From "Why Doesn't Our Baby Open His Eyes?"
Jeff and Karen Jacobson felt it the moment they laid eyes on their newborn son—that pang in the gut that tells you something is wrong before the fact registers in the brain. As the doctor lifted the squirming baby up and over the drape blocking their view of the cesarean, the couple noticed their son’s eyelids were unusually small and were scrunched tightly shut, like buttonholes on a shirt. Everything’s fine, they were told as the nurse scuttled the baby away. But as Karen, 35, lay there, groggy from drugs, Jeff at her side holding her hand, all they could think was, Why doesn’t our baby open his eyes?
Karen Jacobson, the type of woman who likes everything to go according to plan, was getting pretty good at dealing with life’s surprises. In 2002, after two unsuccessful attempts at artificial insemination, the former schoolteacher and her husband, Jeff, an accountant, decided to take a break. Wouldn’t you know it? Within a month, she was pregnant with twins. Strawberry-haired Zachary and blonde Danielle, the spitting image of her mother, were born in January 2003. It was happy news when, just 18 months later, the Demarest, New Jersey, couple learned they were expecting another completely natural set of twins.
Karen underwent all the tests she had during her first pregnancy, and then some: an amniocentesis, a blood scan for birth defects, numerous ultrasounds. By all indications, she was expecting two healthy, perfect babies.
May 24, 2005, was the big day. With Jeff at Karen’s side, obstetrician Sharon Patrick performed Karen’s planned C-section. Within ten minutes, Dr. Patrick scooped out baby Jenna. Jeff snapped a picture before Dr. Patrick clamped the squealing girl’s umbilical cord, cut it and handed her to a pediatrician. Minutes later, Dr. Patrick carefully lifted out baby No. 2: Dylan. Soon the mood in the operating room started to change.
The pediatrician checked Dylan’s lungs and gave him a cursory once-over. The baby’s eyes were still closed, but since that’s not too uncommon for a newborn and because everything else checked out fine, she turned him over to nurse Sharon Mendez to tag and footprint. As Mendez worked on the baby, she noticed a small, sunken look around his eyes. She had an idea what was wrong but needed to call the pediatrician back for a second look. She returned with the doctor, who, upon taking a closer look, confirmed her suspicions.
The Jacobsons noticed the nurse whispering, staff walking in and out of the room, the lighthearted chatter turning hushed. “Is everything okay?” Karen and Jeff repeatedly asked Dr. Patrick, still partially hidden behind the drape.
“I think everything’s fine,” Dr. Patrick said, stalling. “Let me just focus on you, and then I’m going to take a look at your babies.”
After putting gauze over Karen’s stitches and covering her with a blanket, Dr. Patrick pulled up a chair and sat down next to her. Looking Karen straight in the eye, Dr. Patrick started to speak. “There’s a problem with your son,” she said. “He doesn’t appear to have any eyes.”
Still under the fog of sedatives, Karen tried to let the doctor’s words sink in. She just lay there and stared, unable to speak. Eventually the tears started to come.
When Jeff came in after removing his scrubs down the hall, Dr. Patrick broke the news to him. Shaking his head, he looked at Dylan, asleep in the bassinet, then turned to Dr. Patrick: “How could this happen?”
Dr. Patrick explained that something had gone awry very early on in the baby’s development.
“I was so distraught and emotional, I didn’t quite grasp what everyone was telling me,” Jeff remembers. “I wasn’t sure if that meant the eyes would form eventually, or if they could do some sort of transplant, or if there was anything in there at all.”
The Diagnosis
A nurse wheeled the babies to a treatment room near the OR. There Richard Koty, MD, a pediatric ophthalmologist, revealed the official diagnosis: severe microphthalmia—small or nonexistent eyes. “Unfortunately, there’s nothing in the eye socket that’s healthy, that’s viable, that the baby would be able to use to see,” Dr. Koty explained to the parents.In the recovery room, Karen and Jeff were finally left alone to absorb the shock. Still dazed, Jeff walked over to Dylan’s bassinet at the foot of the bed, picked him up and placed him in Karen’s arms. “Part of me was thinking, How am I going to do this? And why did God do this to me?” Karen recalls. “But then I saw how cute he was—like a helpless little bear cub that hadn’t opened his eyes yet.” And for a moment, grief gave way to a smile as she gazed into the face that could not look back and pressed her cheek against Dylan’s.
Severe microphthalmia and anophthalmia (absent eyes) are extremely rare, affecting only 1 out of 10,000 infants a year. No one is sure what causes it. Many doctors suspect a genetic defect is passed from one or both parents, though exposure to environmental toxins—like certain drugs, viruses or pesticides—during pregnancy is another theory.
Nothing could have been done to fix Dylan’s eyes in utero. Whole eye transplants aren’t possible now, though doctors are working on advances that may repair parts of the eye, such as retina transplants and optic nerve regeneration. All this could lead to other vision breakthroughs in the future.
The Jacobsons had even more to worry about. Some of the genes that control eye growth also program development of the brain, heart and kidneys. Dylan would need scans to rule out other potential health problems. And equally pressing was the issue of Dylan’s skull growth. The head grows and molds around the eyes. Without any eyes, Dylan’s orbits would not grow properly and his skull would soon start to take on a narrow, sunken-in look.
Back at home, between changing diapers, timing feedings and carving out time for their other three children, Karen and Jeff tried to regain a sense of normalcy. Jeff returned to work a few days after the birth but was so distraught, his boss sent him home. Karen glued herself to the phone. She needed to get Dylan on the list for therapists and state services as soon as possible, but more urgently, she needed to find him a specialist who could tell them what they needed to do next.
One night Karen flicked off all the lights and closed her eyes. Arms and hands extended, she felt her way from the couch to the tops of the green wooden kitchen chairs, bumping along the wall in the dining room, around the corner to the hall and along the oak banister up the stairs, trying to imagine the challenges that lay ahead for her little boy.
When Dylan was two weeks old, Karen took him to the office of a prominent New York City pediatric ophthalmologist. The doctor took one look at the baby and said, “Well, this young man’s never going to fly airplanes.” He referred Dylan to another ocular specialist, but after seeing how young he was, and being put off by the first doctor’s comment, Karen kept searching. Then a family friend told Karen’s mother about a top pediatric eye doctor who had recently treated several Bosnian and Middle Eastern children whose eyes were injured during the wars.
On a warm summer day, Karen carried her son into the second-floor office of Pamela Gallin, MD, director of pediatric ophthalmology at the Edward S. Harkness Eye Institute at New York-Presbyterian (Columbia) Hospital. “What a beautiful baby,” Dr. Gallin said when she unwrapped the blanket. She had seen microphthalmia before, but usually it was just one eye that was missing. Two eyes was a very uncommon case.
Hope for a Happy Life
Dr. Gallin knew the best person to help Dylan: Michael Kazim, MD, a cutting-edge ophthalmologist specializing in complications of the eyelids, who would prepare his sockets for artificial eyes and make sure Dylan’s head grew to the right size and shape. Dr. Gallin ordered a battery of tests to rule out further issues with his heart and kidneys (all eventually came back negative for other defects). More important, she told Karen what she most needed to hear: “You have a beautiful child who will go to college and get married and live a productive, happy life.”Three weeks later, with Dylan under general anesthesia at Morgan Stanley Children’s Hospital of New York-Presbyterian, Dr. Kazim inserted small M&M-size pillows called hydrogels into each of Dylan’s eye sockets and stitched the lids shut. These fillers mimic the presence of eye tissue so the face forms the way it should. Dr. Kazim replaced them with larger hydrogels a month and a half later.
When the doctor unstitched the eyes and removed the second pair of fillers, after another six weeks, he and Dr. Gallin felt that Dylan’s facial measurements had reached normal proportions. But the now-five-month-old Dylan seldom opened his lids, except for an occasional flutter when Danielle handed him his favorite toy, an orange Koosh ball, or when he got a lick from Niblet, the family’s cairn terrier. Most people thought he looked like he was sleeping even when he was awake. So the next step was to visit an ocularist and start the process of making the blond boy beautiful blue artificial eyes that could increase in size as he grew.
As Dylan neared his six-month birthday, Karen sat at her kitchen table with a box of doughnuts, several cups of coffee and two state therapists. She was concerned. Dylan’s twin, Jenna, seemed to be way ahead of her brother developmentally. She was already crawling, while Dylan just sat up and rocked back and forth, a typical self-stimulation technique. Krista Petersson, a social worker and physical therapist at St. Joseph’s School for the Blind, reassured Karen that it’s normal for many blind children to be behind sighted kids on such milestones, since much of what a child learns at this age comes from visual cues. The therapists were more concerned about Karen. Managing four kids under four, one of them disabled, plus a part-time job at an insurance agency, was a lot for even a supermom to handle. But Karen says she’s not ready for a support group yet. Like Dylan, she needs to take one small step at a time.
On a mild day in February, 20-month-old Dylan takes the hand of his therapist, Mi Koo, and walks to a small table in a narrow office at the Jewish Guild for the Blind in Manhattan. Mi Koo hands him a brown coffee tin with colored wooden blocks and plastic shapes. Dylan, his head bent slightly and eyelids still closed, takes out two shapes and bangs them together. He isn’t wearing his artificial eyes today. As soon as Karen puts them in, he takes his finger and pops them out in one quick motion.
He’s also annoyed by the new green-and-blue wool sweater he’s wearing, a gift from his grandmother, and he keeps reaching to scratch his neck. Losing interest in the blocks, he finds the can, drops the blocks in and pushes it away.
“That’s good, Dylan, but don’t push away. When you want to say finish, do this,” says Mi Koo, taking the palm of his right hand and rubbing it over the top of his left, then the left palm over the right, to remind him of the proper sign. Dylan’s newest challenge is learning to talk, another milestone that can take longer for blind children. In the meantime, he’s learning sign language to communicate. He’s become so frustrated at not being able to express himself that he’s started banging his head on the floor.
Cutting Through His Darkness
“You’ve got two weeks. By my birthday, you have to say Mommy,” Karen playfully tells Dylan after his session, as she grabs her 23-pound boy under his arms and hoists him to her hip. Dylan may be taking longer to find his way, but watching his progress from crawling to walking over the past year, the Jacobsons are filled with new hope for their son. In fact, Dylan can navigate the bottom floor of the house as well as his sighted twin, Jenna. And Dr. Gallin, who continues to coordinate Dylan’s care, still believes technology might find a way, someday, to give Dylan sight, and consults with researchers around the world about him.At home, Karen is scrambling to make dinner. Zachary, Danielle and Jenna are all playing in the family room. Dylan alone is in the kitchen. Though his siblings don’t purposely exclude him, his limitations have hindered his social skills. Talking will be a turning point. Once he starts making words and sentences, which should happen over the next year, his social and practical skills will catch up quickly. He’ll learn Braille and attend kindergarten in a mainstream classroom just like Jenna.
For now Dylan seems content to explore the world on his own. His little hands feeling along the kitchen wall, Dylan makes his way to the hall and to the gold knob on the closet door. He fumbles with this new discovery and, hearing the click of the latch, pulls the door open. Giggles. He pushes it closed. Click. More laughs. For 15 minutes, he opens and shuts, hysterical laughter cutting through his darkness. Watching from the kitchen, Karen smiles as she realizes her little bear cub is opening his own doors and becoming a little less helpless every day.