The Pirate Years, Part II: Let the Trauma Begin!

What happens next, after your 3-year-old has cataract surgery, having, so far having lived more days with obscured vision than without?

She is unhappy, miserable really, in pain, and mostly unable to understand the what and the why of 12 eye drops a day, a plastic shield taped to her face, and a whole lot of medication. (For all of that, she could be as adorable as ever. Here's a little movie to prove it.) The eye drops were the worst--some were painful, some not, but the lesson is quickly learned, and within a few days it took two people to pin her down to get them in. As we weaned off them, we were able to start giving them to her while she was asleep, a tricky task which sometimes ended in nightmarish battles that we joked (but really feared) would give her a twitchy sleep complex later in her life. A year later, eye drops are still the worst possible punishment for her--a bout of pink eye, and the 3 times per day eye drop medication for it, revealed that she has not forgotten, may never forget, the trauma of the surgery and the month of physical recovery which followed.

After that month, though, the drops were over, the pain was gone, and the long term reality set in. The cataract removed and replaced with a plastic lens, Joey is left with, in effect, a case of amblyopia, commonly, "lazy eye." This condition can develop for a variety of reasons, but the ultimate result is that the neurological pathway between the eye and the brain has not developed fully, so that the brain "ignores" the signals that the eye sends. The physical effect of amblyopia is a wandering or "lazy" eye. The therapy for amplyopia dictates that you occlude the vision of the strong eye in order to force the brain to use only the signals it receives from the "lazy" eye. This involves "patch therapy," a seemingly primitive sort of medicine in which a patch is placed over the strong eye to block its vision. It sounds obvious and simple, and yet it is a most incredibly difficult thing to do.

Upon first patching Joey, we discovered that Joey's condition of amblyopia was more severe than the norm, an indication that her cataract had been present very early in life. So not only did her eye "wander," it did not have what they call "central fixation," which keeps the eye centered. With the guidance of her good eye, her amblyopic eye would stay in place; with her good eye patched, her amblyopic eye would do this:

Creepy, isn't it?

Okay. So a few things might bear explaining. The patches can be decorated, and one of Joey's favorite patch decorations are googly-eyes. Unfortunately distracting for this example, but it is the only good picture we have of her from this period. Putting that aside, one can see the other eye shooting off to the side. She is not trying to look off to the left--this is all her eye could do, for almost a month. When she wanted to look out of it, she would have to turn her entire head to the side to align her eye with what she wanted to see.

We had one more month of hand-wringing and waiting: in this period, the eye would either fix centrally, or it would not; if it did not in the short term, it most likely never would. One can only imagine the scrutiny, the hoping, the waiting. Time did its job, in this case, and though it still desperately wanders whenever she is fatigued, her eye did ultimately "fix" centrally.

Unlike other cases of amblyopia (as I understand them), Joey's is unique in that the vision in her impaired eye is so poor--20/300--that her brain quite simply wants very little to do with the signals it sends. (See a simulation of impaired vision here.) Even patched, Joey's brain will sometimes prefer to see the back of the patch rather than see what her impaired eye sees. She will sometimes say that she can't see anything, or that she can only see "black lines": light seeping through the patch onto her good eye. Simply put, there is no reward or punishment in a preschoolers little world that is enough to combat the discomfort, the limitation, and the frustration of not being able to see when, in all truth, and in her own mind, she knows that, in any given moment, she can. Not surprisingly, patching is an effective treatment for amblyopia, but compliance is the greatest barrier to its success.

A year has passed. Nothing has changed. Rather, a lot has changed: Joey is now four, and why no one speaks of the peculiar nightmare that is raising a four-year-old girl I do not know. Despite (or because of) intermittently successful patching, Joey has had no improvement in the vision in her eye. We tried dilation therapy for a short time, and alternative therapy in which the good eye is dilated with atropine--just like in the eye doctor's office--in order to minimize the contrast between being patched and not patched, thereby (in theory) decreasing the frustration with wearing the patch. (Dilation therapy can be used as an alternative to patching in patients whose vision is better than Joey's, but in our case dilation alone would do not good. As it turns out, dilation and patching also did no good.)

In a rare moment, Joey plays fairly successfully at a playground in her patch.

 

Joey is proving herself a fierce opponent to patching. Many who know the sweet, light child she can be would be horrified at the monster unleashed at "patch time." Parents always endure unspeakable assaults from their children, sometimes expected, sometimes not. When Joey threw her boot at my face to avoid being patched, I think something broke inside me, and while I continue to do my best with the therapy, I struggle with how much psychological pain I am willing to inflict (both on her and on me), and what lengths I am willing to go through to implement a therapy which has such an uncertain chance of succeeding.

Over time, the pathways between a child's brain and her eyes become less pliable, and the window of opportunity for change will close. Sadly, Joey's doctor is somewhat pessimistic about her ability to improve; in his experience, fierce battles against patching do not cause a lack of improvement necessarily, but rather seem to indicate (as he put it) some innate understanding that there is no improvement to be had. Maybe this is something that he says to parents who find patching too hard of a battle to fight, to make them feel better about losing that battle more days than not. I don't know. The only thing left to do is to feel horrible about the days when we lose that battle, feel like the days when we get a little are not good enough, and always try for more, against the possibility that even perfect compliance may bring us no change at all. What else is there to do?

Next Up:

The Pirate Years, Part III: Fashion Patches are (Not So) Fun, or, Five Dumb Things I've Done to Get My Daughter to Wear a Patch

The Pirate Years, Part I: "Oh, yeah, my grandma had a cataract, too."

Given my penchant for using writing as cheap therapy, I was somewhat surprised to discover that I haven't written about my daughter's eye surgery. Maybe because I feel like I'm still living it, and sometimes living it takes all I've got. But the approach of the one year anniversary of the beginning of the whole ordeal has me pensive. I think it might be time to sit on the leather couch....Maybe doing so will help someone else.

The Discovery
Not quite since birth, but pretty close to it, Joey squinted. She seems pretty normal here, in September 2008, at 4 months old:

By 7 months, we have a lot more pictures that look like this:

It seemed not at all unusual for a child to squint in the sun. She was known as "Pirate Joey." Everyone seemed to agree that it was cute and quirky, and not one person ever suggested that it might be an indication that something was wrong.

Apparently, squinting is an indication that something is wrong. It is common among people with cataracts, and while cataracts are typically a condition brought on by age and use, children do develop them congenitally. (There is an entire public health campaign in the UK targeted specifically at childhood squinting. Not so here.) I will forever wonder how we made it two years without a single person suggesting a problem, or ourselves wondering about it enough to mention it to her doctor.

Two and a half years passed. I started to notice her eye drift a little when she was tired. Working in her preschool classroom at the end of the school year, I observed her watching her teacher, and noticed that she seemed to look in the direction of her teacher, but not really at her. Bad vision runs in the family, so it would have been no shock to find out that she needed glasses.

This photo is of Joey and Colin, around her 3rd birthday, in May 2011, just weeks before the discovery. Even here, I think few people would notice the squint if not pointed out. (One could argue that Colin is squinting equally with the other eye.) I guess I can take some comfort that it wasn't so obvious, even right before we learned what was happening.

Luckily, Joey's three-year-old routine doctor's appointment was coming up, and it is at this appointment that they typically do the first eye test. (I presume that, at three, kids can finally follow instructions well enough that their answers to "What do you see on the chart?" can be trusted.) Right eye, all is well. She sees a star, a boat....Left eye, and I get a look from her like we just changed languages. Eye chart? What eye chart? her expression asks. Off to the specialist we go.

This alone might have been scary. But oddly enough, we had been to this same eye specialist before, for Colin. (He has a bump on the white of one of his eyes, a nerve, we learned, that just happens to end on the surface of the eye, creating a water blister-like spot. It's not painful, and is completely harmless. He's likely to have it for life.) So we had been there before with not much to worry about. The chit-chat was cordial and calm, some talk of glasses running in the family. Then the ophthalmologist dilated her eye. Suddenly, the conversation changed. She has posterior lenticonus: in simple terms, a cataract, like what older people get as their eyes age or if they have had repeated damage through years of use. Her vision is completely blocked. How long? Hard to say. Not since birth, but probably starting shortly after, some time in the first year. She needs surgery? When? As soon as possible. Within five minutes, I'm in a cramped little office, stacked with papers, talking with an assistant about surgery dates and discussing how to convince a just three-year-old that she can't have breakfast the morning before the surgery.

The Surgery
What else can be said? It was horrible. Worse than horrible. If given the choice, I'll never watch one of my children be put under general anesthesia again. They try to warn you....Anyway, half way through the surgery, they had to call me out of the lounge area....I cried all the way up the elevator, in a panic, having no idea what was wrong. A woman, concerned about the health of her child, who stood beside her, pressed me on my sniffling....Was I sick? Did I have allergies? If not, then what was the matter with me, and could I give it to her child? No, my daughter is in surgery, having her left eye's lens removed, a plastic implant inserted if all goes well, and maybe, maybe getting the chance to see normally. Thanks for asking, though.

About six weeks later, at the end of July, 2011, we find ourselves here:

Looking at this picture just makes me cry.

Next Up: The Pirate Years, Part II: Floating Eyes and Fashion Patches