June Bugs

No sooner had school ended, Audrey and the rest of us came down with one of the worst super bugs-- a nasty strain of rhinitis, the common cold-- I have seen in my life.  Poor Audrey was struck down first.

Our next dilation was scheduled for the first week in June in Boston.  I took her to the pediatrician when she first was sick, to check to see if the infection was in her lungs and it was not.  Still, her post nasal drip cough was keeping her up at night horribly, to the point that Justin and I tried the Combivent out of desperation to see if it might give her some relief, and to our suprise (and some dismay) it seemed to help quite a bit.  The morning of our flight, we had spent all night comforting Audrey through her coughing and she had been on the Combivent for 24 hours, so I called the anesthesia team in Boston and we agreed it was time to reschedule.  In spite of my hesitation, it all worked out to be very much for the best, because we all were sick as dogs for many many days. 3 year old Gideon became so sick that he had me scared for a day or two, and the doctor sent him for x rays in addition to prescribing nebulizer treatments to get him through the worst of the infection.

At the pediatrician with three out of my five. Both Gideon and Eden tested low oxygen and needed nebulizer treatments. Audrey has two ear infections but her O2 saturation and temp were fine. She is pointing at them as if to say "what gives? I usually get all the special stuff at the doctor!"

That was the most athletic doctor appointment ever. Audrey had a full out melt down for the last forty minutes of our two hour appointment, trying to bite and pull my hair while I was trying to listen to the doctor's instructions.  She was exhausted beyond all reason.  This bug really whomps the energy out of you... and out of me!  I'm trying to figure out how I'm going to get Gideon over for his lung xray, Audrey to her nap, and essential errands done. But at least I'm not on an airplane!

Later that day while waiting for Gideon's x ray and helping him in the bathroom, I had a nightmarish experience of getting a thick lump of junk caught in my airway and could neither breathe in nor cough it out for several seconds.  It was absolutely terrifying.  After all the exhaustion of that day, I wound up taking myself to the doctor that evening too.  We all took it easy for the next couple of weeks.

JUNE 12:
This has been a rough couple weeks... I'm going on thirteen snotty days of coughing all night with this stupid viral sinus infection, but thankfully Audrey is better and the other kids almost too, and I think I'm finally nearly done myself, this one hit me hard. Thankful for Justin's help and patience through my fatigue, he fixed meals and helped with kids and house without complaint. We all have been feeling better enough that we went to play at an awesome park in Pleasant Grove today and enjoyed free pizza thanks to one of the special needs clubs, United Angels, and tonight I drove to Salt Lake and back to pick up a new charger chord for Audrey's feeding pump thanks to the generous mom on the special needs Utah Kids board who had an extra. Probably my favorite moment from the past week though was playing Apples to Apples with the kids Sunday night. We each had to play a card from our hand that was the best match for the word Adorable, and hope the judge picked ours as the best match. JJ hadn't won a point yet in the game but he had the luck to get a "choose your own item" card that round and put it down saying confidently, "my little sister." We all groaned loudly and laughed, because everyone knew there was no hope beating that.

Our "All Girls Club" of Broken Bones

We've always had strong bones in our family. Seriously, we go through a gallon of milk in one day at our house.  Never a broken bone in any of us, nope, not ever, our entire lives.  Well, Audrey broke that perfect record back in February, and it seems to have set a trend.  Her broken elbow from February healed beautifully and she has been playing along merrily this spring, but to our dismay our other daughter, 12 year old Eden, had an unlucky fall last week in the backyard and managed to break her left wrist.  The bone was twisted badly and the setting of it was enormously painful for her, but the doctor got it set and casted in the office, only to discover two weeks later at her followup x-ray that the bone had slipped out of alignment again and would need to be re-set under anesthesia.

At Eden's followup x ray, Audrey was fascinated to see the process from the outside for a change!

At least I have been able to be home continuously for a few weeks so I could help with this stuff, thanks to Dr Manfredi's willingness to let us try a low-key dilation in Utah between visits to Boston, making it possible for me to be part of my children's end of school year stuff.  Here was my entry for May 29:

MAY 29
Two wonderful days volunteering at the schools and enjoying the end of year stuff for my kids; today I was in charge of the muddy slooshy water station at the boys' field day for three hours and had a blast. In the morning the kids have just a couple hours of school for their final day, and Eden will perform in the elementary school dance festival for her seventh and final time! Going to be an emotional goodbye to the neighborhood school we've grown to love so much, and next year she'll go to a different school than her friends so that makes it a double whammy. Today after school she had an ice cream party with five girlfriends and I got some darling pictures of them being silly as only young girls know how! Then we raced over to her ortho appointment for her second followup x-ray on her arm and learned to our shock and dismay that her arm bone has re broken, and she will require minor surgery on it tomorrow under anesthesia. They tried to schedule it for the morning and I begged them to wait till afternoon so she wouldn't miss her final hurrah at elementary school; just means she'll have to keep fasting all morning till we report at the hospital tomorrow afternoon. There is some strange irony in the fact that I was supposed to be taking my other daughter on a plane tomorrow for surgery of her own in Boston, but insisted that I needed to stay here to attend Eden's last day instead. Very glad I will be here for this even bigger day than expected, man, but crazy that I'll be going to the hospital after all. If you see Eden please give her an extra hug. She is very frightened and I'm so glad there are so many nice things to distract her.
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I sweet talked them into letting me take her back into the operating room, it is helping her feel much less scared about going under general anesthesia. She has asked so many questions and talked so much to the doctors and nurses I think we threw of their schedule a wee bit, but she needed to understand very clearly every aspect of what was going to happen to her.

MAY 30:
Eden is out of surgery, no pins or incisions were needed to put the bones back in alignment. (Yay!!) She has been talking to me nonstop about the sensation of going under and coming out of anesthesia. One of the first things out of her mouth was "poor Audrey! I would go crazy if I had to do that over and over again."

AND THEN....
Audrey broke her own wrist, June 20, story coming up.  And I broke my finger in mid July.  We girls just wanna have fun.

 

Love Will Steer the Stars

Yesterday rocked. 12-year-old Eden gave her first talk at church, and did an awesome job. She had written the whole thing herself, spending eight hours to write a beautiful eight minute talk about covenants; the great victory in this is that she finished it mostly without nagging, while battling her huge struggles with ADD, so maybe two thirds of that time was spent staring into space. She also overcame intense shyness and spoke up even though it was her first time at the pulpit in front of the entire adult congregation. We are so proud of her. The babies also made a mark in our family history book in another way. On Saturday I'd brought Gideon a cute pair of bright red Angry Birds crocs. He loved them so much that he wanted to wear them to church, with his little formal black suit, to which we said no. Justin and I were busy getting the final things ready, turned to leave and couldn't find the babies. Anywhere. We were getting very worried but since Eden had to be on time to give her talk Justin sent me ahead with the older kids and kept looking for the little ones. I walked the two blocks to our chapel only to find Gideon and Audrey sitting down prim and proper in the front pew. That funny clever 3 year old had figured out how to get away with wearing the angry bird shoes to church! I can just see him grabbing Audrey's hand and saying "come on Audrey, let's go church" and toddling on their merry way, alone the two blocks and crossing the parking lot. Thank heaven they were safe! Thank you to our young friend Andrea S. for noticing they were alone and going to sit with them until we arrived! We were so relieved that I even forgot to correct him; so I guess you'd call that a clean getaway. Then in the evening we had an impromptu visit from Justin's sister and her family who live an hour away; we visited the cemetery together to pay our respects to his grandfather and to our friend Mae Roberts who had devoted her life to helping raise funds through collecting people's discarded soda cans to recycle for Primary Children's Medical Center, with her bent back and sore neck she pulled her wagon ten miles a week and in that way raised over a thousand dollars a year for the hospital, on top of this she also walked or hitchhiked to worship at the temple every morning before it was light; she passed away just before Audrey was born and our own journey with Primary began. This was one of my first times joining Carmen and Jared for their annual tradition to visit graves, and it was pretty moving to see how busy the cemetery was, literally packed with happy visitors putting flowers on graves and remembering their loved ones. The atmosphere was like a big celebration of life and gratitude for our ancestors--I saw one woman's grave from the early 1900s buried next to her seven babies, all died the year they were born except one who lived one year. It's remarkable how the depth and breadth of the stories contained in a cemetery can cause you to re-center on the most important things, simultaneously reflecting on the past, being more intensely grateful for blessings in the present, and planning for the future. We had so much fun afterward eating homemade scones with Justin's sister and her family that I forgot all about looking at the night sky after sunset to see the dawning of the Age of Aquarius (in case you missed it too, Jupiter Mars and Venus supposedly formed a bright triangle in the sky just after sunset). Crud. Maybe the planets could arrange a bonus performance tonight? But either way, I guess even the planets agreed that yesterday was pretty epic.

May 23 Dilation in Utah

Waiting for our turn to go back to the Operating Room!

Audrey's dilation procedure at Primary Children's Hospital went smoothly. It was crazy how relatively low key things were. Having it done in Interventional Radiology instead of Surgery meant she was A) put under semi-conscious sedation instead of general anesthesia, so no breathing tube was placed, so our level of monitoring was vastly less than we're used to, it was kind like going from Major League to Junior Varsity; B) a nurse walked in when Audrey was still wide awake and said "I'm going to just place a little IV now." and I sort of panicked and had to explain to her that Audrey is a tough stick due to her hundreds of past IVs scarring the veins and that it is usually done under sedation; C) they gave her Versed as a spray in both nostrils at once and marveled saying most kids cry when they do that; D) once "under the influence" the nurse placed her IV on the FIRST TRY while Audrey complained a bit but never cried, I was so proud of her; E) while we waited Audrey was pretty sweet, she loved having me read her the Disney Princess storybook and kept pointing at things in the pictures, she was so excited to point out the "hohssies" and "fishies" on the pages, and then on the cover she got really excited and pointed at it saying "I!!" and when I didn't react she insisted again, "I, Mommy!!!" and I suddenly realized she was pointing right at the letter I in PRINCESS. How did she know that? And while on drugs no less? Huh. I guess PBS Kids might be worth its salt. So in their honor I suppose F) is for the fabulous Dr Feola. We talked about what had been done in Boston and when he came back to talk to me after the procedure he said they had obviously done a great job, she was wide open and it looked beautiful in there. He said it was still open to 9mm before he started, he measured to be certain, and he only opened her to 12mm because we had agreed he probably shouldn't try to go past where Dr Manfredi had taken her three weeks ago for the risk of tearing, but he said it opened up very easily. This makes it a bit of a mystery why she had been getting food stuck so often the past week; I don't know if it was simply those three millimeters made such a difference, or something about her not chewing enough, but regardless I was excited to let her have whatever she wants to eat again. 
 

Soon after waking up, Audrey asked for cookies. the doctor allowed her to have a popsicle, and she insisted on holding it herself.  She said firmly, "I do it Mommy!"

Ready for discharge!

We were discharged just three hours after arriving at the hospital, and she felt so well that I ended up driving the carpool after school and taking the carpool kids on a play date to see the baby ducks. 

At home Audrey ate a popsicle and positively REVELED in her slice of watermelon!

She was enjoying it so much that I was surprised when she regurgitated twice while eating the watermelon. Maybe she wasn't chewing enough, I don't know. We are hoping for improvement with the swallowing in the next couple of days, but overall it's been unexpectedly great news and a good experience with our "Junior Varsity" dilation!

Birthday Cake and Story Time on the Night We Returned from Boston

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!

A is for Apple

This is why I said we may need to lock up the apples! We have a sneaky little blonde mouse who is determined to get herself an apple, even though the skins get stuck. She took a nibble out of every apple in the bowl! After she had regurgitated a throatfull of pieces earlier, Justin found her with a mouthful of apple skins a second time, this time carefully chewing and swallowing the juice but not the skins. She turned on the charm, smiled and put the skins from her mouth into his hand.

There's virtually no keeping them from her; it's adorable how she gets so excited about apples and will do whatever it takes to get one.  She can reach up onto the table from the floor, and climb on chairs and reach things from there. We have given her little bits of peeled apple and sometimes she accepts that and does a good job chewing, other times even that is too much for her esophagus either because it is too tight or because she gets impatient and doesn't chew enough, and of course we juice a lot and make smoothies, but the bottom line for her is it isn't good enough. She wants an APPLE, you know? A whole apple. She just craves that satisfaction of chomping into a great big apple.