Back in Boston, May 2-5 -- UpSWING

Fun in Boston on Saturday

We just got back from our fourth boomerang trip from Utah to Boston (out n' back, out n' back) since coming home in February after seven months of living there.  Audrey strictured only half of her width in the past three weeks-- from 12mm to 6mm -- versus three quarters like the time before.  So.... it's not that great, but it's not that bad either. Dr Manfredi said he is still very optimistic about kicking this suckah without major surgery. Ok well, not those exact words, but you get the gist!   Our trip to Boston this weekend was pretty quiet; we met yet another sweet EA family (Heather and Elliott!), and we were very excited to learn that baby Amos and his parents Katie Ann and Aaron (our Amish friends) had finally gone home from the hospital after 18 months of setback after setback.  Their family is among our closest friends we've made through our time in this little club of the EA/TEF Long Gap families who seem to have all the fun, but we kept in touch mostly in person because they use only very minimal technology as part of their culture and faith, and do not allow photographs to be taken of them.  I will write more about them on another post as I continue to catch up on the blog.  I've been burning the midnight oil and putting the home reclamation projects on hold for the past couple of weeks, trying to fix the early blog posts that had all the photos of Audrey's NICU days-- there were hundreds of photos on there that got "eaten" by a change in the programming code, and had to be replaced.  It meant lots of bug-eyed greasyhead pajama days, but the up side was that I went through our NICU photos, a sweet and humbling experience in itself, and in adding them back I included many more of our favorite photos and captions I had written underneath them back then, from an album that was formerly reserved just for our closest friends and family.  There are now approximately 400 of our favorite personal photos of Audrey's first five months, when she lived in the hospital before coming home for the first time. Special thanks again to Tamara and Dana for your help putting together this blog and keeping it updated in those first stressful months, and to Steve for his help this week with some programming glitches.

This past Friday's dilation did yield some changes that were rather significant.  First, Audrey wants to show off the cute Sesame Street band aid on her thumb.  With her veins having been used so many times for IVs in the past, the IV team is having to get creative.  Ouch.  And that purple bruise on her cheek is her souvenir from bonking herself pretty hard on the side of her crib last week when she threw a tantrum at bedtime.  She was OK but it broke our hearts!  I've stopped her from bonking herself that way a couple of times before by putting my arm between her and the edge of the crib just in time, but this time Daddy was putting her to bed and he was unprepared for her fast little fit, and unfortunately she managed to get herself pretty hard.  Poor baby.  I guess the one good thing is, she hasn't done it since.

Here she is in the post-surgical recovery unit at the hospital on Friday

Audrey's prescription of compounded Prevacid, which despite how great it works has given us a lot of headache over the past eighteen months because of how difficult and expensive it is to have filled, is also easy to forget when we leave home because it has to be refrigerated.  I've gotten into a pretty streamlined rhythm about traveling with her and am usually very prepared, but this trip I managed to forget the Prevacid in the fridge on the way out the door.  Dr. Manfredi saved the day by writing us a prescription for something I didn't know existed-- a dissolving Prevacid "solu-tab" tablet that can be worked into a drink or soft food.  Sometimes it's the little things that have great big effects, you know?  I've trekked half the day across town on public transit and foot many times, in all kinds of weather, driven hours to reach special pharmacies, been told it couldn't be filled for numerous reasons, had my first-ever panic attack last fall over trying to make it to the special pharmacy before they closed on a Friday, all over trying to get this compound medication filled.  Inconvenient doesn't begin to describe our general experience with trying to get compounded Prevacid to administer through her G tube.  And now, solu-tabs and improvements in her ability to take drinks by mouth might mean I never have to do it again.

One morning a couple of weeks ago, I came in to find Audrey's G-tube wrapped around her neck three times from rolling over in her sleep.  She was perfectly fine, but it scared me.  I related this story to Dr. Manfredi on Friday and he agreed that it is time to make a change.  He suggested that we try discontinuing the night feeds, and see if we can get her to take enough by mouth during the day to replace her nutrition.  This is a huge step in the right direction, and at least for the first week or two following a dilation, I'm hoping we can be very successful!  Already she has blown me away with her improvement in chewing, though she's still sometimes too impatient and gets herself backed up. But for example, in the Boston airport on Sunday she ravenously ate some big bites straight from my pesto chicken sandwich at lunch, and then later during our dinnertime layover in Denver she ate several big bites off my slice of Hawaiian pizza.  I still mince her grapes and break each macaroni noodle into at least five pieces, but all of this is major and exciting progress.

Audrey loves this photo.  It shows one of her favorite things about visiting the hospital, the yellow socks with rubber treads they have her wear.  We call them "Ducky feet!"

Her oral aversion is virtually nonexistent most of the time these days, with an occasional exception that can be pretty funny.  Last week we took her to an elementary school carnival and though at first she acted very interested in trying her brother's cotton candy, when he gave her a clump she outright refused to put it in her mouth.  You could see on her face that the texture was just plain wrong, and it made me wonder whether this was a normal toddler quirk or if it reminded her of having actual cotton pushed into her mouth in the hospital.  I'm also still having trouble getting her to consistently drink much of anything but water, which puts my smoothie diet plans frustratingly on the back burner. 

I love travel, and I love Boston.  But I have to admit, the flying 7 hours each way every two and a half weeks for four days away from home, then either catching the "T" train for forty minutes with a suitcase plus stroller (in Boston) or (in Utah) driving for 90 minutes to reach my destination, is getting a little old.  So my heart leaped a bit when I first realized that the surgery date they have us scheduled for next, May 31st, is actually four weeks after this past Friday's dilation instead of three weeks like we've done the past four times.  But then my heart sank again, as I realized there is probably no way we can make it that long without her stricture clamping down enough that we have to refuse giving her solid foods.  While refusing a child food might work in some cases, in her two-year-old world that spells major strife (and a whole lot of noise).  I suggested, and Dr. Manfredi agreed, that we do a simple balloon dilation in two weeks in Salt Lake City, then travel to Boston as scheduled for the complex procedure with the needle knife and injections. This will effectively make the time between dilations two weeks, but give her more time to heal between the cutting and injections and more time between travel weekends.  He agreed that was a workable plan, so we are kind of excited about it.

Sacked out sweetly on the plane ride home.  This was her first trip having a ticketed seat all to herself, and she rode like a champ.

3-22-13 BEFORE

That reminds me, I'm not sure if I've ever thoroughly explained what exactly Dr Manfredi is doing inside her esophagus every three weeks.  I actually have a photograph that shows the before and after images of her stricture, that hopefully will help explain things.  Her stricture is basically a narrow circle-shaped scar down inside her food pipe that grows very quickly; it grows closer together and then needs to be stretched back open.  A simple balloon dilation works a lot like an angioplasty someone might have done to relieve a heart attack; with Audrey under general anesthesia, doctors insert a collapsed balloon down her food pipe into the narrow area, then inflate it to the desired size to push on the walls of the scar and force it to go more open to better match the rest of the food pipe.  When they push on that scar tissue, very tiny tears occur that are normal and necessary for the scar to open up, but if they try to push it too wide after it has grown too narrow, deeper tears can form which can be lead to infection, sometimes even creating a hole (called a perforation) in the wall.  When a perforation occurs, spit and food can leak into the body cavity around the food pipe, leading to dangerous infections such as the one she had last June.  In order to avoid deep tears and perforations, the doctor uses a stepped approach and only inflates a little each time, spacing the time between dilations closely enough that hopefully we are gaining ground with each one. The needle knife is used to make very shallow cuts into the circular wall of the scar tissue that then open up in a V-shape to help the stricture relax and open up more easily when the balloon is inflated.  It's kind of ingenious.

 

These "After" images from March 22nd show the V-shaped areas where Dr. Manfredi released the tension in the ring of scar tissue that forms her esophageal stricture, by making shallow incisions with the needle knife.  In addition to the needle knife incisions and balloon dilation, he also injects some scar-reducing medication into the stricture.  He usually injects steroids, but twice in the past he has injected tiny amounts of Mytomycin, a chemotherapy drug that has worked in the stubborn esophageal strictures of other patients.  On our next visit Audrey will receive another injection of Mytomycin.

 As I explained at the start of this post, at her narrowest point she was dilated to 12mm on Friday, having strictured down to just 6mm in the previous three weeks.  Her stricture used to be long and complicated, taking up a lot of the length of the esophagus, but ever since her stent therapy over Thanksgiving week we only have one tiny spot that is still giving her trouble, the actual point where the two ends of the esophagus were reconnected during August's resection surgery.  Our dream is to have it open to at least 15mm and stay open between dilations. 

All toddlers are fascinated by tiny things and naturally want to put them into their mouths.   Back last summer and fall we used to have to be constantly worried and vigilant about little items on the floor; as a not-able-to-eat-chunks crawler Audrey was especially prone (and quick!) to putting little things in her mouth and swallowing them and getting them stuck.  We still worry about things like that, but she's been getting so much better about understanding what is food that I was surprised and dismayed to find her sucking on these.  I bet these buttons are about 10mm, just right for getting stuck!

Ok, that's enough ick.  Here are a couple of fun pics I snapped of our visit to the Jamaica Plain "Wake Up the Earth" spring festival on Saturday.  Audrey had a grand time banging on buckets at the junk band area, and playing with the chalk pieces at the drawing wall.  When she'd had enough, we went home for her nap time and I, you guessed it, entered more NICU photos into the 2011 posts.

Soon after takeoff in Boston on our flight home, I took this photo of the harbor from the air.  I wish I could have taken it sooner, when we were over the ocean, but they ask passengers to keep our electronics off during that time!  The magical sights we get to see from the air are one of the things I've loved about this chapter of our adventure.

 

When we arrived home on Sunday, to my surprise we found a clean house, delicious homemade strawberry shortcake, and this sign on the refrigerator waiting for us.  Team Audrey for the win!