The Nature of the Beast

Over the past 6 months, we have learned a lot.  The biggest thing I have learned is that the MAPCAs component of Mireya's condition makes it much trickier to manage.  She is constantly throwing us curveballs.  The most recent one cancelled our gtube surgery Monday, a surgery we were really looking forward to having behind us.  We came in for a gtube and will come out with a cath instead.  This is the nature of the beast.

Sleeping peacefully, blissfully unaware of the stressful day ahead

What I thought would be the last photo with the NG tube

  

Monday's updates from the ER probably made it sound a lot scarier than it actually was at the time.    I was pretty anxious about this surgery going in, almost like I knew something wasn't going to go as planned.  I had a really hard time settling on Sunday.  I was worried she was coming down with a cold as I had seen a few pretty juicy looking boogers.  I knew that a cold would cancel the surgery.  Monday morning, our report time was 6, with a 7:30 surgery time.  I got up at 5, and we were on our way by 5:40.  We checked in, everything went as planned and headed back to pre-op.  No boogers.  They started to do the work-up and when they hooked up the O2 monitor, they had a hard time getting her sats.  They weren't getting anything over the mid-70s.  I knew this was trouble and was a little surprised that they were so low.  The surgeon came out to meet me and it looked like we were going to go ahead.  However, when the anesthesiologist came to check in, he confirmed my fear.  He didn't feel comfortable going forward with the surgery.  He explained that by going forward, they could probably get through it, but they could also potentially make her very sick.  He said he had seen kids in her position end up on ECMO (life support).  That was all I needed to hear, as ECMO is one of the big fears heart parents have.   He thought it best for us to find the root cause of her low sats and since it was early in the morning, nothing was open.  So they hooked her up to oxygen and off to the ER we went, so we could at least get the ball rolling.   She was never really in any danger (she wasn't even blue!) and it was never scary, just a little frustrating and disappointing.  Once in the ER, they did a more thorough work-up - IV, labs, chest x-ray, echo.  Chris calls the ER Mireya's little house of horrors.  She was pretty pissed off, but mostly stable.  She didn't really start to fall apart until we came upstairs.  The final verdict was that her awesome growth, along with the reconstricting of the pulmonaries, was the cause of low sats.  Ironic, considering two months ago, her lack of growth ended us up here.  Something we all wanted for her was the cause of her current problem.  Basically it seems she had outgrown her pulmonary arteries.  

Tuckered out  in the ER

While she was getting a work up in the ER, they were securing a bed for her up on 8 East.  We were in the ER all morning.  Chris tapped me out around 11:15 so I could run to an appointment and by the time I got back, they had just gotten upstairs.  For once, we got a decent room.  At first it looked like we got a window, which was good enough for us, but upon getting up here, we learned that this room is actually a single!  It used to be a double, but when they opened the new wing, they turned a few doubles into singles and this is one of them.  It baffles me how they chose which ones should be singles, as this seems like a larger room than some of the doubles we've been in (Bucknell people - think Swartz dingles), but whatever.  It is so much better being in a single room.  

Once we got up here, we were reunited with so many old friends.  Our nurse had been our nurse back in December when we were in for the poor weight gain.  A few other nurses stopped by to check in.  We got the news about one of our favorite nurse's new baby boy, just born on Saturday.  Yesterday, some others stopped in to check on us too.  It's nice to see familiar faces and everyone, including her cardiologist was amazed by how "big" she has gotten (She's still only 12.75lbs and 3rd %).  

 

We went through the litany of questions and spent most of the day trying to get her sats stable.  She was all over the place.  Anywhere from 50s to 80s.  They tried different amounts of O2.  Every time they went up on the O2, I held my breath because the threshold for staying out of the CICU is 2L.  Once they go over 2L of Oxygen, they kick you off the floor into the CICU.  Normally a trip over to the CICU for this reason wouldn't bother me too much, but it wouldn't be likely that we'd get as good of a room upon our return from the CICU, so I was praying to stay here.  They bumped her up to 3L a couple of times and I was sure we were on our way to CICU, but knowing her history and the fact she's done this exact thing before, they gave her a chance and eventually she leveled out.  While she definitely needed the extra support (her sats dropped into the 50s without it), it doesn't seem to make a difference whether she is on 2L or 3L.  Aside from outgrowing her pulmonaries, one cause for this wild ride was that she had had nothing to eat since 2AM.  We asked multiple times, but they wanted to keep her NPO in case they wanted to take her to the cath lab right away.  They finally realized she had been 12 hours without food or fluids.   This is especially dangerous in the case of her tiny collaterals because when she becomes dehydrated her vessels constrict.  So we fed her and they pumped her full of fluids.  Through all of this, she was pretty good, not too fussy, though it was clear that she needed a nap.  She couldn't seem to settle, probably because she was dehydrated and hungry.  She took two short naps, and both times she woke up, got upset and immediately desatted, causing everyone to come in to check.  The second time, we had left her all peaceful to go out for a walk and we came back there was a party in our room.  But overall she was a trooper.  

This morning was pretty quiet as we waited to go down to the cath lab.  We were second case, so we had to wait for first case to be over.  It was a long case, so she didn't go back until 1:30.  She was pretty patient up until the last hour, and even then, she wasn't that bad.  Multiple nurses commented on how good she had been.  

Sleeping off the dehydration hangover

Ready for the cath lab. Loves her soft toys

The news from the cath lab has been overall good.  I wanted to post Tuesday night, but I didn't have the full report from her cath.  All we knew Tuesday night was that they had put a stent in both pulmonary arteries, that her direct measured O2 levels were in the 60s when they went in and 80s when they came out.  Dr Lock, who performed the cath, stopped by yesterday morning to give me his report and that was followed by rounds.  There was a lot of information and I needed some time to process it and find the motivation to write about it.  Dr Lock threw me for a loop when he came in.  He walked in and started by telling me there was a little bit of pneumonia in her left lung.  I asked him why and his response was simply "kids get pneumonia."  I was confused.  Then he told me the left lobe was almost gone when they went in, but they were able to recover it.  He then made it sound like he was worried about how much they were going to be able to accomplish when he went in, but that he was "encouraged" by how well it went, and that her next cath should be in 6-8 weeks.  And then he was gone.  I tried to ask questions, but as the day has gone on, I have learned that he is a man of few words...so it wasn't just me.  While he may have had better evidence of pneumonia, the team does not actually believe that she has pneumonia, nor do they think we should treat her for it.  Clinically, she does not present as having pneumonia.  No cough, no fever, her chest x-ray and lung scans looked normal.  The only indication that she might have pneumonia would have been her low sats, but that was also indicative of her narrow pulmonary arteries and collaterals.  Dr Friedman explained to me that in measuring, he should get a 100% oxygen reading from her lungs.  He got a 70%.  This could indicate pneumonia, but could also indicate other things, such as a partially collapsed lung, which he believes is more likely given her situation going in.    As far as his left lobe comment, the attending (Dr Saleeb) suspects he meant the left pulmonary artery, which would explain why they put stents in.  Dr Saleeb could sense my worry and assured me that everything they are seeing in her is normal course for a MAPCAs baby.  They told me "she is fine."  You may have noticed she is still on oxygen in my posts.  This was another source of worry for me - overnight Tuesday night I had a slight freak out when I looked at her sats and thought she was still on 1L.  I thought for sure we'd be coming home on oxygen.  Again, Dr Saleeb to the rescue.  As Boston Children's is a teaching hospital, rounds always includes a bunch of residents that are still learning the field, so she took the discussion of her O2 needs as an opportunity to teach.   The cath doubled the flow to her lungs.  In doubling the rate of flow, it will take some time for Mireya to adjust to the increased circulation, causing her to have higher oxygen needs for a day or two.  This is only temporary and she will adjust.  Throughout this visit, my impression has been that Mireya, because of her MAPCAs, is a particularly interesting case to residents.  There has been a lot of teaching, which is really interesting and also explains why we have become so well-versed in cardiac medicine.  The doctors here do their best to make sure families are well-educated.  

Off to the cath lab

Recovery was a little rough.  She woke up from the cath groggy, but a little wild-eyed.  She had a lot to say, but no voice to say it with.  She was clearly hungry and was sucking down sugar water.  We came upstairs around 8 and thought I would nurse her, to settle her down.  Upon getting up here, she fell asleep.  She had a pretty fitful night's sleep.  She was very unsettled.  Upon waking, it was clear she was not in a very good place.  She had a couple spit-ups and a couple of vomits.  We suspected nausea from the anesthesia, so she was treated with Zofran.  We finally got her settled into a three hour nap and she was much happier when she woke up.  We even went out for a little walk around the floor.  We got her settled back into bed, and she was back asleep shortly.  We had to interrupt this nap to go get a lung scan, but she happily went back to sleep when we put her back in her crib.  She slept for an hour or so, and woke up just in time for the 6PM fussies.  We had some friends drop by to visit, and she was happy to cuddle, and eventually we were able to settle her down and get her to sleep for the night, so we could go to dinner.  

And she's out!

Not feeling so hot, day after cath #4

Feeling better post cath 

It looks like we'll be in a little longer than originally planned, so I decided I would come home last night to gather some things and sleep in my own bed.  When we came back from dinner to check in, Mireya was still sleeping.  Throughout the day, I had gotten into the habit of not watching her sats because I found them to be disappointing.  So I didn't even look at them.  I went into the bathroom and when I came out, Chris told me to look at the monitor.  After hanging out in the low 80s all day, she was comfortably resting in the mid-90s!  She had finally adjusted to the flow.  We found out our nurse for the night was one I had not particularly cared for back in November.  She was new at the time, and very green.  She lacked the typical confidence of the nurses on the floor and did things a little strange.  She particularly liked to give baths in the middle of the night and was a big fan of going on a schedule and waking us up according to the "schedule".  I am much more of a let the baby wake you kind of parent.  I was relieved that I hadn't been planning on staying but also impressed with the level of confidence she had gained in 3 months.  I am actually looking forward to having her as our nurse tonight, so that I can catch up and see how much she has learned.  Anyway, when we left, the plan was to start to see if they could wean her off of the oxygen. I am sure she got a bath, too.

So when will the g-tube be placed?  We were thinking of aiming for the end of the month because we are leaving for Florida on Tuesday and I wasn't sure that would give her enough time to recover.  After a few discussions with the team today, it sounds more doable than I thought.  A resident came by this afternoon to tell me they were scheduling the surgery for Friday.  When I asked him about Florida, he said without hesitation that that should be fine.  A little bit later, I had a discussion with Dr Friedman, who thought we were still leaning against it and asked me if I was comfortable with it.  As I told him, if they are comfortable with it, then so am I.   We'd all love to get it done this admission.  It would mean we don't have to come back at the end of the month.     So we are booked, I signed the surgical consent, and will sign the anesthesia consent today.  

Overall, its been a productive stay.  I thought we'd be going home yesterday, so its a little frustrating that we're still here, but if it means we get to have the g-tube placed and still go to Florida, it will be ok.  While I've been a little bored then last few days, it really hasn't been bad.  I was able to have lunch with another heart mom and her daughter, who has the same condition as my girls, on Tuesday.  I learned that there is a gym across the street that is actually pretty nice, and even more importantly, has nice showers (for the next time we're in a double!).  Having a single has really made a big difference in quality of life while in the hospital.  

I am currently working on another post updating on all the things we've been up to since December and am hoping to get that posted before the end of this hospital admission.  The hospital is a good place to write because there is a lot of downtime and no two year old to distract me.  In the meantime, I will keep you all posted via Facebook and Instagram.