PWS Awareness Day 9: On Doctors and Buses

Minutes after Oscar was born, our doctor proclaimed that Oscar would never ride the little yellow school bus.  His muscles were weak, but his mind would be fine. 

I admit I took solace in his words at the time.  But it didn't take long before the full meaning and insult of his words sunk in.  And when Oscar finally did ride that little yellow bus I wanted to send him this photo and tell him just how wrong he had been.  That I was proud of Oscar for riding that bus, by himself, home from school each day. (And that he should be careful about what he says to terrified mothers in the delivery room).

We've had to part with a couple of doctors in the past thirteen years. One for insensitive comments like this, another for his lack of willingness to consider the parental perspective.  I love the doctors who light up when they see Oscar, excited to ask him how he's doing. I love the doctors who've done their research but value input from me and Paul.  Thankfully, we've had FAR more of the latter than the former. 

PWS Awareness Day 8: Reading at Scribd

My awareness raising happened at a podium tonight.  I was honored to be at the Scribd Author Series event in San Francisco with my fabulous writing group the Write On Mamas, reading an excerpt of my essay about skiing with Oscar.  (The full essay, "Slide Show", appears in our newly published anthology Mamas Write.) 
 
As I practiced this afternoon, reading my words over and over, and trying to coax my voice to convey the right amount of excitement at the proud parts, gravity in the scary parts, and levity at the one almost-funny part, I felt really connected to and protective of Oscar.  In my allotted four minutes I wanted convey to the audience why a seemingly disastrous afternoon of skiing was actually one of my proudest moments.  I wanted them to get it.

Six mediocre read-throughs later I found myself hunting around the house for a picture of Oscar to take with me, to remind myself why I am reading a deeply personal essay to a room of strangers.   Again.

I am doing this -- this writing thing, this reading thing -- so that other parents will feel less alone when their baby is diagnosed. So that other parents will know, that even though raising a child with special needs is challenging, there will be joy and celebration again. So that more people know about this crazy syndrome.

Here's that photo of Oscar. I kept it tucked into my book, and peeked at it just before I stood to read.

PWS Awareness Day 7: Infant Stimulation

Oscar qualified for occupational therapy starting in the hospital and physical therapy followed soon after.  An early intervention specialist came weekly to our house. 

Pretty soon our schedule was full of appointments and my head was spinning with things I needed to do to stimulate his brain and help him build muscle strength.  Babies learn from from sucking on their fingers, from accidentally batting at the toys hanging near them, from shifting their weight and finding themselves in a new position.  Oscar couldn't do any of these things so we did them for him.

His trunk and neck were too weak to sit upright, so I learned how to support his chin while stroking his back to activate his trunk muscles.  He couldn't lift his arms or legs so we laid him on an inclined wedge and propped up his extremities to help him fight gravity a little easier.  We tied loud bells to the bouncy seat and moved the bar so close that any movement would be rewarded.  We laid him on his side and supported him with a rolled blanket so he could see the world from a different angle. We gently rolled his body back and forth and around, and kicked his legs for him.  We put him on his belly and rested his mouth on his hand so he could explore his fingers.  We placed toys and mirrors all around him. We played classical and jazz music all the time.

We tried our best to help him see and hear and feel the world like a typical baby.  But it was exhausting feeling like there was always something more I could and should be doing to help him develop his muscles and his brain.  So sometimes we just had to let it go, let him sleep too long, let him lie in one position too long and focus on Abe or ourselves.  Finding the right balance, one that can be sustained, is one of the hardest parts of having a child with special needs.

PWS Awareness Day 6: Never Full

This is what "never full" looks like:

Oscar's siblings would tell you about the repetitive scrape of metal on ceramic, a spoon chasing the last shreds of cabbage in tonight's soup bowl.  Or the sneaky finger swipes through the cup of yogurt at snack time.  The apple reduced to seeds and a stem.

"I think you got it Oskie," I've heard us all say a million times.

PWS Awareness Day 5: Siblings

Abe met his baby brother for the first time just a few hours after he was born.  We entered the NICU, Abe clutching a huge green stuffed frog under his right arm -- his gift for Oscar.  Dr. Sandhu, the neonatologist, was examining Oscar, checking his muscle tone. He held one of Oscar's arms at the elbow, let go of his fingers, and shook his forearm.  Instead of staying tightly curled in a fist, Oscar's fingers and wrist flopped limply back and forth. I stood there, paralyzed, panicking, not knowing what any of this meant.

Abe saw something different.  "Look Mom, the baby's waving at me!" he said. 

Dr. Sandhu heard us then, and smiling, he waved Oscar's hand once more, making Abe giggle.

Even then I understood the importance of this gesture.  Even then I knew that Dr. Sandhu saw that Abe's relationship with Oscar trumped the need to figure out what was causing Oscar's symptoms.  Even then I recognized that we became a family in that moment. 

Here are a few of my favorite early photos of Abe and Oscar. 

(I can't believe I let a two year old feed Oscar. His suck/swallow wasn't coordinated and he could have choked. Yikes.)

PWS Awareness Day 4: Quiet

"What a great sleeper!"
"You're so lucky!" 

"He's such a good baby!"

Strangers said these things to me while in line at the grocery, while pushing Abe on the park swings, while on walks in the neighborhood. Oscar would be slumped in his stroller or sagging in the baby bjorn with a little towel to support his head.  He slept all the time.  He never cried.

Some days I could just smile kindly and respond, "Yes he is."  

But other days I couldn't control myself.  "It's part of his disability!" I would shoot back, regretting my sharp words as soon as I registered the shocked looks staring back at me.

I'm not proud of those moments.  But there was something in me that needed to let people know we were not celebrating the quiet.

What did we celebrate?  Moments like these:

I don’t know why Oscar was crying here but I do know that it was his first real moment of upset.  I called Paul and we stood there watching, stunned and proud.  Our baby was crying!  Tears streamed down my face as I picked him up and comforted him. 

“Shh, little O, it’s ok. Mama’s here,”  I murmured into his cheek. 

He calmed immediately, but that brief moment in which he needed me sustained me for hours.

PWS Awareness Day 3: Feeding and Satiety

Like most babies with PWS, Oscar was unable to nurse.  He was too sleepy, too weak. He couldn't sense hunger and couldn't latch on.  He had an ng tube and was fed through his nose for the first two weeks before he graduated to a special bottle.  I still remember how I needed to support his neck and head with my right hand while my left rhythmically squeezed the bottle and stroked his cheek.  Feeding could take an hour, but the nurses advised us to stop after thirty minutes - at that point he would be burning more calories by attempting to suck than the milk would provide.

Oscar was five or sixth months old when he started to gain weight a little too rapidly.  All the literature had warned that he would be hard to feed for the first few years. Then a "switch" would flip and he would be constantly hungry.  We expected hyperphagia and rapid weight gain to set in anytime between ages two to five.

But at six months?

I panicked.

Our pediatrician restricted his calories to two-thirds the usual amount recommended for his weight, as is typical for people with PWS.  We recorded how many ounces he drank at each feeding and made sure to stay within the daily limit. When he started eating solids we measured his portions and created a calorie spreadsheet.  (Eventually we would learn to eyeball it.)

I don't know if it was Oscar's slow metabolism or his lack of satiety that led to the early weight gain.  But I do know that as soon as he was able to sit in a high chair and eat solid food, he was eating everything we put in front of him.  Every steamed carrot, every cheerio.  He never, not once, threw anything overboard.   He didn't appear to be always hungry, but even at this early age it seemed like he was never full. 

Here's one of my favorite photos of Oscar at seven months, around the time of that weight gain. 

(By the way, this smile melted away an iceberg's worth of grief and worry.)

PWS Awareness Day 2: Birth

We knew something was wrong the moment Oscar was born.  His arms and legs hung at his sides. He didn't cry.  He was blue.  In the five days before the geneticist suggested we "rule out" Prader-Willi syndrome, Oscar racked up more symptoms.  His rag-doll appearance was due to hypotonia, or low muscle tone, and that, combined with his excessive sleepiness, inability to nurse, downturned mouth, almond-shaped eyes, and undescended testes pointed to a serious issue. 

Infants with PWS exhibit most or all of these symptoms to varying degrees.  Oscar was the first baby to be diagnosed at our local hospital in many years.  But within a month they were testing another baby, and by the following year four babies total had been diagnosed.  I do wonder how many were missed before Oscar came along.  Before improved genetic testing and increased awareness of these early characteristics, many children were not diagnosed until the more well-known signs of PWS -- insatiable appetite, morbid obesity, and behavior challenges -- appeared. 

Here Oscar is, just a few hours old.  His hypotonia was significant, especially in the trunk and arms. He felt loose, as if his bones were just shoved into sleeves of skin with no muscles to keep them in place.  I want to go back and pick up this baby again now.  Now that I am no longer afraid, now that he is much more than a list of scary symptoms. Now that he is thirteen and joking around at the dinner table with his equally goof-ball siblings.

PWS Awareness Kickoff: Before

May is Prader-Willi awareness month and many of my facebook friends are committing to posting daily about PWS to raise awareness about the disorder and how it affects their child and their family.

I'm going to give it a shot on my blog (on an almost-sort-of-daily basis), and am kicking off the month with what I call the "before" picture. 

Paul's dad snapped this photo of us with Abe (age two) as we headed off to the hospital. Oscar would be born 12 hours later.

This is me before I'd ever heard of Prader-Willi syndrome. Before I knew what low muscle tone and insatiable appetite meant.  Before I really knew what pain, grief, and even joy, really were.

I used to want to go back to this moment, to climb back into my younger self's innocence and relish it.  But that's the thing about innocence -- you can't really appreciate it until it is gone. 

I don't have a plan for this month of blogging but I'm hoping it will give me a chance to share little snippets from our daily life and how we've approached this complex disorder.  Stay tuned!

Home

“Mom, I had such a good time in Yosemite!  It was amazing, so AMAZING,” Oscar shouted as he stepped off the huge white tour bus last Friday afternoon. I wrapped my arms around him even as he was still talking, eager to share every detail.

I’d already spoken to one of his teachers, who had arrived five minutes early. “He did so great!” she said before I could even ask.

There was just one sticky time, on Thursday morning. “My mom said she’d come get me today if I needed to go home,” he’d told her with tears already threatening. But she knew the deal. “Nope, I don’t think so Oscar,” she’d said kindly.

He started to cry then, and argue. He may have flopped to the ground. “He was tired,” she told me, “but I knew you hadn’t said that.” She told me she stepped away with her phone, pretending to call me, and returned using the vocabulary she has learned is most helpful for Oscar. “I checked with your mom, Oscar, and ‘the plan’ is that you’re going to stay.” He turned it around right then, she said, and went on happily with his day -- hiking, chatting with friends, and painting with watercolors down by the river. By removing the possibility of going home she had freed him up to engage the rest of the day.

As Oscar was gathering his luggage from the bus another chaperone approached me. “I had the best time with Oscar. I just love talking to him,” she said. I just smiled and smiled, so thrilled that Oscar manages to find these people that appreciate him. I worried though, did Oscar spend the entire trip glued to an adult? Did he engage with the other kids at all?

I stopped doubting and let the trip’s success sink in during the half-hour car ride home. Oscar and his friend M (an 8th grader) shared their favorite moments while Ruby and I listened in. Oscar added on eagerly to M’s stories about the hike to Lower Yosemite Falls, and recounted his own, about the nature center, the gift shop, and the kids he hung out with. (The kids he hung out with!) He made appropriate comments, showing enthusiasm for the details that M added. “Oh yeah! That was so fun!” he would say. He was confident, quick, and socially engaged in a way I rarely see with peers. 

I sighed, letting out that little bubble of anxiety I’d stored up while he was away.   The trip really had gone well.  (And Oscar is already talking about next year.)