Little Miss Adorable’s Story
She was born on a Wednesday, put into the Special Care Nursery on a Friday, and by Monday the Chief of Genetics was involved. On Tuesday my husband was signing paperwork to expedite genetic testing to Washington DC and 15 days later we had out diagnosis – Prader-Willi Syndrome. We were lucky to get such excellent care and an early diagnosis. I know of some children with Prader-Willi Syndrome who are not diagnosed until their teens. We are really lucky.
That’s the story we tell people about Little Miss Adorable’s diagnosis. Just the facts, and not even really a description of Little Miss Adorable. We leave out most of the details because they are hard to tell to ourselves, never mind curious acquaintances.
On that Wednesday Little Miss Adorable was born by caesarian section and had Apgar scores of 9 and 9. She was pink, little and plump. I have to say that a C-section was one of the worse experiences of my life – lying on my back strapped to a table while a sheet blocked my view of the people working on my body. I know what they are doing and I was trying so hard not to launch into a full panic attack. I could feel tugging and tried to not think of the anatomy that was being opened and closed.
I felt nauseous during the operation and by the time the doctor had sewn me back up I was vomiting. I clutched a garbage can, vomiting on the gurney. Little Miss Adorable was swaddled and tucked by my legs. I vomited for 14 hours. I was unable to get out of bed and had tubes attached to me. My husband held Little Miss Adorable and cuddled her during that time.
By the time I stopped vomiting I knew there was something wrong with Little Miss Adorable. She was too quiet, and not interested in feeding. She couldn’t latch on my breast and couldn’t feed from a bottle. The nurse spent the night finger-feeding her. Little Miss Adorable flopped like a rag doll when we changed her diaper. She did not cry. Something was wrong. On Thursday the nurse agreed, and called the pediatrician. The doctor said the baby was mucousy from the C-section and recommended nose drops. Little Miss Adorable was floppy, not feeding and barely opening her eyes. We tried the nose drops and finger-fed her all day Thursday. By Friday a new pediatrician was on call and the nurse stopped her in the hallway. Their conversation drifted into my room, “I’ve been a nurse for 25 years and never seen anything like this…”
The pediatrician arrived in my room, examined Little Miss Adorable and announced that Little Miss Adorable would spend the weekend in the Special Care Nursery (NICU). Tests would be done to identify any infection, and then on Monday Genetics would be involved. Little Miss Adorable was rushed to the NICU and put under a warmer awhile the nurses struggled to find a vein to attach an IV. Little Miss Adorable was dehydrated. She did not cry while she was poked and prodded. Eventually the nurses attached an IV to the vein in her head and we were asked to leave the room while they did a spinal tap.
I was told to pump breast milk and that Little Miss Adorable would be fed through a nasogastric tube. This tube was inserted through her nose and entered her stomach. Her stomach would be filled for her.
We did not know if Little Miss Adorable would get better or worse. We did not know if she would leave the hospital. We simply knew something was terribly wrong. My husband called his parents to ask them to come over and stay with our (then) 2 ½ year old son. What they said remains forever etched on my heart. They couldn’t, because my father-in-law had tickets to a local hockey game. The only time in our lives that we asked for some babysitting help they won’t do it because of a fifteen dollar ticket to a local hockey game. Hubby and I were equally shocked. I called my sister, who lives 1 ½ hours away. While on the phone with me she threw some clothes in a bag and ran out the door, burning rubber down the highway.
I slept while my husband picked up our son from daycare and packed him up to visit with his aunt. When I woke I went back to the Nursery, where I was told I couldn’t touch Little Miss Adorable because care is given every 3 hours (ie. 0300, 0600, 0900) and I was between these times. I could give her ‘a loving pat’ and come back later. I looked at the well-meaning nurse who clearly did not have children of her own and died. A couple days ago Little Miss Adorable was happily a part of me, and now she’s alone in a clear plastic box under fluorescent lights with tubes and wires running every which way. My whole body was aching for my child, and I was told to come back later.
I was demitted from the hospital the next day. The morning nurse (again, someone without children) unceremoniously removed staples from my abdomen and told me that I needed to pack up and leave, someone else needed my room. I was incredibly confused – what about Little Miss Adorable? She would stay here. I would go home. I could come and visit her in the Special Care Nursery.
I now realize I was functioning in a complete state of shock. My husband and I managed to pack up and get my post-caesarian section body into the car. It was a silent drive home. When I entered my home I sat on the living room sofa, and dazedly looked at the cheery Christmas decorations I had put up before Little Miss Adorable was born. My son was out for the day with my sister. Our home was silent. I remembered bringing my son home from the hospital – the giddy feeling that song birds and cherubs were hovering around, celebrating his arrival. The only thought that filled me was that leaving the hospital without your child was profoundly unnatural and wrong. Every fiber of my being protested the separation. I was here, in a room full of stupid grinning Santa Clauses. She was alone, in a plastic box surrounded by beeping machines.
I went into the washroom and cried. Every part of me was crying for my child. Milk poured from my breasts in terrible sympathy. I ached and raged against the injustice of her being left behind. I did not care what was wrong with Little Miss Adorable or how the medical staff were helping her, I just wanted her with me.
As I write this I recall when my newborn son was readmitted to the hospital for jaundice. He was a couple days old and the outpatient nurse who admitted him described how I would stay on a cot beside his crib. She told my husband what to pack for me and immediately sent us to the hospital. My son slept in a plastic box under the UV lights wearing ‘baby sunglasses.’ I lay beside him on a cot, watching him through the clear plastic, changing his diaper and nursing him. We would cuddle in the cot together. At the time I referred to our stay in the pediatric ward as the ‘baby gulag’ – it was cold and I missed the comforts of being waited on by nursing staff. I did not realize how good I had it.
I wish I could have stayed on a cot beside Little Miss Adorable. I could have watched her through clear plastic, changed her diaper, and pumped breast milk. I could have cuddled her in the cot and felt her tiny body beside me. I realize there is simply not enough room in the nursery for 20 parent cots beside 20 baby cribs. Parents would be in the way – hogging the washrooms, talking too loudly, asking the staff questions, and cuddling their children. Maybe even singing and laughing. But that is what babies need – movement, noise, joy and love.
I firmly believe that now as I did then.
We settled into a routine of sorts. After my caesarian section the nurse who removed staples from my abdomen gave me instructions. “Don’t vacuum.” I was ok with that. “Don’t lift anything heavier than your baby.” Ok, I have a 2 ½ year old who can barely walk, how am I supposed to do that? “Just be a princess, and let everyone else do everything.” Ok, who is going to ‘do everything’? My family lived about two hours away, and we already saw how helpful the in-laws were. Was my husband supposed to ‘do everything’? What could I do? What should I not do? How the heck do you ‘be a princess’ anyway? I talked to a friend who said I needed to rest. She offered to drive me to the hospital the next day. My husband picked me up after his work. The next day I drove myself, dropping husband and son off at their respective places – work and daycare. I found the walk from the car to the Special Care Nursery to be excruciating. It felt like I trudged down miles of hallways. I lugged breast pumping equipment, snacks and various items I might need. I sat in front of Little Miss Adorable’s plastic box, staring at her limp form. She breathed. She had reddish peach fuzz hair and clear blue eyes. She was a lovely rag doll, flopping in every direction, and still as death. I held her for feeding (pouring breast milk into tube that ran to her stomach) and carefully untangled wires to put her back in the plastic box. At the end of the day I would race to pick up my husband so he could visit Little Miss Adorable before we sped to pick up my son from daycare. Dinner was a frozen lasagne. We would collapse into bed to do it all again the next day. And the day after that. And after that.
We lived on a treadmill – each day the same, with different doctors and nurses drifting through our lives as shifts changed. We consulted with the occupational therapist, the Chief of Genetics and the endless parade of doctors and nurses. Tests were done – ultrasound, MRI, muscle enzyme. The initial tests for infection came back negative, so did all the other ones. Little Miss Adorable breathed, silent and still. She struggled to take a bottle and was tube fed. She looked like a living corpse. Her muscles sagged, making her head take on strange shapes. On a lucky day she opened an eye and looked toward the sound of my son’s voice. And then nothing. The highlight of my week at bathtime. Little Miss Adorable came alive. Her eyes opened and limbs moved in response to the water and rough washcloth. After bath she would collapse, exhausted and limp.
We were told of Little Miss Adorable’s diagnosis on the International Day of People with Disability. My husband and I sat in the Geneticist’s office, hoping against hope that the test would come back as inconclusive. The Geneticist said Prader-Willi Syndrome. Insatiable appetite. Morbid obesity. We were shocked. Little Miss Adorable’s muscles would continue to develop, and she would get stronger. All gross motor milestones would be delayed, but she would eventually meet them. But she would not decline and was, for the most part, healthy. As the Geneticist described the traits people with PWS have, she emphasized this: all Little Miss Adorable has in common with people with PWS is the one deletion on chromosome 15. The rest of her entire DNA sequence is a blend of ours. And we cannot predict the impact environment will have on her development. The Geneticist also said that Little Miss Adorable’s older brother would help provide a rich, stimulating environment.
After our Geneticist meeting, my husband and I went for coffee , purely out of habit. We sat holding paper cups and struggled to make sense of Prader-Willi Syndrome. Soon it was time to meet the pediatrician who was overseeing Little Miss Adorable. As we sat in a storage room (the only quiet and private place on the ward) the doctor asked us what we wanted to do. My husband said he thought a couple more weeks in the hospital using the NG tube would be fine. I said I want to go home. The doctor looked at me, and said ok.
What followed was a week of Little Miss Adorable struggling to drink from a bottle while we learned to use the NG tube. Learning to check the tube for placement was easy (‘Listen for the pop of air’). For Little Miss Adorable learning to use the bottle was not. The efforts in drinking exhausted her for hours after. Bathtime remained our highlight where Little Miss Adorable came alive.
The doctor on call was reluctant to let her come home with the tube in place. We felt that if feeding was the only concern we could do just as well at home and provide her with a rich environment. Little Miss Adorable was spending her days staring at the ceiling in a little plastic cot surrounded by the hum of machinery. This was not a place for helping her learn.
Near the end of our time in the nursery I had a dream about a shopping mall. I was walking through an empty, dark shopping mall opening door after door and trying to find my way out. There was no natural sound or light. Gleaming tiles, flickering fluorescent light, white walls. Each door I opened led to another darkened hallway. My feet echoed through empty halls and there was no way out. I was lost and alone.
I do not remember the day she was released. I have snapshots of giving the nursing staff cards and cookies. I do not remember the drive home. I do remember our oversized calendar marked with an endless string of medical appointments – some nurses coming to our home, some hospital and clinic appointments. An occupational therapist visited regularly. In February that year we had 28 different medical appointments – in 20 days! We would go to three appointments a day, and race to the local Ontario Early Years Centre on our time ‘off’. Little Miss Adorable became the darling of Mother Goose and Infant Massage Classes.
Little Miss Adorable used the nasogastric tube for feeding for 5 ½ months. Her muscle tone slowly developed. At the age of 12 weeks Little Miss Adorable flashed the world a blinding smile, and has been charming the world since.