Life as a Leach

Oh CHOP... We have had an ongoing disagreement with CHOP orthopedics over which pulmonologist we should see.  We adore our pulm at Hopkins.  She has been with Abby since the beginning and really gets the whole picture.  She truly cares about Abby and we feel like she always has her best interests in mind.  CHOP ortho really only seems to value the opinion of their own pulmonologist with the Center for Thoracic Insufficiency team.  We have nothing against him!  He is a very nice guy and very knowledgeable, but we see no need to switch pulmonologists when ours is wonderful and 2 hours closer.  We don't care for the attitude of superiority we sometimes feel at CHOP and feel like all of her doctors should work together to give her the best care possible, no matter where they work! (Not to mention, Hopkins is not a two bit hospital!!) With Abby's recent lung decline and us feeling that a surgical intervention is needed, Abby's pulm has been communicatin...

Abby had a pulmonary appointment today, and it was awesome! I shared all of the pulmonary data we collect each day, and showed her how her high respiratory rates are trending down.  Abby also did another Pulmonary Functioning Test.  For the first time in a year, she was actually able to do the entire test without getting too exhausted to finish!  Her lung volumes have increased to 30%, which is amazing!  (It's all relative, but 30>15!!)  After discussing with her pulmonologist, we decided that we could cut the morning bipap time and also reduce the amount of saline nebs we do per day.  This gives us SO much more freedom and makes her respiratory treatments so much quicker!! Our pulmonologist was just ecstatic about how much better Abby was doing!  We are so thankful! The icing on the cake was that Abby's blood draw was super quick and on the first try.  We were in and out in 5 minutes!  Side note:  A year ago today, Abby cam...

We got home late Thursday night (sorry to those of you who aren't on Facebook and didn't know that!  I'm terrible about posting here after we get home and things are crazy.)  Abby has had a relatively good couple of days, although today her respiratory rate was a bit higher.  We are trying a different technique with her vent to see how her body reacts to it.  It's called a "Sip and Puff," and no it is not illegal activity!  Basically, it's a concentrated bipap setting that gives more pressure for a shorter amount of time.  The idea is that once she gets used to this, she may be able to do this instead of spending hours on the bipap every day.  She can just "sip and puff" for 10 minutes or so as she needs it.  She is adjusting to it pretty well, but it requires you to only breathe through your mouth and that's pretty weird for anyone.  For right now, we are doing the sip and puff in between treatments and still doing the longer times on regula...

There's no way to sugar-coat this.  Abby is in respiratory failure.  Her health has declined over the last few months to the point that her tiny lungs can no longer sustain her.  None of the "bandaids" they have tried have done much, including oxygen.  See, it's not an oxygenation issue....it's that her lungs are more or less deflated.  Giving her oxygen doesn't really help that. They're using it to get her levels up a little higher when she is sitting, but it doesn't seem to be helping at all when she is walking.  We haven't even attempted stairs yet. We have had lots of serious conversations with our team here and at CHOP.  Their dedication and sensitivity in all of this has proven to us once again just how blessed we are to have two fantastic teams working with Abby.  Because our plan hasn't been finalized yet and seems to change every time someone walks through the door, we aren't quite ready to share publicly.  Once there is a solid pl...

Well, we're still here.  Abby had a great night and I was very encouraged.  But then she had high rates throughout the day, including a respiratory rate of 95 after walking just halfway around the unit.  We are going to try stairs tomorrow to see what she does with that.   But they can't figure out any rhyme or reason to her high RRs, as they sometimes happen when she is hustling lying in bed.  The one pattern we have identified is that she does better right after a respiratory treatment, but that is to be expected.  I can't limit any movement to only immediately after treatments!!   I'm hoping for answers tomorrow...and to see our pulmonologist. 

Abby continued to need bipap off and on today, in addition to her regular scheduled times.  It seems to be about 3 hours after treatments that she starts needing bipap.  But she was in a pretty good mood and loved her visitors!  Matt and Caleb came this afternoon, and then my mom and dad brought Anna and Lucy this evening.  Abby was excited and surprised! Now, she's enjoying Descendants 2 on Disney Channel and getting her treatments.  :) As is typical for the hospital, things don't get done as quickly on the weekends.  Pulm ordered an echocardiogram to check for pulmonary hypertension (always a concern), but only emergency echos are done on the weekend.  They want to talk to CHOP about her thoracic insufficiency, but that can't be done until Monday.   Pulm did increase her pressure support to the highest setting in hopes that putting more air in her lungs will help.  We will hopefully see a difference tonight. If not, there will be many ...

Abby came back to Hopkins last night after talking to pulmonology.  Her respiratory rates have been very high again when doing literally nothing.  She was admitted to the floor.   It was a Rough night. The respiratory rate was 68-70 sustained on bipap, when she is typically in the 20s while sleeping on it.   Heart rate was in 150s.  She was very asleep, but so fast.  When we repositioned her, she woke up and slowed down a little, but then went right back to where she was when she was sleep.  Pulm came in and saw it too.  They called the attending and increased her breathe rate on the vent to 14, but it didn't help. We thought they might send her to the picu.   10 min off bipap this morning and she is 70 laying in bed, so she is back on and getting a treatment.  Hopefully it will be better after that.  They won't let her eat or drink on bipap so I hope her numbers come down so she can eat breakfast.  

So, the good news is that Abby came home today!  Discharge actually happened pretty quickly and what we ended up waiting on was the lunch that we had dalready ordered and the respiratory treatments she was due to get at 12.  As soon as they were over, we headed out!  Caleb was surprised and thrilled that we were home (we never tell him unless we are certain because we don't want him to be disappointed if something changes), and the kids happily played together before dinner.  Then they had a puppy visit! Unfortunately, the bad news is that the doctors don't really know why the very high respiratory rates are happening.  They were able to rule out viruses and high CO2 levels, but they weren't able to pinpoint the cause.  The pulmonologist we saw used the dreaded words, "Progression of Disease."  I seriously almost hear "dun dun DUN!" music each time it is said because it's such an ominous phrase.  It is certainly not something the parent of a med...

I brought Abby to the Hopkins ER this evening at the request of her pulmonologist.  I called her this morning to fill her in on the weekend and to tell her it was continuing.  Then, I let her know that even on bipap, her respiratory rate is still very high, which was new to us.  Usually, bipap does the trick.   She told us to come here, so here we are.  Hopkins has great expedited ER time when the doctor calls ahead for you, so we went almost immediately to a room, and then she got a chest X-ray shortly after.   The X-ray showed that the Right lung is diminished, which isn't surprising.  "Noticeable deformity of spine and ribs."  Oh my gosh!  Seriously?  We had no idea! 🤣. That one made me chuckle.  I really hope the radiologist didn't think that was new information for us! An IV was finally put in on the 3rd try and her blood gas (CO2 level) was good, so that was reassuring.  But her respiratory rates and heart rate are still...

Our CHOP appointments last week were productive.  Her plastic surgeon was thrilled with the progress of her wound.  It should be closed by the end of the month!  We will see him in mid-October and if everything goes well, Abby will be cleared to proceed with the VEPTR reinsertion!! We had some fun at the aquarium after her appointment.  Abby really loved being out and it was the perfect scenario--the place was empty!! She had her MRI the next day and recovered well from that.  We came home Thursday night to a very happy boy and dog! This weekend has been kind of rough, sadly.  Abby's respiratory rates have been very high and her saturation levels have been low...we want them the other way around!  She's been on her bipap a lot this weekend and we have had to stay inside.  Even then, her respiratory rates have been in the 70s.  She has been SO out of breath and has been really frustrated with herself. She is literally gasping for breath while ...

This week is going to be a busy one around here.  Abby has 2 appointments in Baltimore on Wednesday.  She needs her hearing aid mold redone (it squeals constantly!) and she has a pulmonology appointment.  Not too much has changed with her breathing--she is usually still requiring daytime bipap, and sometimes needs it twice during the day.  There doesn't seem to be a rhyme or reason to it, except that it is always in the afternoon.  We have kept the temperature in our house lower in hopes that heat will not be a factor, but the weather has really been pretty mild anyway.  I don't think our pulmonologist will change much at this appointment, since there really haven't been improvements.  I have a feeling we will just "stay the course" with daytime bipap as needed, respiratory treatments and airway clearance 4x a day, and inhaled steroids 2x a day. On Friday, we have appointments at CHOP with the infectious disease team and our orthopedic team.  Th...

We went into this pulmonology appointment today so nervous and anxious about what would be discussed.  As you all know, Abby's respiratory status has really declined with no indication that it is going to improve.  It's more obvious to us every day that the rib-to-rib VEPTR is the key to Abby's lung volumes improving, but that won't be able to be done for quite some time because her body has to heal from the trauma of the last year.  We discussed that today with our pulmonologist and she is hoping that she can provide support to Abby as a bridge until the rib-to-rib VEPTR is able to be put back in--whenever that may be. The question is, how much support is Abby going to need?  She's needing additional bipap during the day now and seems to decline after every surgery...and she has another one next week.  What will she be like after that?  The bottom line is that we aren't sure how much more her little body can take, or how long she can continue with such l...

The questions are written, the pulmonary data is collected, and the CHOP records are in a stack.  Physically, we are ready for tomorrow's pulmonary appointment. Emotionally, we are a wreck.  Tomorrow will hopefully answer a lot of questions, but it could also involve some really difficult conversations.  We are anxious. Thankfully, today was full of visitors and fun! This morning, Abby's teacher and assistant came for a tea party and they even brought a craft!   We all made very fancy hats--even Caleb!  Later, her friend Facetimed her from Sweet Frog to get her order, then delivered!  They had a great time playing and I got to spend some time catching up with my friend (her mom)!   Abby's appointment is at 12 tomorrow.  Please join us in praying for lots of answered questions and positive news.  

Abby woke up really well after surgery and was watching Mary Poppins in the PACU.  We were happy to be able to go back to our room on the 4th floor instead of the PICU!   We are here for a few more days while we figure out Abby's breathing once again.  It's been mentioned that this respiratory rate of 80-100 breaths per minute might be the new normal, which I can't accept!  I didn't even like the new normal of 60 bpm!!  If her rate increases after every surgery...well, we can't have that. The pulmonologist reiterated the concern of her catching a minor cold being very bad for her.  Looking at her tiny little lungs on an X-ray today was disheartening. Caleb was able to go down to the atrium today for Video Game Day with Microsoft.  He was pretty much in heaven playing Minecraft for 2 hours!  I was shocked that there were open gaming systems the entire time we were there!  I thought it was going to be packed!  He also got a nice drawstring...