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.