Life as a Leach

I've determined that Abby's pancreas is a drama queen, just like her.  As I've mentioned, Abby had what we thought was the stomach bug. When she just kept throwing up everything (even 6 hours after drinking it) with absolutely no other symptoms (she's a wild child!), we thought there might be more to it.  Our GI said to keep in touch if it didn't get better, so I sent her an email over the weekend.  She ordered blood work, which came back today as elevated liver functioning.  Of the two enzymes that point directly to pancreatitis, one is actually below normal and the other is a slow test that hasn't come back yet.  So... Abby's going to have an ultrasound tomorrow to check for gall stones.  Tonight, I put her on just pedialyte.  I started at a very slow rate and am increasing by 5 ml/hour each hour until I get to 50 ml/hour.  (It's gonna be a long night!)  If she doesn't tolerate that, then we are to head to the ER in the morning with the e...

That's what we hear from Abby all day long because the girl can't hear a thing!  We were hoping that this hearing loss was a result of a malfunctioning tube, but Dr. T. checked out her ears during her jaw surgery and everything was a-ok...except that her left ear now has a substantial hole in it that will need to be fixed when she is older. So since the hearing loss wasn't fluid related, we were anxious to hear what the audiology report was.  Abby had a hearing test yesterday and cooperated very well.  The audiologist was very good and worked hard to get all of the information she needed.  I feel confident that it was a valid report. Abby's right ear has always been worse, but this time her left  ear showed moderate to severe hearing loss and her right ear showed moderate.  Her left ear has never been anything but normal (her last test was in July), so Dr. T. thinks this hole is causing more problems than he thought.  Because of her age and the gro...

I got a call this morning from the nurse practitioner who works with Dr. C at CHOP.  After I called them last week to let them know about the upcoming jaw surgery, Dr. C spoke with the anesthesia team to discuss whether Abby's December 30th VEPTR date would still be okay.  Everyone felt like Abby needed to have everything related to the jaw completed before the VEPTR to keep her as safe as possible during anesthesia.  Since the metal bars will stay in until early March, the VEPTR will need to be postponed until early April.  We also tentatively scheduled the left side of the VEPTR for early August...but we know all too well how that can all change! I should be used to this by now... But, I am actually super glad that we have a little break after the jaw surgery instead of jumping right into the VEPTR.  I just don't want to bump these anymore after this.  The VEPTR needs to be done.  And, we have a little girl with big plans to be Elsa in Disney Wor...

Abby has been losing speech sounds and asking us to repeat what we say a lot.  Since she doesn't have an audiology appointment until December, I had the pediatrician check her out.  Turns out, her tubes are sideways in her ear canal, blocking her ear drum!  Yikes.  They aren't being very helpful that way and are actually causing trouble, so they need to come out.  I'm going to call tomorrow to see if they can remove and replace them during her g-tube closure next Thursday.  This will be her 4th set of tubes in 2 years.  I kind of doubt if that will all work out to get it taken care of next week, but a girl can dream! In other, but related, news...I found my first gray hair the other day.  I named it Abby.

I never want blogging to become a chore.  When I don't have time or don't feel like writing, I don't.  That's why I'm not a money-making blogger; I can't commit to blogging that much.  I like to write and it's therapeutic for me, but it is also put to the side when other things take precedence. That being said, I've been short on time the last few weeks during our travels to and from Philadelphia and I have lots of medical stuff to share! *  Abby had a dynamic MRI done on May 29th.  Our concern wasn't the MRI itself, but the intubation that needed to happen.  She was successfully intubated on the first try in just 15 minutes!!  We had anesthesiologists from the "Difficult Airway Team" (that in itself makes me happy!!) who were excellent.  They took detailed notes of what they did and what they saw the whole way down, and I couldn't have been more pleased.  Abby came out of anesthesia just fine too.  Once she woke up, she was in a ...

We met with anesthesia today.  Although we weren't supposed to meet with a doctor (we didn't know this until the day before and were rather irritated about that!), once the nurse practitioner heard about her airway issues, she got an anesthesiologist to come speak to us.  We were really impressed with the doctor, who acknowledged how educated we are on Abby's issues and immediately started working on the game plan.  In just a few hours, she had orchestrated everything and was able to secure an OR so that Abby could be intubated there rather than in the MRI room.  This is much safer and allows the anesthesiologist to have everything he/she needs to perform a fiberoptic intubation.  This is the only type of intubation that Abby can have (she has never been successfully intubated, but our Ent tested this out and felt that this was our best bet.), so having everything set up is very important.  We were pleased with how serious this doctor took Abby's case and w...

Pictures from the sleep study! Abby was really great about getting everything set up so she could "sleep," and she didn't fuss at all over all of the stuff on her. In fact, she even wanted to take pictures with my phone! The last step in the long set-up process was putting in a nasal cannula to measure CO2 levels as she slept.  Abby absolutely HATED this!  After Abby tore it off several times, I had to hold Abby down while the tech taped it to her face.  She was so mad at me! She eventually cried herself to sleep settled down and fell asleep.  She snored all night and coughed quite a bit.  I don't really know if the snoring is normal for her, because we all sleep with air purifiers that drown out that noise.  But we'll see what they say.  

We got some not so great news yesterday. Our pulmonologist called last night.  (That concerned me right there--it's usually a nurse!). We got the results back from Abby's sleep study, and it showed "severe upper respiratory obstruction." She had an average of 12 episodes an hour where she stopped breathing, as well as 2 central apneas.  . We have never had anything like this show up on previous sleep studies. Our  pulmonologist is recommending cpap and /or getting her tonsils and adenoids removed--before veptr surgery! Previously, our pulmonologist was very hesitant about VEPTR. She said last night that this is all showing us that we really don't have a choice but to go for the VEPTR. We were not expecting this sort of news at all!!  This sleep study was supposed to be just baseline information for her surgery, so we were pretty caught off-guard with the bad news.  It makes me want to sleep in her room!  We have also been told to monitor her oxygen level...

So much has happened and changed in the last week...where to begin?   We saw our feeding therapist last Tuesday and decided to end therapy until after surgery.  This made me SO sad because Abby has made so much progress in the last eight months, but we really have to focus on getting as many calories as possible into Abby without burning more chewing higher textures.  We have met with a nutritionist several times last week and she gave us some good ideas.  Our goal now is 1,800-2,000 calories a day...typically kids eat 1,000-1,400.  The feeding GI (whom we love) met with us to discuss putting Abby on an appetite stimulant.  They had always been reluctant in the past, but now everyone agreed that this was the best thing for her.  She's been on it for 3 days now and we haven't seen much of a difference, but we are still working her up to the full dose.   Her weight was down at that appointment, and I was so concerned.  How could she be l...

We are home from our visit at CHOP.  I'm going to apologize in advance if this isn't my usual style of post.  I'm exhausted, both mentally and physically! Abby ended up having five appointments/tests done:  CT scan, Pulmonary Functioning Test, X-ray, and appointments with the pulmonologist and orthopedic surgeon.  We managed to get to our appointments on time on Thursday.  After debating whether to call a cab, drive, or even walk, we decided to see if we could drive in.  It ended up being fine. After the CT and PFT, we saw Dr. M, the pulmonologist.  He went over the results and shared his thoughts, but deferred to Dr. C, who would be the one doing the surgery.  So we really had to wait until Friday to get the answers we wanted. We saw Dr C this morning, who went over the CT and PFT, but then asked why we didn't get an xray.  (We were told during the scheduling process than an xray wasn't necessary...)  So, back to radiology we went...

I have one tough little girl!  She deals with so many doctors' appointments and medical procedures.  Through it all, she has a smile on her face and a hop in her step!  I'm amazed that a child with so many obstacles in her life is still so happy! We've recently started talking openly about Abby's ribs with her and how Dr. C is going to fix her ribs.  We're obviously not going into detail about the surgery, but we wanted to start an open discussion so that she would get more used to the idea if and when we actually move forward with surgery.  It seems to be helping, because she's been telling everyone that Dr. C is going to fix her ribs!  (Say it in a sassy voice with your hands on your hips to get the full effect!) Today's adventure was ophthalmology, where we discovered that Abby's eyes are still crossing quite a bit.  After discussing giving her bifocals (for a three year old!!! ) he finally decided that it would be too hard to explain to her how...

Since Abby's tubes were put in her ears January 15th, she has continued to have a lot of ear issues.  Her ears began bleeding again well after they had stopped post-op, so our ENT put her back on Ciprodex.  (If you are looking for a good stock purchase, Ciprodex might be it.  I think Abby is responsible for most of its financial success.)  The nurse practitioner said to call if she wasn't better in a week, and she would see her when we were coming up for Abby's feeding evaluation on Thursday. Welp, they were no better, despite pouring Ciprodex into her ears twice a day.  In fact, her right one was definitely worse.  So I called ENT on Wednesday to make an appointment for Thursday afternoon.  Dr. T. wouldn't be able to see her because he was in surgery all day, but the NP could.  We went straight there from the feeding clinic at MWPH (a 10 minute drive with an extra 20 minutes of driving through the parking garage trying to find a space!!!) ...

We headed to Philly yesterday for an appointment this morning with pulmonology.  We went through a whole bunch of medical history with the pulmonologist, then began discussing the upcoming pulmonary functioning test.  Our ENT was adamant that we have the test done in the OR in case something went wrong and she needed to be intubated.  This pulmonologist wasn't able to do that, so we decided to have Abby do the test awake.  It may not be the most accurate the first time, but we figure we'll try and at the very least she'll start to get used to it...she'll be doing them the rest of her life! This pulmonologist shared his perspective on having the VEPTR surgery , so it ended up being a "bigger" appointment than we anticipated.  From his standpoint, he feels that doing the surgery before the breathing becomes an issue is better.  He explained that once she starts to lose her pulmonary functioning, she most likely won't get it back.  This makes sense, and...

Last week, we went to audiology and ENT.  We were following up on Abby's not-so-great hearing test from last March because our ENT wanted to make sure that her hearing really was getting worse before we put her under anesthesia for tubes again.  With the airway being such a huge concern, we really have to weigh pros and cons.  In March, her left ear was border-line and her right ear was showing moderate hearing loss. Well, the decision this time was pretty quick and easy because Abby's hearing in her left ear was significantly worse and the right ear stayed equally as bad as it was in March.  So now that Abby's showing moderate hearing loss in both ears and we're noticing issues at home as well, our ENT said it really was time to do tubes again.  This will be set #3 in two years.   He wants to get the tubes in soon because, as we know, the hearing issues also affect the speech.  However, we're waiting until after our upcoming pulmonology appoint...

I have to say, genetics went much  better than I expected!  I actually really liked the geneticist, as well as the fellows that also came in.  They were all very social with Abby and identified themselves as pediatricians  with a specialty in genetics.  BIG distinction there. The only picture they took of her was for her file so that they could put a face with a name...just a regular old cheesy grin!  No pictures of perceived imperfections, or even mention of them. Another thing I really appreciated was that the geneticist recognized that I have a lot of knowledge about Abby's syndrome and didn't try to talk down to me.  She answered questions and explained things well, but she also acknowledged that I may even have a better understanding of CCMS than she does!  She especially loved how much contact I have had with other CCMS kids and was very interested to hear how they were doing. We talked at length about the choice we have to join a rese...

Abby had three appointments on Tuesday:  audiology, ENT, and feeding therapy.  It was a long day full of information...some good, some not so great. Audiology was first.  Abby gets her hearing checked every six months because kids with Pierre Robin's Sequence (Abby's secondary diagnosis, since CCMS isn't studied) are at a much higher risk for hearing loss than other kids.  In fact, I just read an article that said that PRS kids have a 77% higher risk of developing significant hearing loss than other kids who have isolated cleft palate.  Some of this is fluid-related, which is usually fixed with tubes, but it can also be because of "interesting" anatomy. If you've been reading this blog for long, you know that Abby has battled many ear infections.  The anatomy of her ears is especially "interesting" and small!   It makes it very hard for tubes to do their job.  Our ENT finally took the tube out for a few months and put it back in during her la...

Abby has a genetics appointment tomorrow. I am so not excited about this. We saw a geneticist at UMMC many times when Abby was a baby, and I just started feeling like it was pointless.  Countless genetic tests showed absolutely no abnormalities.   We even did testing that isn't approved in the U.S. and had to be sent to Canada!  (I really am not sure how our insurance paid for that one, but we never got a bill!)  Her genetic make-up isn't going to change, and we can't find any issues.  So why keep going? Plus, I am not a huge fan of someone whose job it is to point out every single imperfection in my daughter.  When Abby was a newborn in the NICU, I had to endure a lengthy examination by two geneticists where they oohed and ahhed over all of her abnormalities.  The last thing a stressed-out mom wants to hear is a laundry list of the things that aren't "right" with her daughter--and there's something downright wrong about hearing almost exciteme...

We aren't sure what Abby has/had yet, but we do know that it is NOT C-DIFF!!! The only culture that has come back so far is C-Diff, so we are still waiting for the other parasite testing and blood work.  Regardless, this is good news!!! Now maybe I can stop obsessively cleaning everything Abby touches with bleach. Thank you, Lord, for answering our prayers.  And thank you, my prayer warriors! Now if only we can get our dryer fixed...Sears said they won't be able to come out for a week and a half!  Most of the stuff waiting to be washed is now stuff that wasn't necessarily "dirty," (coats, gloves, hats, etc.) but they are now since they've been in baskets with other dirty stuff!  Oh well.

I feel like pretty  much every blog recently starts with an apology for a delay in posting.  But, such is life when your life is busy like mine! This time, my excuse for not writing sooner revolves around bleach, disinfectant, and laundry. But let me back up a bit. I took Abby to the GI on Friday, and she feels like it is C-Diff.  Boo.  She had Abby do blood work while we were there, and we should get all of the results for the tests they've run either Monday or Tuesday  I'm hoping for Monday! Since she did feel like it was C-Diff, our GI went ahead and put us on precautions.  Basically, that means that Abby isn't going anywhere for a while, we wear gloves when changing her diaper, giving her a bath, etc., we are washing our hands with antibacterial soap and hot water very, very often (C-Diff is alcohol resistant, so hand sanitizer doesn't work), and we are cleaning everything with bleach.  When I say everything, I mean everything.   It is...

I'm going to give the very short version of my very long day today.  I'll also spare you some of the grossest details.  Let's just say that this was not the most glamorous day ever. In the middle of the night last night, Abby woke up writhing in pain and was inconsolable.  It took forever to calm her down and we finally disconnected her feed for the rest of the night.  She was up for several hours in severe pain, but she finally fell asleep and seemed calm.  I'd already planned on checking with her pediatrician in the morning. This morning when she woke up, there was blood in her diaper.  Many phone calls, another bloody diaper, and a popped g-tube later, my pediatrician told us to get in touch with GI to be seen. Many phone calls, two trips to the outpatient lab, and a really gross collection later, our GI told us to come in tomorrow--even though we already had a scheduled routine appointment on Tuesday.  These issues, coupled with the delayed ...