When Jackson was born six years ago this December he didn’t open his eyes much the first day, and when he did, he opened his left eye more than his right eye. This worried me a little bit, but as a mom I figured I was being paranoid.
The next day, one of the times I was holding and staring at him, as new moms do, he opened his eyes to look around and I noticed something weird with his right eye. It looked cloudy or reflective but by the time I told my husband, Jeffrey, about it, Jackson had closed his eyes again. Jeffrey also thought it was likely first-time mom paranoia and we moved about the day.
Throughout the next several days Jackson was opening his eyes more and more, which allowed Jeffrey to see his right eye and agree with me that something seemed off. The on-call pediatrician came in every day to check Jackson and finally on the third morning (the day we could have been discharged had we chosen to) we mentioned our worry to her and she pried his eye open to look and said a couple times she didn’t see anything and then eventually said (I think just to appease us) ‘Oh yeah I think I see something.’ She told us that on our 3 day visit at the office our primary pediatrician from the same practice would assess the situation. We made our appointment to see the pediatrician and just had to wait until then.
So, of course, since we had a few days until his appointment, the worrying and googling began as we looked to see what may be ahead of us with Jackson’s eye. I can’t quite remember exactly what I googled but I think it was something along the lines of ‘cloudy spot on infant’s eye.’ Luckily only two things stood out to me (I say luckily, because it could have been much more):
Congenital cataract – lens opacity present at birth
Retinoblastoma – eye cancer
Both seemed likely as they were characterized by cloudy/reflective pupils. Now, it was just a waiting game. We were enjoying our time with our wonderful (and mostly quiet) baby boy but there was still worry in the back of our minds.
Finally it was pediatrician appointment day! She came in to meet Jackson and do a once over. She looked at his eye and agreed that there was definitely something. She assumed we had googled and asked what we found. I told her the two things we found and she said those were the two likely candidates but that since she wasn’t an ophthalmologist she couldn’t tell us which it was. She sent us on our way with our one week appointment scheduled (3 days away) and said she would reach out to the local pediatric ophthalmologist for us.
The one week appointment was at around 11 in the morning and Dr. Amy Kryder said everything looked great. She asked us if we had heard from the pediatric ophthalmologist’s office yet and we told her we hadn’t. So this time she sent us off and told us she would reach out to them again and to let her know if we hadn’t heard from them within the next two days.
We got home from the appointment and I had just been nursing Jackson when my phone rang. It was the ophthalmologist’s office and they were asking if we could be there in about an hour because they had a cancellation and if we couldn’t make it today they were booked until April (yikes!). Well this had already been worrying us and we weren’t about to wait FOUR months to find out what was wrong with our baby so we hopped back in the car and headed to the office.
When we were called back for the appointment we met the Pediatric Ophthalmologist (PO), Dr. John Facciani, who was performing the exam while spouting out numbers and foreign words for his nurse to put in the computer and he was talking to us too. Then came time for him to explain what was wrong. Jackson had a cataract, but his right eye was also smaller than his left eye (microphthalmia) which led him to believe he had something known as Persistent Fetal Vasculature (PFV).
He wanted to send us down to a colleague at Duke Eye Center in North Carolina because she is better equipped for the surgery for PFV. So he sent us on our way and told us we would hear from either his office, or Duke’s office to let us know when to expect surgery. So there we were, Jackson is eight days old and we now have a diagnosis (definitely a cataract and microphthalmia, possibly PFV). We left and I sat in the back seat with Jackson (sleeping of course) and let him hold my thumb while I tried (unsuccessfully) to hold it together while Jeffrey was my rock driving and comforting me and patting my knee all the while.
The next month, we received an appointment/consultation and had surgery scheduled for the same weekend at Duke University as we were traveling from Virginia. Our first time meeting Dr. Sharon Freedman was the day before Jackson’s first surgery. She confirmed what our local PO had said. Jackson had PFV, microphthalmia and a cataract in his right eye. She explained PFV to us more thoroughly and assured us that it was just one of those fluke things and it wasn’t anything we did wrong in pregnancy. For those unfamiliar with Persistent Fetal Vasculature (PFV), when the baby is in utero, there is a stalk that forms between the front and the back of the eye. This stalk is supposed to dissolve on its own in the third trimester which allows the eye to continue growing. Jackson’s stalk did not dissolve on its own in his right eye. Dr. Freedman walked us through the surgery and explained that she would go in and remove the natural lens and the stalk.
The next day was surgery day. Jackson was 7.5 weeks old and it was very difficult for us to hand him off to strangers who were going to put him under anesthesia to perform a surgery we didn’t even know existed seven weeks prior. My husband, his parents and I all anxiously waited in the waiting room while Jackson was in surgery. They had a helpful display board letting you know what stage of the process your child was in. The surgery seemed to be taking longer than expected and finally when Jackson was in the final recovery phase of surgery the surgeon came out and called my husband and I into a room to tell us how everything went. She explained that it was a more complex surgery than she had anticipated which is why it took longer than expected but that everything went smoothly and Jackson was well enough that he didn’t need to stay in the hospital overnight and she would see us the next morning for our post-op appointment, which went great so we were sent home with a strict eye drop regimen for the next 10 days.
After we followed up with our local PO, Jackson started wearing a contact and a patch shortly after his first surgery. He was around 9 weeks old. We started out with patching 3 days a week. We went through all the phases – fussy, ripping patches off and needing to wear socks on his hands or arm constraints to get all necessary patching hours in, days where he would just sleep when patched, etc. It has been a very long journey to say the least! Jackson was also fitted with glasses at 21 months of age, but we were fortunate that he adapted to them pretty easily and didn’t constantly try to take them off.
About a year after Jackson’s first surgery his pupil started getting smaller and smaller to the point where he was barely getting any light into his eye. So in February of the following year, we went back to Duke for a second surgery to widen his pupil.
We learned somewhere along our journey that Jackson has nystagmus in both eyes and that he has a slight head turn/tilt. Our PO believes that strabismus surgery will help with the nystagmus but of course we are hesitant to do that as that surgery would be on his stronger eye. As the nystagmus hasn’t seemed to slow him down yet we haven’t made it a priority and we just keep an eye on it every time we go back to our PO for a checkup. He has also mentioned we should probably wait to do the strabismus surgery until after an IntraOcular Lens (IOL) is placed but we want Jackson to be the one to make the decision whether or not he wants an IOL so we are hopeful that we can wait until he is old enough to understand all the pros and cons of another eye surgery to decide if he wants to continue using a contact or get an IOL.
Jackson just turned six years old in early December. We have been patching since he was 9 weeks old, with the only changes being to the schedule (we now patch 5 days a week) and his cooperation with patching. He still goes through phases where he complains about having to wear a patch but he will put the patch on as he is old enough now to understand we are doing the patching to help improve the vision in his weaker eye. He started kindergarten this fall and is doing such a great job, even while patching! We were worried that his patching would hinder his learning or social experience but so far everything has been going so well! At our last PO appointment he suggested that our patching journey may be close to an end as Jackson’s eye has been pretty stagnant at 20/125. I am not super happy about that being his permanent visual acuity and I am hopeful that we can continue to patch and strengthen the eye further. We want to believe that it’s more that he’s just still not 100% cooperative with the visual acuity tests.
To all the parents out there with similar journeys and/or just starting out – hang in there! It is a long and sometimes difficult journey but know that you are not alone!
(These pictures are all from 2022)