My Journey So Far...

 My Journey So Far:

A TIMELINE

2006: And so it begins…

When I was about 18, one 50p sized mark appeared in the centre of my back. It was grey in colour and slightly indented. It appeared so suddenly and randomly that my Mum asked me if I had bruised myself. It wasn’t itchy or red, it wasn’t sore and if Mum hadn’t noticed it one day when I got out of the shower, I wouldn’t have even known it was there. I went to my GP, Dr Flasz who didn’t know what it was, and he refered me to The Harold Wood Medical Centre to see a dermatologist called Dr Carrabott. I had a biopsy performed by another dermatologist named Dr Kakad and subsequent tests rule out skin cancer. Dr Carrabott said it could be a condition called Morphea, and explained that it is a form of Scleroderma that is limited to the skin. He explained to me that there is no clear cause, and although it is regrettably incurable - it isn’t life threatening. He said that it could get progressively worse, but he didn’t explain to me that this was extremely likely to happen. He said that he would like to arrange a clinical meeting where other doctors could take a look too, and together they could work out the best form of treatment. For the meantime, Dr Carrabott gave me some creams that are usually prescribed for Psoriasis. Over the next few months the creams didn’t seem to have any effect whatsoever. The date of the clinical meeting came around, and I went to The Royal London Hospital in Whitechapel, East London. I sat in a room while around 45 doctors and student doctors came in to take a look. Many of them asked me questions and others didn’t even acknowledge me. I found the experience daunting, and invasive. A while after the clinical meeting, Dr Carrabott informed me that the doctors where unanimous in their diagnosis of Morphea, and they wanted me to continue the cream and monitor me at Harold Wood Medical Centre. Like any girl of my age would be, I was concerned about my appearance, people had started to notice it and ask me what had happened. To the untrained eye the scars looked like burns. I started carefully selecting clothing that covered me, and I started to grow my hair longer and longer in an attempt to cover my back.  

2006/2007: A delay

After months of use, topical steroids were doing nothing other than making my skin sore. By this point the one small patch had spread, it was now two larger patches of grey indented areas, which to the untrained eye looked like burn scars. I returned to Dr Carrabott who asked me to try a different brand of cream. He explained that some Ultra Violet light (UVB) could be used to treat Morphea, but the treatment would require going to a hospital several times a week. At this time I was studying at DeMontfort University in Leicester, and so if I was to receive this treatment I would need to undergo it at The Royal Leicester Infirmary. Dr Carrabott told me to go back to University and he would write to the Royal Leicester Infirmary to arrange the treatment there.

2007/2008: A complaint

I am not too sure, what went on around this time, it is a bit of a void, but I never underwent treatment at the Royal Leicester Infirmary. Whether Dr Carrabott never made the request I do not know. I lodged a complaint with the General Medical Council because of the unreasonable amount of time it had taken to move my case from diagnosis to treatment – the appearance on my back had worsened substantially in this time. What I now (2013) know is, that even if he had transferred me it would have been unlikely I could fit the 3X a week treatment around my university timetable anyway. And with the benefit of hindsight – even if I had this treatment wouldn’t have helped anyway. I turned a blind eye to what was happening, and concentrated on my studies instead.

2009: Not a typical case?

By this point there are three large lesions on my back, they are still grey and indented and look like burns. Interestingly, they are all on the right hand side of my back, starting at the spine and spreading across to the right. By this point I have researched Scleroderma thoroughly on line, I have looked at research papers and even emailed doctors from the USA. Around this time I started to question the diagnosis of Morphea. Unlike typical Morphea, my lesions have never started as a rash, they’ve never been pink or purple, and their grey indented ‘burnt out’ appearance doesn’t happen slowly, but rather - suddenly. I have started to question why I am being passed around dermatology departments when the root of the problem lies in the immune system – surely I should be seeing an immunologist? I have now finished University and moved back home. Having lost contact with Dr Carrabott, I decided to return to my GP to request a continuation of treatment. He re-starts the process and refers me to a dermatologist called Dr Piras at Queens Hospital, Romford. Dr Piras takes the time to listen to my problems, and says that she would like me to try an immunosuppressant cream on the lesions in the mean time. I return to University in Leicester with creams in hand – I can’t wait for university to finish so I can go home and be treated with phototherapy. While I am studying I find myself struggling to cope, and speak to my course leader who is deeply concerned about my mental state. I go to a doctor who diagnoses me with severe depression. I am prescribed anti-depressants to help me cope, and I am advised to attend counselling.

2010: Shining some light on the matter

In spring 2010, after the immunosuppressant creams did not work, Dr Piras agreed to try UVB treatment. Administering this kind of treatment would not be easy in the summer months, as it required controlled exposure to the sun. She explained to me that I would need to keep my body covered at all times. Before I started the treatment I also had to sign a disclosure to accept that my risk of getting cancer could increase. I was then referred to the dermatology nurses at Queens Hospital, Romford – their names are Nicki and Sally. Nicki has a gentle demeanour and I instantly like her, strangely I have met Sally before, she is the Mum of my brother’s childhood friend. I have an emotional breakdown when Nicki explains that I will need to attend Queens Hospital 3X a week where I will take off all my clothes, but on a pair of goggles and a helmet and step inside a phototherapy unit. This unit looks like a stand up sun bed, and essentially it is, but a lot more powerful and for medical purposes. Once inside the unit a nurse will check you over and then start the power, while it is turned on I have to move my arms and legs in a circular motion to ensure even coverage. The thought of practical strangers seeing me naked, scars and all, was too much to bear. But in retrospect the thought of doing it was far worse than the reality. The side effects of this treatment were unpleasant, my skin felt sore – the best way to explain it is imagine if you had been lying in the sun without sun cream on for a while, you’d feel raw afterward. I had about three months of this treatment, and returned to Dr Piras for a consultation. The treatment had definitely not slowed the process. She said that the lesions had softened to the touch, but they were much darker and more noticeable. The next step was to try a deeper form of the same treatment. I returned to Nicki and Sally and they explained that I would be having a treatment called Bath-PUVA. As before, I would keep my body away from sunlight and sign a disclosure, although this treatment would involve a higher cancer risk as the UVA rays penetrate much deeper than UVB. As before I would be naked in a phototherapy unit with the goggles and helmet, but this time I would soak in a bath immediately beforehand, the bath contained a chemical called Psolarin and would be controlled and timed by the nurses. Psolarin encourages deeper penetration of UV rays. I underwent this treatment three times a week for several months, and the side effects were as before, but much more severe. My skin burnt almost every time I had treatment, it was incredibly painful to do simple things like walking, because my clothes would rub against my sore skin. I exhibited symptoms of sunstroke, such as headaches, tiredness and fatigue. I endured the treatment for almost three months. Each time before commencing treatment Nicki or Sally would look over my body and make sure all was well, one day Nicki noticed a new lesion appearing on my lower back at the top of my bum. Obviously, the treatment had not worked, and there was concern that it could even be agitating the condition!!! She consulted Dr Piras who said to discontinue Bath-PUVA immediately and return to using immunosuppressant creams while she sought advice.

2011: Moving things on…

As I have mentioned before, I felt that I wasn’t in the right place being treated solely by dermatologists, and explained to Dr Piras that I would like to receive treatment from an immunologist. I told her that I had researched more invasive treatments on the internet and there had been trials with more involved drugs that were showing promise. She agreed to look in to the matter. Dr Piras frequently attended dermatology meetings at The Royal London Hospital in Whitechapel, East London. She asked me if she could take my case to the next meeting, which I agreed to. In my absence she discussed my case with professionals including Professor Cherio, a leading research doctor in his field. Eventually I was transferred to be under his care. I met with Professor Cherio frequently throughout 2011. I had suspected a misdiagnosis for years, and Professor Cherio said he believed that I didn’t have atypical Morphea, but rather a rarer form of the condition called Atrophaderma of Pierini Passini. Where there is a lack of red / purple inflamed rash like lesions, but rather a grey brown scar with cliff top like more defined borders. I approached the issue of taking drugs to try to control the formation of lesions, but Professor Cherio wasn’t keen. He felt that there hadn’t been enough thorough research to declare those drugs ‘safe’ for people of my age and sex. He said that taking drugs for Morphea wasn’t a good idea, basically the side effects were more serious than the condition itself, I could be left infertile and the drugs could cause liver failure too. He gave me a topical steroid cream and asked me to try it while he considered the next step. I later returned to him, and he was concerned that the topical steroid had not slowed down the formation of lesions. He asked if I would be willing to undergo a clinical meeting. I was reluctant to do this, because I felt that I had been exploited when I did this for Dr Carrabott back in 2006 and it had got me nowhere. Professor Cherio explained that this would be different; it wasn’t for diagnosis purposes this time. He wanted to see if the majority of doctors agreed with his decision to avoid administering drugs. I agreed to return for the clinical meeting, and he asked me to rotate the use of the topical steroid and the immunosuppressant creams in the mean time. When I returned to the Royal London Hospital for the clinical meeting I was devastated to see Dr Carrabott in attendance. I felt that I had moved in one huge pointless circle, and got nowhere – and I bore the scars as proof. After the clinical meeting I met with Professor Cherio – who told me that the consensus was that my condition, although progressive and unstable is not life threatening, and therefore dermatologists at The Royal London Hospital were unwilling to prescribe drugs at this stage. He asked to keep monitoring me. I was forced to accept this decision and went home feeling hopeless. A few months later I attended a routine monitoring consultation where I met Dr Gulati, Professor Cherio’s registrar. She was concerned that I might be showing symptoms of Lichen Sclerosis – which affects the genital region, but may be too embarrassed to say anything about it. She took a look at the area and was satisfied that there was nothing wrong there. I had been experiencing severe back pains frequently, and the aches were such that normal painkillers did not help. My GP had prescribed several painkillers that had also not worked, and eventually he had prescribed a low dose of morphine. I explained this to Dr Gulati and begged her to consider the possibility that my condition wasn’t Morphea – but rather that it was Linea Scleroderma, that was now affecting my muscles. Once again I pushed the issue of drugs to stabilize the lesions that still continued to form. Dr Gulati said that she would refer me for thermo-imaging. She explained that this would show how ‘active’ the condition is, and if it was particularly active the doctors might reconsider the possibility.

2012: In safe hands…

I was referred to The Royal Free Hospital in Hampstead Heath, North London – a leading hospital in researching Scleroderma, and one of the only places that use thermography to treat it. I was extremely pleased to learn that I would be treated by the Scleroderma Clinic in the Rheumatology department, under the watchful eye of Professor Denton, a name I recognised from my research. Upon my first visit I met with Dr Kevin Howlett, a thermo-imaging expert, and had my first photograph taken. Here’s how it works; because the lesions were on my back I first had to take of my clothes and bra and allow the area to cool to room temperature, after about 20 minutes the area is ready to be photographed. The special camera produces a greyscale image that is then transformed into a colour version with various layers of colour. The camera picks up levels of activity and displays it as colours ranging from blue (coolest and least active) – through to yellow and orange (warm and some activity) – and red (hot and most active.) Using this image doctors can establish how active the condition is, what stage it is in, and also what treatment would be most effective in slowing down the process. I met with Dr Quillinan, Professor Denton’s registrar. She was extremely sympathetic to my situation, she took a long time asking questions that others before her hadn’t even though to. She was extremely thorough and gave me the distinct impression I was in safe hands. She listened to my complaints of back ache and agreed that it was connected to scleroderma. Unlike so many doctors before her, she did not dismiss my request for drugs and discussed the options with me. She explained that the phototherapy showed enough activity to prescribe drugs – but that it isn’t without risks in women of my age. She explained that I could take a low dose of Methotrexate a chemotherapy drug that is used to suppress the immune system. This would be taken once a week and would (hopefully) slow down the condition in the long term. Because of the serious side effects that Methotrexate can cause, I would need to have my blood monitored. This involved weekly blood tests, for the first month and then monthly after that. I was absolutely petrified, I had a huge phobia of needles, and the thought of this made me feel sick. Alongside this she wanted me to have a steroid to attack the activity that had ‘flared up.’ The drug, which was called Methylprednisolone, would be administered in hospital by UV drip; I would require three pulses at weekly intervals – great, more needles! Dr Quillinan listened to all of my concerns and put my mind at ease, she said that provided I was monitored effectively – I would be safe and they would stop the treatment at the first sign of anything potentially going wrong. I believed her, and left the hospital feeling more positive for the first time in the whole entire time I had had the illness. I started taking Methotrexate 7.5mg immediately, and I took to it well, I didn’t experience many of the nasty symptoms that others had complained of, but I was more tired than usual.  I was subsequently prescribed folic acid to help me cope with fatigue. While I was having this treatment I didn’t touch alcohol for six months – I tried to look after myself the best I could, and all things considered, I felt relatively ok. I handled the steroid pulses well too, each session took about 4 hours to run the drug through my body by drip, and I would sit in a day ward while it happened. The side effects meant I was left with a nasty metallic taste in my mouth, and I felt sick and tired for a few days after, but I was still able to go to work and live my life relatively normally. My weight fluctuated a little around this time, the difference was around half a stone, I would go from having no appetite at all, to eating constantly and insatiably and piling on weight, and once that weight was on it was hard to shift. My hair started to fall out, and became limp and lifeless, it wasn’t noticeable to other people though, and I was thankful for that. Three months later I had another thermo-imaging session with Dr Howlett, and the doctors felt it showed a little improvement. Dr Quillinan increased the Methotrexate to 10mg per week and told me that I wouldn’t need more Methylprednisolone for the time being. As winter approached, I started to notice differences in my body; due to my weakened immune system I caught illnesses very easy, such as coughs, colds, sore throats and water infections. I was given a flu jab by my GP to try to combat this. I met with Dr Quillinan every three months or so for a consultation, and each time I had a thermo-imaging session with Dr Howlett. It was during one of these consultations that I complained of worsening back aches. Dr Quillinan referred me to a physiotherapist at King Georges Hospital in Goodmayes, East London. I had a thorough consultation with a lovely therapist called Angeli – she explained that the condition had fused my muscles all along the right side of my back, and that as a result of this I needed to build up the muscles on the left hand side to help balance it out and carry some of the strain. By this point flexibility had become a real issue; I am extremely stiff and can’t bend and twist the way I once could. Angeli was concerned because I had a new lesion appearing on my right shoulder, which was slowly restricting my movement. Angeli has worked out some exercises to help me cope with the changes that were taking place – I now meet with her every two weeks, although she says it will take a long time to see feel any major differences.

2013 - PRESENT: A turn for the worst…

I returned to The Royal Free in February 2013 for routine monitoring and had another thermo imaging session. Dr Howlett explained that the image was more symmetrical than had been seen before, and that it was a positive sign. On this occasion I didn’t see Dr Quillinan for my consultation, but instead I met with an associate doctor of Dr Ong’s. The doctor was pleased with the thermo image, and said that I was to continue taking 10mg of Methotrexate for another three months. As I was about to leave the room, I said “It’s probably nothing, but I have a some marks appearing on my forehead and on my chin – I just wanted to bring it up to put my mind at ease.” I had noticed these particular marks appearing in the summer of 2012, when the sun seemed to make them greyer and more noticeable, but I had never pointed them out to doctors as I felt they were probably irrelevant. Sometimes it was hard to tell if they were nothing more than veins close to the surface. The doctor asked me to remove my make up and shone a light on the areas – she seemed very concerned and went to get Dr Ong himself. The two doctors looked at my face closely and decided that given the visibility of the area in question, it was best to act quickly, in order to take no chances. As I had so far responded well to Methotrexate they asked me to up my dosage to 15mg per week. They also said that they would schedule more intravenous steroid treatment; however they wanted to hit the activity hard and fast – so instead of undergoing one treatment a week for three weeks like previously, this time I would be admitted in to hospital and would receive one pulse per day for three days. I was told to go back to Dr Howlett and have my face thermo imaged. As Dr Howlett was looking at the image and talking me through what he it showed, the doctor came in to the room and took a look. She explained that there was (red) heat activity shown in the image – but as the forehead is a hot area with veins close to the surface, the image was somewhat unreliable. I felt devastated – until now I had been able to cover my scars – but now my biggest fear had become a possible reality.