My Story in Short.
I want to make this brief and informative rather than a long biography, although I might try my hand at one of those too at some point; but for now I will stick to the main points of medical interest.
Ages 3 – 7
The first incline that something was wrong came when I was 3 years old and became very lethargic, experiencing fevers and chills and becoming hesitant to use certain limbs – at times my arms would go limp, my feet would turn inwards or I would hold my neck awkwardly. Eventually I was seen by a Paediatrician called Dr Sills at Alder Hey Children’s Hospital in Liverpool; he specialised in rheumatology and diagnosed Reactive Arthritis, possibly due to a past virus or food allergies. Looking back however, I know that the way I felt back then was exactly how I feel during flares now, only I was too young to voice it.
Treatment consisted of Naproxen, diet changes and extensive physiotherapy. At one point I was supposed to have surgery for Bursitis of both ankles but this never went ahead. Instead, I was fitted with special splints for my lower legs and a neck brace – I still have the little wax elephant that my mum and the therapists made to distract me while having this done 🙂 Either these things worked or I eventually grew out of it, as I don’t look back on this time as being only one of illness; and by the age of seven I was pretty much the same as every other kid, into everything and anything.
Age 14 – 19
Up until the age of 14 my life was very ‘normal’; in fact I was sporty, active and on the go all the time. I probably had more aches, pains and xrays than the average child but nothing to worry me. Then shortly after receiving our TB vaccines at school I started to feel lethargic, extremely weak, sore and tender, plus I began to get fevers alongside a speckled rash that was prevalent under my eyes and across my knuckles. When I was finally referred to a Rheumatologist called Eilleen Baildham at Booth Hall Children’s Hospital in Manchester (and after lots of different tests) we were given the diagnosis of Dermatomyositis.
I don’t doubt this diagnosis, even though it has changed since, because at that time it was certainly my muscles that were involved more than anything else. Initial treatment consisted of a sharp burst of high dose Prednisolone (80mg) to be gradually tapered over the following year. I did show some improvement in time, although I missed almost all of my first GCSE year and had to homeschool myself. Just as we had almost completely stopped the steroids, something new happened quite out of the blue. One morning I woke up and both knees were the size of footballs, bright red and hot to touch. This marked the first joint involvement.
Over the next few years we tried high doses of Methotrexate and Immunoglobulin (Ig) Infusions to get things under control, but they never really were. Multiple joints became affected, although my knees were always the worst and needed aspirating / injecting with Cortisone approximately every 4-6 weeks. At the age of 16 I was rushed into hospital and treated for a Pulmonary Embolism; at 18 I had my first surgery to fuse the damaged tib-fib joint in my left leg and a year later at 19 I had the flare from hell but was now too old to be admitted under the Children’s Hospital.
19 – Present
At this point I had no other option but to be referred to an ‘adult’ Rheumatologist – Dr Snowden – at the nearby North Manchester General, where I was immediately admitted. This was probably the worst flare I have ever had – severely underweight, losing my hair, barely able to move due to pain and swelling, unable to eat without vomiting (including vomiting blood numerous times), covered head to toe in a rash and experiencing horrendous fevers that required ice to cool me down; as well as having a swollen spleen/liver and other organ involvement that showed up in my bloodwork. At one point my Liver and Kidneys showed signs of failure due to damage, but thankfully this was reversible. I can’t really portray just how horrible this time was, but at one point I really felt like I was dying and I am by no means a drama queen.
All the tests that I’d had prior to my Dermatomyositis diagnosis were repeated and this time the Rheumatologist diagnosed Still’s Disease. It is possible the two conditions overlap or that one evolved into the other, but again there was no doubt about it. The Methotrexate was stopped while I was in hospital and we tried Arava (Leflunomide) instead but I showed no improvement, so then we turned to Cyclosporin and thankfully that turned my life around.
In time I improved greatly, was able to finish college and look forward to starting University. However, I had only been there a month or so when I became ill again and needed emergency surgery for abdominal bleeding, which resulted in quite a serious infection. After that, the Cyclosporin didn’t keep things at bay so much on its own and in July 2004 I started Enbrel (Etanercept) alongside it, which seemed to benefit me almost immediately.
I returned to university in the October and had a brilliant few years with just the occasional glitch in my health, graduating with first class honours in 2007. My right shoulder began to cause me some problems in my last year and I had my third surgery in March 2008 to resculpture the ball joint and remove the synovium, as well as tightening the surrounding ligaments, which were damaged due to dislocating so many times and probably by me putting it back in myself rather than going to A&E. Again the surgery worked wonders and despite the surgeon’s concerns (and with lots of physiotherapy), I managed to get almost a complete range of movement back.
My most recent problems started after about a year of working full time. I just seemed to get less responsive to treatment and needed more steroids, including Cortisone injections for my left shoulder and elbow. We tried increasing the Cyclosporin to its maximum, which worked for a while but gradually impaired my kidney function. We tried Anakinra (Kineret) in July 2010, optimistic that it was proving beneficial to many Still’s patients… but it was like injecting water for me and I became much worse without the Enbrel.
Starting back on Enbrel shortly after, we decided to try and replace the Cyclosporin instead and turned to Mycophenolate Mofetil (Cellcept), which brought about a complete personality change and depression, so I requested this was stopped. After being admitted into hospital in December 2010 due to episodes of fever, rash and semi-consciousness, I started Azathioprine (Imuran). This left me feeling very nauseous but, desperate to feel better, I persisted until being rushed into hospital with chest pain and finding out that the drug had wiped out my bone marrow, causing Neutropenia and, in turn, Pericarditis since I couldn’t fight off any infection.
Once I had recovered from this episode, I restarted the Enbrel and Cyclosporin combination (alongside varying doses of Prednisolone), while my Rheumatologist and his registrar decided on a new plan of action. A drug called Tocilizumab was initially considered but in the end we went with Abatacept (Orencia), since it has a similar mechanism to Cyclosporin and the hope was that it would be equally successful. I started this at the end of June, alongside Methotrexate, and seemed to benefit in that it helped my fatigue; but my joints and blood test results became increasingly worse.
After over three months on Orencia, we decided that it wasn’t worth persevering with and so switched to Tocilizumab (RoActemra), the current ‘wonder drug’ for Still’s Disease, with Cyclosporin instead of the Methotrexate. I started the Tocilzumab at the end of September and initial signs were promising – my inflammation markers were lower than they have ever been and we were hoping that my joints would eventually catch up. However, despite the perfect bloodwork, I deteriorated physically until being admitted into hospital on numerous occasions. The flare reached its peak in February 2012, when I was hospitalized for a fortnight, and again we were forced to rethink my treatment plan. Which brings me almost to the present:
During this hospital stay I had a minor procedure on my hip; when I returned home I was wheelchair bound and could barely move. For the first time in my Still’s experience, I needed carers to help with my daily living and medical aids to sit up in bed, go to the toilet and bathe myself. At the end of March 2012 I started high doses of another IV Biologic called Infliximab (Remicade), alongside Cyclosporin. Over the past few months I have made huge improvements and, after lots of physiotherapy and determination, am once again walking without assistance. So I think it is safe to say that the Infliximab is doing the trick.
As you can probably see from reading the above, life with Still’s can be very difficult and unpredictable at times. There are good times and bad times. Every person with Still’s is different and so it can be a challenge, for both doctors and patients, to find a treatment that helps. What works for one person may not work for another; it really is a case of trial and error. But as frustrating as this may be, please don’t ever give up hope.
My disease is described as ‘extremely refractory’ (unresponsive to treatment) and I have had several doctors comment that I had literally run out of treatment options, having tried each ‘mechanism’ or type of treatment available. However, I was determined that I would keep on trying every single drug, down to the very last one if I needed to, even if a similar drug had proved unsuccessful, and my Rheumatologist agreed. Thank God we did! I don’t think anyone really expected Infliximab (an anti-tnf drug like Enbrel and Kineret) to work but it seems to be doing the trick and I’m not complaining!
I am very lucky to have such a fantastic Rheumatology team looking after me, as well as the support I have from my fiance, family and friends. Support is just as important as the medications in dealing with this disease and I couldn’t do it without them.
Anyway, I hope you enjoy reading my blog and find it useful in understanding and living life with Still’s Disease 🙂
Medications I Have Tried (With Links For Further Information):
Naproxen & Diclofenac (NSAIDs), Plaquenil, Gold, Sulfasalazine, Prednisolone, Methotrexate, Immunoglobulin Therapy (Iv Ig) , Leflunomide (Arava) , Cyclosporin (Neoral) , Enbrel (Etanercept) , Kineret (Anakinra) , Mycophenolate Mofetil (Cellcept) , Azathioprine (Imuran) , Orencia (Abatacept). Tocilizumab (RoActemra), Infliximab (Remicade).
Pain Relief: Ibuprofen, Co-Codamol, Tramadol, Nefopam, Zomorph, Oramorph.