Through Emma's Eyes

Emma's always had her share of special challenges. Her low responsiveness continually worried the doctors before she was born. Her delivery was an emergency induction when she was clearly in distress. She was born at one of the busiest times of our lives, and lived her first 20 months in the background of boarding school life. She struggled with fluid in her ears and nearly constant ear infections from about 14-20 months old until she finally got the ear tubes she needed. When she turned two and still wouldn't say a word, we called in the early intervention speech therapists who didn't seem to have a category for a smart child who understood everything that was said to her but couldn't seem to get a word to come out of her mouth.

Her third year was characterized largely by frustration and weepiness. Life just seemed to be such a challenge for her. She would "hum" what her eyes told me were long complex sentences in her mind, and then she would either roll her eyes at us or break down in tears when we couldn't understand her. Thankfully, by the time she turned three last September she had learned a little more of our language and we had learned a little more of hers. We could understand each other much of the time and Emma began to blossom. She is bright-eyed and happy, singing her little songs and developing her own personality now, but some things still puzzle me.

Why isn't she speaking any more clearly as the months go by? She is no longer labeled as having a speech delay, but an impediment, probably speech apraxia, where the brain doesn't communicate with the speech muscles correctly. But there are other puzzling behaviors as well.

The first time we visited our new house Naomi and Hannah ran up and down the stairs, but Emma froze at the top and began screaming as if the world had dropped out from under her. She was trembling all over by the time I rescued her and showed her how to place her hand on the wall and walk down. She had been up and down the flight of stairs at our previous house ten times a day, so why the fear here? Why is she so clumsy? Why does she sometimes walk right into something as if she doesn't see it? Why would she rather collapse on the floor sobbing for her sippy cup than stand up and see that it's sitting in plain sight on the table?

With my concerns mounting, I asked my pediatrician for a referral to an ophthalmologist to check Emma's eyes back in January. She questioned the need and looked at me like I was hopelessly paranoid, but agreed to refer Emma. We left the office satisfied, but she never sent the referral. When I brought the concern up again in April, Emma was finally referred to an ophthalmologist.

Tuesday afternoon Emma was patient and cooperative as machines measured the curve of her eyes. She tried to name the cake, horse, duck, and hand pictures that grew ever smaller. She let them put in the drops to dilate her eyes without a peep, and she tried, unsuccessfully, to identify the 3D creatures while wearing goofy glasses on her face. And, once again, I am glad I pushed for the referral.

It turns out that Emma's left eye is extremely nearsighted with a large amount of astigmatism too. She sees around 20/200 in that eye, which is being able to see the huge E at the top of an eye chart and nothing below that. Her right eye is getting her by with decent vision, somewhere between 20/40 and 20/60, enough to barely pass a driver's license test. The ophthalmologist was upbeat, saying he was glad that we caught this early, and that often, with correction, the vision in the bad eye will improve. I guess if we help her eyes to see more clearly those pathways in the brain may become stronger since she is still young. She has some adorable purple glasses on order, due in next week.


I feel a familiar mix of vindication (I told you she had trouble seeing!), relief (maybe life will get easier for this poor child now), and regret. So that's why she spent all those cumulative hours screaming that she couldn't find her sippy cup--maybe she really couldn't. I have no doubt that she screamed at the top of the stairs because, with a combination of poor vision and almost no depth perception, the world really had dropped out from under her. The doctor says we caught it early, but, just like the Celiac disease with Naomi and Hannah, I wish I had caught it sooner.

Somewhere in the back of my mind lurks the fear that her eyesight is actually on its way downhill fast. One 2009 paper that was published from the ARPKD/CHF study that Naomi and Emma are enrolled in showed a few of the several hundred kids in the study also showed features of a more involved neurological syndrome, and a subset of kids with that syndrome experience retinal degeneration at about Emma's age. The ophthalmologist couldn't rule that out, but didn't want to jump to that conclusion. He will check Emma's eyes again in July and will expect to see improvement with the glasses. If we do not see improvement at that time, we'll be referred on again to the children's hospital for more in-depth evaluation.

But that is July, and this is May. Next week Emma will be sporting some cute purple eyeglasses, and, hopefully, fewer tears of frustration.