6 months since decannulation!

I haven't updated in about seven months. The big news since the last time I posted an update is that Zadie was decannulated (had her trach removed) in October 2014.  As I re-read what I wrote in September, I see I was resigned to the fact that Zadie was going to have her trach for another winter.  Thankfully, we saw a wonderful doctor, Dr. Atlas, who is a pulmonologist in at Goryeb Children's Hospital in Morristown.  At our first meeting, he seemed as confident as we were that Zadie didn't need the trach anymore.  We were given the blessing to begin capping 24 hours a day (previously it was only approved for waking hours) and Dr. Atlas encouraged us to leave Zadie's trach out for a few minutes each time we changed it.  We made an appointment for a few weeks later to see how she did and to possibly schedule a bronchoscopy/decannulation. 

A little more than a week before the appointment, I did a regular trach change and let Zadie run around with a "naked neck" for a few minutes.  Well, she enjoyed it so much that she wouldn't let me put the new trach back in.  It ended up to be about 15 minutes that she had the trach out.  She was doing fine, but until we were absolutely sure she was ready, we had to put the trach back in.  When I finally got her to let me put it back in, it wouldn't go in.  Her stoma had closed enough in that short of a time that the trach size she had been wearing for almost two years wouldn't go in.  I got a little anxious, but I got the backup trach in.  It was a Sunday, and since she was fine, I just waited until the next day to call the doctor.  When I did call the next day, I was able to get Zadie's follow up appointment moved up to that week. 

Since she was doing so well with everything, a date was scheduled for a bronchoscopy (a scope of her airway done under anesthesia) and possible decannulation: October 22, 2014.  She went in for her bronch and came out pretty upset, but trach free!

Not feeling too great right after waking up from anesthesia; a nurse once called it "anesthesia angry" and it is pretty accurate!

 

Dr. Atlas was a little concerned about the floppiness of her airway (something that Dr. Javia had mentioned in the past) so he couldn't guarantee that the trach would stay out, but we were hopeful!  After keeping an eye on her oxygen saturations in the recovery room for several hours, Zadie was moved to a room, still trach free!  The plan was to stay overnight and see how she did.  She did great, despite some puking and a fever.  I was worried this would cause them to keep her another day, but I think they viewed it as just post-anesthesia effects.  The most important part, her oxygen, was great!  She was discharged the next day and has been great since! 

Naked neck finally!!!

 

We have to see Dr. Atlas for a follow up appointment soon.  Her stoma is still open and most likely, she'll have to have it surgically closed.  Dr. Atlas said he would wait about a year before deciding to close it, which is the same amount of time that Dr. Javia said to wait.  It would have been nice to have a closed stoma before the summer, but I'd rather wait until the right time. 

MRI and beyond...

Since Zadie's last sleep study showed some central apnea (the first time in her life that central apnea has been even mentioned), the doctor wanted her to have an MRI of her brain to make sure there were no issues there that would possibly cause central apnea.  Being that she has a trach, she would have to be under general anesthesia for the MRI.  And because she'd already be under, they decided to do an MRI of her spine as well.

When we arrived for her MRI, they asked several questions about any implants or devices.  The trach Zadie normally wears, a Bivona, has metal in it, so that can not be worn in an MRI.  I was aware of this and had brought a back up in a different brand, Shiley, which is made of only plastic.  They also asked about the ear tubes Zadie has had since she was 11 months old.  Those tubes were put in during her cleft palate repair at St. Joe's Hospital in NJ, not at CHOP.  Apparently, some ear tubes have metal components in them, and no one thought to ask ahead of time for the operative report to confirm that there was no metal in them.  So, CHOP had to contact St. Joe's and send over a release signed by me to get the operative report.  That was really frustrating, but only turned out to be an hour or two delay.  She was in the MRI for about two hours, and was pretty cranky when she woke up.  Some positive feedback I received right away from the anesthesiologist was that he didn't see any apneas while she was under anesthesia.  He had mentioned ahead of time that if there were any apneas while she was under, there was a possibility of her having to stay overnight.  Thankfully, we avoided that.

Since we had the MRI on Friday and a follow up appointment with a pulmonologist on the following Monday, both in the Philly area, we decided to make a weekend out of it and drove out to Lancaster, PA.  Zadie was really hungry and thirsty when she woke up, and she drank juice and ate a bunch of cheez its, and it probably was a little too much too fast and she puked all over herself in the car.  But, that seemed to be the only ill effect of the day.

We had a great weekend in Lancaster, and woke up early on Monday to be back for the appointment with the pulmonologist.  Right after we left, I got a phone call from the sleep doctor's office with the results of the MRI.  They were normal, thankfully!  I mentioned to the woman that we had an appointment for a follow up with the pulmonologist and she checked the doctor's schedule and said we didn't have an appointment.  I had a funny feeling that was the case, since I hadn't received a confirmation call.  So, instead of going to an appointment, we had a fun last day of our trip and spent the day in Hershey.

We were able to schedule the follow up appointment with a doctor in New Jersey, and we're going to that tomorrow.  The ENT's office wants the pulmonologist to look at the results of the sleep study and make a recommendation for further steps.  Since her O2 levels were low for part of the study, the pulmonologist might just want her to be on some nebulizer treatments on a regular basis to open up her lungs.  Then we'll have another appointment with the ENT at CHOP in October.

Dr. Javia does not decannulate in cold and flu season, and since we will next see him in October, I've accepted the fact that Zadie will have her trach for another winter.  This enterovirus that's in the news right now is a bit scary and all the more reason to keep the trach for now.

Not the answer we were expecting...

The nurse at the ENT's office told me that Dr. Javia was "pleased" with the results of the sleep study.  She said he wanted us to meet with the sleep doctor, Dr. DelRosso, (since this doctor works out of the pulmonary department, I mistakenly thought she was a pulmonologist - she's not) to go over some numbers to see if she agreed that Zadie was ready for decannulation.  We were pretty encouraged and even though I tried not to get my hopes up, we really thought we would get a green light to decann.  We were wrong!

Getting vitals!

First, the doctor didn't even seem to know why we were there.  She asked if we had done the sleep study to see if Zadie was ready to be capped (Zadie's been capped for over a year).  She also said more than once that she hadn't reviewed the study.  We made this appointment over two weeks ago, why hadn't she prepared?  In the middle of the appointment, she left the room for about twenty minutes to take a better look at the results.  She said that Zadie now has central apnea, which she never had before.  While her obstructive sleep apnea is now greatly reduced (an AHI of 1.8 at this study compared to an AHI of 75 at the last study), she now has central.  Central apnea is when the brain doesn't tell the body to breathe.  There is no surgical fix for it.  And there is no obvious cause of it for Zadie.  Dr. DelRosso said that reflux and asthma are two causes of it, neither of which Zadie has symptoms of.  She also said it was possible that it wasn't true central apnea, but just oxygen desaturations.  Out of the whole night (7 or 8 hours of sleep), Zadie's oxygen saturations (sats) were below 90 for 3.5% of the time, or 15 minutes total.  To us, this doesn't sound significant, but apparently it is.  The doctor also said that Zadie's sats dropped pretty dramatically during short pauses in breathing.  Whereas most kids can hold their breath for 20 seconds and have no desats, Zadie's sats dropped significantly during 5 second pauses in breathing.  Again, no reason why this could be happening.  Dr. DelRosso's only theory is that now that Zadie's airway is open (and was previously obstructed), her body is not used it and is overcompensating.  

The first step was to get a chest xray to make sure that Zadie's lungs look ok.  We had that done yesterday while we were at the hospital.  They also took an xray of her head so the doctor could look at her adenoids to see if they had grown back at all (they had grown back somewhat between her adenoidectomy in September to her tonsillectomy in May).  Since xrays are all digital now, we went back to the doctor's office to see if she could look at them right away.  She did come back and see us and looked at the xrays while we were there.  She said everything looked good.  The next thing would be to decide if Zadie needed an MRI of her brain to rule out anything going on there that would possibly be causing the apnea.  Her office called me today and said that Dr. DelRosso had consulted with the sleep lab director and they decided that it was best if Zadie got the MRI.  Because she has the trach, she has to be under general anesthesia.  They will be doing an MRI of her spine at the same time since she'll be under.  

Chest xray

Head xray (you can see BJ's fingers holding her head back!)

Meanwhile, I had left a message for Dr. Javia's (ENT) office.  I wanted to talk to him about how blindsided we were about the results.  Dr. Javia actually called me himself and said he was sorry for the miscommunication.  I'm not sure what I was looking for from him, but I'm just very upset that I feel like we're getting nowhere.  We're spinning our wheels trying to get Zadie decannulated and new stuff keeps popping up and keeping her trached.  We want to get another opinion, but at the same time, to start over with someone new would absolutely delay decann.  I feel like we've invested so much in CHOP that we have to keep sticking it out with them.  Dr. Javia called me back a second time this afternoon to say that he had spoken to Dr. DelRosso and she said that decannulation is still a possibility, but they want to rule some stuff out first.  I don't know if that means decann is a possibility this year, but we're already in August, and they will not decann during cold and flu season.  I have a feeling we'll be seeing another winter with the trach.  

The good thing is, Zadie is good.  She's happy and healthy and doesn't know anything different. I just hate to see her continually have to go through all this stuff.  This will be her ninth time going under general anesthesia, if I'm remembering correctly.  

I'm interested in hearing from others where central apnea has popped up out of nowhere and what was the cause and outcome!

Sleep study

Zadie had her third sleep study a few weeks ago.  I went with her this time and it was all pretty uneventful, which was nice (unlike her first one, which I went to with her, and her second one, which BJ went to with her because Beatrix was three days old).  I was very nervous leading up to it, since she's 3 now and has tons of attitude and getting her to do anything she doesn't want to do is difficult.  I also thought she'd be upset going to the hospital, getting the hospital bracelet on, etc, after her rough time after the tonsillectomy.  But, she took just about everything in stride.  The tech attaching her leads was great and had the perfect personality and really engaged Zadie.  And the other tech came up with a paper doll book from somewhere which Zadie loved and kept her occupied.  She barely cried at all, in fact!  She started off snoring pretty significantly, but after the tech and I adjusted her position a couple of times, she settled down and had a fairly restful night.  She woke up whimpering a few times, but that was about it.

Waiting to get started

Stacking rolls of tape

New habit...chewing on her finger

Happy!

Bunny ears

Nasal cannula was the tough part last time...this time the tech told her that since she had the bunny ears, she needed whiskers like a bunny!  It worked!

The tech told her she had to squeeze her nose anytime it itched (rather than rubbing it and possibly messing up the cannula)

The next week, I talked to the nurse from the ENT's office.  She said Dr. Javia was very pleased with the results!  I couldn't believe it!  I never expected her to say that.  She said that her AHI (apnea hypopnea index) was 1.8!  The last study, it was 75!  Unbelievable improvement.  I'm not sure we can thank the tonsillectomy totally for that, but it just might be that all that pain and suffering actually accomplished something for her.  The nurse told me that Dr. Javia wanted Zadie to have a consultation with the doctor from the sleep lab to see if he agreed that the numbers were good enough for decannulation!  I still can't even believe that this conversation is happening.  I'm still trying not to get my hopes up too high, but it's hard not to.  We're going to meet with the pulmonologist tomorrow and we'll see what he says.

Finally recovered! (I think)

Last Thursday, two weeks and two days after Zadie's tonsillectomy and two days after her last ER trip for bleeding, she woke up early in the morning and was coughing up blood.  This time, it was coming out of her trach as well.  It was a pretty large amount of bright red blood that she coughed into a paper towel.  I called the ENT's office when they opened at 8 am (the ENT resident on call didn't return either of my calls...hmmmph) and they asked if we could come down there to their ER (at CHOP, in Philly.)  I had already assumed that would be their suggestion/request and I didn't really want to go back to St. Barnabas since they didn't know what to do with us anyway.  So we headed down there.  Since it's a two hour drive, the nurse told me to call en route if there was more bleeding and they would possibly reroute us to another, closer hospital.  Luckily, we didn't need to do that.

CHOP's ER monitored her and the doctors told us they were deciding whether to take her into the OR to try to figure out exactly where the bleeding was coming from and cauterize the area or just admit her for observation.  Either way, she was not going home that night.  One of the doctors told us that bleeding is normal after a tonsillectomy, although it's rare, occurring in about 1% of cases.  When bleeding does occur, it's usually earlier in the recovery period, around 7-10 days post op.  And even though bleeding is normal, it's always taken seriously because of where the tonsils are located.  Because of the OR possibility, they wouldn't let Zadie eat or drink anything but clear liquids.  The worst part was her getting an IV.  They were good enough that they got it in on the first try, but it was pretty awful.  She was hysterical, and kept crying "Mommy, Mommy!"  I felt terrible.

Sleeping in the ER

She finally got into her room around 7 pm.  We decided BJ would go home with Jonathan and Beatrix and I stayed with Zadie.  It's really hard to be so far away from everyone!  On Friday morning, they allowed Zadie to have regular food again and she ate a bit and drank and was cleared to go home, although we had to wait awhile for BJ and the kids to make it back.  Traffic was terrible thanks to the start of Memorial Day weekend.

Feeling pretty good

Since Thursday morning, there has been no more bleeding and (knock on wood) I think we're in the clear!

14 days post op

Zadie had her tonsillectomy two weeks ago today.  She is eating and drinking pretty much like normal, she hasn't had any pain medication for over two days, and her breath smells much better.  So I figured we were past the whole recovery period.  Then this morning, I walked into her room to check on her one more time before work and I see this:

It was pretty shocking to see the big blood stain on her pillow.  She didn't have a night nurse last night, but I do check on her periodically through the night, and she's hooked up to a pulse ox that alarms if her heart rate goes too low or too high or if her oxygen levels go too low.  She had a quiet night, and she must have had her head on this stain the first time I checked on her this morning, because I definitely didn't see it.  BJ checked on her in the morning as well and didn't see it.  I noticed dried blood on her lips, so I could only assume that the blood came from her mouth.

I called the ENT's office when they opened and the nurse there asked how big the blood stain was, and when I described it, she said that Zadie should be looked at in the ER right away.  She said while it wasn't usual to have bleeds that long after the operation, it wasn't unheard of.  So once again, we go off to the ER!

Zadie really hates the hospital now, and cries even when they're putting the hospital band on her ankle and when they're weighing her.  She was really upset after getting weighed, and that's when the tech took her temperature with the forehead thermometer, and it came up as 101.  Zadie's nurse and I were both surprised at that, since she hadn't had a temp earlier.

When the doctor came in, he didn't really know what to do.  He looked at the tonsil area as well as she would let him and said he didn't see any blood.  He listened to her lungs and asked about the congestion (she has a bit of cold/allergies right now).  He suggested a chest xray to rule out pneumonia because of the "fever" and her congestion!  I suggested that we try the temp again when she's calm before we go running to get a chest xray, because I was pretty sure she did not have pneumonia.

After that, he really didn't know what to do, and even asked me who he should call.  When I hesitated, he said, "well, since CHOP is so far away, maybe I'll call the pediatrician."  I told him the ped is not going to know at all what to do, so CHOP is probably the best bet.  Then he asked me for CHOP's phone number.

Later on, the tech came in to take Zadie's temp again, and it was 98.2.  I was glad that I knew enough not to just immediately agree to a totally unnecessary chest xray!  Meanwhile, he had consulted with CHOP, and their suggestions were the same as a week and a half ago, transfer her to CHOP (no thanks...just for the distance) or admit her for observation.  This time I asked what we could do to avoid the admission.  We agreed that we would hang out in the ER for a couple of hours and see how it goes.  And Zadie entertained herself!

It was clear that Zadie was quite well, so she was discharged with a "You know where to find us, we're always here!"  

We don't really know why she had more bleeding.  But hopefully that was it!

Tonsillectomy

It's been a rough week!  Zadie had her tonsillectomy at CHOP on Tuesday.  She was scheduled to stay over one night.  The whole family went down to Philadelphia for the surgery.  She was scheduled later in the day, which was nice for getting a later start, but since she had to fast for the surgery, we all had to fast!  The surgery went fine.  Her adenoids had grown back a bit, so Dr. Javia shaved them down again.  I know that adenoids can grow back, but it's only been seven months since her adenoidectomy!  I hope they'll stay small.

Immediately after surgery, before she woke up

Cracking a little smile! A couple of hours after surgery

The plan was for BJ to stay in the hospital with Zadie overnight.  Jonathan, Beatrix and I were lucky enough to get a room at the Philadelphia Ronald McDonald House.  I will write more about the RMH in another post, because it was such an amazing place.  It was my first time at a RMH, and while I've heard nothing but good things about them, I just couldn't believe it.  For a "suggested donation" of $15 (meaning they will take less if you can't swing the $15), you get a room that's just as nice as any hotel, plus a fully stocked community kitchen that's open 24/7 and several play areas for the kids.

Jonathan and Ronald

Zadie required a little bit of oxygen overnight while sleeping, which is not unusual for her after a surgery or when she's sick.  We're fully equipped with oxygen at home, so this didn't concern us.  Of course, the hospital wanted her completely off oxygen before she was discharged.  I understand their position, but for a kid like Zadie, to keep her in the hospital just for something like that (when we can perfectly handle it at home) is silly.  She was drinking fine, which was the big requirement.  They had also put her IV in her foot, which really bugs me.  She's almost 3 years old, potty trained, and she can't get up and walk around!  She's always been very hard to find a vein, but I think they could have tried harder to find a vein in one of her arms/hands.  They do it once the kids are under anesthesia, so it's not like they're fighting a wiggling child.  I kept asking them about taking it out, but they wouldn't take it out until they knew that she was being discharged.  But, I wasn't going to let them keep her another day if that meant she was confined to the bed/chair that much longer!

They finally released her and we were on our way.  I was kind of (no, really) missing her Gtube, just because she was really fighting us to take her pain medication.

Thursday and Friday (two and three days after surgery), she fought the pain medications really hard.  We had to force her to take it just about every time, so she definitely wasn't getting full doses.  Probably because of that, she wasn't drinking as much as she should have been.  So it was a bit stressful.  She was drooling horribly a couple of times, which indicated her pain was out of control, since she wouldn't even swallow her spit.  I felt bad that we weren't helping her more.

Friday evening, she took a late nap and woke up about 7 pm, very miserable.  She was drooling and I went to wipe her mouth when I noticed bright red blood in her mouth.  Everything we were told and all the paperwork we were sent home with said any amount of blood is cause for concern and to call the ENT immediately.  She coughed up a big load of mucus and it was streaked heavily with blood.  I called the ENT resident on call (since it was 8 pm on a Friday night) and as soon as I told her what was going on, she said, "you have to take her to the nearest ER right now."

Waiting in the ER

So off Zadie and I went.  There was no more blood by the time we were at the ER, and the ER doc looked at her tonsils and saw no blood back there.  He said he'd call CHOP to consult with them.  I figured that they'd send us home.  I was wrong!  They wanted to admit her to keep an eye on her in case she started bleeding again.  Apparently, sometimes there's a small bleed and then later on, a larger bleed.  I would normally fight against her being admitted, but I had done enough googling while waiting that I was scared enough to agree.  I read about kids who had died from bleeding to death after a tonsillectomy.  The tonsils are very close to major arteries, and sometimes, when the scabs start coming off, a kid can bleed out.  A friend of mine messaged me on Facebook to tell me about a scary experience her daughter had after her tonsillectomy.  Very scary.

Like she owns the place!  Waiting for discharge, roaming the halls.

The hospital kept Zadie until about 3 pm on Saturday.  There was no more blood, and she was drinking pretty well.  We had finally gotten to a point where she would take her meds willingly if she could give them to herself (push the syringe), so from then on out, things just improved.  She would take her meds when she was supposed to, she was eating a little here and there and drinking better.  We started gradually spacing her meds out longer and she did well.  Yesterday, Saturday, about 11 days after surgery, was the first day she didn't have any pain meds and did great all day.  She's definitely lost weight but I'm hoping she'll re-gain what she lost.

Next up, another sleep study, which is scheduled for July 10.

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

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!

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.

Sleep study...

Zadie had her sleep study on Sunday night.  It started off pretty eventfully, as her Gtube balloon (the tube is held in by a water filled balloon - read more about it here) burst and the tube fell out.  I didn't have a replacement with me and the sleep study staff insisted that we go to the ER to get a replacement.  I was pretty upset, because I didn't know how long it would take for the replacement, and I really wanted the sleep study to happen.  But, the CHOP ER staff was wonderful and we had a new tube in within 2 hours and we were back to the sleep lab.  I have learned a lesson: always take a replacement tube!

Zadie showing off her new tube

So now that we were back in the sleep lab, we started to get ready for the study.  Since she didn't get a good nap that day (she slept for a total of about an hour, in two increments, in the car) and we already had to mess with her to get the new tube in, I was worried she'd be very uncooperative.  She did great while the respiratory therapist (RT) started putting the electrodes on her head and body.  She got to pick which color wire was put on next.  I said to the RT, wow, she's being really good.  She said, yeah, this part isn't the hard part.  We found out what was.  The nasal cannula.

All smiles for first electrodes

Wrap my head up in gauze?  Ok!

A gauze hat with bunny ears?  Great!

Nasal cannula...not cool.

Once the nasal cannula was in (it measured the carbon dioxide she was breathing - it had a little attachment for her mouth in case she was mouth breathing), she was very unhappy.  She cried on and off until she fell asleep.  The RT brought her a coloring book and crayons and she would be happy with that for a short time but then start crying again.  We really tried to keep her from crying too much, because the cannula couldn't get too wet and neither could the tape.  I really didn't want her to have to replace the cannula.  Zadie would just think she was getting it off and then to have to put it back on would be awful.

Occupied with a new coloring book and crayons..

Zadie didn't know what to do with herself.  She was crying on and off, she wanted to be off the bed (but she couldn't be, since she was all wired up and connected already), she wanted me holding her, she wanted me on my own bed, she wanted her milk, she didn't want her milk.  I finally got her to lay down by telling her I'd come back to her bed if she put her head on the pillow.  She did, and I did, and she fell asleep soon after that.  The RT told me when it was safe to move (she could see from her readings when she was in a deep sleep).  She slept fairly well, but she did snore a lot (just like Daddy!).  I knew that wasn't a good sign.  But she would sort of wake up every so often, crying.  I finally got to sleep, and then she woke up about 4 am and puked.  Her cup of milk was still on the bed, and I think she drank it and it wasn't very fresh.  We got her cleaned her up and she went back to sleep pretty quickly.

Despite it all, a smile at 4 am!

We were woken up at 6 am.  The RT cleaned up the wax out of her hair as best as she could and we were sent on our way.  Zadie was so happy!

So happy to be heading home!

I got a call from the ENT's office today and she briefly went over the results.  Zadie did have some apnea and that has to be addressed.  We have an appointment to see the ENT on August 12 to discuss the plan, but there's no way I'm going to be able to wait that long to find out what the plan is, so I'm going to call tomorrow and try to get more information.

The sleep study is scheduled!

The sleep study is scheduled.  Unfortunately, it's not until August 21!  I guess the doctor wasn't kidding when he said it would be about two months away.  The lady I talked to said that there are always cancellations and that I can call every other day to check.  So I guess that's what I'll be doing for the next two months!  Hopefully we'll be able to get in sooner.

CHOP update

We all took a ride down to Philly today. It was a crazy rainy day which made the trip even longer.  Because of the weather, I guess a lot of the appointments before us were running late.  Zadie's appointment was at 11:20 and we didn't see the doctor until after 2!

Dr. Javia looked at Zadie's ears.  She has an infection in her left ear, which we suspected.  Since she has a tube in there, it didn't seem to be hurting her, it was just draining a lot and she kept picking at it.  She was prescribed antibiotic drops for that.  The tube in her right ear fell out and is still sitting in her ear canal.  He said it will eventually fall out of her ear.  Even though she didn't pass her hearing test in that ear in April, possibly because the tube is out, we will just monitor it for now and will not take any action.

Since Zadie is doing so well with the capping, we are now cleared to get scheduled for a sleep study.  Unfortunately, Dr. Javia said it might be a couple of months until we can get in.  But, he recommended that we keep calling to ask about cancellations, etc.  If the sleep study goes well, then Zadie will be scheduled for decannulation.  When she had her bronchoscopy in September, Dr. Javia noticed that her adenoids were rather large, and they will possibly be a problem.  We won't know that until the sleep study, though.

I'm waiting for the sleep study department to call me with a date.  I'm very anxious about all of this.  They will not attempt decannulation in the winter, so we have a limited amount of time to get everything done.  It will all happen eventually, though, so if it doesn't happen this year, all is not lost.  I'm just really hoping it does!

As all good trips to Philly end, we stopped at Pat's for some cheesesteaks.