Finley's Retinal Appointment

Today I took Finley to Massachusetts Eye and Ear for her annual Retinal Specialist appointment.  It is an all day appointment and she does not love it.  But she was a trooper today, and the appointment went quickly.

We got good news that Finley's vision seems to be remaining stable.  Although we have seen her move closer to the TV and tilt her head a little more, according to her test today her vision in her right eye is 20/60 and her left eye 20/120.  Dr. Pierce seems to think that Finley is getting older and better able to do the testing, so before her answers were probably not as accurate.  She has always had one eye a bit worse than the other, but this was quite the stretch.  This is as far apart as they ever have been.  The right eye also seems to have gotten better acuity, which we were told can happen sometimes.  It will get a bit better for awhile, and then take a decline.  But for now - things look good.

They did an ERG (electroretinalgram) which she really hated.  The last time she had this done, she was asleep, but she is old enough know they decided to try it awake.  It is a pretty uncomfortable procedure, but she did well.  The ERG is how the doctor determined Finley had LCA and not something else.  She has a classic ERG - very little response from the retina when light bounces off.  A normal person's level is about 200, and Finley's is 35.  And this is consider good for LCA, but LCA none the same.  So - she had a stable ERG (it hasn't changed since the last time she had it) but it isn't GOOD.  Make sense?  Still very little active recognition from the retina, but she has some!

They had to put drops in her eyes three different times, and the drops they use sting.  She was about to lose it the last time, but she let them do it. I felt so bad for her.  They had so many test they had to do today, they had to keep putting drops in her eyes to keep them dilated, etc.  It was not fun.

She also did a dark adaptation test -where they test her ability to see in the dark.  She did really well with this.  This always surprises us and Dr. Pierce because one of the signs of RDH12 is night vision loss.  This is the one thing Finley has never had.  There are a few other children with RDH12 that do okay but most of them don't have any night vision at all.  So we consider ourselves lucky.  She had to wear eye patches over her eyes for 30 minutes before we did this test, but she kept herself occupied listening to Junie B. Jones on the Ipad.  Thank goodness for that Ipad.

They took pictures of her retina, and the black areas are still there - of course (not like we thought they were going anywhere) and she has decreased pigment and thinning vessels, BUT they have not changed in the last year.  This was great news.  She has LCA, we know.  She is going to go blind, we know.  BUT - right now she is in a stable period, so we are so, so happy.

They didn't do the vision field testing part of the day because we are having a very extensive vision field test next Tuesday.  So that got us out of the appointment a little early as well.  We are very anxious for that visit because that will tell us where her holes are in her vision.

We had a nice talk with Dr. Pierce and his whole team (which has about 8 people on it - researchers, geneticist, doctors......).  LCA is still a rare disease even in the opthomology field.  Each doctor took a turn looking in Finley's eyes because you don't get to see LCA retinas that often.  Finley thought this was hysterical - that they all wanted to have a look.  She was really good about it.

 We talked about how LCA is still such a mystery.  That we can guess all day long what her path is going to be.  We have a pretty good idea from past RDH12 cases, but each kid is different.  Finley has pretty good vision for an LCA kid.  But there are kids that have better.  And there are many - even among our RDH12 kids - that have worse.  He told us that she is in a "plateau" period, but that one day she will go down and they don't know when that will be.  And then she will plateau off again for awhile.  It could be next year, or in 5 years.  We just don't know.  We talked a lot about the foundation and our work and he was impressed about the money we have raised.  Never stops making me smile to see the surprised look on the researcher/doctor's faces when I tell them what a power of a parent can do.

So a good day.  Finley is wiped out, but happy that it is over.  We are home and relaxing and enjoying the fact that for now - we are doing everything right.  She is remaining stable, we are protecting her eyes, and we are given the gift of time with her vision for now.