global stage, as well as raising awareness and funds for the Scleroderma Unit at The Royal Free Hospital.
I have a new dream now – a #sclerodermafreeworld….. imagine that.
This article was written and published in September 2016 for my Column
To read my articles:
- the importance of an early diagnosis click here
- the role of medical research click here
- the importance of expert specialist centres click here
- diet and nutrition click here
- taking part in research trials click here
- the UK guidelines for management and treatment of Scleroderma click here
- my skin is cured from scleroderma click here
Please DONATE to help fund medical research at the Scleroderma Unit at The Royal Free hospital where 100% of your donation will be used to fund vital medical research. Thank You.
Living the dream, scleroderma style, hoping for a cure
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