Keratoconus Cornea – the journey so far

By a parent member

It was early in 2015 when I visited the opticians for my 20 year old daughter’s routine eye appointment that staff first noticed something unusual evident in her left eye.

Our daughter wears glasses and has regular check-ups with the hospital opticians annually. The opticians asked us to refer to the hospital ophthalmology department for further checks.  The appointment came through in September.

Our daughter was given an eye test using picture symbols and then a variety of tests after eye drops were administered. She was fine with the drops and they appeared to give her no discomfort.One of the tests involved using an instrument that takes a picture of the back of each eye. Due to my daughter’s severe learning difficulties she constantly moved about so the nurse was only able to get a picture of one eye. Later our daughter managed to keep her head steady, with support, when examined by the ophthalmologist. She was expected to be very still and perch her chin on a shelf…it helped when I stood behind her and held her head gently. The opthalmologist shined a light into the back of each eye and spent some time looking carefully at both. All these tests lasted about an hour and a half in total.

The outcome was devastating for our daughter…the opthalmologist diagnosed keratoconus corneas in both her eyes!

In keratoconus the transparent ‘window’ at the front of the eye grows abnormally thin and into a cone shape, causing distorted vision and often begins in the teenage years. The prevalence in young people with Down’s syndrome is very high in comparison with the general population.

In my daughter’s case, one eye had quite severe scarring. We were then asked to see a consultant opthalmologist who did another test to see the actual extent of the damage. No eye drops were needed for this test.  It was very quick and the result was that her left eye was too far gone to consider any other treatment but a corneal graft.  In our daughter’s case this procedure would be too difficult as it would involve extensive aftercare. We will seek a second opinion.

Her right eye was within the upper and lower limit to do Cross-linking (CLX) eye surgery, an efficient type of newly available surgery that hardens the cornea. In most cases this cannot repair or make better the vision but can halt the deterioration.  Sometimes contact lenses can be used but this was unlikely to be satisfactory for our daughter because of the complexity of her learning disability.

Being told the left eye was virtually inoperable was such a blow…

We had a period of reflection at this point. Had we missed something? Was there more we could have done?

We looked back and saw that we had been more vigilant than was necessary in taking her to the opticians every year. Neither college, school, family, support workers or respite staff had ever noticed anything either. Our daughter was just herself! But this was the crux – her learning disability had masked the problem because she was unable to understand the deterioration and show us or talk to us about it.

We then watched her each day and realised there had been signs which we had put down to her learning disability. She had been accommodating the eye problem.  These included not lifting her head when we wanted to take her photograph, favouring one direction of looking over another, looking down a lot, spending time indoors and out of the lovely sunshine (she used to be an outdoor girl!!), using the edge of her foot to feel each step as she descended the stairs, not watching videos so much or TV.  We just assumed she was moving on and favoured listening to her music!  In all her activities she seemed fine including dancing, trampoline, swimming.

I telephoned the Keratoconus Group for information and support and they were very helpful. Their information on the internet and by post covers what happens in cross-linking and corneal eye surgery. As it has only recently been accepted as a treatment on the NHS not all hospitals do the surgery. We were relieved that our hospital does do the surgery and have had a follow up appointment at which this will be discussed. The Down’s Syndrome Association have also been very helpful by raising awareness and publishing  this vital information on keratoconus cornea and telling health professionals.

Our daughter will need a general anaesthetic which will not be easy due to her level of understanding and needle phobia. To ensure it is in her best interests to have the surgery, we have asked the Community Team for People with a Learning Disability to be involved as they can devise ways to communicate the procedure to our daughter to help her be aware of what is going to happen. We are hoping for the surgery to happen as soon as possible and will keep you posted as to what happens during the next stage.

Until then…..

Read Rachel’s story of being diagnosed and treated for keratocounus.

Find out more about keratoconus here or download a factsheet version (pdf) to print off and take with you to your local opticians. Click here to download the file.

Help us raise awareness among professionals.

SeeAbilitySeeAbility have a searchable database of optometrists and dispensing opticians who have shared information on their services for people with learning disabilities.

Find out more and access the database here.