I thought I would start my diary with a little bit of information about Keratoconus and some background on myself.
Keratoconus (KC) is a progressive, noninflammatory, bilateral (but usually asymmetrical) disease of the cornea, characterized by paraxial stromal thinning that leads to corneal surface distortion. Visual loss occurs primarily from irregular astigmatism and myopia and secondarily from corneal scarring. All layers of the cornea are believed to be affected by KC, although the most notable features are the thinning of the corneal stroma, the ruptures in the Bowman layer, and the deposition of iron in the basal epithelial cells, forming the Fleischer ring. Breaks in and folds close to the Descemet membrane result in acute hydrops and striae, respectively.
I have had very poor vision my whole life, I believe I have had keratoconus since about the age of 11 but I wasn’t officially diagnosed until I was in my early 20’s. When I got my first set of RGP contact lenses (after many pairs of glasses that just didn’t cut it) it was incredible. I have no idea how I managed to get through school, learn to drive and start working with the vision I had. With the lenses I could actually see colours that were bright and bold instead of washed out and faded looking. I could see the leaves on the trees and the number on the front of the bus. So many times I walked past people in the street and didn’t acknowledge them. Not because I am rude, I just didn’t see them, now I could see so many people it was almost overwhelming.
I guess I wore RGPS for about 5 years then my eyes started to drastically change. I lost so many lenses because my corneas were so steep they kept falling out. I remember one time being in a meeting at work and both fell out and rolled across the boardroom table!
At this time I was living in the UK so went to see an Optometrist by the name of Ken Pullum. He basically reinvented the modern scleral contact lens. Scleral lenses literally saved my life, without them I would not be able to work, drive, read or do any of the other things that I love to do. They are a large lens that fits over the whole of the front of the eye. As they sit under the eyelids they can’t fall out and they give excellent vision. They feel really bulky at first but once you get used to them they are very comfortable. For the first few months of using them I had a massive phobia about putting them in and taking them out I found it really difficult and stressful. As you can see from this picture they are a lot bigger than regular RGP lenses.
After about 12 years of wearing the scleral lenses, in 2005 I moved to Australia and continued to go about my business and day to day life. Life was fantastic; I was literally living in paradise. I thought that my KC was stable and that would be the end of it. I was so wrong!
My vision steadily got worse over the years but then in 2014 I hit an all-time low. I developed scleritis in both eyes. Scleritis is an extremely painful inflammation of the sclera which is the white of the eye. At times it was so painful I would just lay there moaning and crying wanting to rip my eyes out of my head. Because of the massive inflammation in my eyes I started to develop corneal neo-vascularisation. The corneas don’t have blood vessels in them they are clear windows; they get the oxygen they need from the air. Well my eyes were so inflamed I started to grow large deep blood vessels. These have caused a lot of complications. Because they are deep in the cornea they block the light from entering the eye and reduce my vision. At times they leak blood and fluids and my corneas go a hazy white and I can’t see at all, sometimes for weeks until the haze clears. Thankfully this usually only happens in one eye at a time. This is a picture of my scleritis eye.
I am being cared for by an awesome eye doctor and rheumatologist. My scleritis is being difficult to treat, it is stubbornly refusing to get better. I have been on many drugs including Methotrexate, Azathioprine, Rituximab and Cyclophosphamide. So far nothing has worked well enough to give me long term relief from the pain of scleritis.
It’s a stalemate because my doctor will not give me the corneal transplants that I need until we get the scleritis under control.
Fast forward 3 years to 2017 ….
I am still struggling with scleritis, my vision is getting worse and I desperately need to get new corneas. My doctor has agreed that we have nothing to lose by grafting my left eye now because I pretty much have no vision in it. I am struggling at work and I no longer drive at night. I see washed out faded colours and can no longer enjoy my hobbies. It’s really limiting my life. Before the surgery he is going to zap the blood vessels with a laser because if he does the graft with the vessels there it will be a disaster and the transplant will more than likely reject.
I am due to start a new treatment in the next few days. Either Humira injections or Infliximab infusions. My doctor says that once I am on this and the pain is controlled somewhat he is happy to proceed.
I seriously cannot wait, I am sad that someone has to lose their life in order for me to get the gift of sight but after being seriously vision impaired for so many years I am just so excited at the thought that I could get near normal vision. I am 48 years old and I have never had anything near normal vision so this is a really big deal for me. A life changer.
This is the start of a new journey for me, I hope that my experiences can help someone else with their own journey.