2015 in a Nutshell

Audrey's 2015 

canceled a surgery

broke an arm

refused a local dilation

checkup in Boston

confirmed 2 fistulas

scheduled surgery for January

AND

ate a gummy bear successfully for the first time, in small bites

hugged Cinderella and Darth Vader thanks to Hope Kids

turned four years old

swam in the "Frog Pond" at the Boston Common

became a Junior Ranger in Costa Mesa National Park

rode a horse for the first time

visited a beautiful castle to watch a scene from the Nutcracker at "Clara's Tea Party"

Visited Disneyland for the first time

Two New Fistulas

Audrey's esophagus as lit by barium. You can see the connection point, the shelf where food gets stuck, the changes in direction, and that subtle black webbing on the left side is where her fistulas were passing barium into the lungs in response to Dr Manfredi squirting barium directly into them. 3 days later, she's still finishing coughing out that stuff. We need to know if food and drink are quietly causing her lung damage over time, or if the fistulas are remaining closed when she eats. That will determine the urgency for the resection surgery, whether we need to do it as soon as possible or can wait until June like I'd prefer due to my responsibilities at home.

Be Still and Know

I've re-learned a valuable truth this week. Sometimes, the healthiest thing for a worrier is for things every now and then TO go just as wrong as they worry it will, and then see that the world keeps on turning and things will be OK. On Thursday afternoon we were supposed to have an important test at the hospital, but the trains broke down for a while during the time we needed to travel. I called a cab and was told they'd be there in 15 minutes, but it never came. A second cab also did not come. A third cab, from a different company, offered us a ride and then changed his mind when we were half loaded, because I only use a card instead of cash. The swallow study had to be canceled, but luckily, the world kept turning.

When I left my house in Utah, I left a meticulous list of each day's responsibilities for my oldest kids, with boxes to check off to help them keep track and reminders for things like practicing their instrument. Arguably the most important was Eden's sweet harp teacher coming to our home on Friday to give her lesson in lieu of us coming to her--a valuable opportunity also for her to help Eden learn to tune and repair our harp. Eden woke up, got herself ready, practiced her instrument, went to the back room for something, got sidetracked, and missed the knock at the door. The harp teacher went home. Hearing about this has again, for many reasons, made my worry level for my oldest daughter spike above that for my youngest. But, the world kept turning.

Our long day at the hospital yesterday was very informative. Audrey has not one, but TWO fistulas that have grown connecting her esophagus to her lungs. The shape of her esophagus at the connection site is the likely culprit for food getting stuck so often; there's a distinct bend in the road and a wide shelf at the point of the angle. No dilating was done because of the risk of widening or rupturing the fistulas. The resection surgery we sidestepped in January needs to go back on the schedule, and we're waiting on the results of a chest x-ray to determine how urgent the surgery is. They are looking for signs of food debris in the lungs which could lead to permanent lung damage. Audrey hasn't had any issues with aspiration pneumonia yet from the fistulas, knock on wood, and since the number of moving parts in my family that hinge on my being home right now is unreal, my preference is to put off surgery until school's out for the summer again. I also learned that she still does not have full function in her vocal chords, meaning we were right to worry when she coughs so often while eating. Luckily they were able to do the modified barium swallow test we'd missed on Thursday, after Audrey was awake enough from anesthesia, and that was helped by the fact she hadn't been dilated so her throat wasn't too sore. Those amazing ladies in the feeding team had Audrey feeling like a movie star as they closely recorded her swallowing function. She ate grapes, watermelon, chips, applesauce, and drank an entire 2oz barium-laced apple juice box, and every single swallow was ABSOLUTELY PERFECT. Considering 3 years ago she'd been aspirating with every swallow, and those vocal chords aren't closing all the way, this news was a gargantuan relief.

We left the hospital in the beautiful sunlight, took a train and walked through the public gardens full of pink flowers, listened to my favorite street musician play his Spanish guitar, went to the playground and splashed together in the water until sunset. Because we had been prepared to stay overnight at the hospital, I even had a dry change of Audrey's clothes for this spontaneous adventure. It was wonderful. So yes, she needs major surgery again. And yes, the future is uncertain as to how long that will be and when, and I pray we will find ways to keep the family healthy in every sense during that time. Yes, I'm OK. More than OK. And the world is still turning.

Trusting our Gut, Against the Grain

I was chilled to see yesterday's Timehop from 3 years ago, and today's is even worse. Was it a coincidence that it happened the same day? I hesitate to share, but some may not remember why the bougie dilation proposed so confidently by our Utah GI, Dr H, yesterday has us so worried for Audrey. Our wonderful former Utah surgeon, Dr Downey, did Audrey's last rubber bougie dilation 3 years ago before we switched to doing the gentler balloon dilations and, eventually, twice doing stents. It was at UVRMC, close to home, no sweat, dilation number twenty something probably. He was stretching her esophagus gently when he suddenly felt it give way, and knew the tissue had torn. I was not worried. We'd had tears before. It meant some antibiotics, right? He was more concerned than I and transferred us North to Primary Children's because he wanted the more experienced team to place the pediatric chest tube he knew she'd need to drain the anticipated infection from saliva leaking into the mediastinem or pleural space just outside the esophagus, I might be messing up these terms. Basically you don't want a tear in your esophagus. Anyway, we were admitted, they started the IV abx, placed a chest tube, and instead of getting better she kept getting worse. It turned into one of the most traumatic hospital stays we've had by far. Turns out the chest tube team had not accounted for her prior surgeries that had caused the lung to adhere to the chest wall during healing, so her lung was punctured during placement, and no one caught the mistake for more than a day. By the time I begged them to take another x-ray because she was looking awful and struggling to breathe, there were 60ccs of air pressing on her heart. The infection kept us inpatient for almost a month, and we missed what would have been my last lengthy visit to see my grandma C, last before her stroke. Worse, I know that we almost lost Audrey that stay. All from a routine procedure that went just a little bit wrong.

That was also when we found out that Brayton, another EA child in another state's recent passing had come from a cascade of complications from an esophageal stent, and that revelation rocked us mere hours before a stent was suggested for Audrey's brittle, tight esophagus.  It led us to pause, to question, and ultimately to gently decline the suggested course of treatment in favor of seeking a second opinion in Boston. It was too much at once, and we were (correctly as we now understand) frightened that the stent could tear her open.  With as weak as she was, I honestly feel that that decision to refuse the stent, give her a little more time and seek the second opinion where we learned her esophagus was hanging together by a thread of scar tissue, might have saved her life.  In this world of medically fragile kids, so often there are little procedures and seemingly small decisions with big ramifications.  The decision to refuse a doctor's recommended course is not an easy thing to do, especially when you trust and admire them like I do Dr H.  But we have decided to cancel the local dilation and learn what Boston can see at her next scope.  

Earning her Ice Cream

9am: Waiting for the big test; she's still fasting and doing great. We had the option to wait another 90 minutes and get Versed to help with the trauma of this test, or muscle through getting the NG tube down her nose and strapped to a board for the high volume barium study without it. I described to Audrey the choices, without glossing, and suggested we could get ice cream after, and she shouted "ice cream!!" Here we go, brave girl.

12pm: Well, it hurt like the dickens, but she did the test without sedation. There is no sign of a leak. Off to get this girl some strawberry ice cream! (Right after she catches a few winks.)
So thankful for the wonderful staff at Primary Riverton for making a tough time easier, especially Steph with Child Life who worked hard to distract Audrey from the intense pain. Audrey thought the bubbles holder looked like a strawberry ice cream cone, so she asked very sweetly if she could have some after we finished.

As we were leaving the hospital, Audrey waved and called "Bye! See you tomorrow!" Clearly the toys and people had left an impression.
"No sweetie," I laughed. "We won't be back tomorrow."
"Oh. Next week then!"
"No, my love. These are very nice people but we won't be back next week."
"Why not?"
"Because your test went well."
"Oh. What do you mean?"
"The pictures of your throat look very good. The doctor doesn't need to see you for a while."
"But I like the doctors!"
"..."

Whole Framily

Yesterday was supposed to be Audrey's major surgery in Boston.

Remarkably, I'm still here at home, and instead she'll be tested this Tuesday at Primary to see how much her esophagus is leaking. She's had ups and downs this winter so far but is really doing great. All week I've been feeling an incredible sense of gratitude, a heightened awareness of the good things in my life, like when you've been hungry for a very long time and then sit down to enjoy a simple meal and suddenly it's the best thing you've ever tasted. My eyes are open to a million small joys. Eden working on a coloring book as Audrey directs her to use different colors and exclaims, "It's so beautiful! Brown hair just like mine!" JJ and Aaron giving me random hugs and obediently doing the chores that I ask, the laughter we share over silly inside jokes, their enjoyment of the food I make. Eden teaching Audrey steps from her ballroom dance class. The ambient sounds of the toddlers playing together. Their begging me to read them a story and then bringing me an armful of books. Gideon jumping up and down after leading singing at Family Home Evening and begging for us to sing yet another song, then directing us not just with his arms but with a whole-on vigorous dance to lead us in the music.

Last Saturday, after dinner Justin and I were still sitting at the table talking after everyone had left, and Audrey came and snuggled into my lap and hugged me. Then she wiggled over onto Justin's lap and hugged him. She reached out and touched my fuzzy sweatshirt thoughtfully. I smiled at her and said, "Do you like my soft shirt, Audrey?" She looked directly in my eyes and said, "I like YOU, Mommy. I LOVE you. I love my WHOLE FRAMILY."

Both Justin and I had tears in our eyes and couldn't speak for a few moments. This 3 year old child has an old soul. A beautiful, regal, powerful soul. We are so blessed.

Simple Gifts

This is what pure joy looks like. Right after Pink Blankie came out of the dryer last night. She went from tired and grumpy to singing "Blankie! Blankie!" over and over when I brought it out from the laundry room.

Certain Uncertainties

Just got off the phone with Michelle at Boston Children's. She left a message yesterday asking me to call her back, and for some reason I found it really hard to do so. I knew what she was calling about. Were we canceling surgery on the 23rd for sure? Justin is convinced it is the right choice for now, but I hesitated. I want so badly to be sure that we're not hurting her by waiting, not causing permanent harm to her lungs like Dr Manfredi feared last September when they first found the leak. I've learned to respect the worry in a doctor's eyes. I saw that look in Dr Miner's eyes during NICU rounds at PCH when he was the lone voice counseling against our then-team's plan of substituting a shortage of the usual TPN calcium by giving calcium citrate via peripheral IV back in May 2011, when tiny week-old Audrey was one of the 4 worst injured babies in the PCH NICU by the calcium fiasco. Her hand still bears a deep, ugly scar from the burn that ate away her flesh and took 3 months to heal. We should have listened to our guts and read the signs then, and gone against the easy course. Is this another one of those times; are we being foolish to cancel and think it's OK to wait? Or is Justin in fact protecting her from unnecessary surgery, potentially numerous followup dilations and another extended separation of our family?

So this morning I procrastinated calling Michelle back until she finally called me again. I apologized and tried to explain my hesitation, thanked her for her work to set up these appointments, and finally did cancel. I should feel elated and relieved. Right?

In November Audrey was eating by mouth extremely well. In December, whether due to a series of viruses or other reasons, she stopped eating by mouth almost entirely. Not for lack of wanting. Last week she was virtually 100% dependent on her g tube. And then, it shifted again and she has been steadily eating more and more by mouth until last night she blew me away by jumping up and down with excitement begging for cucumbers. I was very hesitant at first, and carefully gave her the smallest, thinnest slice to nibble. She had one or two brief "stuckies" where she coughed and gagged and worried us, and then amazed us by finishing it and begging for more, eventually eating not one, but 10 slices.

Wow. This girl just refuses to be defined.