Limbal cell failure and advanced KC.. help!

[Lyricsoprano1]

Hi everyone! Thanks for having me...

I’ve had Keratoconus 17years and copped with RGP’s the whole time. My eyes recently started getting lens intolerant, then 4months ago I suffered Hydrops in my left. I also have Ehlers Danlos which has seemed to complicate things as my RGP’s have caused significant scarring (central scarring made deeper on hydrops left eye).

Anyhow... I recently saw a corneal surgeon privately for topography as I’ve not had it since diagnosed. My regular optom is the contact specialist at our local university hospital.

My corneas are around 300MICROMETRES in the centre
The Kmax readings are 63.5 (right) and 75.8 (left)
And ‘some limbal stem cell failure has been seen inferiorly’
My VA can’t be improved any more in my left (hydrops eye with central scar and 75kmax reading) with vision only improving to 6/60 and my right eye is corrected to 6/36 but I can only wear the RGP for short periods.

I’ve been referred on to Moorfields as with all of the above and the way my skin scars/heals due to Ehlers Danlos, he doesn’t feel he can treat me.

My question: will the stem cell failure around the limbus mean I can’t have a graft in the right eye? Does anyone know the treatment for this..

Is the left eye likely to develop it? As I was really hoping this eye could be grafted due to not being correctable at all.

I’m feeling a little overwhelmed!
Many thanks for listening!
Lyric soprano x

[gsward]

Hi, Lyric soprano and welcome to the forum.

I think the best advice we can give you at this stage is to wait until you have been to Moorfields. They have a huge range of specialists and one way or another it is likely they have seen something like your situation before.

There are other lens options like sclerals that can be effective after hydrops. You don't mention how recently your hydrops was but they do continue to heal over 6-12 months.

So for now "park" your thoughts and concerns, and don't panic.

Graham

[Lyricsoprano1]

Thanks Graham,

Thankfully I’m going privately. I’ve been advised today (by my optom) that the limbal stem cell deficiency in my good eye is likely down to the long term use of piggy back lenses and the dry eye/allergy conditions I have. The advise is to leave that lens out for 6months to see if the cells will regenerate. I think scleral’s may eventually help this eye as it has quite bad scarring from the RGP’s so that’s a good call, thanks! The only issue I have have is frequent scleritis of an immune origin but I like the idea of new lenses for that eye. Sadly, I have a mix of conditions which don’t seem to work together well and so my eyes are very injury prone.

The hydrops happened almost 6months ago but has left a deep, atrophic scar - I scar with a thin papery middle and wide edges due to a collagen defect so have been advised this is permanent. This eye is contact lens intolerant and when I tried the RGP again the stromal oedema returned. It has a scar sized area of vision missing but I’m not so worried as know this could be fixed with a partial thickness graft.

My concern now, is I don’t have a lens in either eye and vision below 6/60 so I’m contemplating how on earth I’m going to work as a music teacher - I think I may need to take a break.

Thanks for your reply! Much appreciated.

[gsward]

That's interesting, piggy-backing is often offered as a solution for intolerance to RGPs but for some of us it does have side effects too. The advantage of sclerals is they vault over the cornea completely and the cornea is then only in contact with saline. Let us know how things work out.

All the best.

Graham

[Anne Klepacz]

It's great that you're seeing someone at Moorfields - as Graham says, there is a huge amount of expertise in KC there plus all sorts of exciting research on stem cells and other solutions for complex cases. As for your work, are you an employee or self-employed? Do have a look at all the information on our website https://www.keratoconus-group.org.uk/in ... s-to-work/ if you're an employee (though I realise that reading music is 10 times worse than reading print when you have KC)! All the best, Anne

[Lyricsoprano1]

Graham, I love the piggy back system, it’s been amazing and saved my sight for so long but I also have mast cell activation syndrome with the EDS and suspect it’s a mix of lack of oxygen plus chronic inflammation - don’t want to worry anyone as for most, I’m certain this doesn’t happen. I’m a trouble maker! Lol. I really appreciate you taking the time to come back to me.

Out of interest, did anyone find the balance between close work and long distance sight difficult whilst wearing RGP’s? I suspect I have a regular, eye muscle co-ordination thing going on too but the more we add to my prescription for the myopia, the harder close work is.

Hi Anne, I’m freelance but teach at a school. I can’t get there for now as obviously can’t drive so I’ve been teaching online with one lens in, but removing the two as has been advised, I can barely see the laptop and cabt see the pupils’ faces to assess technique. I think performing is over at least for a little while as can’t confidently travel or read the scores/figure out blocking (especially with stage lighting and me seeing odd shapes out of the halos). Performance wise, I’m just going to really struggle interacting with other singers as they move to the other side of the stage and they’re gone to me. I’m at peace with my limitations as I’m certain they won’t be forever but I am going to miss it. (Also have five children at home, so partially sighted Opera is just too stressful right now ). I got lost the other evening walking to the toilets because of the odd lighting and ghosting and it really has knocked my confidence regarding doing anything without lenses.

I’m very much looking forward to having happy corneas and have heard great things about Moorfields, so just trying to muddle along for now.

[gsward]

Often with KC it is not possible to get both eyes perfectly in balance for all the things you might do. So for example I have a pair of glasses to wear for driving that corrects a mild astigmatism which you generally cannot do with lenses. I have another pair for reading and screen work, but if I am on a mobile phone at close range I can read the screen fine. Maybe after they have done the best they can with lenses you will need a normal refraction sight test to see if more can be "squeezed out".

[Lia Williams]

Out of interest, did anyone find the balance between close work and long distance sight difficult whilst wearing RGP’s? I suspect I have a regular, eye muscle co-ordination thing going on too but the more we add to my prescription for the myopia, the harder close work is.

This could be presbyopia and a pair of reading glasses may be required! For people who don't have keratoconus there are multifocal contact lenses which like multifocal glasses allow one to see clearly at all distances. I believe that it is possible to get multifocal contact lenses for keratoconus, but given the time taken to prescribe single vision contact lenses for keratoconus, multifocal ones are rarely prescribed.

One solution that can work is monovision. This is where the dominant eye is prescribed a lens for distance vision and the non-dominant eye one for close vision. I find it works well for me and I only use reading glasses for small print in poor light.

My concern now, is I don’t have a lens in either eye and vision below 6/60 so I’m contemplating how on earth I’m going to work as a music teacher

Do you have any back-up glasses? Although glasses will not give you the same standard of vision as your contact lenses they may give you some vision. So often people are told that glasses are no use for them, when they would actually be of some use when contact lenses can't be worn. Or just to give your eyes a rest. Without my lenses I can see very little but glasses allow me to function when I'm not wearing my lenses. I wear them first thing in the morning and last thing at night.

It's worth noting that to get an optimum prescription for glasses takes time. The normal better or worse questions are not easy to answer with keratoconus as on each line some letters are clearer and some are worse. The high street optician I go to allows a longer appointment time for me.


Lia

[gsward]

Lia, useful points you make here. The multifocal option for KC is usually avoided because of the inherent instability of KC. It becomes a nightmare keeping everything in balance over time.

Some of the optometrists at Moorfields do indeed promote the monovision approach, once the patient has got their head around the idea! However, it seems to only work well for a minority of patients.

The refraction team at Moorfields will take as long as it takes, and I have been an hour getting it 'right'. They also really won't mind if you want to take your prescription to the high street if you prefer.

[Lia Williams]

The refraction team at Moorfields will take as long as it takes, and I have been an hour getting it 'right'. They also really won't mind if you want to take your prescription to the high street if you prefer.

I've known people spend two hours in the refraction department.

Often people are only seen by the contact lens clinic and are reminded to see a High Street optician for a full eye test as the contact lens clinic only "examine the front of the eye".

As for getting glasses prescribed to wear on top of contact lenses this is where it gets difficult. The High Street optician wants "permission" from the hospital to prescribe glasses to wear with hospital prescribed lenses.

My reading glasses are from the pound shop.

Some of the optometrists at Moorfields do indeed promote the monovision approach, once the patient has got their head around the idea! However, it seems to only work well for a minority of patients.

With presbyopia any solution is going to be a compromise. Monovision works well in the early stages of presbyopia where there isn't much difference between the two eyes. It works well if both eyes are getting good vision. If one eye still has residual ghosting, which is not noticeable when both lenses are worn, this would be noticeable if the prescriptions were changed for monovision.

LIa