Lots of updates!

Zadie had her bronchoscopy/sleep endoscopy in March at CHOP.  Dr. Javia said her airway looks great, with no obvious issues!  This is good news, but also bad news in a way.  At least if there was an obvious issue, there might be an explanation for her obstructive sleep apnea (OSA).  But, as it is, there are no clear reasons.  Dr. Javia did say that her tonsils were bigger than the last time he saw them (which was when he removed her adenoids.)  That's the last possibility for a cause for her OSA, other than just a hypotonia of her airway, which is something that can't be surgically corrected, she'd just have to grow out of it.  We are taking her to see him this coming week so he can look at her tonsils again and think about having them removed.  We'll be pretty upset if she has to go under for another surgery, when he could have taken her tonsils out when he was in taking out her adenoids in September, but at the time, he didn't think it was necessary.  Dr. Javia also said that there is the possibility of decannulating (removing the trach) and putting Zadie on CPAP.  We are very interested in this possibility.  I can't imagine it will be easy to get her to wear a mask on her face to bed, but I know other kids do it.

A couple of weeks after the CHOP visit, Zadie developed what looked like a pimple on her belly, right under her Gtube site.  It got bigger and redder and I ended up taking her to the doctor.  He took a swab of the pus inside and put her on antibiotics.  It turned out to be a staph infection.

Staph infection by Gtube

She ended up having a reaction to the antibiotic, Bactrim, so we stopped that, but her pediatrician felt that she'd been on it long enough and it was ok to stop.  

Red cheeks - reaction to Bactrim

Rash on chest that spread everywhere - reaction to Bactrim

Meanwhile, she was weighed when she went to the doctor for the staph infection, and she had gained weight since January, when she went to see the GI!  Between the weight gain and the fact that the Gtube site was getting yucky (and, I imagine, contributed to the infection), we decided to pull out the tube.  We waited until morning so that her stomach was empty, and just pulled it out.  It was a very exciting day.  I wasn't sure what we'd have to do to keep it from leaking, but it closed up very quickly, within a few hours, and we never had any leakage from her stomach.  Since she had some granuloma (scar tissue) built up around the site, it's not looking the prettiest, but I'm hoping as she grows that the scar tissue will lessen and be less noticeable.

One tube down, one to go!

In other news, Zadie has had two evaluations with the child study team from the school district so far.  One was with the speech pathologist and the other was with the learning consultant.  From what I understand, she did very well in the evaluations, which is great, but it also means she most likely won't qualify for the district pre-school.  It is pretty impressive that a kid who has had so many factors against her barely even has a speech delay.  Both Pierre Robin Sequence and having a trach are major causes of speech delays and issues.  But she is doing really well!  I'm back to work at the daycare now and when her nurse went on vacation last week, I took her to work with me.  Even though the class I have is a little younger than her, she wanted to stay with me and she did very well with the whole preschool/daycare thing.  She loved wearing her backpack and having a lunch box and she loved playing on the playground with the other kids.  One way or another, by the fall, I would like her in a preschool so that she gets out in the world!  She'll be turning 3 next month, so her speech therapy with Early Intervention will end at that point.

She's currently obsessed with Frozen, just like every other little girl in the world.  She's going to have breakfast with Elsa tomorrow morning, which should be a lot of fun!

I'll leave you with a picture of Zadie and her big brother and baby sister from a photo shoot we did about a month ago.  Super cute!

Jonathan, Beatrix and Zadie

Early Intervention transition

It's hard to believe, but Zadie will be 3 years old in less than four months!  Since Early Intervention only covers children until they're 3, we had the option of getting her evaluated to transition to the school system for preschool.  We decided to see if she qualified.  We had the initial meeting with the child study team for the school system last week, and they agreed to do a psychological, social, educational and speech evaluation on her.

We're not sure whether she will qualify, since she really is doing quite well, with speech being her only issue at this point, but her medical issues may help her get qualified.

In nursing coverage news, the insurance company approved Zadie's nursing hours until April, when they will be reviewed again.  They usually approve in six month increments, so I'm not sure why it's a shorter period before another review.  I do wonder if it has to do with the bronchoscopy she'll be having in March; they might be waiting to hear the results of that.

I had posted this on Facebook and haven't mentioned it here yet, but Zadie's GI doctor has discontinued her overnight feeds!  She weighs over 30 pounds now, which is around the 50th percentile.  This is amazing progress considering just over a year ago, she was in the 5th percentile for weight.  We have to make sure that she at least maintains and ideally gains some weight in the next month or two.  If she does, we have the option of completely removing her feeding tube!  This is such exciting news!  We will most likely keep the feeding tube for at least some time, since she still has the trach and will be having procedures and possibly surgeries in the future.  The feeding tube is a very good thing to have at times.  I'm really proud of how far she has come.  When she was born, she couldn't even suck from a bottle, and there were several months where she took nothing by mouth.  Just 2 years later, the fact that she's thriving without the aid of the feeding tube is really remarkable.

Zadie's current favorite word is NO and her current obsessions are turning lights on and off and closing doors!

Zadie turned 2!

Zadie turned 2 on Friday!  She had a fun day, spent some time at Jonathan's preschool, then we went to Chuck E. Cheese and then out to dinner.  For the first night in over a week, she didn't need oxygen while sleeping.  Yesterday, Grandpa and Uncle Sammy came over.  I made cupcakes and we attempted to sing happy birthday, but it was confirmed that she really doesn't like people singing happy birthday to her.  She gets very upset!

Just an update on her progress...she is talking a lot. She still gets speech therapy once a week, and is saying all sorts of words, including uh-oh, oh no, water, wow, mouse, and the old favorites mama, dada, baba and baby.  She's getting big, too.  She went to see her pulmonologist the other day and she's now 24 pounds, which is great.  She's moved up in percentiles.  She's eating more and more.  She's also getting very fresh, scuffling with Jonathan and throwing toys.  Typical 2 year old, I guess!

Fresh out of the oven May 31, 2011

May 31, 2013

You can really see the difference in Zadie's chin from her newborn picture to her current picture.  One of the things about Pierre Robin Sequence that made it difficult for Zadie to eat and breathe was the recessed chin (micrognathia).  If she was born at another hospital, she probably would have had a jaw distraction, but St. Barnabas doesn't really do them and they opted to let her chin grow out on its own.  A plastic surgeon at CHOP wanted to do the distraction later on in order for her to lose the trach, but we decided to wait and see what happened.  So far, I think we made the right decision.  Her chin has grown out a lot and her trach is capped all day long, meaning she's breathing through her mouth and nose.  We're hoping she is decannulated (loses the trach) this summer!

Hearing test

I took Zadie to get a hearing test on Friday.  Her ENT at CHOP wanted it done, and rather than trekking all the way to Philadelphia, we were able to visit their specialty center in Princeton.  

The audiologist checked the placement of Zadie's ear tubes first.  The left one was in place and unblocked.  She could see the right one in her ear, but it's either blocked or not in place.  Therefore, she has some hearing loss in that ear (which is hopefully temporary).  

I talked to the ENT's office, and we are going to try to unblock the tube by dropping hydrogen peroxide into her ear 2-3 times a day for up to a week (or if she starts showing signs of pain, we will stop sooner).  Once we are done with that, we will be dropping ofloxacin drops in her ear for 5 days.  Hopefully this will unblock the tube.  We are seeing the ENT in June anyway, so by then, he'll be able to look in her ear and see what needs to be done.  

In other news, Zadie is doing great wearing her Passy Muir valve.  She wears it all day while she's awake (we take it off for naptime) and she's even building up the time that she wears a cap (which completely blocks the trach and forces her to breathe only through her mouth and nose.)  Since she's been wearing the Passy Muir so much, she's been vocalizing a lot, and is really building up her vocabulary!  I'm hoping that by the time we see the ENT in June, she is wearing the cap all day and we can schedule a sleep study to see if we can get rid of the trach!

Early Intervention Annual Review

Zadie had her annual review yesterday for Early Intervention.  Early Intervention is a state program that is designed to help kids with developmental delays catch up in time for school.  She's been receiving services through EI for over a year now.  She gets speech therapy and occupational therapy.  We had two meetings to update things yesterday.  During the first one, a test called BDI (Battelle Developmental Inventory) was administered.  The therapist asked me a bunch of questions about different things that Zadie could or couldn't do and gave Z tasks to try to do, like stacking blocks and using nesting cups.

Little Z did great on the test!  She improved greatly since the first test was given over a year ago.  All of her scores improved.  In fact, based on that test alone, she does not qualify for EI.  However, since she has the trach and the related speech delay, they used a clinical assessment to determine that she is still eligible to receive services.  She'll continue receiving speech therapy once a week and occupational therapy will be reduced to once a month.  She babbles a lot and she is starting to say words.  The three big ones right now are mama, dada and baba.  She is starting to mimic sounds we make, and she said "duck" yesterday with the therapist while looking at a book with ducks.  She's still signing pretty regularly and still picking up new signs, so all in all, she communicates pretty well.