UK Keratoconus Self-Help and Support Association

Hi Everyone

Just posting as at the moment I am feeling alittle bit shell shocked.

A little bit of background info is that i am 47 years old and was diagnosed at the age of around 19 with KC in both eyes.
Over the years I have worn RGP's mostly in the right eye as the left has always been a problem.

Anyhow about 2-3 years ago my now 21yr old daughter was diagnosed with KC in her right eye. After numerous attempts to fit a CL at the local hospital (to be honest I dont think she ever really preserved for long trying it) but the CL fitter did say it was difficult for a pt to tolerate a lens in only one eye - we decided to get a referral to another local hospital.

We have been today and this is the thing that has shocked me - they have tested her eye and found that she is extremely short sighted but the optician said the astigmatism wasnt as bad as she thought it would be - apparently her topography showed that eye to have a thickness of 350.

Anyhow the consultant thought with her short signtedness and astigmatism he didnt think they would be sucessful in fitting her with a CL. He thinks the way forward would be a graft on the right eye - (he says to think about trying to sort that eye out before the other eye shows any decline - apparently her left eye vision is still perfect but still on the thin side of 480.

I just feel soo upset - i thought the appt was just to fit a CL and never ever imagined a corneal graft would be mentioned.

We have said we want to trial contact lenses even tho he said he wasnt confident that they would beable to get a acceptable fit.

Does anyone know if intacs would be a option with a corneal thickness of 350 - or CXL.

To be honest I was soo shocked I now have all these questions and fears that I didnt ask him when i was in.

Sorry for this long message but needed someone to talk to.

Any advice would be appreciated.

Libby

Hi there Libby

I'm always disheartened to read a post from who's having a bad time (either personally or in connection with a family member). But well done for asking some questions. Sometimes things aren't as cut and dried as you're first advised -- I've posted before on how sometimes the NHS system doesn't always click into place without a little nudging ! So, don't be too fretful if you can help it, and I think you're doing a fantastic thing supporting your family member. That will be a big help to them -- just knowing they're not facing things alone.

Turning to your specific questions -- I can relay the information passed to me when I was in the same situation. The criteria for Intacs is (from memory) 310 microns of corneal thickness (give or take -- I don't recall the exact number, but it's in that range -- I remember because I was 290 and thought to myself "darn, just outside"). I think you're daughter is in the right area if they're 350. Not always made clear when the Pentacam is run and you get the output given to you (that's the bright concentric rings thingy you might well be familiar with where you look at the dot in the middle and the machine prints out a coloured "map" of the eye) but readings can and do vary. It's not an exact result, so the quoted 350 may well be pessimistic. And it might only be a very small peripheral area nowhere near where the Intac might reside. I'm speculating, but you can probably tell that a specialist who is really knowledgable about fitting Intacs would be able to tell you far more than you've been advised thus far.

I'm less sure about CXL i.e. whether or not it is worth doing with that degree of thinning. I'm sure that other informed opinions will be along soon with an answer. It's worth keeping in mind that even post-CXL, the CXL won't typicaly alter the shape of the cornea or reduce the cone, if present. But then you can then consider LASIK or similar to reschape the (now hopefully stabalised) cornea. So, that's another avenue.

It's definitely worth thinking about consulting with a surgeon on these points. Skilled optometrists are the closest thing to miracle workers in my humble opinion, but they usually defer to surgical expertise once you're outside the realm of contact lenses and their fitting.

Another option is non RGP lenses. Let us know if you want a bit more guidance about what's available in that line.

It's scary stuff sometimes when you need your vision fixing but the options on the table aren't suitable for you. But please don't worry -- and ask your family member not to either (they probably still will, but at least you can be the calm and reassuring one )

Best wishes, and as I say, shout up if you want anything more specific on any of the points I've mentioned above.

Chris

Thanks Chris for you kind reply it was very helpful.

We are going to go down the CL route and try everything going before we even think about transplant.
Next time we see the consultant Im going to have a written list of questions ready so I dont forget anything. Even then no disrespect to the consultant we have seen today but think we will ask for a further opinion before going ahead.

Dont mean to sound pessimistic and I know that we should be grateful that grafting is an option (and I am grateful) but I want to make sure that we have exhausted all other options before going ahead.

Again many thanks
Libby x

Hi Libby,
You're right to be making sure that all other options are explored before thinking about a graft. But according to the various consultants who've talked to us at conferences and regional meetings about Intacs and CXL, 400 is considered the minimum thickness for both these procedures. It is possible to artificially swell the cornea for CXL to 400 if it's a bit below, but I'm not sure that would be possible with a thickness of 350, given that part of the epithelium then has to be scraped off before doing CXL.
But have all contact lens options been exhausted? It sounds as though your daughter has only tried the standard rgps - but there's piggy backing, hybrid lenses, semi scleral and scleral lenses as well (and possibly the special soft lenses for KC though if she's very shortsighted they may be more difficult - wearers of these lenses would know better than me). And in terms of getting used to lenses, it may be easier to have lenses for both eyes rather than just for one, even if the non KC eye has a lens with little or no power. So worth exploring the contact lens route further while she thinks things over. Though if it does end up being a graft in the end, there's lots of us here that can tell you it's worked well for us!
All the best
Anne

Hi Libby,

I agree with everyone else here that grafts should be seen as last resort and that as other types of lenses have not been tried, I feel these ought to be explored first. A soft lens would give least sensation if used in one eye - and they can be supplied up to -30D or even more so power is not an issue. If these do not work, then scleral or semi scleral tens to be tolerated better than smaller lenses.

Anne is correct about cross linking though it is worth saying that research is ongoing in this area, so that holding off a decision on grafting using contacts while seeing if CXL could be possible in a year or so seems like a good idea. What I would emphasise though is that although the other eye is fine at the moment, Have it monitored regularly and if it shows further signs of thinning or progression, go for CXL on that one.

Lynn

I dont know the exact details but when I was referred for possible Graft surgery I ended up with my scleral and to be honest once you have gone thru the process of fitting and the 'Oh my God I will never get used to this' phase my Scleral is my best friend its just a case of sticking to your guns persevering not giving up and having people to talk to who understand ie Us.

(WARNING – LONG POST – SIT DOWN WITH A CUP OF TEA ADVISED! OR COME BACK WHEN YOU’VE GOT TIME TO SPARE )

Hi again

Just to return to the subject of "what's the minimum corneal thickness recommended for Intacs" question... I did want to really pursue this option with my surgeon because it was -- seemingly -- a win-win solution (better vision and freedom from lenses potentially). So I asked to explore the limits of what would be ethical to consider having them fitted as a procedure. That's where the c. 310 micron figure came from -- that seemed to be the "pushing the envelope" area, and then only if the patient really wanted to go down that route no matter what. Lots of sucking through teeth on the part of the surgeon (the one I had was very good -- didn't explicitly say "oh no, you're not having that" -- but did let me know when he thought that something was ill-advised) so I took the hint.

Worth recapping on what the US jurisdiction has to say on the subject of Intacs. They are not prohibited, but they are only given "Humanitarian Device Exemption (HDE)" which I think means in layman's terms "look, we're not exactly convinced this is suitable for universal adoption (that would be given the 'Safe and Effective' tag) but in some cases they do some good and they are rarely a complete disaster -- and you can always remove them and in the worse-case you can still have a graft later on which usually isn’t complicated by previous Intac insertion/removal. There are some complications and you (the practitioner) needs to be straight with the patient about the risks and not sell them as a cure-all"

One of the best methods I've found in a assessing what procedure carries what risks and what potential benefits is to use information from the insurance industry (that's the industry I work in). This sounds incredibly perverse, but actually there is some logic to it. Bear with me and I'll try to explain. Medical insurance is incredibly tightly regulated. Insurers can't refuse to pay out for a procedure that will be benefit a policy holder (subject to the policy's T's & C's not ruling out the condition for other reasons which gets complicated but aren't a factor for the point I'm making here). But they can legitimately refuse to pay out on untried or very marginal benefit treatments. They can also refuse to cover a practitioner for professional indemnity if they think that there may be hoards of miffed patients (or worse, deceased ones !) pursuing claims for malpractice.

A vast amount of legislation and case law governs the decision making process. What it boils down to is that the insurer can set very explicit (down to incredibly low level medical detail) on what -- for a given treatment for a given condition -- claims can be made and what can't. The precise margin at the boundary and the exclusion criteria get set out for the avoidance of all doubt. Doubt can and does result in legal proceedings. These can be by either policy holders (patients if they are seeking for a treatment to be paid for under a policy they took out) or patients if they have undergone a treatment which they think was not performed correctly or unwarranted against an insurer covering malpractice against a practitioner. All parties try to avoid this because it's costly and the publicity can be awful for the insurer who might have a perfectly reasonable justification for not honouring a claim for a dubious treatment, but then has to show up on the steps of the court with a lot of shroud waving from the other side. No insurer ever wants to go there !

So, where you have a procedure and a condition, you'll almost certainly have clinical qualifiers from an insurer. This is much more true in the US because of their insurance-based healthcare system but the same holds true in the UK too (in the absence of anything else we'll tend to be guided by FDA rulings on this, although NIHCE carries more weight -- but is not such a comprehensive source of guidance at present).

Right, still with me ? Good. So let's look at Intacs. Here is the clinical guidance issued by an underwriter:

http://www.omic.com/products/bus_produc ... -18-09.pdf

Lots of insuro-medical waffle, the main action is mid-way down page 2:

To qualify for the treatment of keratoconus, patients must meet the following eligibility criteria:
+ Patients must be contact-lens intolerant.
+ Patients must have clear central corneas or only minimal central scarring. (Patients with central scarring must be advised of the risks and/or side effects they may experience as a result of their scarring.)
+ Patients must have corneal thickness of 450 microns or more at the sides of the segments.
+ Patients must have only PKP as an option to improve vision

(As an aside, I've picked Omic for the purposes of example. Omic is a malpractice insurer, not a retail insurance product provider. They produce such explicit guidance because the procedural risk sits with them. Most retail insurance policies lay off the actual policy risk onto a re-insurer which makes it devilishly tricky to see what a particular policy would cover in extremis. And then we get into the whole “on label” and “off label” issue. But I could write many more pages on that lot and try your patience even further. Suffice to say this is a good publically available example of an insurance policy document)

Different policies may be subject to slightly different clauses (depending on the insurer or re-insurer carrying the risk). But I’d say the above was pretty typical.

Each of these criteria is quite interesting in their own way because perhaps inevitably subjectivity tends creep into what should be a mechanical black-and-white decision tree and you get wriggle room. Take, for instance, “Patients must be contact-lens intolerant.”. What does that actually mean ? I hate the little buggers sometimes, but does that make me “intolerant” ? In order for the professional indemnity cover to be effective, worse case, the surgeon would have to be able to prove with a balance of probabilities level of evidence that the patient was indeed “intolerant”. So a patient could show up and have gentle whinge about how much of a nuisance their lenses were. They wouldn’t be ruled “intolerant”. The same patient could tell the surgeon that their lives were made a complete and utter misery by the lenses and couldn’t manage another day with them. They’d be classed as “intolerant” most likely.

Okay, turning to what’s of interest to us here, namely corneal thickness. The exact wording is “450 microns or more at the sides of the segments”. So, as long as you’ve got 450 microns where the rings sit, you’re okay. You could have a central cone way down in the 200’s, but provided there’s thick tissue available at the perimeter you’re not ineligible. Of course, common sense and experience really should get a look-in here – the surgeon would probably want to consider overall thickness and the possibility of migration of the Intacs along with a load of other factors.

Bottom line for me is – when considering any treatment option – if you really want to consider it, then do. Be wary of “one size fits all” guidance – especially if it’s trying to boil down complex inter-related criteria to oversimplifications. Trust your gut instinct. Then, get the best advice you can and be prepared to listen to and follow what you’re told by an expert in that field. Do what feels right for you.

Which is I think where you came in Libby!

Sorry to have taken up so much space, hope it’s of use to some people.

Kind regards

Chris

Thank you everyone for your kind words and support.

Lynn I was wondering in your opinion would we be sensible at thinking about CXL on my daughters good eye at the moment so to hopefully stop any more thinning?

Although I sight is perfect at the moment in that eye they did say it was thin with a thickness of 483 - I was just thinking perhaps to try to halt any progression that could arise!

Regards
Libby

Hi Libby

This is probably the most difficult question to consider.There are two schools of thought and if they were discussing your daughter's case, it would go something like this:

As her KC eye has deteriorated so quickly, it would be wise to have CXL on the good eye so that it is preserved in its present state.

KC can be monocular and the good eye may never deteriorate. It would be wise to wait until that shows definite signs of progression.

Ahh, but progression can be very sudden - see how the KC eye progressed very quickly?

Yes but we do not know the long term effects of CXL...

But we do know what happens if the KC in the good eye does progress quickly!

And so on....

It is very much a balancing of pros and cons but here are my own thoughts:

I have double checked with a surgeon experienced in CXL and he says it is perfectly possible to have CXL on the worst eye using hypertonic drops. It is definitely worth exploring this option, as this would then stop the progression and give your daughter some breathing space. Other contact lens types can be tried once the CXL has been done and often, fitting is a little easier post CXL.

Once that eye has been done, further checks should be done on the good eye and on balance, I would say it would be sensible to do CXL on the good eye. This is partly due to the fact that otherwise, the whole family will be on edge waiting to see if the KC starts up in this eye and this can cause untold stress.

This suggestion is, of course, with the understanding that I have not seen your daughter's eyes. If you consult a surgeon experienced in CXL, he or she can give you detailed information about the procedures and informed advice.

Lynn

Hi Libby

Although its generally worth exhausting all avenues before considering a graft, there may be good clinical reasons why your daughter's consultant is recommending a graft and perhaps you could discuss these at your next visit. For example, he might think further thinning would increase risk of hydrops, and she would eventually need a full thickness rather than partial (DALK) graft which is generally believed to have much less rejection risk than full thickness graft. By all means ask for a second opinion as its a fairly major decision before making up your mind. In my experience, most patients who have corneal grafts regard it as a positive experience, so its not all doom and gloom and the results can be very good in the hands of an experienced surgeon. Good luck