ENT visit

We took Zadie to CHOP in Philly today for a visit with her ENT, Dr. Javia.  After her bronchoscopy and sleep endoscopy in March, he said there was a possibility that the tonsils need to come out, since they were pretty large.  This was pretty disappointing since she was just in the OR in September to have her adenoids removed.  Apparently, at that time, the tonsils were so small that he didn't feel they needed to come out.  Recovery from a tonsillectomy is much worse than from just an adenoidectomy, and I do appreciate that he only does what he feels is absolutely necessary.

However, now that Dr. Javia has confirmed that he feels she needs a tonsillectomy, I do wish he had just taken them out in September.  Two birds, one stone?  She's scheduled for the tonsillectomy for May 6, which is just over three weeks away.  After that, we'll be able to schedule her for another sleep study to see if there is any improvement to the obstructive sleep apnea.  Since her airway looked beautiful in the March scope, if the tonsils don't prove to be the problem, then hypotonia of the upper airway will be the likely culprit.  There is no surgical repair for hypotonia, it is just something that Zadie will have to outgrow.  Since there's no other reason for the trach right now besides the OSA, Dr. Javia said that decannulating (removing the trach) and putting her on a CPAP mask is a possibility.  She would have to get used to the CPAP mask before decann, though.  I'm not sure how she'll do with a mask on her face!

I hate to put Zadie through another surgery!  I know it's for the best and she won't remember much (the older she gets, the less we can say "she won't remember any of this!") but it's just no fun.  The older she gets, the worse things like surgery and hospitalizations and blood draws get.  But she's a trouper and soon enough, this will be in her rearview mirror.

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!

ENT visit

This past Monday, we took Zadie to see her ENT, Dr. Javia.  Since she had failed her sleep study so horribly, we went to see what the next steps would be.  Unfortunately, Dr. Javia doesn't really know WHY she failed the study so badly.  It could be hypotonia of the base of her tongue/upper airway, so that it falls back and partially blocks her airway while she's sleeping.  There is a possibility of more granulation or scar tissue since the adenoidectomy in September.

So Zadie will be having a sleep endoscopy and a bronchoscopy in March.  The sleep endoscopy is similar to a bronchoscopy in that they're looking at her airway, but from what I understand, the anesthesia is a little different so that it mimics sleep so that they can see what is going on inside when she's sleeping.  If they find any scar tissue, they can take it out then.  If nothing needs to be done, she should be able to go home that day.

Unfortunately, if it's the hypotonia, nothing surgically can be done and we would just need to wait for her to grow out of it.  And there is no way to know how long it would take for her to outgrow it.

Meanwhile, we are waiting to hear from the insurance company about her nursing hours.  The ENT's office sent the company the notes from this week's visit.  Since there are no plans for decannulation at this time, I am hoping that we will be able to keep at least some of our hours.

On another note, we saw a few other trached kids in the waiting room.  And we met a little girl (not trached) with PRS!

Zadie is a big sister! And other updates...

Zadie and Beatrix!

It's a while since I posted anything.  The big news in our house is the arrival of Beatrix Rose, Zadie's little sister!  Zadie really, really loves her and always wants to help with her.  Obviously, having Beatrix has made things a little more interesting around here.

A few days after Beatrix was born, Zadie went to Philly with her daddy to have her follow up sleep study.  Since she did badly on the one in July, her ENT, Dr. Javia, suggested we remove her adenoids.  He was hoping that they were the cause of her apnea.  We were hoping that the follow up sleep study would be improved enough that decannulation would be a real possibility.  Unfortunately, less than an hour into the study, the technician removed Zadie's cap.  From then on, she was sleeping as she does at home, with the trach open, and therefore not really getting any benefit out of the study.  It was pretty discouraging, since there was no point in being there any longer, but they stuck out the night (Zadie was sleeping already anyway).  We held out some hope for a positive result, but when I talked to the nurse a couple of days later, she told me that the results were much worse than the results from July!  The apnea hypopnea index (AHI), which is the number of recorded apneas or hypopneas per hour, was 12 in July and 75 in November!  The link I provided has a different measure of what is severe from what CHOP considers severe, but on either scale, 75 is severe.  And that's why they took the cap off.

No like sleep studies!

I don't know why it was so much worse.  She was a little congested, but not enough that we thought we should reschedule.  I really wonder if there were any errors made in setting up, but there's no way to know.  We will be heading down to see the ENT again; he wants to look at her airway in the office and then decide the next step - maybe another bronchoscopy or maybe another sleep study.  Neither is a really easy option.  Zadie would be put under for the bronchoscopy and sleep studies are really miserable, so I feel really bad she has to go through either (or both) again.  And, since Dr. Javia is at CHOP, it's a two hour drive to do anything.

Otherwise, Zadie is doing really well, health wise (knocking on wood).

Letter from insurance company

This past week, we were informed that our insurance denied Zadie's nursing hours (again).  Again, they said that Zadie's care was purely custodial, meaning basically babysitting, which is outrageous considering we can't leave her with just anyone.  Dr. Javia's office at CHOP took the first step in appealing with the peer-to-peer review.  They spoke to the medical director at the insurance company, but they still denied the hours.  The ENT's office then filed an expedited appeal on our behalf (they were really proactive about it, which I appreciate!) and the insurance company was supposed to give an answer by this past Friday.  Instead of an answer, they reinstated the nursing hours temporarily while the appeal went to medical review.  So, for now, Zadie has her nurses back.  I still haven't gotten the official denial letter from the insurance company.  The only correspondence I've seen from them is a really poorly written fax that was sent to the nursing agency.  It's kind of mind boggling how someone who apparently doesn't know the word tracheostomy (instead, it's referred to as a "tube in her neck for breathing") can determine whether anything is medically necessary!

"I do!"

Little Miss Independent wants to do EVERYTHING.  Every step of the pottying process (lifting the lid, flushing, turning off the light, etc.), pouring her own drinks, opening the door for the dogs. Her latest thing she wants to do is her own suctioning. She doesn't need suctioning too often anymore, but she usually needs it first thing in the morning. I do let her help a little bit. She also wants to be the one to pull her gauze out from under her trach every morning and night. She gets very upset if we don't let her do things, and says, "I do!"  Just a typical headstrong 2 year old with some atypical things she's learning to do!

Follow up on Sleep Study

We went to Philly on Monday to see Zadie's ENT to discuss the results of the sleep study and to talk about the plan going forward.  The nurse on the phone had told me that Zadie had moderate obstructive sleep apnea, but Dr. Javia told us it was actually severe.  He thinks the culprit might be her adenoids, so she is scheduled for an adenoidectomy on September 30.  Her tonsils have always looked fairly small, but there is a possibility that they will be taken out at the same time.  While she's under for that procedure (or procedures) she will also get a bronchoscopy to really look at the airway.  About 4-6 weeks after her surgery, she will get another sleep study.  Even though we'll already be in November by that point, Dr. Javia didn't totally shoot down the possibility of decannulation (losing the trach) this year, although he usually doesn't do it in the cold and flu season.  I'm not getting my hopes up for decannulation this year, though.  I think chances are pretty good it will have to wait until the spring.  It would have been nice for the trach to be out before the new baby comes, but it is what it is.

Zadie showing off her boo boo from getting blood drawn.