August 2023

I have always been a poorly child. I had constant upper tract infections: ear infections, tonsillitis, colds and lots of time off school. One day when I was 9, I woke up with a very swollen and discoloured right eyelid which I couldn’t open. My mum took me to my GP who prescribed me some antibiotic eye drops for conjunctivitis. After a week, the swelling was no better and we returned to the GP who then suspected orbital cellulitis, and I was given a course of stronger antibiotics. After 2 weeks, my eye was still no better which is when my GP then referred me to my local hospital. At A&E, I was seen by a junior doctor who told my mum it was an allergy and sent me home. I was referred to the Hospital again by my GP who requested that I would be seen by an ophthalmologist. I was seen by the same Junior Doctor again who still persisted that it was an allergy. My mum says she refused to leave until someone took me seriously. After a second and a third opinion by ophthalmologists, I finally had an Anca blood test and a CT scan. My mum received a call the same week by a consultant who had seen my results and told my mum that I was being referred to Great Ormand Street Hospital (GOSH).

A week later I had a biopsy taken of my eye at GOSH, where I stayed for 2 weeks. I received IV pulse steroids during my stay and was sent home on steroids, antibiotics and eye drops. Once home, my mum got a call from Professor Paul Brogan. ‘Well do you want the good news or the bad news?’ he asked my mum. ‘Good news is it’s not cancer, but the bad news is its GPA’.  That week I was seen as an outpatient at GOSH where I had photos taken of my saddled nose which they had noticed at this time showed nasal septum collapse. I then took part in an 18 month clinical trial called the MYCYC trial in which I was randomised to Mycophenolate mofetil with Prednisolone, followed by Azathioprine. I then became in remission after my treatment. I was able to go back to school, but I looked different. Having been on IV and oral steroids, I had become quite cushingoid, and I had what they call a moon face. I did hear the word ‘fat’ thrown around a few times at school, but I knew what I had been through and how special I was.

It was during this time that my mum received a call from the Junior Doctor who misdiagnosed me in A&E who apologised profusely and asked if he could write a journal on my GPA diagnosis to teach others about how GPA can present in different ways.

Within 18 months I had relapsed. I had constant nose bleeds and was admitted into hospital on New Years due to low platelets and E-coli in my urine. I was still on the MMF and steroids for a further year. I relapsed again when I was 12. It was Easter and my mum was unable to get hold of anyone at GOSH for advice, so my GP had to prescribe me some MMF and Steroids to get me through Easter. I had a short stint on treatment, and was able to stay in remission for a long time.

I was discharged from GOSH as an Outpatient when I was 16 and then was under the Royal free London. When I was 18 I was able to have reconstructive surgery to fix my saddled nose. This improved my breathing and my confidence a lot and I was also able to lose my steroid weight I had carried with me through my teens.

In January 2021 (when I was 21) I got Covid. I was in remission from the GPA and wasn’t on any medication at the time. I did not have any symptoms of the covid at all. However, around May 2021, I started to become very breathless and had a sharp pain in my chest when I breathed. My GP saw me and was shocked how ill I was. I went straight to A&E where a CT scan picked up that I had suspected hypersensitivity pneumonitis. After learning of my history of GPA, the Respiratory consultant was convinced it was pulmonary vasculitis, meaning that the GPA had spread to my lungs. I had a 10 day stay in Hospital as I needed pulse steroids and oxygen. When I was well enough, I was referred to the Royal Brompton Hospital as an outpatient where they re-started my mycophenolate mofetil and oral steroids.

This leads me to now – 2023. I am able to live a normal life and I work full time as Pharmacy Technician in a busy Hospital in West Sussex. Unfortunately, I have not lost the weight I have gained since my most recent flare-up but I am working on this and am just glad that I am happy and (at the moment) healthy.