Just Another Day In Boston

 Hello Friends,

Not too much to update today. Yesterday they tried to condense her feed schedule down from 60ml over an hour and a half to 60ml over an hour and fifteen minutes. She didn’t like that at all, and had several large spit up’s throughout the day. Last night they went back to the hour and a half and she “only” had one bigger spit up event. Other than that, it was a really good day. She was happy and playful, so I guess she got all the sedatives from her operation Thursday out of her system on her sleepy Friday. Also, she got to go outside for the first time, ever! Not including when she was transported from hospital to airport to hospital to get to Boston. She wasn’t too fond of it, so we’ll have to work on that.

EGD #2

 Well, Lily had her second EDG yesterday and the results weren’t exactly thrilling. The upper portion of her stricture had reduced in size to even smaller than it was before they dilated last time. The lower portion was only slightly bigger than it was before the last EGD. They did a steroid injection at the upper portion, to discourage scaring and hopefully help it maintain size, then dilated again.

They also asked that we allow them to include her in a study group where they are performing ultrasound scans of kids with EA so that they can hypothesize if certain kids might have more or less trouble post EA surgery based on thickness of the esophagus walls. We agreed, so now going forward whenever they do these EGDs they’ll also do a ultrasound. It probably won’t do much for us or Lily, but hopefully will be of some benefit to other kids in the future.

Currently, our hopeful discharge date is around September 19th.

Another Delay

 Hi Friends,

Just a quick update on Lily. We just found out today that while they've been saying she would need three of the EGD's (where they dilate the stricture in her esophagus), what they meant was that she needed three after the initial one. So once again, we thought we had a discharge date, and now it's a week later. That puts her discharge sometime around September 18th. New best-case scenario, she's coming home after eight months in the hospital. 😔

Other than that, her heart rate is still higher than they would like it to be. Her prior normal was around 130 and for the last week or so, it's been fluctuating more around the 170 range. They put her back on the Clonadine and have been slowly weaning off that again. However, being back on it hasn't really had any effect on her heart rate. They're going to consult with cardiology and see where we go from here.

Here's a cliff notes version of Lily's life so far:

January 20th - Cora and Lily were born
January 24th - Lily has her g-tube placed, starting out at 1ml feeds 
January 27th - Lily's heart surgery to band her PDA is scheduled for Friday the 31st
January 30th - Lily's heart surgery is postponed because she spiked a fever
February 1st - Lily's feeding tube came out and needed "emergency" surgery to replace it
February 3rd - Lily has her heart surgery to band her PDA

February 11th - Cora goes home

February 28th - Lily has her first esophagus gap study, measured at 4cm

March 11th - The last time Rose was able to see Lily due to Covid visitor restrictions

April 10th - Lily has her second gap study, measured at 3.5cm

May 12th - Lily has her third gap study, measured at 4.5cm

May 22nd - Lily has open-heart surgery to repair her VSD

July 12th - We drive out to Boston, thinking we'd be there for around three weeks then take Lily home

July 14th - Lily flies out to Boston, initial surgery scheduled for June 16th

July 16th - Lily has her Foker I surgery and her gap is measured at 6cm

July 18th - Lily's gap is measured at 2cm, and she has her first traction procedure to stretch her esophagus

July 24th - We find out Lily has a blood clot in the right side of her neck/chest area

July 28th - Lily has her Foker II surgery, connecting the two ends of her esophagus. We are told Lily would need to be there for around a month after the surgery for recovery.

August 4th - Lily has her first attempted extubation which did not go well and they intubated her again

August 6th - Lily has her second attempted extubation which is successful, but she's put on a CPAP

August 11th - Lily has her first esophagram and were told there was no leak or major narrowing of the esophagus evident

August 13th - Lily is off the CPAP

August 19th - We thought we'd be able to take Lily home by August 28th

August 21st - We thought we'd be able to take Lily home by September 4th

August 26th - Lily has her EGD and they found her esophagus diameter to be around 3.5mm, instead of the 10mm or so that it should be. We found out the dilations have to be done at BCH so with three dilation procedures required, we think we'll be able to take her home by September 11th.

September 1st - We found out the first EGD doesn't count as one of the three, so we might be able to take Lily home by September 18th. Almost ten weeks, which is just a few more than our initially planned three.

Plan.. E?

 Change of plans.. again. We were under the assumption that Lily could discharge as soon as she was weaned off her sedation meds and clonidine. The nurse practitioner with the esophageal and airway team told us today that unfortunately, Lily will need 2 more dilations, each a week apart.. And they don't want her to leave until after that. Because her esophagus is so "freshly" post op, they don't feel comfortable with anyone else doing her dilations. Even though the surgeons in GR are familiar with EA kids and dilations. The Boston team is very EA specialized, so I get it I guess. But it's still a hard pill to swallow knowing it will be another 2 weeks. Joe is going to fly home on Friday and we will figure out a new plan for the next couple weeks. I know she's in good hands, and I am grateful for her progress. Sorry if I seem ungrateful. I just miss Lily and can't wait for her to be home.

Video chat with Lily :)

Cora loves to eat!

Rose had her first s'more this week. She liked it :)

So Close, But Also Not

 Well, yesterday Lily had her EGD. They do an X-ray, and also put a scope down her esophagus to measure the inner diameter of the esophagus. Her’s measured about 3.5 and a normal baby her size it should be 10 or 11. They said around 60 or 70% of the time after the Foker process kids will need some dilation of the esophagus at the connection point. I talked to the surgeon who did her Foker and he said 3 or 4 is about the average they see, so it’s not concerning.

As of yesterday she was officially weaned off her morphine drip, and they started weaning her off the clonadine.  She was fussing yesterday after the EGD so they ended up giving her a morphine bump. Apparently they gave her another bump last night.

We’re working on getting her eating schedule up to speed. Adjusting the amount she gets and how often she gets it, through her g-tube. 

Her pic line is supposed to be coming out this afternoon, which just leaves her vital leads and her g-tube. Least amount of accessories she’s had in quite a while!

It’s been a very frustrating couple days. The general fussiness due to weaning off the medications. The fact that it seems like the general expectation is that we will just always/only be a patient of BCH. “Oh, we’ll do that in a couple days. Oh, we’ll do this next week, then probably again the week after that. Oh, we’ll just take care of that when you’re here for the 3rd EGD in three weeks”. Um...yeah, I don’t live here and I’d really like to go home someday if that’s okay with you guys.

Other than that, Lily is doing really well. It’s just hard to be so close.. We both just need to go home. I’m really missing my other girls. Hoping she’ll be home before her 1st birthday! Ugh.

This is the extent of me being a tourist. Whatever is on my way to/from the hospital, or to/from wherever I get dinner 😉 

Detox Is Not Much Fun

 Well, I guess yesterday was our free pass day. She had a good night last night, and pretty good this morning. But this afternoon I guess the morphine cut caught up with her. She’s been pretty sleepy, but with a lot of crying bouts in the mix. But as long as I hold her just right and rock/bounce her just right and don’t move my arm or scratch my nose or breathe wrong...then we’re good! For a little while anyway. Trying not to think about what the next week or two will be like if this is just the beginning. But we have to do it. She’s gotta get off these meds so we can get the heck out of Boston. 

From taking with the pain management team today, it sounds like maybe next weekend isn’t going to be our go home date. We’ve gotta get off the morphine, then on to the clonidine. They said maybe 4 or 5 days to get off the clonidine. That’s not what we were expecting. So based on that, it may push is into the next week to get home. We’re so close..but also not. Every time someone tells us it’s a day, two more days, three more days, longer than we thought, it feels like it might as well be two months. And nothing is for certain, which doesn’t help. It all depends on her, on how she handles things, how her body responds. So we’ll see what tomorrow brings. Or maybe just see what the next 5 minutes brings and start with that.

Here’s Some Bonus Pictures

 Because Lily gets most of the attention here, and these two need some recognition for how dang cute they are.