Born with aniridia

When I was seven years old, an ophthalmologist told me that I was born with aniridia. It is an eye disorder characterized by a complete or partial absence of the iris (colored part of the eye). This abnormality reduces visual acuity (sharpness of vision) and increases sensitivity to light. I inherited it from my mom, and everyone in the family (brothers and sister) has this eye condition, even my nieces and nephews.

This condition developed cataracts, dry eyes, limbal stem cell deficiency, and many eye complications as I got older. Hence, I was always seated at the front of the class from elementary until college to easily read the lectures written on the blackboard.

When I started working, I felt the blurriness and had my regular checkups with an ophthalmologist. I was advised to immediately undergo phacoemulsification (cataract extraction surgery)—right eye then left eye after a week.

I had my right eye surgery in October 2020, but due to the COVID surge then, my left eye surgery was extremely delayed until last month.

According to my ophthalmologist, my left eye was going to be a very risky procedure with two obstacles taken into consideration. First, the delayed procedure caused the hard-thickened cataract. It is like ketchup spilled on the table and exposed for a long period of time that it has hardened and is difficult to scrape off. Imagine the delicateness of the eye and how challenging for the surgeon to remove it without inflicting damage. Second, the presence of the big white mass cataract hindered the opacity to see through my eye.

My aniridia condition is a very rare case that made it more complicated and very challenging for my cornea and retina specialists to do the procedure with many factors to consider.

I was on six-hour fasting (no food and water intake) and arrived at the hospital at 6 a.m. A few minutes before I entered the operating room, my doctors explained to me the risks and the three possible options to extract my cataract.

First was to put artificial cornea; second, remove my eye then put an artificial eye for the extraction then return my real eye after; third, if the worst happened, proceed to Boston Keratoprosthesis.

No assurance, but my ophthalmologists committed to doing everything to keep me safe and restore my vision. I prayed to God for protection and guidance, and wisdom for my doctors as the anesthesiologist put me to sleep.

I woke up around 1:30 p.m. in the recovery room with a plaster strip on my left eye. Clueless about what happened, I was so hungry and medically intoxicated. I was so curious if it went well and which among the options materialized. The doctors told me that the entire procedure took four hours. None of the three choices was done.

It was totally a different, rare approach. Surprisingly, as they opened my eyes, the jelly-like liquid in the back part of my eyes (vitreous) unexpectedly disrupted and released fluid substance. It is not a good thing, but they took it as an advantage that this jelly-like liquid softened the hardened cataract. However, it was very challenging to scrape the cataract off with the opacity of the white mass in my eyes. They decided to switch off the lights and plunged a sort of mini-flashlight under the jelly-like surface of my retina to light up through my cornea.

The doctor explained it was the same logic as driving a car through stormy weather when you cannot see through the windshield unless a car in the opposite direction switches on its headlights. So the flashlight submerged underneath my retina helped a lot to lessen the opacity and made it more visible to scrape off the cataract.

When my doctor removed the plaster bandage over my eyes, I saw several floaters (they looked like spider cobwebs inside my eyes). It was a natural thing because of the disruption of the jelly-like fluid, but after a few days, the floaters solidified and disappeared.

This kind of medical procedure will always be a part of the lives of our family from one generation to the next bearing such bad chromosomes of aniridia.

Jonalyn Kaw, 29, is an alumna of the University of Santo Tomas and a resident of Calapan City, Oriental Mindoro.