Update on Charlotte's hydrops and visit to hospital

[Charlottes mum]

Well here's the latest on Charlotte and her hydrops. Its day 15. Yesterday she had an appt at Hull Eye Clinic with the corneal specialist. Unfortunately he was on call so we saw one of the doctors instead. We've never actually ever talked with the consultant yet. Bit miffed about that but I understand his schedule is busy........We have another appt for Charlotte on 5th March and I've asked that given her current setback and that she's 3+ years since diagnosis, that we do actually see the consultant at that nx appt.
Dr checked Charlotte's eye. No real news, still very cloudy, not so much pain, more discomfort especially when she is tired. We asked about position of scar but he explained that he cannot say at this stage. We expected that too really. Her eye looks nice and clean he said, and that although her cornea is obv very irregular there looks to be no rupture to the surface of the cornea. So that's good to hear too. Charlotte was very good and patient and we were proud of her as she was obv not happy to be back there. Bless her.
We asked the Dr about future treatments, he went a bit "mumbly" when we mentioned the poss looking into CXL on the right eye (which he said looks stable).We are keen to look at all viable options for Charlotte but realise that as her left eye is still cloudy that it's difficult to make plans until we know how it will be after the hydrops. We can discuss options for soft contacts at next clinic visit he informed us. I know the dr and the optom are keen for Charlotte to wear her right eye contact (RGP) but she is not at all happy to have ANYTHING in her eye at the moment. I am not willing to insist/try to persuade her to wear the RE contact while she is in discomfort in her left. It would cause her further distress and that's just not fair. Her Dad and I have discussed the wearing of it with her and she it quite sure she doesn't want it in! So that's that for now with the wearing issue.
I guess it's still a waiting game as to how the hydrops will affect her eye, months to heal they said which we knew anyway. I know that Charlotte's left eye vision was "not best" prior to hydrops and that she hasn't gone from 20/20 to her current very low vision (only weird bits thru top bottom and sides she tells us) but I still feel physically sick with worry that she will not regain much back in the months ahead. I know her RE is working extra hard now which is why we want to look into all poss for her to keep that one going strong.
Another thing is that prior to the hydrops when I looked at her LE in profile I could see the cone and it was very pronounced but since the hydrops it's not as much. However when she has her eyes closed I can see more of the shape beneath her closed lids on that LE. I mentioned it to the Dr and he said it's just all part of the swelling and the hydrops. Her RE in profile is only very slightly conical, hardly noticeable at all. I know but that's because I know what I'm looking for now but other people wouldn't notice.
I think I need to "get a grip" as I do realise that I am in danger of becoming ill myself with worry and then I'll be no use to anyone.
Feeling very low and frightened for my daughter, Angie S.

[longhoc]

Hi, just to say I think you're handling everything very well considering !

In this sort of situation, I always think of those information presentations that flight attendants give when you're on a plane... "fit you're own emergency air supply mask first, then attend to infants who need assistance". They don't spell out why, of course, but the implication is that you'll keel over through lack of oxygen and then you'll be in no fit state to help anyone else. So easy to say, of course, when it's not you in that position...

Difficult for me to think exactly what is best for Charlotte, I am reminded of how I react sometimes to what the professionals tell me what I should or shouldn't be doing. When it is a clear case of medical need, I always go along with them. But I too was advised to keep wearing my RGP lens in my right eye after my graft to maintain "some vision". Well, I tried, really I tried. However, I ended up with headaches, tiredness, dizziness and poor co-ordination no matter how much I attempted to compensate for only having semi-good vision out of one eye and pretty much nothing out the other. And I could rationalise that though I was suffering, it was "for the best" which lessened the distress -- Charlotte may not be able to make that cognitive connection so might be even more distressed than I was.

I asked the optometrist what, exactly, what the necessity of doing that course of action and the answer wasn't particularly clear cut, just some generalisation about "it will make adaptation easier later on" and "better to have the very best vision you can get even if it's not that brilliant". I wasn't, ah-hem, especially convinced and there wasn't any medical justification so I stopped the (for me) unpleasant regime of one lens only in one eye.

So, my take on this is, from what we keep getting told, we should always listen to the child and listen to the patient and unless there's clear medical evidence to the contrary be guided by what they/we want. After my graft, I'd been through quite enough already without any more aggravation and felt a lot better by being able to make choices which were the right ones for me rather than what anyone, however well intentioned but who didn't actually have to endure what they were asking me to endure, said I should do.

Maybe for Charlotte, taking a break from the lens for a while, if that's what she wants to do, can give her a sense of control back in what has been a situation completely outside what she can influence. Perhaps after a bit of time has gone past, she'll want to get a bit better vision back again and will ask for the lens. Okay, you'll probably then need to re-establish tolerance times over a few weeks but it might be worth it in terms of Charlotte's quality of life.

Have a real worry I'm completely out of my depth here and talking about things I haven't really got a clue about, not being in the position of such a caring mother to Charlotte so I'll quit with the advice giving here ! *

Good luck to both of you.

Best wishes

Chris

* Except to add ( ) that sometimes it doesn't hurt at all to be a bit stubborn and bloody minded so don't hesitate to put your foot down and insist that Charlotte gets seen by the ophthalmologist. Only s/he will have the expertise to give you proper guidance on what the next steps should be.

[Lia Williams]

Angie

You said

Maybe for Charlotte, taking a break from the lens for a while

Did Charlotte use her glasses for back-up? And is she able to use these now? I ask because I for one didn’t bother keeping up a pair of glasses and then when I did get a pair they were effectively useless. In fact for years I understood that glasses would be of no use to me at all. The problem is two-fold:

Firstly ‘spectacle blur’ is caused by RGPs and some demoulding of the cornea is needed after wearing lenses to have optimum vision with glasses. This why some people have their eyes tested late afternoon for back-up glasses if they want to wear glasses in the evening, and others in the morning if they want to wear glasses before they put the lenses in.

Secondly if glasses haven’t been worn for some time (in my case it was more than 20 years) the brain takes sometime to get used to them. I have a high –ve prescription which means that there was a lot of barrel distortion so while I could see with them while I was sitting still, I found it very difficult to walk round the house wearing them as the whole world seemed to swim round – most disconcerting. It was easier to exist in a total blur when I had removed my lenses. I did learn to use the glasses again but it did take time – an hour a day for a few weeks.

So if Charlotte is resistant to wearing glasses you could suggest that she wears them to watch to TV where she’ll notice an improvement. Also if she did have soft lenses in the future the problem of spectacle blur is much reduced so it would be easier to switch between lenses and back-up glasses.

If Charlotte is offered soft lenses but is resistant to trying them you could use the analogy that soft lenses are like a comfy pair of slippers whereas RGPs are like a pair of high heeled shoes!

Lia

[Charlottes mum]

Thank you Lia and Thank you Chris,
We very much appreciate your replys, Lia, Charlotte hasn't worn specs for over 3 years now since diag in nov 2009. she'd been wearing off the end of her nose for ages before diag which is what prompted us as parents to get a referral back to the clinic because we knew "something wasn't right" (and if I had a penny for every Down's kid I see nowadays who does the same and I obv wonder if they are heading down the KC road too!!), I wish Char could fit a script for specs but I do understand the KC complications with glasses. We will be asking all about that at our next appt in 3 wks tho.
So thanks again to all who have read about Charlotte and your kind wishes, we wish you all well too and Charlotte says, "Hi".
Angie and Char x

[GarethB]

I am the type of person that takes no deterioration to be good news and getting better as excellent, so it is good to know Charlotte is no worse which is a start.

If you are seriosly thinking about soft lenses, the longer Charlotte can go without an RGP in her other eye the better as all RGP's have an influence on the cornea either flattening or drawing out to a more conical shape depending on fit (even an 'ideal fit!).

Glasses even if they don't give ideal vision, Charlotte might appreciate having some improved vision even if it is limited just to ease the straign on the right eye. From what you describe she has made quite a rational decision in deciding she would rather go without lenses at the moment and bearing up well.

When I first tried a silicone hydrogel lens for KC I had to ask if the lens had been put in, it is that bigger change from an RGP lens with respect to comfort. I was on a cource once with optometrists learning to fit lenses to KC patients. One tried a normal RGP lens for his normal eyes and within 15 seconds he was wanting the lens removed! After that he had an appreciation of what I meant that you learn to tolerate an RGP lens, after this he tried a soft lens where the comoft is at the other extreme of the comfort scale.

I wish Charlotte and yourself the best of luck in her recovery.

[Charlottes mum]

Thank you so much for that Gareth. It is good to hear of your experience with the soft contacts. We feel that we need as much info as possible so that we hopefully have a better chance of making the best decision for Charlotte. So cheers for that!