Whoopsie Piggle can be found on “Down syndrome Blogs,” a site that aggregates Down syndrome blogs by category. One of the categories where Whoopsie Piggle is listed is “Dual Diagnosis” because Lyra was born with congenital cataracts in both of her eyes. Since her birth, the idea of my child being blind has been far more worrisome for me than her diagnosis of Down syndrome and while I hope this essay appeals to a variety of readers, I most wish to share what we have learned with parents of children who have both Down syndrome and vision impairment issues.
This past fall I finally gave up. And when I did, my relief surprised me.
“You know, even in kids without Down syndrome, somewhere between 18 months and four years old they typically stop tolerating contacts,” said Lyra’s ophthalmologist more than a year earlier when we started having problems keeping contacts in Lyra’s eyes. When she was a baby the same doctor had said that he would prefer to keep Lyra in contacts for as long as she tolerated them. The word “tolerate” when used in a medical setting sounds like a biological event over which there is little control, such as when organ transplants are tolerated or rejected by the recipient’s body. But in the case of contact lenses “tolerate” is a euphemism referring to behavior.
Lyra began wearing contact lenses when she was six weeks old. Because she was born with cataracts in both eyes, her natural lenses were surgically removed. Unlike when an adult has cataract surgery, however, synthetic lenses were not swapped in where the natural lenses had been removed. That’s because Lyra’s eyes continued to grow for at least another year after her surgeries. When her eyes were done growing, transplants were theoretically an option, which one of the younger doctors in the ophthalmology practice suggested.
“Young doctors love to operate,” said Lyra’s surgeon, who is the head of the practice, “but when you get older you recognize it is sometimes better to wait. Now if she comes to me at eighteen and says she wants implants, I’ll do them. But if she can do fine with contacts, it’s better to wait.” I agree for three important reasons: First of all, intraocular transplant surgeries in children under the age of five have far more complications, resulting in additional eye surgeries up to 72% of the time. Secondly, accurately determining what strength to give a surgically implanted lens on a child who is preliterate and unable to perform the typical acuity tests (Which one is clearer? A or B?) is never ideal, and, finally, I believe by the time Lyra is eighteen the field of intraocular lens transplantation will only have improved, perhaps significantly.
But back to tolerating contacts. The first two years Lyra wore them, I occasionally found one of her eyes nakedly free of any contact. Usually when this happened, the lens was ready to be replaced, which we did with the staff at the ophthalmologist’s office every three to four months. A new lens is shiny and clear, but over time protein accumulates, dulling the surface. I’m sure as they get dirtier, the contacts are less comfortable and I was not surprised when Lyra rubbed them out from time to time. This started happening more frequently after Lyra’s second birthday. Then this past spring, a few months before Lyra turned three, the pollen count rocketed higher than in most years. Lyra and everyone else in the house became sneezing snot buckets. Contacts disappeared weekly and at roughly $150 per lens, replacing them is not something to sneeze at. “It’s time,” said the doctor, “she’s spending too much time wearing only one lens and it’s too stressful putting new ones in this often.”
Two things in Lyra’s life have never been easy and have only gotten harder as she’s become bigger and stronger: quarterly blood draws to monitor her thyroid levels and contact lens changes. When she was a baby, I sat with Lyra on top of my legs with her head on my knees as one nurse put the speculum in her eye while the other one removed her old lens and put in a new one. The speculum was always necessary because Lyra clamped her little eyelids closed so tightly it was impossible to keep them open using fingers alone. When she was larger, I held Lyra sideways in my lap while one nurse held her head, another operated the speculum and a fourth nurse conducted the lens change. Always, Lyra screamed herself hoarse and became slippery with sweat. After doing this weekly for a month I lost all determination to keep her in contacts.
“Many kids love glasses and stay in them for years,” said the doctor when they removed her final contact lens. Even though there was only one lens to remove, afterwards Lyra remained upset and refused to wear her new glasses in the doctor’s office, wanting nobody and nothing near her eyes. I tried again later that day when we were home and she promptly pulled them off her head and flung them across the room. Now what? I fretted. She can’t go around with absolutely no correction. Her prescription is +19 in one eye and +20 in the other, qualifying her as legally blind (for comparison, I am quite nearsighted and my prescription is -3.75 and -4). I contacted the vision impairment specialist who used to come to our house with Lyra’s county caseworker. She sent me a list of tips for getting kids to wear their glasses, which assumed you could get them on the kid in the first place. I went to bed that night believing we might need implant surgery after all.
The next morning when I got up and went to the kitchen for coffee, Max was sitting at the table. “Look at Lyra,” he said. Because she sits at the end of the table, her back was toward me when I entered the kitchen. I came around and there she was, wearing her glasses while eating her breakfast. With the exception of the following day when she hid them in a pot inside the oven of her toy kitchen, Lyra has willingly worn her glasses ever since. In fact, when she gets up in the morning she often says, “Hi! I want axes.” Which is how she pronounces “glasses.”
Why was I so resistant to abandon contact lenses? What I told myself was that Lyra would not be able to see as well in glasses as she could in contacts. For anyone who has worn both glasses and contact lenses, it is often the case that contacts give a more precise, if not significant, correction. In part because the lens is directly on the eye and not a few centimeters away. But it was Max who hit on the larger reason. Max, who took Lyra’s diagnoses in stride since the very beginning and never grieved over Lyra’s Down syndrome because, as he told me, he just loves her so much as she is. That hasn’t changed, but with fierce love comes the desire to protect. “I wish she didn’t have to wear glasses because it’s one more thing that identifies her as different,” he said to me about a week after Lyra switched to glasses.
Though variation is substantial, people with Down syndrome, unlike other conditions, bear identifiable physical features. Chief among them are almond-shaped eyes due to epicanthal folds of the upper eyelids and flatter facial profiles. In the morning when she sits on my dresser in front of the mirror where I brush her long blonde hair, Lyra looks glamorous. But she cannot work with hair in her face and she cannot see without her glasses. Once her hair is smoothly secured in colorful elastic bands, I place her glasses over her head and around her neck like a necklace, then pull her hair over the safety band that connects the stems. Quickly Lyra grabs the frames and perfectly positions them on her face. “Hi!” she then says every time, looking me in the eyes.
Her glasses are so thick they feel almost like marbles when I rub them clean with soap and water, as I do many times a day. In contact lenses, Lyra often squinted, making her eyes look small. The magnification of her glasses is so powerful her eyes appear owlishly big, drawing attention to her face. Her vision impairment is now as patently obvious as her Down syndrome. More than before, in public people comment on how cute Lyra is. Like three-year-old boys in ties and sports coats, glasses on such a small girl looks endearingly adult. But what happens when Lyra is a teenager and, later, a woman? I have long felt more at ease with Lyra having Down syndrome than I do with her vision issues. And here’s why–Lyra is healthy, a social extrovert and very bright. Understanding that Down syndrome effects cognition, at three-and-a-half years old Lyra is no longer an enigma. She will do well in life with the right support, which we give her and will do so for as long as we can. Beyond us, Lyra is blessed with four adoring brothers whom I have no doubt will look out for their sister when Max and I are dead. (It happens and we don’t pussyfoot around in talking about it.) But my daughter as someone who is legally blind? What that means long term is not so clear.
In Ohio, services are provided at the home by the state, through the county of residence, until a child is three years old. Then the child is eligible for public preschool and it is there that services continue. Lyra is fortunate to have as her classroom teacher a vision impairment specialist. We have long heard that children with Down syndrome have strong visual memory but after raising two kids with severe dyslexia, I was not expecting Lyra to read before five as do many other children with Down syndrome, including our pediatrician’s daughter. Yet today Lyra recognizes and verbally identifies almost the entire alphabet, numbers one through nine, and at least five colors. For now, the letters and numbers she looks at are quite large, certainly not as small as the print in a typical children’s book, which leads to what comes next. When we recently saw Lyra’s ophthalmologist for the first time since her last contact lens was removed he was thrilled with her vision and said, “Somewhere between 12 and 18 months from now we will make the switch to bifocals. I want to wait a while because it’s a difficult transition, the kids fall a lot and it takes a while.” On the other hand, he also told me it would take two to three weeks for Lyra to adjust to glasses and that only took 24 hours.
Meanwhile, Lyra’s teacher is exposing her to braille and signed her up with a non-profit that sends us braille books every few months. The ones that are typical toddler books with clear braille stickers overlaid upon the text we keep at home. Others are simply white 8.5 x 11 pages covered with bumpy dots. We put those in Lyra’s backpack and send them to her teacher.
Like so many changes resisted in life due to fear of the unknown, I wish we had switched to glasses sooner than we did. We would have saved Lyra several traumatic contact lens changes. She still drops to the ground in an unfamiliar setting if she is unable to discern the topography of the ground or floor but otherwise seems to see as well as most other three year olds. An added benefit is her glasses have transition lenses, meaning they get dark when exposed to sunlight. In contacts, I could never get Lyra to “tolerate” sunglasses. Now sunglasses are just part of the package and she looks pretty cool.