A begrudging homage to Aniridia!

My Aniridia Story

A bit of a backstory . . . . .

I’ve never let my eye sight be a problem, that was mainly due to growing up as the
youngest of six and a child of parents who didn’t wrap me up in cotton wool and protected me from the big bad world.  I was a typical boy; football, climbing trees, scrapes, bruises and ultimately stitches! 

Yep of course there was the realisation that I couldn’t do certain things, but I just got on with it, maybe that was the northern boy in me! 

Bizarrely I’ve always been proud to have Aniridia - I liked it that much I gave it to my daughter as a birthday Present! - as a young boy the amount of times at an eye appointment the consultant would usher in a trainee nurse or doctor to see the boy with the rare eye condition, ‘Wow, no iris!’,  It was normal to me.

For a Partially sighted person, my sight wasn’t bad at all, throughout my life it was a steady 6/60, that meant I could see more than most. The aniridia helped in the dark too, every scrap of light would be noticeable, enabling me to plot a path through a busy night club to the bar, it had its benefits!

Things started to go wrong when I was 16, the inevitable Glaucoma made an appearance, both eyes had to have eye drops and a regular six-monthly check-up cycle started that didn’t stop well into my 30’s.

In my early 20s the glaucoma was having an effect on my right eye and I had a Trabeculectomy operation in the early 90’s (at Liverpool University Hospital) to control the pressure.  That was it for about 10 years; college, marriage, kids, employment at RNC and a further Trabeculectomyoperation on the left eye – this time at Hereford - went past the eyes easily and steadily.  But by this point the internet had arrived and that brought information.  I knew that due to knowing more about Aniridia, my eyesight would worsen.

In my late 20s onwards I’ve been under the care of Dr Deutsch at Hereford County Hospital.  My sight in my right eye was starting to worsen, I couldn’t see things clearly, the cause; another Aniridia friend, the Cataract!  In the space of a few years I had cataract surgery on both eyes and additional pseudophakiaoperations on both eyes.

The right eye was a constant problem with ulceration cornea scaring, which resulted in my sight being down to 1/60 in the right.  Dr Deutsch and his team had been brilliant, but they couldn’t do any more, Aniridia was winning the war, my eye sight was beginning to worsen in both eyes.  At that point it was a scary time, I’d never stopped to think about the consciences of losing my sight, but now, it was getting real!

The last operation I had at Hereford was a cornea scrap! I was awake and could see everything . . . not the best operation I’ve had!

Dr Deutsch looked for specialists who could possibly help, and I was firstly referred to Birmingham Queen Elizabeth Hospital. Unfortunately they couldn’t help, but sent me to another Hospital in West Sussex, and there the battle turned . . . . .

I would of gone to the ends of the earth to find any hope that my worsening eyesight could recover and at that point I was hoping that the mist would lift and I could see clearly, I wasn’t thinking about stem cells and transplants, they were things I would never see in my life time surely.

I was always told that I couldn’t have a cornea transplant, there’d be no point, the reason being; another Aniridia effect, stem cell deficiency which ultimately means the cornea is defenceless to attack from the white cells of the outer eye, Step forward into the spotlight Dr Hamada. 

In early 2014 I walked in to Queen Victoria Hospital East Grinstead with low expectations, I couldn’t have been so wrong.  I was told if initial treatment went well, I would go on to have stem cell replacement and then a cornea transplant.  The positivity and the support from the team was great, it would be a long road, but I just hoped everything would go right.

The First operation was to clear the scaring and ulcerated damaged layer on my right eye,Superficial keratectomy, that would take a while to heal and thankfully all went well.  Next would be the start of the COMET Process.  

The COMET process as I see it, is, first defend the cornea and reboot the stem cells in the cornea sub layers, if all goes well, a Cornea Transplant is a possibility.

So firstly, in the first half of 2017 blood was taken and a stem cell harvest sample operation was taken from the inner part of the bottom lip (note to self, don’t have salt and vinegar crisps after the operation!) the cells would then be cultivated in the lab for up to 5/6 weeks and step 2 would go ahead.
A COMET Stage 2 Operation was done, this is where the reboot happens with my own Stem Cells being inserted into the cornea.

At this point I had regular check-ups for about 9 months to see if the cells had grown and were doing what they should be doing, thankfully the Cells were at a good level and the eye was more comfortable than it had been for a while.

The right eye was stable and was ready for the big Transplant.  On January 4th, 2018 the right Cornea was partially transplanted and went well.  A few days later I started to notice a sharpness in the vision, there was still blurring and double vision going on, but definitely sharpness, this was to good to be true! 

One year on and the eye has been getting better every day, week and month.  Regular check-up at both Hereford and East Grinstead have shown steady progress. The right eye can see the top letters again, not perfect, but hopefully after the next step this particularly battle may be won, that next step is to come, shaping the cornea to maximise the vision, it would complete the right eyes 20 odd year battle with Aniridia. 

Its now time for the left eye to do battle, hopefully it’ll go the same way.  On the 14thof February 2019 blood and stem cell harvesting was done, quickly followed on March 7thby stage two.  One week in and the eye is a bit sore and irritable, but it’s the start, and I’m confident.

As mentioned above, bizarrely I’m proud to have Aniridia, its defines me, makes me who I am, but it’s never taken over me.  

I’d like to publicly thank Dr Deutsch for all the years of support and care at Hereford and Dr Hamada and his team for bring back my sight, here is to another year of success!


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