Nicki’s story

Horror, terror, disbelief and guilt. These words best sum up my emotions at being told that my then beautiful 9-year-old daughter was considered to be legally blind in her right eye.

I didn’t hear much of what was said after that. Specialist, immediately, inflammation … they all washed over me. All I could think about was how it was my fault. I had failed to take my daughter to the optician for the previous nine years, and here we were three days after her first visit being told by a specialist that she had a disease I’d never heard of.

The guilt remains; but I’ve tried to absolve myself by fighting to ensure that my daughter has since received the best care by the best specialist in order to give her the best prognosis. You would assume that when a child is diagnosed with a sight threatening disease such as Uveitis, that there wouldn’t be a fight involved in the first place. One of the most important lessons I have learnt is to assume nothing.

Do not assume that the Specialist to whom you’ve been referred actually specialises in Uveitis, rather than having a passing interest and do not assume that you will automatically receive appropriate care. The vast majority of Uveitis sufferers are quickly and effectively treated with short-term steroids. The Uveitis goes and the inflammation doesn’t return. For the very small minority of patients, like Olivia, the Uveitis is bilateral and chronic. It cannot be treated with steroids alone and other drugs such as Immunosuppressants are necessary to avoid or limit the damage that both the disease and the side effects of the steroids can cause.

Olivia was referred on to three different more specialised Consultants over a period of five months. Each time we were told steroids were the answer. One specialist in particular was incredibly adept at underplaying the situation. From the very first appointment I was made to feel foolish for asking too many questions. It was made very clear that although Uveitis was an unpredictable disease, it was very curable with steroids. I had by this point, guided by the specialist, accepted that my daughter had probably lost the sight in her right eye forever more. The focus changed to maintaining vision in the remaining left eye, which despite having the same level 4 flare and cells had retained good vision. The approach was steroids, steroids and more steroid. Dilating drops were thrown in for good measure in an attempt to prevent the other lens from sticking to the iris, causing the pupil to become fixed, as had happened in the right eye.

The cause of the Uveitis was seen as irrelevant. JRA was bandied about but nobody seemed very concerned whether this was the reason or not. After all, her joints were for the time being unaffected, and for this I was grateful. Other symptoms were also ignored, and so my daughters dramatic weight loss and constant stomach pains remained a mystery, considered unimportant despite her now weighing just three stone aged 10. Blood tests were not offered and my numerous requests were rejected until I stopped asking.

Active flare would improve and then change for the worse. Cells increased and decreased. With every appointment steroid drops went up or down accordingly, and with no other treatment mentioned I assumed that this was simply how it would be until the disease burnt itself out and that would be that.

Three months after her initial diagnosis I came across an article that suggested there was another way for cases of Uveitis which were deemed to be stubborn: the use of Immunosuppressants. When I brought up this question I was reassured that my daughter did not come into this category and that it was very early days. Unfortunately I now know that the path we were on could have blinded her.

The second Specialist mentioned in passing that Olivia might require steroid injections or steroid tablets further down the line. He also informed us that Olivia had a cataract in her right eye, band keratopathy, her colour vision was reduced to 4/13, there were 3D issues, extensive synechiae on top of, at that time, flare and cells of 3+ in both eyes.

This was all news to me. I requested copies of all communication between the GP and the Specialists and found a wealth of information. I’d been so certain that Olivia was seeing the top people and that I was being told everything I needed to know that I had failed to ask the right questions. Research began in earnest and I turned to the US. In the meantime I received a letter from the Specialist stating that as Olivia was “essentially one eyed” she was an ideal candidate for a chemotherapy drug called Methotrexate. I was truly terrified. It was the first time chemotherapy had been mentioned by anyone. I didn’t even know chemotherapy was used to treat Uveitis.

At the next appointment the Specialist backtracked and said Methotrexate would not be necessary. However, now that I was aware of the dangers of long term steroid use I questioned this decision, but he was adamant that Olivia could safely stay on steroids for the next ten years. It was this statement that finally spurred me on to look elsewhere and led me via a visit to Dr. Foster in Boston, USA, to Professor W. Ayliffe in London.

Blood tests were carried out. The results showed she was ANA positive, and with very high levels together with other criteria the diagnosis pointed to JIA type Uveitis. On examination we were told that the Uveitis was sight threatening and that she needed to start Methotrexate and Ciclosporin immediately. Prof Ayliffe explained that the Metho was a low grade chemo drug, one of the safest to use. Despite the fear, I knew in my heart and head that he was right. Without these drugs my daughter was going to go blind. Olivia didn’t react well to the Methotrexate tablets, the nausea and exhaustion were debilitating. Professor Ayliffe responded immediately by switching her over to injections and prescribing Ondansetron, an anti-emetic, which helped to lessen the sickness
After three months Olivia was able to come off the Ciclosporin, but a flare six weeks later which the reintroduction of Ciclosporin couldn’t control led to the introduction of Tacrolimus. An increase in flare and cells a couple of months later resulted in higher and higher doses of Tacrolimus being prescribed.

This led to remission, but not for long. Humira had been discussed for a while, and with previous drugs failing, it was time to make decision. With cells back at 2+, and the risk of this increasing, Tacrolimus was stopped and was replaced with Humira together with the Methotrexate.

Another six months down the line and the cells were back in both eyes. Methotrexate was swapped for Cellcept. Two weeks later at the next check up, Olivia’s eyes were clear! There has since been two dose increases, but only because Prof Ayliffe acts so quickly. Other specialists consider 1+ cells to be acceptable, but even this low number of cells, if left to fester, can and often does, result in damage to the eye.

I still have nightmares about that first six months following diagnosis. Nearly six years later I still have my days where the tears threaten to fall and the anger wells at the unfairness. It isn’t fair that my little girl has to go through this, and I would do anything to take it away and give it to me instead.

It’s not fair that we live our lives according to a new calendar – the next appointment calendar. It’s not fair that my baby has had to suffer being pricked by needles and eat to a timetable when tablets dictate empty stomachs. It’s not fair that she missed so much school in during the first year following diagnois, that she was the only one playing sports in safety goggles.

None of it is fair. BUT, I’m very aware that she is one of the lucky ones. We found an excellent Specialist in Prof Ayliffe, who is as determined as us to beat this disease. I’m safe in the knowledge that Olivia is in the care of a true Uveitis expert, who will give her the best long- term prognosis, and for that I’m truly grateful.

In hindsight I should have asked more questions as soon as Olivia was diagnosed. I should have researched the subject more fully. I should have found Professor Ayliffe faster. I should have walked away from the previous Drs sooner. The should haves have driven me to distraction, and will probably haunt me forever more. However, I’ve accepted that I’m only human and I did what I thought was the best for my daughter at the time with the information I had.

Olivia was and is the fighting force behind the reason for setting up this charity. Nobody should lose their sight because their Dr doesn’t “believe’” in taking an aggressive stance. Nobody should lose their sight because their PCT won’t fund a particular drug. Nobody should feel that they are alone trying to battle a disease nobody has heard of.

In an attempt to help others get through the frightening stages of diagnosis, treatment and the fear of the unknown, Olivia’s Vision was born. Equally importantly we need to raise money. Money for research, money to raise awareness, money to prevent more adults and children alike from losing their sight to this destructive and little understood disease. If you are able, please donate what you can, and with your help, Olivia’s Vision will do all it can to bring about change.