Part 6 (1/2)
”What if she can't?” I asked Kevin.
”We'll cross that bridge if we come to it. She can do it. She'll do it.”
The harness was lowered down to her. Annabel sat for another long moment while Mike called her name, telling her over and over to take hold of the rope, and after a while, she reached out and took it. Then she looked up at him and smiled.
”Good girl, Anna! Good girl!” Mike called over his shoulder, ”Okay, y'all, she is responding. She's responding.”
There was a collective exhale, and I felt Kevin squeeze my shoulder.
”Okay, Anna, we need you to put one foot into that big loop. You see what I mean? Put one foot in that big loop, and then put your other foot in the other big loop, and just pull the harness up so your legs are all the way through, and it's like you're sitting in a swing, right? See how that works? Give the rope a tug, Anna, so I know you're holding on good and tight.”
Anna still didn't call out to him, but she followed his instructions and got herself securely rigged in the harness with Mike encouraging and cheering her on.
”Yes! Way to go, Anna. You got it, you got it. Okay, we're going to start bringing you up, and we're taking it nice and slow.”
With the Cleburne engine still a good twenty minutes away and Anna dangling in the harness thirty feet below, they began the careful process of raising her up. We waited for what seemed like an eternity.
It should have been easier, considering all the waiting we'd done in the previous years.
ANNABEL WAS IN THE hospital for twenty-two days after that initial surgery, and during that time, we all experienced moments of intense grief and intense grat.i.tude. She was alive, and that came to mean everything. We knew the road would get rougher, but now we knew what to pack. That was something. A lot, actually. For a long time, that was something I'd prayed for: a handle, a set of words I could Google, any clue to the right questions.
When Annabel was strong enough to stand, the nurses told us we had to get her up and walking. Understandably, Anna wanted nothing to do with that. She'd just sustained this ma.s.sive insult to her little body, and she wanted to curl up in her bed with the menagerie of stuffed animals people kept bringing her.
”Anna,” Kevin said as gently and as firmly as he could, ”you gotta walk for us.”
”No. Daddy, I can't. Don't make me.”
”I'm sorry, Anna, you have to.” He stood her up on the bed and looped her arms around his neck. ”I've got you, okay? We can do this.”
”As long as you understand,” she sighed, ”there's no way I can physically do this.”
He lifted her down and set her on her feet, supporting and steering her as she made the terrible trip from the bed to the door frame and back again. About eight steps each way-a huge accomplishment that we celebrated with tears in our eyes.
Cook Children's had a lot of wonderful people on staff, but their child life specialist, Dani Dillard, a young woman with bright eyes and an amazing smile, made a lasting, life-changing impression on Anna. Just a few days after the surgery, she tapped on the door to see if there was anything she could bring Anna.
”A couple movies, maybe? Or some music you'd like to listen to? I have some coloring books with me. Do you like to color?”
”Yes,” said Anna, ”but I have this.” She held up her right arm to show Dani the IVs in her wrist and the back of her hand.
”Oh, we can work around that, easy squeezy,” said Dani. ”Mom and Dad, maybe you'd like to go get some coffee while we work on our coloring for a little while.”
Grateful for the opportunity to leave the room together for half an hour, Kevin and I left the two of them chatting over their coloring books, and by the time we came back, Anna announced, ”Dani is my best friend at the hospital.”
I was amazed at her infinite patience and the creative, drama-free ways she talked to Anna about everything that was going on, physically and emotionally.
”Do you understand what happened to your body, Anna?” she asked.
”I had a surgery,” Anna said.
Using a baby doll, Dani showed Annabel what that meant and how the IV was feeding her just like her body was sucking on a straw, and why she had to keep getting out of bed to walk a little each day. Over the years, she got to know Anna's sisters and loved them as well. Dani became part of the routine that made the days and nights bearable for Anna during that long hospital stay, and beyond that, she showed Anna and her sisters an extraordinary ministry made up of small things that were enormously important to this tiny, vulnerable person and her exhausted parents.
We took Anna home to begin the next leg of her difficult journey, and the following Sunday was Easter. We sat in church together as a family for the first time in many weeks, and that was such an abundant blessing. I let the familiar songs and scripture lift my heart.
Why do you seek the living among the dead? He is not here. He is risen!
We had no choice but to accept the reality that Annabel was in for a lifetime of struggle with this thing, but we would never accept that she was at its mercy. Quality of life-not just for Anna, but also for our whole family-was a goal we would have to define and fight for. It would be a feat of engineering. And it would be worth it. There was no point seeking the full living of our life among the dead ideas of ”normal” and ”supposed to be.” We would have to trust in G.o.d's grace and find a new way forward.
Kevin and I set out to learn everything we could about pediatric motility disorders while Anna relearned the simplest elements of a child's life-how to eat, how to play-and did her best to catch up with a mountain of missed schoolwork.
From the time she was four years old, Annabel had suffered horrific pain and other symptoms. Now she was six. In the months following the catastrophic double surgery, the gastroenterologist finally homed in on the true diagnosis: pseudo-obstruction motility disorder, a rare disorder that has a profound effect on the intestines, preventing the body from receiving the nutrition it needs, and antral hypomotility disorder, which is essentially a paralysis in the stomach.
The hardest words I've ever heard and spoken: ”There is no cure.”
Pseudo-obstruction motility disorder in children is usually congenital, present at birth. Over time, bacterial infections and malnutrition take their toll, along with a variety of issues that directly or indirectly affect almost every part of the body. The exact cause is unknown, and it is a difficult disorder to treat. At this writing, there really isn't one treatment protocol everyone agrees on, because the diagnosis can be difficult to get to, and there's so much variation between the patients.
We were told that it becomes increasingly difficult to maintain a good quality of life for kids with pseudo-obstruction motility disorder and antral hypomotility disorder. Because chronic abdominal pain is one of the main symptoms, they're frequently not able to eat, and even when they can, they don't want to, because they've learned to a.s.sociate eating with physical agony and emotional distress. In many cases, liquid nutritional supplements have to be fed through a nasogastric tube, which goes from the nose to the stomach, or a ”G-b.u.t.ton” that's been surgically placed in the abdominal wall. These effectively replace the nutrition needed for survival but place an enormous burden on a kid-as it would on anyone in a society where so much socializing and celebrating revolves around food. Sometimes it's necessary to remove part of the intestines, and surgical bypa.s.s may be considered. Sometimes a colostomy bag and other extreme measures are needed. In the most severe cases, small bowel transplantation may be considered.
A certain percentage of children with pseudo-obstruction motility disorder and antral hypomotility disorder are able to stay the course after diagnosis; they stay about the same during their lifetime, and continuing treatment keeps them on a fairly even keel. Other kids go through a lot of ups and downs with symptoms in constant flux and treatment constantly being readjusted in an effort to keep up. The overwhelming majority of children with pseudo-obstruction motility disorder and antral hypomotility disorder continue a steady downhill trajectory that has already begun by the time they're diagnosed. Their quality of life erodes as the increasing need for pain control becomes a higher priority. Nutrition via IV or PICC line allows them to survive, but not surprisingly, depression is often a major issue.
As we continued our quest to get the best help for Anna, even the most optimistic doctors agreed that there was no statistically measurable percentage of children with pseudo-obstruction motility disorder and antral hypomotility disorder who fully recovered. They simply did not see patients get well and go on to live their lives free of pain and treatment. Our best hope was to make her life as comfortable and happy as possible as the disorders continued to take their toll on her little body.
A pattern began to form. One step forward, two steps back. Sometimes three steps back. Less than six weeks later, she was back in the hospital, then home again but still pale and in pain, her little belly miserably distended. We were back where we started, only now, on the days when Anna felt only moderately lousy, we felt lucky. On the nights when there was nothing else we could do for her, Kevin tucked her into bed, saying, ”Maybe you'll feel better in the morning.” It felt so empty when he said it, but Anna accepted it with hopeful grace.
We considered it a triumph one afternoon when she went outside to swing, but she came in just a short time later, telling me, ”Mommy, I feel strange. I feel like I can't breathe right.” I put her to bed with all the standard comfort measures, walking on eggsh.e.l.ls for a few days until she rebounded and felt well enough to go back to school.
We began trekking to one specialist after another, determined to find someone who could help her. One of them started her on a drug called Reglan to spur contractions in her digestive tract. The possible side effects included muscle movement problems similar to Parkinson's disease, but it was the best available option.
”The best medication, cisapride, was taken off the market,” the specialist told us. ”Only a handful of physicians are licensed to prescribe it. Gastroenterologists. And veterinarians, of course.”
”Veterinarians?”
He nodded. ”It's mostly used on cats, I believe.”
Happily, I happened to have my very own veterinarian on speed dial, and Kevin dove into research on the drug, but before we had a chance to pursue it, Anna ended up in the hospital again. This time, we opted to take her to Dallas, where we'd already connected with a terrific specialist who was known for breakthrough procedures-including pediatric intestinal transplants-and a wonderful motility specialist, both of whom recognized quality of life as a priority. At this point, Annabel was on eight medications, a pharmaceutical plate-spinning act that made it possible for her system to function on a basic level. We didn't want to take her to a hospital where the doctors who understood this didn't have admitting privileges. So off to Dallas we went.
The first day, she was processed through the ER with the familiar refrain-chronic pain, severe distention, and a general malaise-and they ran the usual battery of invasive tests, which located a possible obstruction. The second day, things took an unexpected turn. An earnest young resident came in and sat down across from me.
”As we were reading the CT scan,” he said, ”we saw evidence of healing on the eighth and ninth ribs.”
Completely missing his point (and now I understand that it was very pointed, the way he said it), I started asking questions about brittle bones and even volunteered that Anna was such a rowdy little otter when she was a preschooler, she'd broken her arm more than once. I asked him, ”Do you think this means her bones are brittle? What would cause that? Could it be related to nutrition issues that come with motility disorders?”
”It doesn't appear to be the result of an accident,” he said. ”In cases like this, we have no choice but to have CPS investigate.”
”CPS,” I echoed. ”You mean... Child Protective Services? You're having me investigated for...”
He curtly confirmed that, yes, indeed, that was the deal. And then he left me there, feeling accused and infuriated and very, very frightened. But as I sat there with my ears ringing, I thought of that day Anna was out on the swing and came in saying she was having trouble breathing. I'd tucked her in with Motrin, a heating pad, and a good book. She never said she was in the kind of pain a person with broken ribs would be in.
”I want to know how that happens,” I raged to Kevin. ”How do a little girl's ribs just break like that-and how do we keep it from happening again?”
He shook his head. ”I don't know. Maybe they're seeing some artifact of the distention or something to do with the nutrition deficits she's gone through.”