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.

I was and am a successful breastfeeding mother!

I'm very excited that Zadie and I were included in a kellymom.com article about the different faces of breastfeeding.

When you think of breastfeeding, it's usually a mom with a baby on her breast, but that's not always the case.  Zadie couldn't even drink from a bottle very well, so direct nursing was out of the picture.  Not only did her cleft palate make forming a seal in order to suck a bottle impossible, her small chin and tongue falling on her airway made everything even more difficult.

I did try to nurse her once, but she and I were too stressed out and I decided that it wasn't that important.  What was important to me was that she got what she needed, however she got it. We attempted feeding her orally every day, just about every feeding, using a Haberman, or Special Needs Feeder.  It has a special nipple which allowed us to squeeze the milk into her mouth while she tried to suck.  We only tried for 30 minutes at a time, because after that, we were told that she would be expending too much energy.  She was primarily tube fed for her first year.  Once her palate was repaired when she was 11 months old, she really got much better at drinking.  

I exclusively pumped for her for 18 months, and the majority of her nutrition for those 18 months was breast milk.  She was eating very little in the way of solids, but I was continually reassured (by doctors and nutritionists) that my milk was all she needed at that age.  We did transition to formula, with plans to further transition to a blenderized diet, but luckily for everyone, her solid food intake increased.

Flash forward to today, and I'm successfully nursing Zadie's 8 month old little sister, Beatrix!

Video of Zadie's trach change

Check out Zadie getting her trach changed! We do this about once a week. 

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.

Third trachiversary

Today (July 5) is three years since Zadie had her trach placed. All day yesterday, I kept thinking of July 4th three years ago, when we were woken up by a phone call from Zadie's neonatologist. She had had a rough weekend, having lots of trouble breathing after her Gtube surgery a few days before. The morning of the 4th, the doctors decided she needed to be intubated and because of her tricky airway, they couldn't get the tube in. We had to give permission over the phone for her to be put under general anesthesia (for the second time in just a few days!) in order for them to intubate her. When we got to see her later, it was rough. That was probably the hardest day up to that point. Her little arms were pinned down to the bed so that she couldn't pull the tube out. I felt awful seeing her look at me, like she wanted me to help her.  It was one of the worst days of my life, seeing her like that, and not knowing what was ahead of us.  

July 4, 2011

Once she was intubated, Zadie's neonatologist, Dr. Ruben, was very nervous about her pulling the tube out, since she was a big, full term baby (much different than the preemies she was surrounded by in the NICU).  Seeing an experienced NICU doctor nervous definitely made me nervous!  She told us that day that Zadie needed a tracheostomy.  This was the first time that a trach was even mentioned to us.  It wasn't a suggestion or something to be discussed.  It just what was going to happen, and as fast as possible.  When the surgeon said he didn't have time until two days later, Dr. Ruben pushed him to make time the next day.  

So, on July 5, 2011, at 5 weeks old, Zadie had her trach placed.  It was the first day since the day she was born that she didn't have any tubes or tape on her face.  I had been wishing for it to be gone, but I didn't want it this way.  Still, it was wonderful to see her pretty face and for her to be able to be on her back comfortably (with her breathing issues, she had to be on her side or belly in order for her to breathe properly.)  

July 5, 2011

Fast forward three years, Zadie's a big sister (still working on being gentle), a little sister (annoying her big brother every chance she can get) and does just about everything any other 3 year old does.  Today, she went swimming!  I can't wait until she gets the trach out, but she's doing just fine with it.  Next step...sleep study on Thursday night!  

July 5, 2014

No more nurses!

After nearly three years of having strangers (at least they're strangers at first!) take care of Zadie, we are officially nurse free.  It feels good!  We had been without a regular night nurse for months, only having one night a week covered, so a few weeks ago, we decided to do away with that one night.  It was silly to have one night covered.  Then, last week, we found out unexpectedly that our day nurse, who had been with us since before Beatrix was born and had become very close to us (or so we thought) wanted to cut way back on her days.  She only wanted to work one day a week, which, again, was silly.  At Zadie's age, we are not interested in introducing her to anyone new, so that was that!  The only problem is that we utilized Zadie's nurses as babysitters over the years, since she has specific medical needs that no one but me or BJ are trained to handle.  But being that she's so healthy and stable now, it should be ok to use regular babysitters.  It'll be nice to be a regular family 24/7 from now on.