When Arthur was born, we noticed almost immediately that one of his eyes seemed a little swollen shut. He would open one eye and not the other. We asked the doctors about it while we were still in the hospital after his delivery. Our resident doctor assured us that it’s common for babies to have some swelling or similar symptoms, especially when they deliver “sunny-side-up”.
The next day during the rounds, we continued to voice our concern over the disproportion and favoritism opening one eye and not the other. The overseeing physician repeated the advice we received already, and to be honest, it’s easy to listen to what you want to hear.
Arthur passed all of his tests. I stayed in the room for each one. He aced his APGAR with a 10—crying before even being fully born. Chaos ensued at home, and we enjoyed our time together before making our way home.
At his follow-up clinic appointment, we voiced our concerns again—nothing was improving, and we were able to look closer at his eye and see different coloring and a jagged-shaped pupil. His eyes were checked, they felt his head, and gave him the all clear again. I gladly listened. Luckily, Alan wasn’t so easily swayed.
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| The photo we captured of Arthur’s eye before seeing the Ophthalmologist, when we were told things were normal |
At two weeks old, Alan was still sticking to his gut that something wasn’t normal with Arthur. I knew that he looked different, but I wanted to believe otherwise. I finally took to Dr. Google (which is SO unlike me to wait an entire two weeks… it usually takes me 5 seconds to web MD a problem and conclude impending death).
Google came up with some scary results:
- Congenital Glaucoma
- Horner’s Syndrome
- Cataracts
- Pete’s Anomoly
- Anopthalmia/Microphthalmia
- And more
After reading about how serious many of these conditions are, we called the Pediatric Ophthalmologist on our own and requested an appointment without a referral. They fit us in the next Monday, and immediately knew something was not right in plain sight. After sending stern warning letters to all of our doctors, our provider’s Ophthalmologist admitted that they have no tools or specialists able to work on babies this small, and we were sent on to a specialist at the University of Wisconsin.
We still didn’t have any answers when we came to the UW. We met our doctor and after a long, long day of examinations and deductions, we knew what we were dealing with:
Arthur had a Persistent Fetal Vasculature (PHPV/PFV) Congenital Cataract and Microphthalmia in his left eye.
Persistent hyperplastic primary vitreous or persistent fetal vasculature is a rare anomaly of the eye where it doesn’t fully form in the womb. When an eye is formed in utero, a stalk connects the back of the eye to the pupil, and once the eye develops further, the stalk then regresses and the eye is left clear to allow light in for vision. With PHPV, that stalk sometimes doesn’t regress—and the eye remains cloudy. A PHPV cataract is the worst type of cataract for vision prognosis, and needs to be removed between 6 and 8 weeks of age for a chance for the brain to make the right connections to the eye in order to establish vision. Microphthalmia can pair with PHPV, but can also exist alone. That condition is the medical term for having a smaller eye. It can vary in severity and in how it affects vision. All together, these conditions lead to increased risk of glaucoma, poor vision prognosis, and a long journey of maintenance and related surgeries.
Hello! Our story is very similar to yours. Living in the Nations capital, Washington DC, I really believed that the hospital doctors seen it all and was relieved when they repeatedly told me that the swelling was normal since I almost had my baby in the car. At exactly 2 weeks, we learned our “new” normal which is that our baby has right eye micro. Not sure how old your son is..ours is 3 years old now. He wears a prosthetic eye and protective glasses. Besides that he is a normal 3 years old, who is known as a leader in his classroom. I know Arthur is also going to do great things!
Sorry my response took forever- it kept glitching on me! It's so crazy that healthcare professionals doubt a parent's concern– why wouldn't they go the extra step to double check things? So glad your little guy is doing so well! <3
Thanks for blogging. I am just starting to learn more about PFV and cataracts after my sweet girl was born. Our first appointment is next week to evaluate her cataract. Has Arthur had surgery yet?
I hope it all goes smoothly for you! Arthur has had surgery already and I'm hoping to unfold more of our story to blog and share with similar stories- I actually just wrote about preparing for his surgery! http://www.homemadehometown.com/2018/02/preparing-for-surgery-advocating-for.html
Hello, my 4 month old son was born with the same condition. After 2 surgeries, unfortunately his retina detached. It’s a really hard thing to process. Especially being a new mom. His affected eye is slightly smaller than his good eye. Doctors are going to monitor it to see if it continues to grow. I hate not knowing what will become of his eye.. and for his future. Your boy seems so happy and it gives my heart some ease. Thanks for sharing your experience. What are some of the struggles Arthur experiences having one eye? Thank you.
Hi Renae! I’m sorry to hear about your son’s retina detaching– we walked the same path with Arthur. He now has a prosthetic shell to stimulate orbital growth and he is on par with all of his peers, enjoying 4-year-old kindergarten with his friends, and enjoyed showing them his “extra eye” at show and tell! I share much more about him on Instagram http://www.instagram.com/kylamariecharles