Amy and Aliyah

Looking back from a future date,

Some friendships seem meant to be.  One day in June 2012 at Primary Children's Medical Center, I was struggling with the stress and anxiety of going against the grain of our team's recommendation for the stent and moving forward with plans to seek a second opinion from the Esophageal Atresia Center at Boston Children's Hospital. I went to the Parent Center to use the computer and chatted with the kind woman at the desk, Cindy.  I'd gotten to know Cindy a little from our long stay there the previous year and many visits since, and so I opened up to her about our difficult decision and how nervous I was, and her eyes got big and she said, "there's another family that just did the same thing. They're in Boston right now, and you should talk to them, they are amazing."  She was right.


Once Cindy passed on my info to her, Amy contacted me quickly, and our conversation allayed so many of my worries about the logistics and details of that scary leap.  We managed to meet them in Boston on our first trip there, and they were a huge help as I worked to figure out the T rail system, hospital and pharmacy for the first time.  But more than that, Amy and her daughter Aliyah have become very dear friends.  We wish we lived closer to them, but just being in the same state is such a comfort, and that added up with all the other things about them makes them feel practically like family. We see each other several times a year at activities for special needs families, we try to stop and see them on our trips to see family out West, and sometimes we've been lucky, unlucky, whatever, to be inpatient at the same time in the same place.  Those times are my favorite, because the hugs and convos between friends who are both in crisis fighting for their children's health are incredibly healing.  Amy is so full of love and faith, it's a joy to be with her. Plus both of these chicas are just loads of fun.  Wherever they are, there's bound to be some sort of shenanigans.

Here are a couple of photos I snapped of them and the kids when we stopped at a fast food place near their house while driving home from visiting our West Coast family in 2014.  Amy and Aliyah surprised Audrey and Gideon with these cute turtle stuffed animals, who were quickly dubbed Boston and Austin by the kids, and those turtles have been among their favorite "fuzzy friends" ever since.

Amy is such a doll.  From my journal that day:  Oct 1, 2014: Met up with Amy and Aliyah for a quick stop on our way home from Oregon late Sunday night. Aliyah surprised Audrey and Gideon with these cute toy turtles, and they have played with them non stop ever since! Whenever we leave the house Gideon puts his turtle in his backpack, and asks if he can bring him in if it's a small place we're going, and asks if he can leave him in the car of its a big place we're going where the turtle might get lost. Audrey and he play with them all day and sleep with them at night. Thank you for being such sweet friends!

Aliyah, Audrey and Amy playing at Esplanade park in Boston, September 18 2012.  Does it look familiar? It was wonderful Amy who took the photo of Audrey and I on that happy day, the anniversary of Audrey's NICU graduation, the photo that's at the top of every page in this blog.

Oct 6, 2014 Audrey pretending she has turtle babies in her tummy.

March 27, 2015: While on a family trip to Zion National Park, Audrey and Gideon dictated this letter to Eden for their special stuffed animal turtles back home. They signed it from themselves and Cookie, his Christmas stocking kitty he currently takes everywhere. They said Cookie missed Austin and Boston, so they did this to help Cookie feel better. I love this little glimpse into their view of our time at Zion National Park.  The letter reads: Dear Austin and Boston, We are at Cliff Rose.  There are lots of pretty flowers everywhere.  It's JJ's birthday, and we had lots of cake and ice cream.  We played in the sandbox, went hiking all the way up a mountain, played with a yellow frisbee, and we watched Big Hero 6 in the night, and ate hot dogs and s'mores.  We had lots of fun.  from Gideon, Audrey and Cookie

Aliyah, Audrey and Amy Playing at Esplanade park in Boston Sep 18, 2012

Giving those toys was a simple spontaneous thing they did, but it meant so much to the kids. This kind of thing is what Amy and Aliyah do routinely, just spoiling the people around them and spreading kindness and love everywhere. Aliyah is a jewel.  Her smile lights up a room, and the children flock to her because she's so much fun to play with. She shines with a light that comes from the naturally lovely person she is, the exhausting battle she's fought, her faith in her Father in Heaven, and the love of her family. Amy also has a deep faith in and love for the Lord, and she shares that too in a way that is beautiful and comforting and welcoming and accepting of all beliefs.  She's so much fun to be with, and she's also a tough-as-nails mama bear when the situation warrants; her knack for street smarts has been a big help on many occasions, especially when advocating for her children.  And with a heart like hers it often seems that every child is one of her children.

Aliyah's medical journey is overwhelming to describe. This lovely teen has been through a long physical war, and her challenges have not become less through the years.  Amy summarized and wrote down her story at my request, and I know it took a lot out of her.  Thank you Amy, for sharing her journey.  Aliyah, we think about you all the time and are sending you so much love and support. I'm so impressed with the powerful young woman you are becoming, and all the ways both of you change the world for the better.  It's a privilege to be your comrades in arms, ladies.

Aliyah's Story, by her mother Amy

Here is our Angel. I have never really written her story before so get ready for a novel.

Aliyah was born March 9th 2001 She weighed 3lb 10oz.

She has the V CTE of the VACTERL Association

Audrey and Aliyah
cruising in the halls
of Boston Children's
while the mommies talk,
September 2012

(V) = vertebral abnormalities

(A) = anal atresia

(C) = cardiac (heart) defects

(T) = tracheal anomalies including tracheoesophageal fistula

(E) = esophageal atresia

(R) = renal (kidney) and radial abnormalities

(L) = (other) limb abnormalities

She tested positive for Methamphetamine when she was born and was then taken from her birth mother. As far as I know she was born at 31 weeks and had type C. She was born in Ogden but was taken to PCMC within hours and had surgery to fix the EA/TEF.  Birth mom was arrested, so this sweet baby only had the love and care of the kind nurses in the NICU. She was in the NICU for almost 4 weeks then went to an emergency shelter home for about 3 days. We found out about her and her 2 year old sister when we were asked to be their foster parents. She was about 5lb when we got her. Aliyah came to us with an apnea monitor on. We were told by the state and her doctors that she would outgrow all her health problems by the time she was one.

We adopted both girls when Aliyah was about 18 months old. She was diagnosed not long after that with failure to thrive. She also got RSV, Pneumonia, and influenza all at the same time. That is when they told us she had IGA deficiency. She has been life flighted and went septic on us. She was always sick and we spent a lot of time in the hospital. She had a NG or NJ tube for many years until 2006 when they did a Nissen on her because she was refluxing and aspirating so much. PH probe showed she had acid up to top of her throat 138 times in 24 hours and 18% of the time it was going in her lungs.

After the Nissen they put a G tube in which has been changed to a GJ tube. Doing the Nissen seemed to set off a different set of problems. She could not swallow her own saliva and would choke and aspirate that for hours and hours each night. She then had 15 surgeries in 18 months trying to resolve these problems. They dilated and redid the Nissen to loosen it but nothing helped, they took it down part way and did Bronchoalveolar lavages to see how much she was aspirating into her lungs. Then they took down the Nissen most of the way. It was either have her aspirate stomach acid or aspirate saliva and anything she tried to eat or drink.

Beautiful Aliyah cheering up Audrey
at Salt Lake Comic Con
April 17, 2014
Seeing her and Amy was the
highlight of the day.

At one point they told us to never let her eat or drink by mouth again because the stuff she could get down her esophagus would go into her lungs and the stuff that wouldn't go into her lungs wouldn't go down her esophagus either. We said, how do you sit at a table as a family and tell one of your children they cannot eat? How do not let them have their birthday cake even if it is only a couple bites? Every holiday, event, church activity, school activity involves food. It was then we decided to let her eat what she wants even if it is just a nibble. Within reason, of course she can't eat a big juicy steak. We learned to tell people if you can and would feed it to a 1 year old you should be OK giving it to Aliyah as a general rule of thumb.

In March 2012 I found out on Facebook about the EA team in Boston.  We did some fundraisers and went out there to see them in June. In Boston Aliyah met her very first EA/TEF child. Finally someone like her. She met many more in the year we were going out there for treatment. They found she had severe tracheomalacia still, her aorta was collapsing her trachea with every heartbeat so they did an aortapexy. Then a few months later they took her Nissen all the way down and found and fixed a Paraesophageal Hernia.

They also did manometry testing while in Boston and found she has zero motility in her esophagus, her stomach is paralyzed and she has little to no motility in her intestinal tract. The doctors in Boston have said they can't make a body have motility so there is nothing they can do to help Aliyah. She just had an Appendicocecostomy surgery done in Oct 2013 where we insert a cath into a hole in her abdomen and flush her system and bowels out each night, and that worked well until recently.  

In November 2014 Aliyah had a Tracheopexy done where they tied her trachea to her spine and they fixed a hole she had at the bottom of her trachea. Her airway has been much better since then. However when she had a high resolution CT scan last year the found another hole so we went back to Boston in May 2016 and had them look at it.  We have opted not to do anything about it at this time. 

Special hugs at the BCH Yawkey Family Inn 2012

They also found in Boston that she still has some Bronchiomalacia. The doctor said "I wish I would have fixed that two years ago but I didn't do it back then."  He also said her lungs are filling with scar tissue, nodules and inflammation because she is aspirating so often.  He told us to have her sleep sitting straight up but when we try that she drops her head forward and cuts off her airway. 

For the past few months Aliyah has really been struggling with tolerating her feeds as well as her flushes not working well.  She is in so much pain with the flushes and feeds that we are not sure what to do. The doctors are not sure what to do either; they believe it has to do with her motility issues and like I said above there isn't much that can be done about the lack of motility.

Despite all these challenges Aliyah is a bright, happy girl who is blessing to each person that knows her. She loves to bake for whoever she can, although she doesn't eat any of it. She loves to sing and was in her first talent show in July 2013 and in her first community play Dec 2013. Music is a big part of her life and brings her much happiness. She loves taking singing lessons and her teacher is amazing working with her and her airway issues.  Aliyah has a very big heart and has raised some money by baking and selling cupcakes for a family whose father had terminal brain cancer. It has not been easy to watch her face so many challenges and suffer so much. At times she will say "Mom I just wish I was normal." I told her everyone's normal is different. God has a special plan for this strong young woman. She has defied all the odds that were stacked against her from day one. If this girl doesn't give you hope I don't know what will. I am honored to be her mother and have learned that through great trials come greater blessings.

We've loved you from the first day we met, Aliyah!
Cheering you on, sending you strength, praying for a cure.

June 25

After waiting all day on pins and needles, finally got the call from Boston Children's about Baby Audrey's case. Dr Manfredi (the Gastroenterologist) feels it is worth a try to give her the steroid and needle knife treatments. He said it would be ideal to have her out there for two months, after her leak is declared completely healed. Nervous but hopeful this may be the turning point for Audrey. Debating whether to do it asap or wait until school starts... *sigh* as Marty McFly would say, this is heavy!

D-Day!!

D-Day!!  We are being discharged today after nearly three weeks in the hospital.  Monday's esophagram showed a definite improvement; her leak is not entirely closed but it is at least halfway there.  We are back on continuous pump feed, but we managed to ditch the TPN a couple of days ago so that she is 100% back on formula for her nutrition.  Still nothing allowed by mouth, and we need to keep her at continuous feeds (a slow steady trickle of milk into her belly) to minimize reflux so that they can keep the leak site clean.  We'll have another esophagram in two weeks, and a visit with our surgeon next week for follow up.  The biggest remaining hurdle is the lingering inflammation in her chest, which they keep tabs on with blood tests, but it is coming down steadily.  We are still on two heavy duty antibiotics, Meropenum and Diflucan.  The Diflucan can go through her G-tube, but the Meropenum is IV only, so they started to prescribe us Clindamycin so that we could ditch the picc line.  Unfortunately we had a terrible experience with that drug in February (and based on the research I did then, I personally feel it is a very dangerous medication) so I told them I would be willing to go home with the picc if necessary, and in the end they felt that was best.  So I am being trained in how to take care of this line that goes straight to her heart-- sterile methods of care and administering medication.  Did I mention I have four other kids.... no pressure, right?  But if that's what it takes to get us home, bring it! 

Thank you to so many for all of your kind words and visits and help, it has made this-- as much as possible--a wonderful stay.  What a great learning experience, and I've had the opportunity to do and see so many things that I never did while we were in the NICU.   I've visited the Parent Resource Center almost every day for free food and great conversation, met the cute "pet therapy" dog twice, had a sweet visit from music therapy,

attended the Take 20 4 U  twenty minute breathing and relaxation class offered once a week (that always was advertised in the pumping room and during that frenetic time last year those signs made me think, yeah right!), visited the busy behind-the-scenes hospital mail room, and I even made it over once to work out at the JCC gym.  Several sweet visits from friends, two wonderful weekends taking my kids on field trips while my husband stayed with Audrey, lots of playtime with Audrey, wagon rides to the patio, a stranger made her day by giving her a balloon.... so many things.  I still wish we were in Oregon right now visiting family and friends like we were supposed to be, but all in all, we're not doing too shabby.  The big suspense right now is wondering what we will learn from Boston. Will she be a candidate for these outpatient surgeries?  I feel like a little kid waiting for their call-- butterfly city, man!

Now we are waiting for Daddy to come take us home, and Audrey is getting a last nap in.  But not before she discovered that she can reach some cool stuff from her crib.  

....Yep, we're ready.  :-)

Picnic Time!

Wednesday, June 20
 In the NICU there was a superstition about the wonderful four-letter word that starts with "h" and ends with "ome"; it seemed like any time there was serious talk about a baby getting to go there, something new would pack a couple more weeks onto their sentence. So, instead they would talk about going on a "picnic." I am thrilled to announce that Audrey and I have a picnic scheduled for THURSDAY!!!!  I spent many hours yesterday getting her medical images from this year (from only this hospital, not even both places we've been treated) put onto disks-- it took eight of them--and overnighting the package to Boston Children's for review by their physicians.  I am anxious to find out whether she is a candidate for the needle knife procedure that could remove the scar blocking her throat endoscopically, meaning they could do it all from tools inside her throat, no major surgery.  It is an outpatient procedure and is performed by a Gastroenterologist, but it is not yet available here.

Hope is the thing with feathers
That perches in the soul,
And sings the tune–without the words,
And never stops at all,


And sweetest in the gale is heard;
And sore must be the storm
That could abash the little bird
That kept so many warm.


I’ve heard it in the chilliest land,
And on the strangest sea;
Yet, never, in extremity,
It asked a crumb of me.
-Emily Dickinson

Monday, June 18

Monday, June 18
Room service just came in asking what Audrey would like to eat. I was polite in explaining her situation and that a mistake must have been made in this morning's orders. I guess one of her cute caregivers must have thought this poor kid deserves a steak!! (So funny. But I want to let out a barbarian warcry!) This morning's test of her esophagus seemed to show big improvement, but you know it probably means another week stuck here till it closes up. She is fine in every other respect, but still not allowed to eat and her picc line nutrition is not a good thing to bring home to my busy household. We'll see what surgery team says. I am so, so frustrated. WE WANNA GO HOME!!

A teenager on the hospital patio gave Audrey this cute balloon, and she was delighted with it!  There is something so wonderful about watching a baby discover balloons for the first time-- they way you can bop it and it bounces back, the sound and the lift of it.  They really are marvelous.  I love the way children re-open our eyes to these things!  I heard the craziest thing last week-- that in 100 years, helium balloons may be a thing of the past because helium is not something that can be produced in a factory, and we are running out-- this crazy thought sounded like some kind of urban legend, but from what I am finding from news articles, it is all too real!  What a strange thought... I guess that makes this moment all the more precious!

One really funny thing I have to tell you: the staff here all wear a portable communication device around their neck, called a Vocera. They can ask it to page whoever they need and talk to them whenever. Well, we've been here six months all told and how did i JUST learn for the first time that a nurse can speak into the Vocera and say "Beam me up Scotty" and it will play goofy scifi sound effects? (Funniest is when it asks you back, "I think you told me to beam you up. Is this correct?")

4 p.m:
Wonderful news, we get to try her G-tube again tonight!!! Only ten ml per hour, so it's only a drop, but it's a place to start! I am soooo excited!!! They are really concerned about protecting her esophagus from food in order to allow that leak to finish healing, so we are starting with continuous feeds at a suuuuper slow rate, but we will increase it gradually over the next few days as she tolerates it; the day before this started she was at 240 ml per hour for half hour increments four times a day (basically like a regular baby drinking four 8-oz bottles a day), so it will take a while to get there again, but hoo to the ray, we are FINALLY approved for some kind of FOOD again!!

Thursday, June 14

Audrey had surgery today to relieve lingering pressure on her right lung, but the mass was too consolidated to extract--apparently that is good news though, and her body will slowly absorb it on its own. Getting to love our interventional radiologist, Dr Feola, what a great doc. He'll probably be doing her next dilation instead of Dr D, with a balloon instead of the Tucker dilators due to her recent leak. Still doing our homework about whether and when Boston is the best choice, but I am 70% there. Thank you again for all of your concern and support!

Wednesday, June 13

So happy to have spent a wonderful relaxing lunch and conversation with friends today. Baby girl also had a fun visit from "pet therapy" and got to pet a giant fluffy Bernese Shepherd! Also a wagon ride and fell to sleep playing with her cute soft owl, a gift from my wonderful nurse who was there when Audrey was born. Also loving the treats from other dear friends. What started out very stressful turned into a great day, thank you for so many kindnesses! Audrey will have another surgery on Thursday. They will sedate her and use an ultrasound to guide a needle to relieve some fluid still pressing on her right lung, that was not relieved by her chest tubes. We are down to just Tylenol for pain; I think today was the first day with no morphine!

Audrey is asking for a rest on my lap.  Peyton is asking for a rest on my shoe.  I feel loved.

Exhausted, Frustrated, But Improving

Chest tubes are out as of this morning!  That means a huge reduction in her pain.  I am told that a chest tube feels a lot like a butterknife stuck in your ribs.  She is finally able to play and crawl comfortably again without morphine!  We will need to be here at least one more week while they continue to monitor and treat an infection in her mediastinum, the space outside her esophagus.  (No trip to Oregon yet, boo!)  Still on TPN, no food allowed by stomach. When I need to eat, I leave the room.  The sight and smell is torture for her.  Waiting on a full second opinion from Boston Children's, and we're now to the point where we're strongly considering either a hospital transfer or a trip there for her care in the near future.

The visit from Justin and the kids was sooo great!

As we'd anticipated, surgery did recommend the stent.  Their plan was to place it, monitor it one week later, then remove it after four weeks.  Although Dr Rollins and Barnhart have used this same procedure on four patients successfully over the past three years, and are in the process of publishing a paper about it, we felt like the details of 3-yr-old Brayton's passing were too close to home, the timing of that story coming to us too much of a coincidence.  We declined the procedure.   Doing so may have extended our hospital stay, but from our viewpoint the risks just weren't worth it.  We did learn that BCH does use stents but only uses them for a 3-day period for healing a diverticulum.

 

She was also removed from droplet precaution today (which means she can go on wagon rides again, yay!) and we are scheduled for a chest CT scan to check for abscesses, which they suspect might be present based on yesterday's labs. Thank you for all the support and encouragement. Yesterday was exhausting.  The esophagram showed no real change in the diverticulum, and the surgeons here initially said they want to keep her as an inpatient until it heals over--- but that could be a month, it could be never-- so I think they'll just reevaluate things next Monday when they do their next esophagram.  That news was a blow.  I had really thought we were heading home in the next couple of days, once the chest tubes were out, but our usual surgeon Dr Downey helped us understand how serious the infection is in her throat, which is still being treated with picc line antibiotics.  Part of my heartbreak is that I will miss a special wedding out in Oregon next week that I've been anticipating for months.  

 

Time with our family together definitely cheered her up!

 We also had our first telephone consult with Boston Children's EA department, but their second opinion was incomplete yesterday because they did not have all of her data yet.  Still, our conversation was great and I was truly impressed.  We are carefully considering the logistics, pros and cons of going there while trying to maintain the great relationship we have with our surgeon here.  We greatly value his advice.  Our surgeon loves Audrey and has spent a LOT of time talking with us and doing all he can to help her; he is wonderful and very protective of her.  Essentially, he is not convinced that Boston Children's Esophageal Atresia Program has more to offer there than we have here, so we are working on doing all of our homework with cool heads and asking a lot of questions of both sides.  It is tough.   I am trying to sort out what is fact and what is opinion. Very stressful.  Boston claims to have the premiere treatment center for Esophageal Atresia, and first hand reports from dozens of families seem to corroborate that claim.  I even asked for any negative experiences, and out of dozens of comments the worst it got was that they might tend to be a little on the overoptimistic side.  If we stick with the plan here, our surgeon will continue a course of dilations as needed, but with a gentler method of using balloon dilators to keep her just open enough for saliva to get through.  In a few months or a year, I think he will consider a resection of her esophagus, but that's not on the radar yet.  I am worried that a resection could result in a loss of her esophagus, because I read a study that said that in a patient three years later after a tight repair, the esophagus was still so tight it was "like a guitar string."  I am frustrated to think of the months ahead, constantly denying her food when she is so excited for eating, of her tears and frustration as she chokes and throws up every time she manages to snatch a bite of something she wants.  Our surgeon gently called it a time for tough love.  But it is so heartbreaking.  I look at her and I see a passionate little  Rapunzel from the Disney movie Tangled, singing, "When will my life begin?"

 Two more happy things: she was totally calm for her CT scan a few minutes ago (great news: no abscess!!) because they let her keep her fuzzy pink blanket that she has been sucking on for comfort all week--we have already snuck it away to wash it three times, haha!-- and I got to wear a hot pink sparkly lead jacket--GROOVY--my inner "hot 80s girl" approved. Plus yesterday Justin and I went to go downstairs for lunch and a volunteer was playing the grand piano and let me hop in and sing with her.  THAT felt SO great, and I felt like I was rockin' dat place with my classical soprano shtick.  Eeeeeven if nobody stopped as if to say, "wow she's so good!".... more like, "oh my gosh who is that lady embarrassing herself at the piano, I can't watch!"  Hahahah!

 

Pieces of the Puzzle

After a wonderful visit from my dear friend Angie and her husband last night, plus a talk with one of our special nurses from NICU days, I should have been all set for a restful night!   You'd think, huh.

Woke up in the night with questions swirling in my mind and jotted them down for later.  Pieces of the puzzle that did not fit, such as...    If they took the chest fluid sample immediately from her cavity right after the chest tube was placed in the OR, it makes no sense that there would be e Coli and other nasty bacteria in it, if it was truly a "contained leak" separated from the chest cavity by a membrane.   The surgeons had blamed the bacteria on the practice of "flushing" the tube which is normal for treating heart patients-- whose blood in the chest cavity coagulates and clogs the tube-- but was not needed for draining Audrey's pleural fluid, so they discontinued that in the first couple of days, which I was glad for since it was painful for her.  But until this morning I hadn't realized the disconnect in the reasoning-- the flushing was not begun until after we left the OR, so the sample for culturing had already been taken.  But then, why would the esophagram show a contained leak if there was a free tear?  I know she was supine, laid out on the table and sedated.  I think about a hard, fibrous scar and the manner in which it would crack.  I wonder if it would be opened and closed by movement.  We should do her next esophagram conscious and sitting up, and "live view" instead of snapshot-style if possible.   (Note: Dr Downey later told me that there is transfer, or travelling of bacteria, between the mediastinum and the pleural space, so we can have a contained leak and still have a bacterial infection around her lungs.) I am in knots over this coming Monday's test.  If the leak is healed, we are on a path to home.  If not.... well, we'll have to see.  She had a kind of rough night, following yesterday's kind of rough afternoon, then another rougher afternoon today-- her pain is increasing and I'm not sure why.  Soreness, hunger, reflux? (Oh my goodness, reflux!  We haven't had reflux meds in a week!  Fixed that.)  Still pain.  So she just had her first morphine dose in days and now she's playing happy as a clam.  They discontinued her Toradol (intravenous ibuprofen) today because there is a limit of how many days they give it before they have concerns about kidneys and liver, so we now have Tylenol instead and I have to ask for it every time, and she always needs it right when it wears off.  It takes SO LONG to get it too!  Like an hour and a half a lot of times.  Poor baby!  When she cries and whimpers I can't think straight.   Awesome nurses tonight and in the morning are planning to get on top of the schedule so she is never without a pain med in her system-- they think it's just the increased movement that is causing her to be sore, chest tubes are supposedly super painful.  Thanks Angie for the awesome dinner last night, made for great leftovers for lunch--got me through the stress today!


Although her pain seems to be increasing, her numbers look good and her chest output has basically stopped-- then at 4am I suddenly had a horrible thought-- what if the chest tubes were clogged??  I got up to help comfort her and help the nurse until we got some pain meds on board, and told the nurse of my worries, and that I wanted to ask for a chest x ray to make sure she wasn't building up pressure again-- turns out it was already ordered for this morning!  Thank heaven!  I knew there was no way I could sleep, so got showered and dressed and then dozed until they were ready to take her down for the chest x ray.  Thankfully it showed continued improvement in lungs and both tube areas.  Other worries were relieved when I posed my questions about the bacteria to the (awesome) Resident, Katie, who basically confirmed my suspicion: yes, Audrey probably did have a free leak at some point (between the contents of her throat and her chest cavity), but it was probably very, very small and has obviously healed over because she is improving.


Spent most of today working out the logistics for my husband and four other kids to come up for a visit this weekend..... whew!  What a production!  The chaplain came to meet me this morning too-- I confess I've been running a bit low the past couple of days, and it was great to talk with her.  I told her the gist of our tale, and summarized my core beliefs, and facetiously asked her for some magic words for dealing my baby's mortality.  She told me that I already have the ideal outlook, beautiful beliefs, and the best attitude she's seen.  Darn it, haha!  We had a lovely prayer together and she left me with the scriptures I'd requested and a promise to stop by again.


Also talked to Boston today. They had not called me back yet and I was getting nervous. Apparently they do their chart reviews on Mondays and are in the OR W-F. I explained what is happening and they are putting our chart on the top of their stack (got the records transferred, check!) and the team will talk to us Monday! Same day as our esophagram. Ohhhh man, I am so nervous, and excited, and nervous, for Monday!


But for now time for a break.  Visits from two sweet friends tonight and then, just before bedtime, my children!!  YAY!!

Monday, Monday, Can't trust that day....

I added all of our updates for the week so far to the previous post, with lots of great photos from the past few days, but a friend told me she hadn't realized I was doing it that way, so I'm going to try doing updates on little posts-- I'm still not a pro blogger, so be patient with me!  I think when I started that post initially, I was hoping things were going to continue to settle down and it would be easy to keep it to a single post.  In theory, if I start making each update a new post, it will have the opposite effect and mean we have less to report, right?  We could be home in a flash!  Sounds good to me.

 One of our friends here at the hospital from last summer brought us some cheerful photos she'd printed off Audrey's blog for us to hang on her crib.  I put the one of Daddy where she could see it and she likes to smile at it.  Two other signs she's been feeling better:  trying to crawl out of her crib, and jealous of my food!

The surgical team came in a few minutes ago and got an onslaught of questions from me.  They are so great.  But my questions are hard.  It is apparently not a simple thing to know where that 60 ccs of air in her anterior chest cavity came from, for example.  They didn't even want to talk about the stent yet, probably because they had lots of other patients to round on and they knew that wasn't going to be a short discussion.  They did tell me that she will have her next esophagram on Monday, so a TON hinges on that day.  But yesterday evening Dr Rollins did come by and discuss the stent procedure with me.  They are thinking of placing the stent (at this point the plan is only if her diverticulum doesn't heal), which would be somewhat similar to a dilation except that they would put this flexible poly-coated titanium ring in there, then theoretically the scar tissue will form in a wider ring, and they will monitor it weekly and remove it at the end of four weeks.  I was so glad that he came to talk with me, because apparently Dr Downey had forwarded my email from Tuesday night to him.  Here is the email I wrote:

Hi Dr Downey,

Thank you so much for your call today!  Audrey is showing some improvement tonight and we were thrilled to see her smiling and reaching for toys.   She did test positive for rhinovirus which may explain the continued fever.  I am writing you because I was surprised to receive an email tonight that seemed very relevant to Audrey's current situation, and wanted to pass it along to you.  A friend of mine is the head of the EA/TEF parent group for Utah, and during a recent conversation I had casually asked her if she knew any more information about little Brayton, a three year old in northern California who recently passed away from complications of his EA/TEF treatment.  I had been following the family's story near the end of his life and was heartbroken when he passed.  Apparently my friend has contact with a good friend of the family, and received this information from them with more of the medical details.  I was surprised to learn that the specific circumstances that lead up to his death were complications from a stent.  We understand that this information is neither complete nor directly from the source and thought you had probably heard of the case and knew better what had actually happened.  We also understand that this is kind of a worst case scenario, but the implications were enough to spook us regarding the stent-- I think I understand now why you had formerly expressed reservations about this approach for Audrey.  Perhaps we could talk again about this in a few days, after Audrey is through her infection and we see where things are headed with this diverticulum.  I will also of course pose our questions to the team here should this become imminent.   Also got the ball rolling with Boston Children's earlier today, and they should be in touch within the week-- we would like to put their specialist directly in touch with you if that is all right, to facilitate the conversation; I will give their Doctor your office number unless you'd prefer something else.  I hope together we can find the best possible solution to this challenging situation.  Your compassionate care, as ever, means worlds to our family.  Thank you, and have a safe trip! 
ps: (See the text copied below)  No need to respond to this email, we can talk in a few days.  Thanks again.
Here is the pertinent section of the email. (I have edited it slightly from the original):
"Brayton was successfully connected, however he kept getting recurring strictures and had to go for weekly dilations 4 1/2 hours away from home. Mom could not keep doing this, so opted to have them put a stent in his esophagus to try to keep the esophagus open and strictures at bay. They sent him home, with weekly monitoring of the stent, but unfortunately the stent still ended up eroding his esophagus and part of his trachea. They ended up removing all of his esophagus and doing a spit fistula as his esophagus was completely destroyed, they then tried to repair the holes in the trachea, but with no success as it was now damaging the lungs as well, and he kept getting septic from the stent eroding in his body. They did ECMO which oxygenates the blood but is always very risky and could cause you to bleed out. Unfortunately Brayton did ultimately bleed out causing him to pass away."

Dr Downey wrote me back briefly expressing his sadness at this tale and that it reminds them to always be very thoughtful in any approach, which I'm sure Brayton's team was.  While welcoming of additional input from the surgeons at Boston, Dr D expressed his caution about both sides of a second opinion, both in the sense that "if you ask 5 surgeons, you'll get ten plans for success" and being wary of blanket statements or guarantees of success.  Each child is so different.  Nothing's to say that what worked for one, or did not, will happen the same for Audrey.  But even knowing this, my mind is reeling with questions about sweet little Brayton's treatment.  How long was the stent in place?  Were there other health issues contributing to his complications?  His mother also sought a second opinion from the Esophageal Treatment Center at Boston Children's, what did they say?  Even if they had gone to Boston, it may not have been the miracle solution.  But perhaps by learning from their experience, Brayton can help, or even save, Audrey.  That beautiful family was irreparably damaged by this experience, and my heart still goes to them in thought and prayer so often. The best way I can think to help them now, and to honor Brayton's memory, is to be strong and do all we can to help Audrey in her similar fight.  At the moment, Dr Rollins has me convinced that the stent has good potential to help Audrey-- they've had success in four previous patients over the past three years, one of which was a long gap situation similar to Audrey.  But that email made my blood run cold, and a little voice in my brain keeps saying that learning more from both Brayton and Boston could change everything.  I love what my friend Candi said, "I suppose the worst thing that could happen is they could say that our Utah Doctors know what they're doing, right?"  haha! That totally IS going to happen.  I just hope we get some fresh ideas going that could help her.

(Update: I just learned that Brayton's severe esophageal erosion happened within just a couple of weeks after the stent was placed, in spite of weekly monitoring by his local (less specialized) hospital.  Ugh.  Looks like we have some really tough decisions ahead.)

The other thing I've been reading nonstop about is jejunal interposition.  Apparently it is another one of the main surgeries used to replace an esophagus, but I hadn't really heard of it until I saw this chart showing the pros and cons of each approach.  Dr Downey prefers the gastric pullup as a fallback surgery if we are unable to save her esophagus--which he hasn't given up on yet--and I know he must have excellent reasons.  (Although the G.P. is a commonly used surgery in these babies, the thought of pulling her stomach up into her throat still makes me nervous and sad.  I need to learn more about it to combat those feelings.)  When I ran across that chart I was surprised to see that the list of  "cons" for each of the traditional surgeries was fairly lengthy while the list for the J.I. was tiny.  I was suspicious that it was simply an incomplete list and wanted to see for myself, but it gave me a glimmer of hope to see something new so I've been reading like a maniac.  If we were to lose Audrey's esophagus through all of this, I wonder if that would be our best next recourse.  Jejunal interposition sounds interesting because it
A - maintains peristalsis (progressive muscle action toward the stomach),
B - doesn't have the fast-growing problems of colon interposition, and
C - follows the natural path of the esophagus because the diameter of small intestine is small enough to fit in the natural spot behind the trachea, but unfortunately it is a very difficult surgery to perform and it appears to be only in the last few years that doctors have been successful with it.

I looked down at Audrey snuggling in my lap.  She's been fussier this afternoon, maybe hungry or sore from her increased movement yesterday, and she had a really hard time sleeping for her nap.  I sighed and gazed out the window for a moment, watching an airplane fly above the city skyline, and for a few seconds the sun flashed off the body of the plane, making it look for all the world like a silver bullet flying through the sky.  Could this surgery, with the likely accompanying plane ride to Boston, be our silver bullet?  Is there something else we can do to save her esophagus endoscopically?

Here is the study I'm currently reading.  http://www.biliaryatresia.org/reference/EA-Jejunaltranspostion.pdf  And here is the second email I sent Dr Downey:
Much agreed.  Thanks for your thoughts.  I am wary of that as well, although I also respect that one second voice on the matter could yield valuable ideas.  Ran across one website in particular last night that intrigued me regarding the potential of jejunal interposition vs. the other major approaches we have talked about for a fallback plan should it become necessary in the future, wanted to send that your way for your thoughts. Shows an interesting comparison and contrast of the different traditional approaches; I am curious whether you agree with the website's assessments.   There were some key advantages to using the jejunum, notably a maintained peristalsis.  Look forward to talking later.  Here is that site if you get a minute and want to check it out, it's very straightforward.  http://www.childrenshospital.org/clinicalservices/Site2807/mainpageS2807P23.html

Dr Downey responded by sending me the following (heavy medical language) info and a recommendation that I visit the PCMC library to read more.  I am hungry to learn, so this was great.  But definitely sobering.

1. J Pediatr Surg. 2009 Oct;44(10):1928-32.

Surgical outcomes of esophageal atresia without fistula for 24 years at a single 
institution.

Holland AJ, Ron O, Pierro A, Drake D, Curry JI, Kiely EM, Spitz L.

Department of Pediatric Surgery, Institute of Child Health and Great Ormond
Street Hospital for Children, University College London, London, UK.
andrewh3@chw.edu.au

PURPOSE: The aim of the study was to evaluate the surgical outcome of esophageal 
atresia (EA) without fistula for 24 years at a single tertiary center for
pediatric surgery.
METHODS: The study used a retrospective chart review of infants diagnosed with EA
without fistula between 1981 and 2005.
RESULTS: Of 33 patients with EA without fistula, 31 charts were available. Mean
birth weight was 2327 g (range, 905-3390 g), and 71% were male. Most common
associated anomalies were cardiac (n = 6; 19%) and renal (n = 5; 16%), followed
by vertebral (n = 4; 13%) and anorectal (n = 2; 7%). The median initial
esophageal gap was 5 vertebral bodies. Six had a primary repair, and 25 patients 
had esophageal replacement at a median age of 7 months. This involved gastric
transposition in 20 (1 followed failed jejunal interposition), colonic
interposition in 5, jejunal interposition in 1 (after a failed colonic), and
repair at another center in 1. With a median review of 9 years, 21 patients had
long-term sequelae with the need for multiple further surgical procedures
including an antireflux procedure in 5. One patient died.
CONCLUSIONS: Management of EA without fistula remains challenging. Most patients 
required staged treatment that included esophageal replacement. The frequency of 
late complications indicates the need for programmed long-term review.

PMID: 19853749  [PubMed - indexed for MEDLINE]


2. Semin Pediatr Surg. 2009 Feb;18(1):44-9.

The surgical approach to esophageal atresia repair and the management of long-gap
atresia: results of a survey.

Ron O, De Coppi P, Pierro A.

Department of Paediatric Surgery, Institute of Child Health & Great Ormond Street
Hospital, London, United Kingdom.

The optimal approach for esophageal atresia (OA) repair and technique used for
long-gap OA repair are controversial. There are few data comparing the outcomes
of the different approaches and techniques. We performed a survey of current
practice of 88 pediatric surgeons and asked experts to provide us with
definitions and rationales behind their management strategies. There were no
differences between UK and non-UK surgeons. Although the majority of pediatric
surgeons perform minimally invasive surgery (68%), only 16% have performed
thoracoscopic OA repair; however, 46% are planning to carry out thoracoscopic OA 
repair. Gastric interposition is the most preferred technique for long-gap OA
when primary anastomosis is not possible, with 94% of those surgeons who use the 
technique satisfied with it. Growth of the esophageal ends by traction is the
other major technique used, but only 76% of surgeons who use it are satisfied
with it. Most surgeons repair < or =2 patients with long-gap OAs per year.
Long-gap OA should be managed by a limited number of surgeons at each center.
Even among experts, there is little consensus on the definition of or the optimum
technique for repair of long-gap OA.

PMID: 19103422  [PubMed - indexed for MEDLINE]


3. Pediatr Surg Int. 2005 May;21(5):369-72. Epub 2005 Apr 13.

Ileal pedicle grafting for esophageal replacement in children.

Bax NM, Van Renterghem KM.

Department of Pediatric Surgery, Wilhelmina Children's Hospital, University
Medical Center, P.O. Box 85090, 3508AB Utrecht, The Netherlands. N.Bax@wkz.azu.nl

Reconstruction of the upper esophagus in small children remains a challenge. Free
jejunal interposition as frequently used in adults is much less appropriate in
children because of the limited vessel size. The use of a jejunal pedicle graft
in children has been described, but gaining enough length may be a problem. A
pedicle graft of terminal ileum may be a better option, but this technique has
never been described. We report a child with esophageal atresia and distal
fistula who had a very short upper esophageal pouch. Primary repair was
impossible. The fistula was ligated and a gastrostomy created. A second attempt
at anastomosis was not successful either, and a cervical esophagostomy was
created. The child was fed by gastrostomy and received sham feeding orally. When 
the child was 10 months old, the upper esophagus was successfully reconstructed
with a pedicle graft of terminal ileum. Postoperatively there was a limited leak 
of the proximal anastomosis, which healed spontaneously. The distal anastomosis
had to be dilated on a few occasions. With a follow-up of 1 year, the child is
eating well without gastrostomy supplementation. On imaging, the ileal pedicle
graft looks somewhat tortuous but contracts nicely. We feel that ileal pedicle
graft reconstruction of the esophagus should be part of the instrumentarium of
pediatric surgeons dealing with esophageal reconstruction.

PMID: 15827751  [PubMed - indexed for MEDLINE]


4. J Pediatr Surg. 1999 Nov;34(11):1630-2.

Reoperation after esophageal replacement in childhood.

Dunn JC, Fonkalsrud EW, Applebaum H, Shaw WW, Atkinson JB.

Division of Pediatric Surgery, UCLA School of Medicine and Department of Surgery,
Kaiser Permanante, Los Angeles, CA 90095, USA.

BACKGROUND: Esophageal replacement is associated with significant morbidity that 
may lead to operative interventions. This study reviews the management and
outcome of children who underwent reoperation after esophageal replacement.
METHODS: Eighteen patients who underwent esophageal replacement from 1985 to 1997
were reviewed retrospectively. Ten patients underwent reoperation. Patient
management, perioperative morbidity, and the dietary intake at follow-up were
recorded for each patient.
RESULTS: Of the reoperated patients, 7 had esophageal atresia, 2 had caustic
ingestion, and 1 had achalasia. Nine patients received a colon interposition, and
1 received a reverse gastric tube as the initial esophageal replacement. Seven
patients required revision of the anastomoses. Three patients required complex
esophageal reconstruction: 1 underwent gastric transposition, 1 underwent free
jejunal graft, and 1 underwent gastric transposition combined with free jejunal
graft. Seven patients were eating well at follow-up. Two patients still required 
partial gastrostomy tube feeding. One patient died 6 months postoperatively from 
aspiration pneumonia.
CONCLUSIONS: Esophageal replacement continues to be a challenging operation
associated with significant complications. Most reoperative procedures were
directed toward strictures and persistent fistulae. Complete graft failure can be
managed by gastric transposition or free jejunal graft. Despite the perioperative
morbidity, most patients have good functional outcome.

PMID: 10591557  [PubMed - indexed for MEDLINE]


5. J Pediatr Surg. 1993 Aug;28(8):990-4.

Development of a technique for jejunal interposition in long-gap esophageal
atresia.

Cusick EL, Batchelor AA, Spicer RD.

Department of Paediatric Surgery, St James's Hospital, Leeds, England.

Primary anastomosis is the treatment of choice in esophageal atresia. There
remains a small number of infants in whom establishment of primary esophageal
continuity fails or is unrealistic due to a long gap. Esophageal reconstruction
then necessitates an interposition/transposition procedure. The most widely used 
tissues are colon and stomach but each has significant limitations. Jejunum is
theoretically the ideal esophageal substitute being of appropriate diameter and
having good peristaltic activity. Its use in the past has been limited by the
precarious blood supply and restricted length which result from the short
mesenteric pedicle. We have attempted to resolve these limitations by using a
microvascular anastomosis to augment the blood supply to the proximal jejunum in 
a series of 5 cases (2 pure atresias, 1 esophageal atresia with proximal fistula,
and 2 atresias with distal fistula). In a sixth case (atresia with distal
fistula) a free jejunal graft was used. In one case initial surgery was confined 
to cervical esophagostomy and feeding gastrostomy, in the remaining 5
interposition was necessitated by failure of a primary repair. The age at surgery
ranged from 8 to 16 months. The development of the technique and outcome in each 
patient is described. We conclude that a free jejunal graft is preferable to
augmenting the native blood supply and intend to continue with this latter
technique.

PMID: 8229605  [PubMed - indexed for MEDLINE]


6. J Pediatr Surg. 1988 May;23(5):483-9.

Long-term results of jejunal replacement of the esophagus.

Saeki M, Tsuchida Y, Ogata T, Nakano M, Akiyama H.

Department of Surgery, National Children's Hospital, Tokyo, Japan.

Since 1969, jejunal interpositions have been carried out in 19 patients for
esophageal replacement. A segment of the upper jejunum was used to bridge the gap
in the right thoracic cavity. The distal esophagus and its sphincteric mechanism 
was preserved in all but four patients, who had peptic strictures. Early
postoperative complications such as total necrosis of the graft, perforation of
the graft, and anastomotic leak developed in three patients (16%). There were no 
operative deaths but there were two later deaths (11%). We were able to follow 12
patients over a long term. Among these 12 patients there were two anastomotic
strictures, one of which was dilated successfully by bouginage, and one marked
redundancy of the jejunum which necessitated surgical correction. Both height and
weight were lower than -2 SD on a Japanese standard growth curve in two patients 
who had anastomotic strictures. Transient stagnation of swallowed barium at the
lower esophagus was the common finding; it was observed in seven cases (58%).
Only three patients (25%) complained of occasional feelings of delay in
swallowing. Stagnation with a mildly redundant jejunum was the common radiologic 
finding in these three patients. Reflux of the gastric content into the esophagus
did not occur. All the patients, except one who still has dysphagia due to
anastomotic stricture, can eat anything they wish at almost normal speed. These
long-term results indicate that jejunal interposition with preservation of the
lower esophagus is a recommendable procedure for esophageal replacement.

PMID: 3379557  [PubMed - indexed for MEDLINE]

In the Hospital for a Week

Baby Audrey and I will be in Salt Lake at Primary Children's Medical Center for at least a week.  Her routine monthly esophageal dilation surgery on Friday morning reopened the previous tear in that thick, brittle scar tissue in her throat. As updates come in, I will continue to add them at the bottom of this post.

Daddy comforting Audrey during their ride to a swallow study checking for leaks following Friday's dilation.

Her surgeon knew right away that something was probably wrong, because her throat dilated right open past the point it had been a month ago.  He asked that she be held a few hours for observation, and sure enough,  as she came out of anesthesia her oxygen needs seemed to be gradually increasing instead of decreasing.  A swallow study confirmed the leak had reopened, and we were admitted for the night.

We spent Friday night (June 2) at our local hospital under observation, and she seemed to be improving dramatically that first evening.  During the night however, fluid quickly built up around her right lung, making it difficult for her to breathe.  Her oxygen needs and pain level continued to increase, and on Saturday morning she was transferred up to Primary Children's for surgery to relieve the pressure around her lung.  The chest tube drain (for her lungs) and a picc line (to send antibiotics directly into her central blood lines) were placed without incident.  We are waiting for tests to tell us whether saliva is leaking into that area or if it is simply inflammatory "pleural fluid".  The tests we have seen so far seem to indicate the second, which is what we want.  Saliva would mean major surgery to open her chest for rescue surgery.  In the meantime, her pain has been gradually decreasing and her breathing is much less labored with the chest tube releasing that pressure around her lung.  Thank you for your prayers for baby Audrey.  We will try to keep you posted.

Audrey's first professional portraits, taken two weeks ago!  Thank you Michelle and Candi!

4:30 p.m. Saturday June 2 (before the entry above) Facebook status:

Audrey is in the Operating Room at Primary Medical Center after an unexpected turn for the worse and transfer of hospitals after her routine dilation surgery went south yesterday. I am alone and very nervous, but on the bright side I did get to see lots of things I haven't seen before today. The inside of an ambulance (well, when I was conscious) and met a really cool paramedic who's been practicing for 25 years and lives his mantra that "life is a test, not a vacation", the inside of the Operating Room (nifty, they had a ginormous monitor and murals on the walls of a Utah Jazz basketball game), and managed to get myself very, very lost in the hospital despite having lived here for five months last summer. And I tried a new kind of soup in their cafeteria, yummy chunky tomato basil. *sigh*

 

Please keep praying for that sweet baby girl. She is being a champ, but she is in a lot of pain. Morphine round the clock. You can do it baby, Go Team Audrey!

10:30a.m. Sunday June 3 update:  
Three great things to report: her (awesome) surgeon just came to see us and said everything is looking good so far, that what we are seeing in the drainage tank is definitely pleural fluid (inflammation instead of spit), and her pain must be lessening some because she has been able to go a little longer between doses of morphine, and best of all (ok maybe I'm a litttttle off on my priorities here), our trip to Oregon that we had planned for mid June is NOT off at this point.  (Yaaay!)

My beautiful girls, oldest and youngest of our 5 children.  Both medical miracles. 

4:30 p.m. Sunday June 3 update:
Audrey continues to rest and get round-the-clock pain meds, but she is breathing and sleeping more deeply and her oxygen needs are definitely coming down, which is great because she keeps trying to take off her nasal cannula!   She has gone from needing 2 liters all the way down to 0.5!  My favorite part was when she started reaching for things again out of curiosity, and sat up in her bed and reached for me.  We even watched a cartoon together that she seemed to really enjoy.  Little by little I am beginning to see a return of our sweetheart's personality.  

Daddy holding her upside down in our newly built sandbox, mid-April

I wrote a lot more on this day, but didn't want to distract from the quick updates, so it is at the bottom of this post.

 2:30p.m. Monday June 4

Last night she seemed to need her morphine more often, and her breathing sounded like it was getting worse.  This morning we went down for an x-ray and wound up instead in surgery, because the x ray showed a massive amount of air pressing on her heart.  They placed a second chest tube drain, got the air off successfully, did her esophagram under sedation, and the leak still appears to be contained, so we don't know where the air is coming from.  Could the air be coming through a micro-fracture that is too small to allow liquid?  I am bracing myself ever more for major surgery.

7:45 a.m. Tuesday, June 5

Yesterday they vented 60ccs of air (a large syringe full) off Audrey's chest cavity around her heart. Their current theory is that a lung was slightly punctured when the first chest tube was placed, which fits with the esophagram studies showing a contained leak. The lung apparently adheres to the chest wall following open chest surgery (which she has had twice in her life) and makes it easily punctured in the case of a chest tube placement. A couple of the pieces don't fit to my reasoning, but I like the prognosis of that much better than a free leak from the esophagus. She is now on three antibiotics--Meropenum, Diflucan, and as of yesterday's culture readings, Vancomycin (any time you see -mycin you know it is a big gun, so I'm thinking probiotics again after February's C-diff, but her system is at rest since she's on TPN so I'll have to wait until we are approved for G-tube stuff again) The biggest new news is that she spiked a fever in the night of 103 farenheit and we don't know why. It responded great to Tylenol though, and came right back down to a reasonable level. Man, this kiddo does nothing halfway. (Where does she get THAT from?) We'll probably do a swab to see if it's a virus, perhaps the one her 2-yr old brother had over the weekend. Thank you again for all the prayers and sweet messages of support and encouragement.

Her esophagram image shows the leak (also called a diverticulum, the black ball on the left side near the top of the photo), but no "effusion" or leaking out into the chest cavity.

9:15 a.m Tuesday, June 5
Just learned: A more likely cause for the fever is a mild pneumonia and atelectasis (I just learned how to spell that) they think they are seeing on the x-ray.  Both normal side effects of what she is dealing with, caused by not breathing deeply enough to inflate the entire lung.  I am so glad, because I really don't think she was exposed much to her toddler brother's bug, which he came down with mostly after we left... (Plus I have tried to be really careful!  Even when he isn't sick! We are always washing and trying not to let them share things they put in their mouths etc.) and she really didn't need anything more to deal with.  They couldn't do much for a virus anyway, and she is already getting all those antibiotics, so yeah, I like their theory better.  But they will probably still swab her nose to test for viruses.

10:30 p.m Tuesday June 5
Too much new Audrey info to write about immediately, but will flesh out asap. For the moment, here's the CNN ticker version: positive rhinovirus results (she has a cold), continued high fever when not on Tylenol or Ibuprofen, no one seems to know how to test her esophagus for an air leak but they'll try once her infections clear, Vancomycin med was stopped following a negative culture (good news), Boston Children's is going to weigh in over the next few days, a stent is the PC doc's current plan if the leak doesn't heal itself, but the more I read about it the more I am worried it is not a long term solution. At mid day I was very down and she was in a lot of pain, but we both got naps and by evening I saw the first smiles in days, took her on a wagon ride through the halls-- she likes the jungle murals, especially the mama gorilla with her baby. Received an unexpected email with some info about a stent complication in another toddler that led to his passing-- brought it home that we are fighting for her life with every decision we make.

By far the highlight of the day-- her first smile!

The shimmering city lights out our window

When it hurts to move, snuggle your mom

 

 

 

 

 

 

4:30 p.m. Wednesday, June 6:

A quiet, pleasant and restful day.  Wagon rides to the courtyard, and unexpected visits from dear friends.  Docs are waiting to see if her diverticulum (leak) heals in a week.  If it doesn't, they want to place an esophageal stent in an attempt to close her leak and keep her stricture at bay.  I am not very hopeful that a stent will work for Audrey's funky anatomy, and from what I've read I definitely do not think it is a good long term solution.  Researching our "what if" options has led me to wonder about a jejeunal interposition; it is a major surgery but appears to be a much better option than those we've previously discussed.  See this link.  That a surgery specialist's website lists its primary drawback as the fact that it is a "technically very difficult surgery" has me wondering whether we are heading to Boston, not because our fabulous surgeon couldn't do it, but because I know he cares so much about her that he would want her in the most experienced hands for that specific surgery if it turned out to be our best option.  I haven't felt up to writing in detail about my feelings and the details of how everything has shaken out the past couple of days, but I want to express my awe that the order of events has been, if you don't believe in a higher power, an awfully huge coincidence.  Information and resources have been falling into my lap at exactly the right moment, courage and strength and the right people to talk to have been at my doorstep at exactly the right time.  Whenever my pragmatic brain starts to kick in--- bam, I am reminded we are not alone. My Father in heaven is always there, leading, guiding, and walking beside us, helping me find the way to help this courageous little girl.

STOP-- PHOTO TIME!!  (cue techno beat)

We took a morning walk outdoors to the children's courtyard!

The courtyard is full of healthy and lovely things

The cheery children's courtyard

 This artwork was made by my young friend Andrea, whose mother generously brought her children and my older daughter to visit me today.  The dragon is breathing fire that holds the words,
"We're with you."
It now hangs on our hospital room wall.

What a treat to see my two girls together today!  I have missed my other kids back home!

LOVE this family!  Thank you for visiting me, dear friends!  Like the dragon Andrea painted, you are truly powerful.  And thank you, THANK YOU to EVERYONE who has come, even if I haven't snapped your photo--- your visits made our day!

****************************************

A few more thoughts from Sunday June 3:
Wanted to share a few fun things with you, just for the record.  First, you miiiiight be wondering how could I possibly get lost in the hospital I spent 5 months living at, right? So, we arrived by ambulance and walked through the ER, up the back patient-only elevators to our room, and down to the Operating Room as soon as we were prepped and they got through with their previous case, through the halls and elevators reserved for gurneys-- I had no parent bracelet, no room number (never thought to check), nothing. Holding and comforting her most of that time. I walked down with her and a temp tech INTO the O.R. (never never expected that! They always made me say goodbye outside before!) and the tech left. The O.R. staff let me help comfort her as they got her ready and on the table, then when they were ready they told me to kiss her good bye and let myself out the door.... and BAM. I was totally alone. It was so weird. I recognized NOTHING of where I was in that part of the hospital. It's the part where the surgeons go, back behind all the waiting rooms, and behind some special doors that lead out into the main hallways and lobby, but it is a complete MAZE back there and I was hopelessly lost for which way to go. No helpful signs, and doors and hallways in every direction. 

  

I actually had to ask a random doctor in the hallway which way to go. I had a moment of panic that I would have a hard time finding her room again, realized I had no parent bracelet or anything-- it was a weird feeling, especially on top of not knowing how the surgery would go. I knew what section of the hospital we must have been put in only because I remembered the fish murals from our G tube training class-- but there are a ton of rooms and hallways back there too. I got back to the right place by remembering the view out the window at the end of the hallway near our room, and looking for the hallway with the window view that matched that memory-- and when I got to the right area a nurse recognized me. How silly, huh? It was like the twilight zone. I have ZERO sense of direction and it gets me in trouble a lot. Hahaha! 

One really fabulous thing about this hospital stay though, is I LOVE that I get to stay with her!!! Some parts of this experience are going to be much easier than the NICU!  When my little two-year-old is over his cold, my children and husband could all visit us at the same time instead of one at a time!  I don't ever have to leave Audrey except for when I need to shower or buy food. Saturday night I had a funny little adventure.  They have a single bathing room out in the hall you take turns at. I must have gotten a shower after others had used all the hot water last night, because it was much colder than I like and I thought from the way they had things set up, it might be a safety lock on the hot water temp or a cost saving thing. I thought to myself, this is nothing!  I can be tough!  My friend in Ecuador has no hot water at all!  This is a blessing to be here!  Think of it like camping!  Better than camping!  But after shivering my way through washing, I dried off and looked at the separate bathtub and an idea occurred to me-- I wondered if it might be on its own water heater--- so I tried it and it worked! (Soooo guilty! What a waste of water!)  But it was HOT and perfect, and I was a mom-cicle!  It was getting pretty late and I knew I was going to have a hard time sleeping if I couldn't warm up, so I got in the tub just to soak for a few minutes and warm up my core temp.   First bath in a lonnnnng time, and I love baths, so it was a really nice way to end the evening.  I thought about nothing except how Skyping (video calling) my silly little boys back home earlier that evening was soooo much fun!  They are such hams for the camera and were so excited to see me and Audrey!  Justin says our little two year old keeps asking to go in the car to come see Mommy and Audrey.   He has a cold so we will have to be happy with Skype for now, but it was so adorable how excited he was on the computer. As I settled in to sleep, I enjoyed the GORGEOUS view out of our hospital room window looking out over the city skyline. Now it is the next afternoon, and I am having a sweet quiet day with just Audrey and me.  I just gave her a sponge bath and she is more comfy (after so much perspiring) and resting more deeply right now, and we get to be alone together in the room most of the time. I might actually get to take a nap this afternoon. Sooo... yeah. We are not doing too bad, all things considered.

Ok, so this is not aaaaactually the EXACT view from my window, but it's kind of close and was better than my photo, and my camera's photo doesn't do justice to how pretty it is. :)  The shimmering city lights, breathtaking mountains, the indigo sky...

But I have to be honest, I'm not going to mince words.  Major decisions are ahead, sooner than I had hoped, and our surgeon is struggling to make those very difficult decisions.  He needs our prayers too.  I think he is going to say he won't dilate her again, that we must cut out the scar somehow.  I am afraid for what that means.  She received a priesthood blessing today, and strangely the blessing said nothing about healing.  Only that she would be a blessing and an inspiration to many, that she would fulfill her purpose here on earth, that she would by her example and story encourage others to become their best selves, to know that they too can do hard things.  I trust the Lord.  All we can do is to do all we can to help Audrey, and trust the Lord to make things work together for his purposes.  In the meantime we are blessed with her sweet amazing self, and focusing on each day, taking it for what it is, and feeling joy at each little miracle.  I also received a priesthood blessing, and it talked about how our family and friends are God's angels to us.  Thank you to everyone-- for watching children, for making meals, for calling and praying and caring and reading this.  You truly are our angels!

Mon précieux chef-d'oeuvre, la raison de tout ce que je fais