New Math

Math test time. Audrey was recently switched to a new formula, and discharged with these instructions: Feed Audrey 48 ml of Elecare Jr per hour plus 10ml per hour of water, for 16 hours. Elecare Jr is mixed per these instructions: add four scoops of powder to five ounces of water to yield six ounces of mixed formula. How many scoops of formula and how many ounces of water should I be mixing per day? No one better cheat and give me mililiters. Ok, go!

First Dilation Since Surgery

Audrey sailed through her dilation surgery today. Turns out, no rocks, sticks, or acorns were down there after all (causing her to reject food last night and throw up her pureed avocado and medicine), she had simply constricted down to a tiny 4mm hole already. That was really, really fast. Surgery wasn't even a month ago. Dr Manfredi dilated her up to an 8mm opening, and plans to do a bit more next week to get her to a 10, then she'll need at least a couple more dilations to get her to the 12-15 goal. Even though I know this is different, I'm having some creepy deja vu.... last year we did this same thing, rapid restricture every time we'd start to make progress on oral feeds, lots of dilations, and then eventually we wound up in a terrible place, with "friable" brittle scar tissue in the esophagus that tore easily and landed her in the hospital for most of June with a life-threatening perforation. This time has to be different. Of course, last time we had a tight repair and the two sections of her espohagus were basically pulling apart and filling in with scar tissue, but it feels so familiar, the rapid restricturing just when we start getting somewhere with eating.... it makes my heart clench in fear just to feel like it's all happening again. Dr Manfredi is going to inject a little steroid into her scar next week, a treatment that is not available in Utah and a major reason we are here, and hopefully that will calm down the scar tissue and keep it from closing off so quickly. We'll also keep on top of her acid generation with her PPI meds, and hope to avoid a major surgery for reflux.

My husband's aunt asked me if she could receive a transplant of stomach and esophagus.  I don't know all the reasons, but that kind of transplant is not done anywhere in the world. I'm sure it is for a major reason, perhaps because the nerves are so important and are too tiny and complex to connect in a transplant. Certainly excellent surgeons must have tried it and have found it is not feasible, I almost don't want to know, it probably resulted in the recipient passing. But thank you for the thoughts, yes we had wondered that too at one time back at the beginning. I'm not aware of any laser therapy for esophagus, but you are right that we are here primarily because of the focused steroid injection and/or "needle knife" therapies that are not available in many places, which I should have mentioned are hugely successful. Audrey will be in the best hands in the world here for this specialized work on her esophagus. Dr Manfredi will do a great job on her, it's just a bit scary to be in such a similar place as last year and having seen where it can lead. It will all work out, it's why I'm here, to give her the best chance of success. We are doing all we can do, and one day soon I have faith that it will be enough.

NICU Graduation Anniversary

I gave her an anniversary present: a pair of soft pink shoes, and she loves them!

Today marks one year since Audrey came home from the hospital for the first time, at almost five months old. In celebration we went to go see the ducks, squirrels and pigeons at the public garden, and played on the swings at the park with our sweet friends Aliyah and Amy. We had so much fun and can hardly wait to see Daddy and the siblings who are coming in just four weeks!  

Unfortunately I think Audrey might have also managed to swallow something like a bark chip at the playground, because she could not swallow her medicine tonight which surprised me. Then, as I was settling in for the night she surprised me again by gagging and retching in her sleep with a terrible gurgling sound and a terrified look on her face. I am trying to figure out what happened... I think it is reflux, but she was drenched in sweat. Briefly considered taking her in, but I watched and held her for a while, changed her and put her back to bed. It was time to turn off her pump anyway for pre-op, and I had turned it up a bit high in order to get her calories in for tomorrow ahead of time, so it was probably my fault. She's been fine since. But still, honestly it scared the stuffin' out of me. I've heard of this before from other EA moms, but never seen it in her. I really do not want to do surgery for reflux on this kiddo. We are just all funned out. 

Busting us out of the Clink

Eeeee! She took one small step today! Feeding team took away their approval for the chopped strawberries when they watched how she was handling them, darn it, but it's just a matter of time before she figures out the chew THEN swallow thing. But we are still on a big upward swing. They're running out of reasons to keep us before next week's dilation, and basically gave me the option of going and coming back-- I was torn, because here we kind of have a little easier time getting her food and nutrition support, and logistic wise it's definitely simpler. But we are sharing a room most of the time with post-surgery babies so it isn't always the best sleep environment.  We compromised to be discharged on the 14th and return for her first dilation next week.  Thank you to our new friends Lexi and Abby for all of your help bustin' us out!

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Lexi keeps an amazing blog at www.MostlyTrueStuff.com, about life with her busy household of four children, one of which lives with Autism and one (darling Abby above) with Down syndrome.  What a fabulous mom and person she is.  We are so blessed to have met her through a mutual friend.  She drove a lot of hours to help us out today, thank you Lexi!

As we were packing up to go, the clowns stopped by our room (for the first time all month!) to blow bubbles and sing silly songs.

Audrey's First Call for Room Service

 Monday morning rounds brought a big surprise: the doctor who will be doing all of baby A's post-op procedures, the gastroenterologist Dr Manfredi, had a family emergency and is out of town until next Tuesday, so we won't be doing her dilation this week after all. We'll be waiting in the hospital until he gets back and proceed with the plan from there. In the meantime, Audrey's Boston surgeon (Dr. Jennings) encouraged me to let her try eating whatever she wants. His thought was that it would help her throat stay open to keep it used.

Hello, Room Service?  I'd like to place an order for... everything.

I got really excited and ordered dairy-free blueberry pancakes and chopped bacon and strawberries (both whole and pureed) for her. I chopped some of the strawberries up into tiny bites, likewise broke up the pancake into small bits, to give her some of everything to try. After a very long (1.5 hr) wait, during which she decided to participate in New York's Fashion Week by emptying her suitcase and trying everything on,

 

 the food finally arrived. It was so exciting to see her sitting down to her very first meal of food without needing to chew through a mesh bag! 

She took it very seriously (way overdue for morning nap by that time, as I had been unprepared for the food prep to take that long) and took her first bites very cautiously, but soon got into it more. 

 She liked the strawberries the best but gave the pancakes a genuine chance. 

 

She seemed to be doing so well with the chopped strawberries that I decided to see what she would do with a whole one. She took the berry, nibbled a tiny bite, chewed and swallowed, and then stuffed the whole thing in her mouth at once, chewed once and then swallowed. I knew what would happen next before it happened-- her throat is only about a half inch wide at the narrowest point, so the strawberry had to come back up. In true toddler fashion, she grabbed up one of the two big chunks and put it back in her mouth, chewed a little more and swallowed it right back down. Then she started working on a bunch of pieces of the pancake, (and wouldn't you know the feeding team came in right about this moment and was chatting with me and observing us doing this,) and then Audrey suddenly got this look on her face like "Oh crud. It's stuck. Wait for it...." bam, the stuck stuff had to come up again too. Me and my big ideas-- 

I blamed the blueberries for blocking up the works, but the feeding team said it is common for kids with EA TEF to have lots of trouble with bready foods. They were gently recommending that we go back to purees only (nothing too liquidy either, remember, she is not allowed to drink anything thinner than honey textures right now due to aspiration in her swallow study) and I could feel some unbidden desperation creeping into my attitude as I argued at how great she was doing on the chopped fruit, so they gently compromised on that as long as it's well chopped. Their thought was that they want to keep it a positive experience for her and not cause her to retch, which could bring back an oral aversion. She is doing well on learning to chew but is not quite there yet, so they want to give her more practice. They suggested that I go get her some dissolve-able snacks like the Gerber cereal stars to practice on, but unfortunately the hospital didn't have any so later that day I walked to the grocery store with a sweet friend who showed me the way, and Audrey now has a well stocked cupboard of dairy free and team-approved treats. 

(She is allergic to the protein in cow's milk, sometimes called casein.) This morning she enjoyed sampling all of her new treats for breakfast (especially the Gerber maple waffle wheels), and even asked for them by name, "treat!"
 

For lunch she happily sampled purees of meatballs, corn, squash, chicken noodle soup, strawberries, and apple cobbler. She got most interested in the meatballs, and insisted on taking the spoon from me after a few bites and giving it a whirl herself. That was so exciting and fun for both of us! There was meatball puree everywhere, but it was sticky enough that she got a lot on the spoon and into her mouth, I was so proud of her! She finished her meal off with a few puffed curls of veggie-flavored Pirate Booty (a salty snack similar to cheese curls but with no dairy). Then we got to have a couple of hours break from her tube feeds, and she and I went down to the garden and she got to crawl around-- she is loving pulling herself up right now, and has just started coming to a stand on her own and is practicing a few seconds without support, might not be long before those big first steps!-- and found something I'd never seen before, a little playground near the garden. Since she was free of tubes she actually got to slide down the slide! It totally made her day, and mine, as she laughed and laughed to go "1, 2, 3, wheeeee!"

Now You Feed 'em, Now You Don't

Just had a nurse come in and tell me we weren't allowed to give her more than tiny tastes of baby food--- so this makes about the seventh time I've had to argue the case for what Audrey is allowed to do based on what was told me directly at her swallow study, not what was written afterward (apparently somewhat vaguely). Ay Yi Yi!!!!! Why do these things always seem to happen on the weekend.  On a great note, she is off IV liquids entirely and they are talking about pulling her central line (although I hope they keep it through her dilation surgery this week because she is so hard to get IVs into!  This week will be #1 of three weekly procedures to train the scar in her throat to behave itself and not try to close off.) For all my positivity, this whole communication-between- caregivers-over-food issue has brought out a bit of the mama bear in me! 

I was sad about the end of summer and her missing the fun of splashing in the water, so got creative and set up a bin of warm water for her to play in.  It was relaxing for both of us.

Swallow Study Report was, well, Tough to Swallow

More exciting strides today! A swallow study in one hour to learn more about if, how and why she might aspirate when swallowing. She is cooperating to the fullest by taking a long early nap, so she can be as helpful as possible when being asked to ingest her treat of radioactive pudding and mutant juice. I'm told she's on her last bag of TPN (intravenous nutrition), meaning formula is finally starting today! Slow continuous feeds planned at first, and going from there. We will see how much her reflux is an issue now that her throat is wide open, but we have not yet discussed any surgical options with the team. Our first (of three weekly) dilation is next Thursday or Fri. I think we might be checking out of the hospital after dilation #1 around next Saturday. Getting very ancy to see my kids and husband when they fly out for a visit.... 5 weeks to go!

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Annnnnnd, the other shoe.

Swallow study results from today: Baby Audrey aspirates a little of every bit of liquid she swallows, she struggles a little with pudding consistency foods but seems to sort it out, but on chewable soft solids she got the gold star, no problems. When she drinks liquid, it travels too far down the back of her throat, and she doesn't coordinate her muscles in such a way to prevent it slipping down

into the airways, and has to eventually cough it back out, which sometimes takes several minutes. A Speech Language Pathologist would be better able to explain what is happening, but my guess is it might be a side effect of having a lot of work done in the back of her mouth, tubes down her throat etc, which have desensitized the gag reflex (which is in some ways a blessing in her case, I can only imagine what it would be like having all she's had done in there, my gag reflex is really strong and it would have been torture-- I can barely even get a strep test! She's such a champ.) Anyway, they think that practice will improve her coordination for liquids, but in the meantime she has been given a prescription for thickened liquids by mouth (the consistency of honey) and soft solids. The funny part of it is, that up until today she had been approved for nothing BUT clear liquids, and now she is allowed to have almost anything except them, a complete reversal. But the test went really well-- she ate her radioactive pudding and drank her mutant juice without much complaint, and especially liked the little Gerber dissolving cereal stars (which were well dipped in radioactive powder, but hey, what can you do). When we gave her the plate of cereal she looked at me and kept turning them in her fingers as if to say, "is this REALLY ok mom? It's kinda weird that you are actually giving me these instead of me having to hunt for and steal them off the floor." When she realized it was ok, she ate every single piece with careful fingers. Then this evening, she enjoyed her very first jar of baby food, and handled the spoon with amazing grace. Happy baby tonight.

Audrey's first jar of baby food, Sept 8, 2012

Such a quiet, simple moment, yet so profound! This is some of the very first food she has ever been able to eat in her life, and by far the largest quantity. One small jar of baby food, one giant leap for baby Audrey!

A dear friend commented on this note, and said:

"and handled the spoon with amazing grace" -- ♥ In the Episcopal Church, the Gospel appointed for today's Office was the second half of John's story about the man born blind healed by Jesus: "He answered and said, 'Whether he be a sinner or no, I know not: one thing I know: that, whereas I was blind, now I see." (John 9:25, KJV) You are so surrounded by love and hope!!! Thank you for keeping us posted.

I replied:

Haha, I was hoping someone would catch that. Thank you dear friend for your ever-inspiring encouragement. I find it amazing that when she was given a priesthood blessing in June, the elders did not pronounce full healing upon her but instead that her life and her story would influence many for good, and that God would reveal his purposes and blessings for her in time and in his own way. I think of the earlier part of that same chapter of John, verses 1-3....

 1 And as Jesus passed by, he saw a man which was blind from his birth.

 2 And his disciples asked him, saying, Master, who did sin, this man, or his parents, that he was born blind?

 3 Jesus answered, Neither hath this man sinned, nor his parents: but that the works of God should be made manifest in him.

 

I see it all of the time here (at Boston Children's Hospital), the works of God being made manifest through these sick kids. The kids who come here are all the "rock stars" from all over the country and the world. I mean, we were one of the worst troublemakers in Utah as far as kids with her condition goes, but out here we're made very aware of our blessings on a daily basis through the families we meet, many of them somewhat famous in their communities through fundraising for their child. The thing is, these kids may or may not get completely well in spite of all these state of the art surgeries and care, but those whose lives they touch are changed forever for the better.  You just can't spend time with any of these kids, without smiling and realizing what life is all about-- not about how long you live, but how well.  How deeply you love.  How purely you laugh.  How each simple gift matters every day.  Life slows down in the hospital, you just work on the simple things and almost everything else just sort of becomes less relevant.  Just last night I was talking with two friends about this, as we were encouraging one who was struggling while her son was in a much longer than expected surgery... even when it may not seem clear, God has a plan for these kids. All these families are walking through fire, and are being challenged and changed in incredible ways.   In spite of the massive challenges we are all facing, I can honestly say that the hospital is not a sad place.  It is a place of work and learning, of simplifying life down to its basic elements, of purifying by fire.  I feel very blessed to be here and getting to know these people, even more so because I am still able to communicate so freely with all of the friends and family who care about us elsewhere. As you said, surrounded by love and prayers on all sides, and we feel it.

Esophagram, Round 2

After a fun Skype with siblings and my parents this morning plus her first visit from the hospital dog, Audrey is napping ahead of another uncomfortable esophagram. They need to find out if her throat has a leak or not, so they can hopefully begin giving her tube feeds instead of intravenous nutrition. Although she's had them many times before, for a number of reasons last week's esophagram was pure torture for her.... so we're praying for a better experience today.

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Later:

No leak!!!!!!! :) Swallow study tomorrow. Better experience today, very compassionate helpers working hard to help her feel empowered and comfortable every step of the process.

Exciting Visit from the ENT Team

We had a bedside scope today by the ENT team and.... drumroll.... Audrey's vocal chords are now working perfectly! This means she can protect her airways while she swallows. She guzzled two ounces of apple juice, almost completely without coughing! Our next esophagram is Thursday, then hopefully approval for..... food. And the next round begins. The child life specialist also surprised me this morning by arranging a free chair massage for me; it was heavenly. Ended the day with a happy visit to Bath and Body Works to use a coupon, with another EA mom who has become a dear friend. But the best news of all, we finally have definite plans for my children and husband to come to Boston! As in, tickets and everything! That's one paragraph that spells massive stress relief for this mama.

Oops!!!

Although I usually avoid eating in front of Audrey, we were having a relaxed morning and after some playtime it was getting late, so I decided to have a snack. I was eating an orange and Audrey was begging me for a taste. She was approved for clear liquids yesterday morning (but had coughed with water so we are worried about aspiration, but that's another story) so I thought, maybe I can just squeeze a little of the juice into her mouth and see what she thinks. Next thing I knew she had darted out with her chompers and was chewing up and quickly swallowing a chunk of my orange. It was too late to fish it out. I couldn't believe what had just happened, and I called the nurse right away to confess my crime. Audrey cried thinking she had done something wrong, but once I got over my panic we praised her and the nurse brought her some apple juice to wash it down. Audrey liked that very much. (For my EA/TEF savvy pals, she does have some strider with swallowing, a sign of tracheomalacia, but it seems fairly mild. And she coughs a little, but not like she's choking. I think I also recognized what was happening as the reflux popped the stuff back into her throat and irritated her sutures, she fussed a little and seemed in pain for a moment, even though she has her G tube open to a down drain right now. It's so weird to watch what normally is such a simple mundane process, with so much to observe and consider the implications. The coughing is a sign of a paralyzed vocal chord, or something else not working right in the swallowing process. That and the reflux will be our major immediate hurdles going forward. The stricture management we are hoping will go well under Dr Manfredi's arsenal of tricks, as he tells that scar to mind its own business.) But overall, a very exciting moment in the life. The orange went down, by gum!