UK Keratoconus Self-Help and Support Association

Hi All. It's been years since I posted anything. My 34 year old seriously intellectually disabled daughter was diagnosed with KC about 5 years ago. It's quite bad but due to her disability she has only had 2 eye exams under general anesthesia - the last being 18 months ago. She was prescribed -12 diopter glasses to try and help her watch TV but she won't wear them. She has the intellectual age of about 18 months so it's very difficult to explain anything to her. She can't speak and the only way we know if she is having eye discomfort is when she rubs or covers her eyes. Her care givers and I believe she has quite a few headaches due to her eyes because on sunny days she is worse than on duller winter days. It's all guesses though. Her eye specialist has suggested he cross links her corneas to try and stabilize them. We are unsure if her KC has progressed since her last exam. The specialist has suggested that she has another GA eye exam and if there is deterioation he'll cross link the worst eye and then do the other eye 3 months later. She was supposed to have the exam done last Friday BUT New Zealand has run out of Riboflavin and until a new supplier is found we have put off the exam. She won't be able to wear a bandage contact lens as she will need a GA to get it out. The specialist has proposed using long acting local anesthesia and antibiotices in her eye then covering it with an eye patch for the 1st 24 hours. I'll also need to restrain her so she can't take the eye bandage off.

I am still in two minds about her having it done. If it was for me then I would but I can understand the procedure, know that my eye will be very painful for a few days and that my vision will be worse until the cornea heals and stabilizes. I have never spoken to anyone who has had the procedure done and so have a stack of questions that I feel the specialist can't answer for me. I understand that a crossliked eye is very light sensitive and that sunglasses should be worn - but Jenny won't wear glasses. I hate to submit her to months of pain / discomfort. She is happy as she is - she got very depressed when her eye sight deterioated and is really only coming right. I have to do the right thing for her but just don't feel like I have all the answers I need. Can someone help??

Looking forward to your replies. Jenny's worried mum, Carleen

Hi Carleen,

I'm sorry but I can't comment on CXL.

However I'm not surprised that Jenny hasn't taken to her -12 dioptre glasses. Two or three years ago I got a pair of back-up glasses which are about -11, and having not worn glasses for over thirty years it took weeks for me to get used to seeing with them, using them for about 1/2 an hour each day. The problem is that with high power -ve lenses there is a lot of barrel distortion so unless you are looking through the centre of the lens verticals appear to bend. In time the brain gets used to straightening up the image - and people whose prescription has got stronger over time don't notice a problem. In addition to the barrel distortion there is also chromatic aberration so looking off centre will give items blue or red edges. I'm not sure what to suggest, also it must be a shock to her to see anything remotely. With a -12 prescription I suspect that Jenny's best vision is less than 6 inches from her eyes. Is there any way Jenny could have a weaker prescription which would improve her vision slightly and as she gets used to the prescription increase it?

Lia

Hi Lia. Thanks for your insights about the glasses. Her vision is probably a lot worse than the glasses are prescribed for because when she wants to look at something she holds it about inch away from her eye. The glasses were only for when she watches TV or is doing activities at a table. The trouble with her is that she hates anything near her face. She won't wear a sunhat either. The only time she will wear sunglasses is in the car when I take her home for a visit. I stop the car if she takes her glasses off so she knows that if she wants me to keep driving, her glasses have to stay on. Funny girl. I also need advice about CXL recovery, pain etc. Hopefully someone will get back to me about that too.

Cheers
Carleen

Hi Carleen,
I haven't had CXL either, so can't offer any personal experience. It must be such a hard decision to make and not helped by the fact that you seem to have had varying advice over the past few years on the best options for your daughter. Looking at your previous posts, I'm a bit confused about the current proposal to treat both eyes, as you mentioned that Jennifer had had a hydrops in the left eye. I'm not a specialist, but I'm not sure if crosslinking can still be of benefit after a hydrops. Maybe one of the experts can shed light on that. For the right eye, the crucial question I guess is how thin that cornea is and whether it has been getting thinner in the past year or so. I think you said in an earlier post that the reason for considering crosslinking was to prevent the possibility of hydrops in that eye. I can understand that you'd do whatever you could to avoid the pain and discomfort Jennifer had with her left eye hydrops. So you're having to weigh the pain of a possible future hydrops against the pain and discomfort of CXL (which, from people's posts on this forum, is perhaps likely to last less time than that of a hydrops). That's a really tough call, and you really need as much information as you can get from the specialist on the likely risks in both scenarios and how likely a hydrops is in the right eye.
All the best
Anne

Hi Anne,

Thanks for your comments. Jennifer has had hydrops in both eyes, worse in the left than the right and both corneas have scarring - again the left is worse than the right. The reason for having the CXL is to try and stop the progression as her eyes appear to be getting worse. Her corneas may be too thin (I'm still waiting for the information to be sent through from her specialist) but the Dr says they can be thickened. . I'm not sure they can be thickened enough to do the treatment though. I'm sure her corneas were about 300 microns 18 months ago. I have read studies that say that very steep corneas may flatten somewhat after CXL so even with stable vision CXL may still be worth while doing.

My main concerns about Jenny having the treatment is management of her eyes, pain, mobility and safety. She won't wear glasses, lives in a care facility with 4 other severely disabled people, has mild cerebral palsy and has no speech so can't communicate if she is in pain or is having problems with her eyes.

I would really love to talk with someone who has had the treatment so I can understand what I am proposing to put her through.

Cheers
Carleen