Advocating When Nobody Listens

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.

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.