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Adventures in Philadelphia

We made it home from Philadelphia!  I didn't bring my camera with me because it was such a quick trip and I didn't want to have to worry about it while we were in the hospital (I do NOT leave it in the car!!), so I don't have any pictures to share.  But we did have some adventures!

After driving 4 hours after church and hitting some traffic at the Bay Bridge (shocker) and on 95, we made it to Philadelphia with really no issues.  We stayed at the Double Tree because we didn't get into the RMH near the hospital.  Sadness.  But the Double Tree was very nice and RMH got us a good rate.  We checked in, got settled, then headed out to find the hospital so that we would know where to go in the (early) morning.  We grabbed dinner at Chipotle and brought it back to the hotel, then decided to let the kids go for a late evening swim at the pool.  After all, swimming would tire them out and make for an easy bedtime, right?  Ha!!!!

I think we all finally fell asleep around 10:30. Abby may have been the last one asleep.  Despite having two queen beds, we finally had to put her on the floor because she wouldn't leave Caleb alone.  He fell asleep soon after we did that, but Abby was wired.  Apparently chlorine makes her hyper.

The alarm went off at 5:45, and I'm proud to say that we made our goal of a 7 a.m. departure!!  Woo hoo!  (This was mostly because we didn't feed Abby before we left; we decided to wait until after the appointment, since it was so early.  That came back to bite us, but more on that later!)

CHOP is really very easy to get to.  Although I know JHH better, I was pleasantly surprised at how simple it was to get to our building, park, and find the orthopedic department.  It's actually much easier than JHH!  We settled in, filled out a lengthy registration packet, met with a researcher (who gave us a greasy used-car salesman speech about wanting to draw blood for genetic testing--no thanks!), talked with a Physician's Assistant, tracked down her medical records that were sent there but not put in her file, and finally met with the doctor.

He. Is. Brilliant.

It was SO nice to finally have someone who knows CCMS, has seen these kids' ribs, and who could actually teach us something rather than us educating the doctor.  This is the first time that any of our doctors has ever had more than one CCMS patient.  He's as much a CCMS expert as anyone in the world!  He has operated on 6 other kids who have CCMS.  6.  That's almost all of them!  Craziness.

She had some new xrays done today, as well as a CT scan.  We didn't think we would be doing all of that, but I'm glad we got so much information in one day.  The CT scan is usually done under sedation at this age, but we wanted to try without it because of the dangers for Abby.  She was perfect!  We had no issues, and we attribute that to the wonder Childlife Specialist who brought in a mini CT scan and showed Abby how it works beforehand.  It was a great experience, thank goodness!

So, basically, here's what he said:

The gaps in her ribs are not currently affecting her respiratory status.  Her respiratory rate was a little high for her age, but she is overall doing very well.  He is more concerned about how her ribs will grow and whether or not they will constrict her lung functioning.  One of the concerns is that the gaps will overlap and constrict the growth.  Right now, the ribs expand and the pieces move out as she inhales.  He doesn't know how long they will continue to do this.

He was impressed with how well she was doing.  He said that she is doing better than any other CCMS kid he's seen, and that, if there is such a thing, she seems to have a "mild case" of CCMS.  If this is mild, I really don't want to see more severe!

He will be following Abby, and we'll go back in 6 months.  At that time, she'll have a pulmonary functioning test under sedation...which adds a whole new level of anxiety and issues.  He'll also check on her rib gaps at that time.  I'm figuring on seeing him about twice a year, unless something changes.

If there is a change, then we'll turn to the VEPTR.  But hopefully we won't have to!

One interesting little tidbit...Abby's spine is the length of an average-sized newborn baby.  Hmm.  Who knew?  He then turned to me and asked if anyone has used the term "Short Stature." Yeah, yeah, yeah.  We know, we know.  The girl is going to be a bit on the petite side.

We are very, very glad we went.  It was worth it just to hear him say that he knows what CCMS ribs look like.  We've waited a long time for that kind of information!  Oh, and she's totally safe to do whatever she wants.  Her ribs are fine.  Great news!!

So, so thankful.  Today was a good day.  Long, but good!


Comments

Mary Lou said…
Julie....
"Abby was wired. Apparently chlorine makes her hyper.". Crack. Me. Up!! ;-D
"It was SO nice to finally have someone who knows CCMS, has seen these kids' ribs, and who could actually teach us something rather than us educating the doctor. This is the first time that any of our doctors has ever had more than one CCMS patient. He's as much a CCMS expert as anyone in the world! He has operated on 6 other kids who have CCMS. 6. That's almost all of them! Craziness.". Wow!! ;)
"The gaps in her ribs are not currently affecting her respiratory status. Her respiratory rate was a little high for her age, but she is overall doing very well.". "He was impressed with how well she was doing. He said that she is doing better than any other CCMS kid he's seen, and that, if there is such a thing, she seems to have a 'mild case' of CCMS.". "She's totally safe to do whatever she wants. Her ribs are fine.". Thanks be to God!! ;-D
Hey. "short" people are the best!! They often think big, dream big, care big, and possess the biggest hearts!! Trust me. I have known of many "short" people like this!! ;)
--Raelyn

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