UK Keratoconus Self-Help and Support Association

[list=][/list he added that its highly unlikely that Charlotte's cornea will ever return to normal. soooo, not nice to hear but we already knew that pre hydrops Charlotte's left cornea was far from normal. Bothers me that he said its the worse he has ever seen because I have read lots mainly on here about hydrops that looked so so major but that the cornea actually looked ok in the end and even surprised the Drs and Optom etc. At this point I feel so very bad for Charlotte because she was never totally happy or comfortable with contacts (RGPs) we had to do an awful lot of cajoling and bribery to get her to wear them. she has marks on her cornea from the contacts which even I could see as far back as up to 2 years ago. post on here somewhere. it was a dimple vale which we now know can be caused by poor fit contacts. the Optom has tried lots of fits and I don't have a problem with her treatment. I just keep wondering how that left eye would be today had we not gone down the contacts route for her because for 6 months last year we said we couldn't badger her anymore and she was ok, I know her visions not best but she was happy, beautiful big blue clear eyes, not sitting right on top of the tv, she was writing nicely, reading ok not book to nose. She's the same now except for that left eye is just so sad looking it breaks my heart. And yet she carries on all singing all dancing as always. So hard.

It could be that your consultant just hasn't seen many cases of hydrops so based on their expereince Charlottes is the worst they have seen but by another consultants standards that has more expereince of this may consider Cahrlottes case as normal. These things are all relative.

What concerns me more is the marks left on Charlottes eyes from the contact lenses and many fits have been tried I am afraid sounds like the way I was treated 25 years ago, but back then all we had were RGP's. I may be wrong but it does sound to me like Charlottes only expereince of contacts is RGP lenses of which there are many but being rigid are far from forgiving to a damaged cornea. My personal view is that they should have already tried soft lenses, hybrids, semi sclerals or scleral lenses which in the case of soft lenses are very forgiving on the cornea which is why I find them so comfortable and the scleral lenses vault the cne of the cornea completly so don't leave any marks.

Hindsight is a wonderful thing but from my expereince back in 2004 when I had to go back to contact lenses and then I was told they were doubtful my vision could be corrected I worried to the point that I had to be treated for depression. An expereince I hope never to have again and it was the help of this group that got me through that phase and it now takes soo much more to get me stressed or frustrated! Now I plan for the future and look at what I can do and could help maintain my vision for the future and I am now aware of many options that are available to maintain my vision in the future. So there was a positive from the depression in that my outlook for the past eight years has been far more positive.

Like you I worry as a parent and my daughter has annual eye tests and topographys so that if she develops KC, we can catch it early and i can give her the support based on my experience. My parents did their best but like you I am sure experienced frustration that they were unable to do more because they only saw the problems that I had despite the fact that I had developed my own coping strategies and I was as chierful as ever.

Many people that post here do so because they are experiencing problems and are wanting help so the more extreme cases of KC are likley to be shared on these forums. You get the same on other forums such as Arthritis, my wife thought because she has developed it so young she would be in for a rough time when in fact she is managing well so doesn't visit the forum as she doesn't need help or support since she got the answers to her initial concerns, but she knows where to go for help I the future.

Thanks GarethB. Yes Charlotte does seem to have developed coping strategies and I thank goodness that she has, I think that I myself need to follow her lead in that because I don't feel that I am coping inwardly - feel helpless at best/crushing guilt at worst.
My priority now is for that eye to clear (how long is a piece of string?????) and to see what the specialist advises. Appt nx Monday morning. I know one thing for sure; I am not allowing any RGPs in her eye ever again, no matter what they tell us. Charlotte's not happy about it, and we're not happy about it so it's not happening. Open mind re the softs as I have read lots about them (positives) but the upshot is the actual getting anything in Charlotte's eyes as she will now equate contacts with horribleness and hydrops - she's already indicated this and putting my feelings aside I can't do it to her, its not fair. I want more than anything for her vision to be ok, she's only 20yrs old and has Down's syndrome as you know, but if at any point Charlotte tells us that she just wants "them" to leave her eyes alone then I'll have to go with that even though I know there are options that could help her vision improve. I never put words into her mouth, she knows her mind and is very willful when she needs to be! Wondering now if that left eye will ever regain much vision even when the clouds gone, given the irregularities on the surface? If not that pretty much rules out CXL on her right eye if it was viable and if she would have agreed (which I doubt she would have) as she would then potentially have both eyes impaired albeit one temporarily. We know that left eye vision wasn't great pre-hydrops but now with the cloud she can literally only tell light from dark.
On Sunday my husband said he thought the cloud looked different, half of it looked lighter but still v.cloudy, (I think the same) and he mentioned this to the registrar yesterday because although he was not in the appt with us I rang him at work and had the registrar speak to him direct so he could hear first hand what he thought as I knew that I was stressed and might not convey exactly what was told to me) anyway, the reg. (who saw cloud for first time yesterday so had no other visual comparsion) said it was likely to be signs of healing.
I haven't asked Charlotte if she thinks it's less because I don't want her to worry if it's not, she will tell us when its improving enough for her to notice and I don't want to "nag" her or cause her to stress over it.
She's gone off to her day centre this morning a bit sorry looking and full of cold but determined to go as she doesn't want to miss her activities, she always has a plan for the day! Proud of how she's coping, wish I was as good!

Hi there Angie

I'm dithering about whether to write this because I agree entirely with Gareth's summary -- with rare conditions like Keratoconus (and then adding on top not universal events like acute hydrops) it's not, as Tom Jones would say, unusual to find even specialists to come out with comments like "that's at the poor end of the scale" or "that's quite severe" when actually it's probably not at the really, really bad end of the spectrum at all, it's more that the people concerned simply haven't encountered enough patients with our condition to accurately place it in context.

However (you knew there was a "however" coming I'm sure ), it might be worth at least getting your heads collectively around what to do if you're in the position of having to opt for a graft for Charlotte in order to regain usable vision in the affected eye or because the best advice you've received from the professionals is that Charlotte's cornea should not be left as-is. It might, of course, not come to that. The hydrops could heal and leave Charlotte with a scar-free cornea or even if there is scarring, it could be well out of the way of the centre of the eye and only affect peripheral vision slightly. And it is also a possibility that Charlotte's vision may be better post-hydrops than pre-hydrops because the scarring may even improve the geometry of the eye as a happy accident. If I remember correctly someone (might have been Anne) told me that in the dim and distant past, surgeons used to induce hydrops in Keratoconus patients because the outcomes sometimes were that vision improved. It was a desperate measure but there wasn't at the time anything else much to be done.

But, looking on the gloomy side, if a graft is suggested either to improve vision or because the cornea isn't thought to be stable in the long term how do you and Charlotte feel about that step ? It's quite okay in my view to not think at all about it until it happens, because it might not. Alternately, if you and the family want to at least ponder on it then you can (when Charlotte has her consultation with the specialist) have some points to discuss that you've already explored together. The main things to consider for anyone thinking about a graft are:

1) Am I doing it to improve vision or because the thinning of the cornea is such that it's likely to leave a residual risk of further hydops occurrences ? (your ophthalmologist can guide you on this one to a degree, but if vision is very poor, you can certainly consider a graft simply to try and improve the vision)
2) Will I be able to manage the immediate post-operative recovery period (eye patch wearing, not rubbing the eye no matter what, medication regime (drops for several months), daily hygiene and hospital visits ?
3) Are the risks worth the potential benefits and will the short, medium and long term implications impact quality of life to an acceptable or unacceptable degree ?

As Keratoconus is a medical condition not uncommonly co-existing simultaneously with Down's, it would be great (and not all that unlikely) if the ophthalmologist has some experience with performing grafts on young people with Down's too. Wouldn't hurt to ask if s/he has.

Like I mentioned, I'm a bit conflicted as to whether it is even worth thinking about the possibility of a graft when it might not be required at all. The flip side of that is, if that's the big scary thing which is at the back of everyone's minds anyway, better to bring it out into the open and at least think through the implications consciously. I don't know if Charlotte has ever had to have a major operation and how it affected her... if not, then perhaps some gentle probing might elicit an initial reaction. If it's a big resounding "no way !" I've a hunch she'll let you know

Best wishes

Chris

Thanks Chris.
I really appreciate you taking the time to read and respond to my post. I agree with what you wrote re the comments from the Dr yesterday and varying degrees of hydrops he May or May not have seen in the past. I did kind of bite my tongue when he said it, not least of all because it was obv right in front of Charlotte!
my husband and I have had the "graft" conversation. we have tentatively discussed hospitals and operations with Charlotte, keeping it simple and casually (whilst inwardly quaking) dropping into every day chat about maybe getting a new cornea one day in the future if the Dr said it would help her see better. she needs plain and simple language and words that she understands as you'll appreciate. it terrifys us tho and she has never had an op before. the whole thing would be so frightening for her. not to mention the recovery/keeping her off the eye etc. even if she had it, explaining to her and getting her to understand the implications of it would be incredibly hard. she would be expecting to wake up from the op to see ok instantly so that side of things would need exploring too. I could do the caring no probs but from her point of view its a massive undertaking. lots to think about tho just in case.
Angie

Chris, it was was quite common in the 60's and 70's to induce a mild hydrops so the remaining scar would strengthen the cornea in that area. Since posting on thsi forum and attending KC group AGM's and meetings I would say it is pretty much an even split between those who have had improved vision post hydrops and those where it has been worse.

I have been contacted by people a week or less post graft cncerned that their vision isn't instantly impoved immediatly they woke up from the operation. For me this is difficult to sympathise with because when I had my first graft (coincidentaly my worst eye now!) at 18 my mother was horified to see me walking up the coridor with no vision correction as if I cold see perfectly normaly!

My vision was good enough that I could see where i was going and I could sit a few feet further from the TV than I used to with unaided vision to wathc the formula one! I did get told off later by the nurses for leaving my room who then got told off later by my consultant for fussing too much as I would need to look after myself sooner or later and what better place to learn than in a hospital.

From your previous posts, it does sound that when it comes to Charlottes health, she is pretty switched on and understands what is happening even if she doesn't understand why it is happening. It does sound as if you are very close and that she trusts you implicitly, therefore I was wondering if it would help if you had an RGP in your eye, so she could see / sense your reaction followed by having for example a soft lens put in your eye before she tries it. For one minute I don't expect it to be the most pleasent experience for you, but it might help Charlotte and I am sure you to describe the experince and the differences between the two.

Having a graft is initially uncomfortable because you can feel the stitches and unlike a contact lens you can't take them out but you do get used to them and for me they were less of a discomfort compared to an RGP lens.

Thanks Gareth.
I have had a soft contact in by Char's first Optom to get her to see me have something in but never an RGP. worth a go tho.
I have today found the icf page on face book which I shall have a good read of.
thanks again to all your support. I don't know where we would be without it.
Angie