Zadie's getting noisier!

We took Zadie to CHOP in the beginning of September for a bronchoscopy.  At the time, they downsized her trach because the ENT saw that she has a very small airway and her previous size trach was pretty much filling up her trachea, which is why she never made any noise.  The hope is that with a smaller trach, she can tolerate a Passy Muir valve (PMV), which will allow her to start vocalizing.  The day after her bronchoscopy and stoma revision (where they removed some scar tissue and fixed her trachea, which had partially collapsed), we tried the PMV while still in the hospital.  She must have still been swollen from the surgery because she did not tolerate it well at all.  We made an appointment to come back to the office in October (a week from tomorrow!) in hopes that the swelling goes down enough for her to tolerate the PMV.  If she still doesn't tolerate it, we will downsize again.

Anyway, gradually over the past few weeks, Zadie has started to make noise!  It's not all the time, but she does get squeaks out!  Sometimes it's when she's crying, but sometimes it's when she's happy and she's clearly intentionally making noise.  This is a great sign, because that means she's pushing air up past her trach and out her mouth and nose.  I think we have a good chance of her tolerating the PMV next week.  And if she doesn't, then I think the smaller size trach will really do the trick.  I hope so!  It's really, really great to hear her making sounds.

In case you missed my blog post from earlier this year about why she's so quiet, here you go: Why My Baby is So Quiet.

First bronchoscopy

Zadie had her first bronchoscopy on Tuesday.   It's done under general anesthesia and it gives the ENT a good look at her airway and vocal cords.  This was Zadie's fifth time being under general anesthesia.  It's never easy to send your little girl off to be put under!  We have to put a lot of faith into the doctors.

Jonathan had come down to CHOP with us, and BJ was entertaining him while I stayed with Zadie during the pre-op period.  The ENT told me that it would take anywhere from 15 minutes to an hour.  15 minutes if he looked in her airway and everything looked good.  It would take longer if there was anything that needed to be done, such as cutting out granuloma (scar tissue) or stitching up a collapsed trachea.  I was nervously waiting for it to be done and watching and hearing other doctors come out to talk to parents in the waiting room about how great their kids did.  The lady running the waiting room came to get me and said, "the doctor is ready to talk to you" and took me to a private room!  She didn't say anything and just left me there.  I really started to freak out - why didn't she say Zadie did great (I heard her tell other parents that) and why didn't Dr. Javia come out to talk to me in the waiting room?  After a good five minutes of increasing anxiety, Dr. Javia finally came in, all smiles, saying she did great.  He said, "I didn't scare you, did I, putting you in the room?"  Uh, yeah you did!

Anyway, Zadie did do great.  He did have to cut out a granuloma, which had been causing her airway to be 50% obstructed!  Her trachea was also partially collapsed, so he put two stitches in to take care of that.  Because of that surgery that had to be performed, they admitted her for the night.  The best part is that because her airway is very small, and the size trach she had in filled up her airway, the doctor downsized her.  This is great because it will allow more airflow past the trach and up through the vocal cords.  This is a better chance for Zadie to make noise, start talking, etc!  I am so happy that we decided to get another opinion and not just listen to whatever one doctor told us.  If we had listened to the ENT at St. Joseph's, she would not have had a bronchoscopy for another 2-3 months, and who knows how obstructed her airway would have become.

Since her trach is now downsized, she has a better chance of tolerating the Passy Muir valve.  This is a special valve that goes on the trach.  Air can flow into it, but not out of it.  Therefore, it forces the person wearing it to breathe out her mouth and nose.  Zadie's never been able to tolerate it, but now we know why.  She had a big obstruction in her airway and her airway is very small and was completely filled up with the trach.  If there's no airflow past the trach, then the valve won't work.

So, on Wednesday, we tried the Passy Muir again, with a nurse practitioner from the ENT office observing and testing the pressures with a manometer.  There are certain parameters that they look for as far as the pressure, and if the pressures are too high, then the valve isn't being tolerated and can not be used.  Unfortunately, Zadie didn't tolerate the valve well at all.  The NP said not to get too upset about it.  Zadie might still be swollen from the surgery and we will try again in another month in the ENT's office.  If she still doesn't tolerate the Passy Muir then, we will downsize the trach again.

This is all very exciting, because these are all steps towards decannulation (getting rid of the darned trach)!  It's not in our very near future, but it is there.  She'll definitely have the trach at least through the winter, but maybe next summer?!?

Meeting with ENT at CHOP...

BJ and I took Zadie down to CHOP today to see Dr. Javia, the ENT.  We had met with him in December and he prescribed the Passy Muir valve (PMV - speaking valve) at that time.  He wanted us to come back to check the pressures to make sure that it was safe for Zadie to use it.  She ended up being sick a lot from January to April, and then had her palate repaired in May, so we didn't get back to him.  Besides, we had pretty much decided to stick with St. Joe's for all of her care, so we didn't really plan on going back to CHOP.

Well, now that I'm not happy with the ENT at St. Joe's, and the craniofacial team there kind of dropped the ball on guiding us through all of this, we're kind of up in the air again.  I feel terrible that Zadie is still not able to make sounds.  My hope for the ENT visit today was make sure that the PMV was safe to use and then we could get working on it.  Unfortunately, since no one has ever looked at Zadie's airway, the doctor wants us to hold off on the PMV for now.  We have scheduled a bronchoscopy, which is a scope of her airway and is done while under general anesthesia.  That will be happening on September 4, which is just a few weeks away.  I'm anxious and excited to find out what's going on in her airway.

The doctors at CHOP are very fixated on doing a jaw distraction on Zadie.  We are hoping it won't be a necessary surgery, so we aren't entertaining the notion right now.  The fact that they are so gung ho about it is a big reason we shied away from  CHOP in the first place.  If we can avoid two additional surgeries (one to place the distractors and another to remove them), plus all the rough stuff to get through the procedure, plus all the risks involved, we would love to do so.  It's not clear to us at this point that it's a necessary procedure.  Maybe after the bronchoscopy, things will be clearer.  I am pretty bummed that today's visit didn't go as I expected.

We are considering getting yet another opinion on a course of action.  We are pretty much resigned to the fact that Zadie will have the trach at least through the winter, but we'd love to be on track to get it removed by the spring/summer of 2013.

On a positive note, Zadie had a hearing test, and did well, so I think the tubes are doing their job.

And on another note, I already know how lucky we are, but being at the Children's Hospital today, and seeing all the sick kids, I was reminded even more about how truly blessed we are.  Zadie's good and happy and I know this all is just a bump in the road.  I'm just looking forward to the day we can look BACK at this particular bump.

One year at home...

Today is the day, a year ago, that Zadie finally got to come home from the hospital! Leaving her there when I was discharged was one of the hardest things I'd ever done! I always heard about babies having to stay in the NICU, and I always said I couldn't imagine how hard that would be. Unfortunately, I found out. What made it a million times worse was that almost every day when I went to the hospital to see Zadie, I'd see a beaming mom sitting in a wheelchair, holding her tiny bundle of joy, waiting for dad or whoever to bring the car around.  It really was like a knife to the heart to see that, every time.  It never got easier.  I often wished that there was some secret NICU parent entrance so we didn't have to pass by that scene every day.  I also realized how lucky I was that I got to experience that once.  I was that beaming mom holding my little Jonathan just three years earlier.  It was a long ten weeks, and I couldn't believe the day had finally come where they would release Zadie!

Zadie with one of her favorite NICU nurses, Patty

Zadie's neonatologist, Dr. Ruben, sending her off

Proud big brother with his baby sister home for the first time!

It was torrentially raining that day, and when we got home, the director of nursing of the home care agency and four nurses were waiting to be oriented on Zadie's case.  From that day on, we've had nurses in the house 16 hours a day almost every day.

So much has changed in the last year!  Zadie has grown, of course, and is happy and healthy and on the go.  She's been back in the hospital a few times.  She no longer has a cleft palate.  She's eating and drinking by mouth, although still uses her tube overnight (mostly because we haven't gotten the orders changed).  We are old pros at changing trach ties, changing the trach itself, getting it back in if it gets pulled out, changing her Mic-key button (feeding tube), putting that back in if it gets pulled out.

Maybe, just maybe, in a year from now, Zadie will have put a lot of this behind her!

How to Travel with a Trach Baby

We just recently returned from an 11 day trip through the south.  We really briefly considered taking one of our nurses with us, but we really wanted our first family vacation to be just us.  We had a great time, and here's my advice and tips for anyone considering traveling with a trach baby!

• There's no such thing as traveling lightly with a trach baby.  I started to put all of Zadie's supplies in a big tote bag, and then decided just to go for the third suitcase.  I had to bring enough supplies for 11 days plus enough extras just in case!  Also, the night before we left, Zadie was desatting a little while sleeping, which means her blood oxygen levels were a little low.  The orders are for her to be on oxygen if her oxygen levels stay below 92%.  We had planned on bringing oxygen tanks, but they only last a few hours each, so if she really needed oxygen on the trip, the tanks wouldn't help too much.  So we decided to bring the huge oxygen concentrator with us.  Wouldn't you know it, she didn't need a bit of oxygen the entire trip!  Though I truly believe that if we hadn't brought it, she would have needed oxygen and we would have been sweating it.  

• Keep the suction machine at arm's length.  This is something most trach parents do anyway, but we were driving for hours most days, and it wouldn't have been great if we had to pull over every time she needed suctioning.  I kept the machine right behind me, and Zadie's seat was in the middle.  If she needed suctioning while we were on the road, I just turned around in my seat and suctioned her rather than pulling over.  We pulled over plenty as it was with a 4 year old who apparently has a bladder the size of a walnut.  

• Don't expect to be in and out quickly of hotels.  We didn't stay in any one place too long; the longest stop was four nights.  Normally, it's not such a big deal to stop at a hotel for one night, pull out the clothes you need for the next day, and zip up the bag in the morning.  But, with Zadie girl, we had a luggage cart completely full for each stop.   Three suitcases, Zadie's humidifier, her apnea monitor, her pack n play, her pillow and blanket, plus toys for the kids.  And getting packed up in the morning took some time, in addition to doing all of Zadie's trach care before hitting the road.  It was nice when we were in a hotel for more than one night and we didn't have to do all the unpacking and packing.  

• Bring an extension cord, a 3 prong adapter and a power strip.  The power strip was key.  I'm glad I thought of it!  Actually, it wasn't me, it was BJ.  But with all the stuff we had to plug in and charge every night, it would have been rough without it.

• Have fun!  We did!

Visit with the surgeon today

Zadie had another follow up with her surgeon, the Italian Stallion, today.  He's Italian, of course, and kind of dashing and he wears red clogs regularly.  Anyway, I didn't expect much of the visit except for him to look at her palate and say it looked good.  I know that it's holding steady up there because Zadie was sucking yogurt off my finger yesterday and has really good suction!  She also drinks really well from her sippy cup.

So, I was surprised that the director of the craniofacial team came into the exam room also.  He began asking me questions about Zadie's progress with eating.  I told him how she's doing, and then he asked who is following her about her trach and what they think about her getting it out.  I thought that was odd, because he's from the craniofacial team who is supposed to be following her and guiding us along.    We don't really have anyone following her specifically for the trach and should we?  I don't know.  We have the ENT, but we've only met him once, and at the time, he said that Zadie will probably have her trach until she's closer to 2, so we won't be looking to decannulate (remove the trach) much sooner than that.

I then mentioned that I was going to be calling the ENT soon to make an appointment to talk about possible downsizing the trach and working on the speaking valve.  Zadie's almost 14 months old and because of this stupid trach, she doesn't make any noise.  She should be talking and saying words by now, and she doesn't make a peep.  So with that, the doctor says, "Yeah, you should be talking to him about that, because we're behind already, she should be making noise and speaking by now."  Well, thanks, pal.  I wasn't upset enough about the fact, now you made me feel worse and now I feel like I'm holding her back because I haven't been pursuing things enough.  Blah.

So, hopefully we'll have an appointment with a pulmonologist next week who we can talk to about the status of the trach.  I'll get an appointment with the ENT and one of the speech pathologists to talk about downsizing and working with the speaking valve.  Then we'll have another meeting with the craniofacial team about the plan going forward.  I really hope that we someday get to the point where we feel like someone is driving the bus and directing Zadie's care!  I had this anxiety months ago, but I thought it was resolved.  Apparently, I was wrong.

I also have to find an eye doctor in our plan to make sure Zadie's eyesight is ok.  There is a genetic syndrome often associated with Pierre Robin called Stickler's and while Zadie doesn't appear to have it, she still needs to see an eye doctor.  Kids with Stickler's often have eye problems.  She also needs to get an appointment with a dentist.  Lots of appointments in the next few weeks!

Happy Trachiversary!

Today marks one year since Zadie was trached.  A year ago yesterday, on the 4th of July, we were woken up by a call from the hospital.  That's never good.  But the call was a few days coming.

On June 30 of 2011, Zadie had a G tube surgically placed.  Her eating hadn't progressed enough and the hospital wouldn't send her home with a nasogastric (NG) tube, the temporary one that goes through the nose to the stomach.  They didn't want to keep her in the hospital just for eating issues, so it was decided that she'd get a G tube.

She went through the surgery just fine.  It was scary for us to leave her with the surgeon and anesthesiologist, but it went quick.  She was intubated for the surgery, meaning she had a tube inserted down her throat to help her breathe through the surgery.  Since she had such a tricky airway (part of her condition, Pierre Robin Sequence, causes the airway to be funky), they were worried about intubating her, but didn't have trouble once she was under anesthesia.  They extubated her (removed the breathing tube) the following day.  She had always had trouble breathing in certain positions, but before the G tube surgery, if she was laying on her belly or her side, she was ok.  She also had a nasopharnygeal (NP) tube, also known as a trumpet, inserted in her nose when she was about 10 days old. The purpose of that was to help keep her airway open more.

Now, after the G tube surgery, she was pretty miserable.  Not breathing easily in any position, and her oxygen saturations were not great.  The surgery was on Thursday, she was extubated on Friday, and by Sunday morning the 3rd, she was on oxygen.  She was holding her own, though, and we kept being reassured that her airway was probably just swollen from the intubation.  I kept asking if the intubation possibly damaged her airway, but the doctors kept saying it was just swollen.

So, on the morning of the 4th of July, we were woken up by a call from Dr. Ruben, Zadie's neonatologist.  She said that Zadie's breathing had worsened to the point that they needed to intubate her again.  They tried to do it while she was awake, but were unable to, so they had to put her under for it.  Since we weren't there, we had to give our permission over the phone for her to go under anesthesia to be intubated.  During that phone call, Dr. Ruben also told us that Zadie would need a tracheostomy as soon as possible.  This was the first time we had heard that word in relation to Zadie!

When we went to see Zadie later on, she was already out of anesthesia and intubated.  They had her little hands pinned down to the bed so that she couldn't pull the tube out.  She was looking at me like she wanted me to help her.  That was probably the hardest day of all the days in the NICU.  It was the first time I was afraid that we were going to lose her.  I was crying and trying to get the nerve to ask a question that was floating around in my head.  I finally got the nerve and asked, "Is she going to be ok?" Her nurse just looked at the doctor, who said, "Her problems are all mechanical.  She's not a sick baby. We just need to fix her mechanical problems."  The fact that she didn't say, "Yes, she'll be fine" was not lost on me, but I did feel better having asked.

Since it was 4th of July, it was hard for even the doctors to get a hold of the surgeon.  When they finally did, he said he could do the trach surgery on Wednesday, the 6th.  Zadie's doctor wasn't satisfied with that.  She insisted he do it the next day, on the 5th.  The fact that she was so nervous and anxious to get the surgery done made me very anxious and nervous!  The doctors in the NICU were used to little bitty preemies, not robust full termers who could easily pull out tubes.  Zadie's doctor was very nervous that Zadie would pull out her tube and it would be so difficult for them to get it back in, that it might be a very bad situation.

So, on July 5, 2011, we walked our sweet little girl down to the OR for the second time in 5 days and she was put under anesthesia for the third time in 5 days.  Again, the surgery went quick and she did well.  When we got to see her, it was like seeing a different baby!  She was laying on her back (previously had always been on her belly or side) and she just seemed happier and more comfortable than she had ever been in her 5 short weeks on earth.

When I think back to that time, I realize how little of a clue I had about what lay ahead.  I understood what a trach was, I knew some people had them for awhile, but I could never have predicted what our life would turn out to be like.  I hope that Zadie is able to lose the trach by her next trachiversary, but I'll be ok with it if she's not.  It saved her life.