I came across a previously published piece I wrote about my daughter, an introduction about OI for some co-workers who were just getting to know me. With the number of new people in my life now, it seems like a good time to repost.
Let Me Tell You A Story
I started a full time job last fall. It’s taken me a while to get to know the people there, but now we’re getting to know each other better, and the subject of my daughter’s disability is coming up more frequently. Whenever I tell new people that my daughter’s in a wheelchair, I get the same response. You OI people know what I’m talking about; the script, the questions that everyone asks when they hear The News. Except for the pity on the faces of the people I’m talking to, I don’t mind telling the story. It’s part of my life, part of my daughter’s life, and gives me an opportunity to remember my daughter as a baby.
So let me tell you a story.
My daughter has a bone disorder called Osteogenesis Imperfecta. She has a severe form of it, though there are worse forms. She uses a wheelchair for getting around. Yes, she can walk with a walker, but she’s very small, and walking’s not practical in the dangerous hallways of her crowded high school. She only comes up to the thigh on most people anyway. So she uses a manual chair or a power chair, when it’s not broken.
She was born with this condition. I knew about it a couple of hours after she was born. No, there was no indication of the condition before she was born, even though I had two ultrasounds when I was pregnant with her. Oh, she was beautiful even then, such a perfect, delicate little face. So different from her brother, who was rugged even as a baby. She had long, gently tapered fingers, a beautiful mouth and a petite nose turned slightly up at the end. Those prenatal pictures made me fall madly in love with her.
When she was born, she wasn’t very responsive to some of those pesky APGAR tests. The nurses told me she had “bad muscle tone”, but that it would pick up in a few hours. They kept her in the nursery. A long time. They brought her in for me to hold, all swaddled and a little swollen. She looked exhausted. I had to put her back in the bassinet for them to wheel out of the room so they could do more tests. I waited.
The doctor came in and told me that he thought she might have a problem. He said they’d detected a broken forearm, which isn’t uncommon in newborns. He said he also heard a “pop” when he did the hip displacement test. He had broken both her femurs with that test. I felt bad for him later. He ordered a full set of x-rays on my baby, and this revealed dozens of fractures, some fresh, some already healing. She had broken bones in my womb.
He suspected she had one of two disorders: Hypophastasia or Osteogenesis Imperfecta. He was pretty sure it was OI. He was right. He brought in the local orthopaedic doctor, who confirmed the clinical diagnosis of OI based on the large fontanel she sported. I worried over that fontanel for months, you know. I was terrified her skull would never fuse together and protect her brain. But I would stroke her scalp for hours, protecting her with my fingers, with my love.
After the doctor told me the diagnosis, he said he was going to have her airlifted to a hospital in the closest big city. They brought me into the nursery, where she laid unclothed, now that they realized swaddling her might cause more fractures. She was in an incubator of sorts, laying alone, uncomforted, in pain. Poor little tiny feet still had ink on them, the Mel Sharples hat askew on her beautiful little head. I couldn’t pick her up.
They handed her to me on a pillow. I held her, now distanced from touching my daughter, from giving her the comfort she needed by the gown they made me wear–how silly to make me wear a gown, when I’d just held her against my skin and she’d survived the experience. But now I was a threat to her, a danger. I held her as close as I could, touched her face, and had to put her back in the incubator so she could get on a helicopter with (caring, dedicated, medical professional) strangers and fly 200 miles away from me.
They let me go home immediately afterward. I didn’t have a baby anymore, so I didn’t belong in the maternity ward. I don’t remember much about the rest of the night. My sister, who had started on her way to come visit as soon as I let her know I was in labor, arrived sometime that evening, and held me as I cried myself to sleep.
It was Christmas time. The day before she was born, I had gone on a Christmas frenzy, buying and putting up the Christmas tree, taking my 4 year old son to see A Christmas Carol performed live, and walking him to the nighttime Christmas parade. At some point, I put the Christmas lights on the tree, which stood leaning against the corner in the only receptacle I could find that was large enough to hold it, a bucket with cinder blocks shoved up against the sides. A Christmas tree adorned only with lights greeted me in the morning, a stark symbol of my new reality.
The next day, my sister, our sons and I drove to the big city. My husband (at the time) stayed home to finish his exams, and would join us later in the week. My body had given over to shock, exhaustion, mourning. The three hour drive felt like years, felt like minutes. To get to my daughter’s side was all I knew.
I flew into the hospital, not caring if I walked into traffic, cut somebody off, bumped into someone. The NICU received me, showed me how to scrub up with those painful brushes, to remove my jewelry and pull back my hair. I had to put on a gown again, yellow crisp paper-like and antithetical to holding a baby.
I found my tiny girl lying on a bed under a warming lamp, completely knocked out with narcotics, wrapped from hip to knee on both legs in soft gauze. I wondered if she was going to spend the rest of her life doped up on pain meds. The bed was tilted slightly, I forget now why, and she would slide occasionally toward the foot. We learned to scoot her gently back into position. The nurses handed my daughter to me on a pillow again, all of the monitor leads taped to the edge of the pillow. The first time, it was difficult to balance this fragile baby and manage all those cords on a stiff pillow. We got better at it, though lifting her back into her crib I was reminded that I had just had a baby. My body hurt.
The neonatologist gave us some more detail about OI. His resident, a doctor with one of the worst bedside manners I’ve ever encountered, did more harm than good. The nurses were my teachers, my examples. I grew to love the smell of Purell, which was not yet out on the open market and was only used in settings like this. I thought it was magical that you could wash your hands without soap and water. In the NICU, I learned to hold my daughter safely (on a pillow), to nurse her, to sing to her. I finally got to put clothes on her, though not the footie pajamas I planned on since her femurs were broken. I put a snap-front jacket on her, gingerly dressing her as I would my most precious torn and tattered Raggedy Ann doll. These were the first OI clothes she wore, the first time we thought about ease of access and light layers for my sweaty girl. And I was so glad she was a girl and could wear skirts when she was broken. So ironic now, because she refuses to wear a skirt. Except for prom.
I couldn’t be with her all the time, since my son needed to be cared for also. I hated leaving her. I hated leaving him. After 10 days, they let me take her home. I was terrified to be without the nurses, without someone to help me move her, or watch her while I slept. But we took her home, driving 5 mph over every pothole and speed bump and train crossing. I still drive slower over bumps now, aware of every jostle of the car. I took her home and placed her, still in her carbed, oh-so-gently in her crib in her white-and-pink-painted room.
There is no history of OI in either of our families. OI is not a result of my having done or eaten or imbibed something while pregnant. I learned from the geneticist who did my daughter’s genetic testing that our form of OI is a spontaneous genetic mutation that occurred in the eggs that were created when *I* was conceived. It just happened.
My daughter doesn’t take pain meds very often. She wears whatever clothes she likes (if she can get it in her size). And that fontanel finally did close up on its own.
My daughter has OI.
My daughter is now 23. She still has OI.