Tuesday, December 11, 2012

RSV & another ER visit

Zadie got a fever out of nowhere last Wednesday.  She had no other symptoms other than excessive sleepiness.  I gave her Tylenol and it went away, but I took her to the pediatrician that day, since pneumonia always comes on fast and is a constant concern.  He listened to her lungs and said they sounded good.  He said it was probably just a virus, but he put her on antibiotics as a precaution so that whatever it was stayed out of her lungs.

She did ok the next day, but then spiked another, higher fever that night, and again on Friday.  She wasn't really eating anything, so I was making sure that she was getting plenty of fluids.  Over the weekend, she was feeling very yucky.  Her breathing was very fast and she was requiring oxygen to keep her saturation levels up during the day as well as at night.  I considered calling the doctor, but I knew that any on call doctor would have us go to the hospital, and most likely, the hospital would want to admit her, and I really wanted to avoid an admission!  So we rode it out for the weekend.  She barely moved from the couch the whole weekend.  She kept spiking fevers, and if we tried to give her anything but Pedialyte or juice, she'd vomit it up.

Monday morning, I called her pediatrician and brought her in that day.  I felt nauseous, because I was positive that he was going to send us to the ER, at least for a chest xray.  And because she had just been in the ER three weeks earlier with pneumonia, I was afraid she was definitely going to be admitted.  I was right about the ER trip, because the ped suspected RSV.  We went right there, and saw our old buddy, Dr. Robbie, the ER doc we've seen way too many times!  Right away, he said she didn't look so bad, especially compared to other times he's seen her, and her pediatrician is just nervous.  He said unless something crazy came up, he wasn't going to admit her.  Huge relief!

The test results confirmed it was RSV, and the chest xray looked normal.  Since RSV is a virus, there's no medication for it, and it just needs to run it's course.  He said it could last 7-10 days, and it's possible that it started last Wednesday with that first fever.

Today was slightly better.  Zadie still slept a LOT, but she did eat a little bit.  She also was smiling more than we've seen in days.  Her breathing was closer to normal, and she didn't need any oxygen.

RSV is a very nasty bug.  At one of our appointments at CHOP, I met a mom and her trached son in the waiting room.  Her son had a trach because of a bad bout of RSV when he was a baby.  Because it's so serious, there is a shot for it, Synagis, that is only given to high risk babies under 2.  None of Zadie's doctors ever suggested we try to get approved for it, for some reason, but the fact that she has a trach makes her a candidate.  I'm not sure if it's worth it to try to get it at this point, but I will ask.

My main goal for the next few months is to keep her as healthy as possible.  We need to be working on the Passy Muir Valve in order to move on to the cap in order to hopefully lose the trach in the spring!  If she's sick, we can't move forward.  Short term goal is to get her feeling good enough to sit on Santa's lap!

Tuesday, November 13, 2012

It's been awhile...

I went the whole month of October without posting anything!  Quick update...as of about a month ago, Zadie lost all of her nursing hours.  The insurance company says nursing is not medically necessary, that it's simply custodial care, and the only reason she would be granted private duty nursing is if she would otherwise be in a facility.  Our first appeal was denied.  We have a second level appeal to file, which will be done soon.

In better news, yesterday Zadie went back to the ENT to try out her Passy Muir valve (PMV).  This is a valve that forces her to breathe out her mouth and nose, and therefore through her vocal cords, giving her the ability to make noise and vocalize!  After her bronchoscopy in September, she didn't do well on the PMV trial, but it was suspected that she might just still be swollen from the surgery.  Zadie is a little under the weather, so I was nervous that it would affect the attempt, but I didn't want to reschedule the appointment again, since the next appointment would have been in a month, and it had already been rescheduled once.

So, it started out rough because she was very upset.  With her current size trach, the pressures with the PMV on seemed to be too high.  We then changed her trach to a smaller size and tried the PMV again.  After calming her down, she did great!  The pressures stayed in the range they were supposed to and her oxygen saturation levels stayed where they were supposed to!   This is GREAT news.  Besides all the numbers looking great, it was so nice to hear her making little noises effortlessly.  We've been hearing more and more from her, but this was more "normal" baby noises.

The plan now is once she gets over her current illness, we can start working with the PMV every day.  We'll start with short time frames, and then increase each day to get her used to it.  In two or three months, when hopefully she's tolerating the speaking valve during all waking hours, we will switch to a cap.  A cap is just a little piece of plastic that blocks the trach so she'll be forced to breathe in and out of her mouth and nose.  We are shooting for decannulation (removal of the trach) in early spring!  I'm trying not to get ahead of myself, though.  I don't want to get my hopes up too much.  But all is looking good right now!  Now we just have to get through cold and flu season.

I'll post again soon!

Sunday, September 30, 2012

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.

Sunday, September 9, 2012

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?!?

Monday, August 13, 2012

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.

Thursday, August 9, 2012

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!

Monday, July 23, 2012

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!

Wednesday, July 18, 2012

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!

Thursday, July 5, 2012

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.

Saturday, June 30, 2012

Tubie Friends

You might have seen the picture of Zadie with her new Tubie Friend that I posted today.  I'm so excited about this teddy bear (who BJ thinks look likes Ted.  From the movie.)

Tubie Friends is a non profit organization that was started by two mothers of kids with feeding tubes.  They want to help other parents and kids who rely on feeding tubes.  All of the "Tubie Friend Surgeons" are volunteers.  All Tubie Friends asks of applicants is for $8 to cover shipping.  If you can't afford that, it's ok.  They'll still send you a Friend.  The stuffed animals (Zadie got a Build a Bear!) are funded by donations.

I sent Zadie's application in on Sunday night and her Friend was on our doorstep on Friday!  Talk about a quick turnaround, when they tell you that a typical turnaround time is 2-4 weeks.  And it just so happens that Beary (as Jonathan named him) arrived the day before Zadie's one year anniversary with her G tube.

Check out their site.  Spread the word if you know a kid with a feeding tube.  And donate a couple of bucks if you have it!

Jonathan, Zadie, and Beary

Thursday, June 28, 2012

No news is good news...

I realized I haven't really written an update about Zadie recently.  The main reason is because she's doing really well and there haven't been any major developments.  She was sick, but seems to be just about over that by now.  She actually started getting sick the day before Jonathan's birthday and I ended up having to take her to the doctor the day of his birthday, which was also the day of his party.  Luckily, that didn't really put a damper on anything, and she got to enjoy the party a little bit.

She has been eating pretty well.  We offer her a sippy cup of milk at almost every feeding (other than when it is a nurse feeding her - the nurses need orders for EVERYTHING, and the written orders right now are to put everything through the tube) and she does really well.  There have been some meals where we haven't had to tube feed her at all!

She's also thisclose to walking.  She lets go of couches or tables for short times and has started bridging distances without holding onto something in between.  No real unassisted steps yet, though.  Soon, I'm sure!  She's doing so well that her occupational therapy through Early Intervention has been reduced to every other week instead of every week. We love her therapist, Patti, and we look forward to her bag of toys every week, but Zadie really doesn't need weekly OT.  We will continue with weekly speech therapy, though.  Zadie still can't make any noise around her trach, so we're working hard on signing with her, so that she has a way to communicate.  It's the cutest thing when she signs!

Zadie signing "more"

Thursday, June 14, 2012

Community Supported Agriculture

Does anyone belong to a CSA?  We have considered joining in past years, but this year we finally did it when I found that Caldwell has one.  If you don't know what it is, you buy a share which is basically an investment in a local farm.  Every week throughout the summer and into the fall, we get a shipment of farm fresh produce.  Our produce comes from Starbrite Farm, which is a certified organic farm in Andover, NJ.

This week was our first shipment.  We ended up with two bags full of beautiful veggies.  I made a stir fry last night using garlic scapes (which are the flower stalks of garlic - they are kind of like scallions, but more garlicky and my hands still smell like them today after multiple hand washings), bok choy, mustard greens and radishes.  I never used three of those four ingredients before!

garlic scapes

I feel a little under the gun to use up all of this produce before it goes bad. But I'm very excited about it, and I'm glad that the kiddies are going to be getting organic veggies all summer!  And we're helping out a local farmer, which is always good.

Monday, June 11, 2012

Exclusively Pumping

It has been quiet around here, so I thought I'd write about something that is a big part of my life.  With Zadie's birthday came an anniversary for me.  I have been exclusively pumping breastmilk for Zadie for a full year.  I never really intended to be still doing it a year later.  I pumped for a short while with Jonathan, but didn't really know what I was doing and didn't seek out any support, so it didn't last long.

Since I had so much amniotic fluid while pregnant with Zadie, and they didn't know why, the doctor was concerned about a possible tracheoesophageal fistula (TEF).  Because of this, it was recommended that Zadie be bottle fed a small amount of water before any breastmilk was fed to her.  The thinking was that if there was a TEF, and fluid got into her lungs, it would be better that it be water and not milk.  Therefore, while we were waiting for Zadie to be brought to my room, they attempted to feed her some water from a bottle and it didn't go well and she was sent off to the NICU.

Since she wasn't able to drink from a bottle properly, it was clear that breastfeeding was not possible, at least not at that point.  Between Zadie's cleft palate and small chin, she couldn't form a seal with which to suck properly.  Even with specially made bottles which allowed us to squeeze milk into her mouth, she never was able to drink much from a bottle.  So, the first night of Zadie's life, I requested a pump from my nurse and the rest is history.

My faithful companion

The milk kept flowing, so I just kept pumping.  I rented the hospital pump because I didn't know how long I'd stick with it, and I was hoping that insurance would pay for something.  Insurance didn't pay for anything, and I still have the blasted hospital pump!  Somewhere along the way, I found a wonderful Facebook group for other moms like me who don't nurse directly, but pump exclusively.  Without those girls, I can't say for sure if I would have made it this long, still being able to provide 100% of Zadie's milk intake.

I had in my head one year as my goal.  I looked forward to "hanging up the horns."  I was counting down the months.  Then, it occurred to me - Zadie doesn't eat like a typical child, so she won't be just moving on to solids and cow's milk like a typical child.  That was when I started thinking about the blenderized diet.  I couldn't imagine switching to a formula after all these months of pumping.  That's also when I realized that I wouldn't be hanging up the horns when Zadie turned 1.

We're working on Zadie's eating, and she's actually doing really well.  I think the palate surgery made a huge difference in her ability to form a seal and she sucks pretty well and enthusiastically from a sippy cup.  In fact, over the past few days, there have been several meals where we didn't have to hook her up to the tube at all!  This is VERY exciting.

She has an appointment with the feeding clinic next week, and I'm going to make an appointment to meet with a nutritionist, but I see the end in sight to my pumping career.  I only pump twice a day for about a half hour each time now, but I'm looking forward to having an extra hour a day!

Thursday, May 31, 2012

Happy Birthday, Zadie!

I really can't believe a whole year has gone by since the day Zadie entered our lives.  It's been quite the crazy year.  I thought today, I'd share more of what we all went through that day.  Because while it was one of the best days of my life, it was also a pretty horrible day at times.

I had a scheduled C section for two reasons.  One reasons was because I had had a C with Jonathan and the other was the fact that I had so much fluid and they didn't know why.  Once they got me open, they were suctioning out all the fluid.  My level of fluid was amazing to everyone in the room, and they had to get a second suction canister.  My doctor told me I had 2 1/2 liters of fluid, which apparently is a lot.  Next, to get Zadie out, there was a lot of pulling and pushing.  She was out!  It's a girl!  I teared up a little when I heard those words.  Lots of hair, just like when Jonathan came out.  They brought her over to the exam table and the neonatologist examined her.  BJ went over to take pictures and I saw the doctor talking to him.  I heard "cleft palate" and I was trying to hear more.  I finally caught BJ's eye and asked, "is she ok?" He smiled and said, "she's fine!"  He told me that the doctor said she had a small cleft palate, but that it wasn't a huge concern.  There would be surgery down the line.  My OB further reassured me, saying she hadn't even seen it (not that she necessarily would).  If only anyone had any clue what we all had ahead of us!

Anyway, I got to hold her and she looked so much like Jonathan!

Next, I had to be closed up and Zadie went off to the nursery to be taken care of.  BJ went with her, and later came to see me in recovery.  When I was ready to go up to my room, they called for transport.  It took FOREVER.  It took so long that BJ actually went to the newborn nursery and "stole" Zadie away so that I could see her.  Later on that day, I would be SO happy that he did that.

I was finally taken up to my room on the 6th floor.  Since I had given birth to Jonathan in the same hospital, almost exactly three years earlier, they had changed a lot.  All maternity rooms were now private, there were no set visiting hours, and you could have as many visitors as you liked.  I was looking forward to these changes, since the day Jonathan was born, I had a roommate and they were strict about the two visitors at a time rule.  My room looked like a hotel suite.  My sister-in-law brought Jonathan later on in the afternoon, along with my niece and nephew, Stevi and Nicholas (future Godparents, although no one knew that at the time).  My father-in-law also came.  Meanwhile, we were all (im)patiently waiting for Zadie to be brought to the room.  I called my nurse several times asking where Zadie was.  Finally, I was told to call the newborn nursery to ask.

I gave birth to Zadie at 11 am.  At this point, it was probably around 5 pm.  I called the nursery and was put on hold.  When the nurse came back on the line, she said, "oh, they tried to feed her, and she didn't do well, so she's going to the NICU."  That was the beginning of one of the best days of my life being turned into one of the worst.  BJ was out of the room at the time and he came back in to see me sobbing on the phone.  I could just barely tell him that she was ok so that I didn't freak him out even more.  Even worse, BJ couldn't go see her immediately.  They had to set her up in the NICU, so he had to wait for about an hour.  He finally got to see her and reassured me that she was ok.

Meanwhile, a plastic surgeon from the craniofacial team at St. Barnabas had been called in by Zadie's pediatrician.  He examined her and was the one who diagnosed her with Pierre Robin Sequence.  He told us that she would have to stay until she learned to eat on her own.  I asked him how long he expected that to take, and he said maybe several days to a week.  A week!  Just the idea of her being in the hospital for a week killed me.  Again, if only we had known what we had ahead of us!

Since I had a C section, I couldn't get out of bed for 12 hours from the surgery, so that meant I had to wait until 11 pm.  I was able to be wheeled down in a wheelchair to see my little girl.  She was sleeping, but hooked up to all sorts of tubes and wires.  It was a relief to see her, but so sad to know I couldn't hold her or feed her or even sleep near her.  Unfortunately, the NICU is on the 3rd floor and I was on the 6th floor, so I was about as far away from her as I could have been.  And this is how I left Zadie on her first day of life.

I'm so grateful that tonight, I get to tuck her into her own crib right down the little hall from our bedroom and tomorrow morning I get to get her up and play with her and feed her.  Happy birthday, sweet baby girl.  You've come so far in the past year and I can only imagine what you'll accomplish in the next year!

Friday, May 25, 2012


I've been thinking a lot lately about what I was doing last year at this time.  I was very pregnant with Zadie, and I was blissfully unaware of what the future had in store for us!  I was going to the high risk group for weekly ultrasounds because of my abnormally high level of amniotic fluid, a condition called polyhydramnios, but none of those doctors figured out why it was there.  I googled that word a hundred million times, but never once stumbled upon Pierre Robin Sequence.  In retrospect, I had noticed Zadie's small chin in at least one of the ultrasound pictures, but I had no idea it could mean anything.  I had no idea it had a name, micrognathia, and that it would turn out to be the main struggle she would face.  So, ladies, pay attention and question everything.  But, don't make yourselves crazy because a lot of times, there's nothing you can do before the baby arrives, although preparation is always nice.  This is the last ultrasound I had before Zadie was born.  Notice the small chin!

Wednesday, May 23, 2012

Swallow Study!

Zadie had her long awaited swallow study today.  The last time she was admitted to the hospital with pneumonia, her pulmonologist suggested that perhaps she was aspirating and therefore should not eat by mouth until she was able to get a swallow study.  That is where they xray you while you eat to see where the food is going (and hopefully not into the lungs.)  Then, Zadie had her palate repair and had to recover from that and finally was cleared last week to get the study.

Today was finally the day.  I pureed up some banana and breastmilk (who wants some?!) in my awesome  Blendtec blender and dug out the sippy cup that she hasn't used in about a month.  We headed off to the hospital without really knowing what to expect.  When we finally got into the room, they sat Zadie in this little chair and strapped her in.  She was so ticked off!  I put on my big lead vest and they set up the xray camera next to her.  It was cute to see her little head moving around on the xray screen.  After they added the barium to the banana puree, I started to feed her.  She was flipping out, probably very hungry and not happy about sitting in that seat at that moment.  But they kept telling me to give her more, so every time she opened her mouth to cry, I'd just stick another spoonful in.  I was trying to watch the screen while feeding her, and I did get to see her swallowing!  Then we tried just the straight breastmilk and she did great with that, too.  It was all very fast-paced and was over pretty quickly.  She passed with flying colors!

With just a week and a day to spare until her birthday, Zadie says, "Bring on the birthday cake!"

Sunday, May 20, 2012

Moving right along.

Zadie had her second post-op follow up with her surgeon last week.  He said she looks great and he said go ahead and feed her anything we want!  Wonderful!  The only problem is, the last time she was admitted to the hospital with some pneumonia like symptoms, her pulmonologist became concerned that all of her troubles may stem from aspiration (breathing food or milk into her lungs) rather than an infection.  Most other doctors, including her pediatrician and the surgeon who repaired her palate, doubt that she is actually aspirating.  I kind of doubt it, too, because every time she's been sick, a dose or two of antibiotics have turned her around almost 100%.  But, regardless, I want to be sure that we are checking everything, so we are holding off on any oral feeds until she gets a swallow study.  This was Zadie's last oral meal, delicious avocado!

Zadie is scheduled for the study on Wednesday morning.  That is a week and a day before her birthday so we're really hoping for good results so that she can stuff her face with cake on the 31st!  She's definitely dying to eat again.  When we eat in front of her, she can't take her eyes off our food.  I hope she rocks the study on Wednesday!

Off the topic of Zadie, today a very good friend of our family received a long-awaited heart transplant.  We're really excited for him and his family!

Sunday, May 13, 2012

Happy Mother's Day!

Last Mother's Day, I was very pregnant with Zadie, and we went to the Turtle Back Zoo.  I hadn't been there in years and I had forgotten how hilly it was!  It was rough with the big baby belly.  It was really fun, though, and we ended up buying a membership that day.  I figured that I'd be home with Jonathan and a new baby all summer and it would be a good thing to have.  It turned out it was GREAT to have, since most of our summer was spent going back and forth to the hospital, and I could take Jonathan to the zoo for a quick trip, even just to ride the train.  

Today, we'll be going out for an early dinner to our favorite restaurant, Don Pepe II.  I'm very grateful to have two really great kids.  I feel very lucky that Zadie is doing so well and hopefully by next Mother's Day, most of her issues will be in the past!  I wish that my own mom and BJ"s mom could be here too, but I know they are watching over us.  

I hope all you moms have a wonderful day!  

Tuesday, May 8, 2012

In honor of nurses week.

I complain a lot about our home care nurses.  However, in light of it being nurses week, I wanted to say thank you to all the nurses who have taken care of Zadie over the last 11+ months and have therefore helped us out immensely.

The nurses in the NICU were amazing.  We had no idea what to expect, and were kind of flying by the seat of our pants in the early days, and we were lucky to have all of those wonderful ladies.  One nurse allowed us to bring Jonathan in briefly so he could see his baby sister, even though it was against the rules.  Two nurses, Veronica and Patty, requested to be Zadie's primary nurses, meaning that they were assigned to her anytime they worked.  We became very close with them, and still are in contact with them.  I could name many more nurses who spent time with Zadie and treated her like she was their own babies during those ten long weeks.  We had to trust them to take care of her, and they made it easy.

As soon as we arrived home from the hospital, there were four nurses plus the director of nursing from our home care agency waiting for us.  It was hard to get used to, and in a lot of ways, I'm still not used to having other people in our house.  But, these women allowed us to get sleep at night and get comfortable with taking care of Zadie at home.  We're old pros now, but in the beginning, we definitely needed the help.

We also know a lot of nurses and nurses-to-be in our personal life.  Zadie's godmother and cousin, Stevi, will be getting pinned this month and Zadie will be at her commencement, cheering her on.

Thank you to all the nurses who have helped Zadie and us on this journey!  I hope for many reasons that she will not require private duty nursing much longer, but while she does, I'm grateful to have them.

Tuesday, May 1, 2012

Morphine is good!

Today was a long day! Up before 5, anxiously waiting and waiting and waiting, and then seeing Zadie post op, swollen and bleeding. Not how you ever want to see your little baby girl, but she was a rockstar. The nurse in the recovery room said she sees kids who have had less done who are much more miserable than she was. Thanks to my PRS mommy friends, Angela and Kris, we had an idea of what to expect. I don't think you can ever be fully prepared to see blood practically pouring out of your baby's nose, mouth and trach! Uncle Geoffrey, Aunt RayAnne, Emma and Grace came for a visit and brought Zadie a balloon. How did they know she loves balloons??? Zadie was standing up and playing a bit in her crib tonight. She might have done a little too much, because she was pretty uncomfortable a little later, and they ended up giving her morphine. No need to be a hero, Zadie! Daddy's home with the doggies, and I'm camping out with Z. Oh, and it's St. Joseph the Worker Day, and we're at St. Joseph's Hospital, so maybe it's a good sign? On that note, good night!


We were up before 5 am this morning, and it was thundering, lightening and pouring rain when we left the house. It was very similar weather the day we brought Zadie home from the NICU, so maybe that's a good sign? She's in surgery right now. I was able to go into the OR with her until they put her under. She was fine. I got to wear a cool onesie outfit to go into the OR. BJ and I are in the waiting room, with our contraband munchkins. Apparently, you aren't allowed to have food in here.

Monday, April 30, 2012

Big day tomorrow!

Zadie has a big day tomorrow.  She's having surgery to repair her cleft palate and get tubes put in her ears.  I'm very anxious about it, but I'm very excited to be heading in the right direction.  This is kind of the first step in Zadie getting past her issues.  Once the palate is repaired, we can focus on decannulation (getting rid of the trach)!  Her ENT said he would start checking her airway when she's about 18 months, so that's the next thing to look towards.  The last 11 months have flown by, so we can do another several months.

We have to be at the hospital at 6 am and her surgery is scheduled for 7:30 am.  It will take about 3 hours.
She won't be able to put anything in her mouth for TWO weeks after surgery.  How rough is that?  She puts just about everything in her mouth now, and her favorite thing to chew on is the stopper on her feeding tube extension.  She chews on that throughout her feedings.  We had to buy these arm immobilizers (aka "no-nos") that she will supposedly have to wear for the two weeks to keep her hands and anything else out of her mouth.  My friends who have been through the palate surgery with their kids say that it wasn't necessary to wear them that long, so we'll see.

Jonathan's having a sleepover at his cousins' house tonight, so he just knows that it's fun time!  He loves his Syme cousins!  Zadie is only staying overnight in the hospital, so we should be home Wednesday.

Please wish her luck and keep her in your thoughts and prayers!

Monday, April 23, 2012

Zadie's weekend away...

Zadie decided she needed another weekend away, so Friday morning, after a week of fighting what seemed to be a cold, she woke up with a fever. Off to the ER we went. She ended up being admitted, although I don't think it's clear what was going on. There are aspiration concerns, so she is on strictly G tube feedings for now, nothing by mouth. It's already not fun, because she's clearly interested in food. When she is recovered, she'll be getting a swallow study. I have asked a few times about a swallow study at her feeding clinic, but they have always said it was unnecessary. I will be mighty upset if it turns out that all her troubles recently have been from aspiration.

 She's still scheduled for her palate surgery next Tuesday, May 1. I was worried this latest illness would affect things, but she's still on, unless she's very congested. So, she'll be back in a hospital (a different one) next week, if all goes well! A nutritionist stopped by Zadie's room on Saturday. She said she looks like she's doing great on breastmilk. I asked her if she had any experience with the blenderized diet. She didn't seem to have too much experience with it, and mentioned how much work it would be. She mentioned that bacteria is a risk of that diet, because food is more likely to be caught in the tube. That seems like just another excuse. She also said that formula is already made to have all of the proper nutrients, etc. It's weird that a nutritionist thinks formula is better than fresh, real food. It seems like, for some reason, most medical professionals are not too enthused. I don't get it!

The nutritionist also said that breastmilk should be enough for Zadie past a year, maybe to 15 or even 18 months! So I had been planning on quitting pumping soon, but I guess it looks like I'll be doing it for a little longer. And I guess that means that I should finally BUY a pump, rather than keep renting the hospital pump. Zadie should be released today. She got her batteries recharged and enjoyed the chest PT from the nice respiratory therapists. She's ready to get out of this crib and back to cruising around the house. And hopefully not coming back here any time soon!

Thursday, April 19, 2012

The blenderized diet.

Since Zadie is getting close to turning 1 (crazy!), I've been thinking about what she'll be "eating" when breastmilk alone is no longer enough for her. Most people who are tube fed are fed formula, and I'm really not crazy about the idea of switching Zadie to formula after all my hard work pumping for the last 10 1/2 months.  I thought I could just puree up real food and put it through her tube (while still working to get her to eat orally).  Apparently, there is something called the blenderized diet, which is pretty much what I was imagining.  And apparently, it's sort of crazy talk in the medical world.  I brought up the idea to the nurse practitioner at Zadie's feeding clinic, and she was very discouraging.  The biggest thing she kept mentioning was how time consuming it would be.  That seems like a silly reason not to do something, if it's something that's good for her.

So I started looking into it more and I found out that it not a common practice, but every parent who uses it swears by it.  A lot of moms whose children were previously formula fed and then moved to the blenderized diet said it was the best thing to happen to their children.  A lot also said that their children were more interested in EATING real food once they had real food through the tube.

One thing I learned was that I needed a good blender.  It's doable with a standard household blender, but a professional grade blender makes things easier and faster.  I emailed the two big blender makers, Blendtec and Vitamix.  I had heard that they both offered a medical discount (their blenders run about $500-$600!) and also donated blenders regularly to tube fed people.  Within a day of emailing Blendtec's customer service, and telling them Zadie's story, I was informed that Blendtec was donating a blender to us!

I've been very excited about this blender's arrival, and BJ's been making fun of me (will he be making fun of me when I am able to make smoothies and shakes and daiquiris?  I don't think so).  Well, it arrived today and without even reading the book, I pureed up some avocados for Zadie.  I fed some to her by spoon, and she did ok with it.  A bunch came out her nose, which is something I won't miss after she has her palate repaired!  Whatever she didn't eat, I blended up some more and put it in with her milk through her feeding tube.  This is very exciting, and while we have a ways to go, I am less anxious about her moving away from breastmilk.  And I'm getting excited to quit pumping soon!

Friday, April 6, 2012

Why my baby is so quiet!

This is kind of an FYI for anyone who doesn't know much about trachs or hasn't come into too much contact with anyone with a trach.  I would say that describes most people!  It definitely described me before 9 months ago.

The comment I notice most when strangers are remarking on Zadie (other than how amazingly adorable she is!) is how *good* she is because she hasn't made any noise!  I always debate about explaining to them why she's so quiet, but usually I just nod and smile and say thank you.

Zadie is a good girl and she's generally very happy and content.  She doesn't cry often, but she's a baby and she does cry sometimes.  However, because of the trach, she doesn't make any noise except for the stuffy, congested noise coming out of her trach.  It's a pretty sad sight to see your baby crying her lungs out, tears streaming down her face, with no sound.  I can only imagine how frustrating it is for her.

A tracheostomy is an artificial airway.  The hole goes directly from the neck to the trachea, below the vocal cords, so the vocal cords are completely bypassed.  Some people can move air past the tube and up through the vocal cords.  I've heard that kids start to figure out that if they block the trach with their finger, they can make noise.  There's also a special valve that can be worn to allow the trached person to make noise.  This is a Passy-Muir valve.  Zadie has one, and we tried it once, but she didn't do well with it.  She has to learn (or be able to) blow air out her mouth and nose, and she hasn't done that in over 9 months.  So that will take some getting used to, as well as growing on her part, so that there's more room around the trach tube.

Zadie gets speech therapy weekly, and we're encouraged to talk to her a lot even though she's not babbling back at us.  We are also starting to sign with her, so that she has a means of communication until she gets decannulated (gets the trach removed) or is able to tolerate the Passy-Muir on a regular basis.

Needless to say, we can not wait until we can hear our little lady babbling, talking, screaming, whatever, all of the above!

Thursday, February 9, 2012

Exciting day...

Today was exciting! Zadie met her new Occupational Therapist and Speech Pathologists from Early Intervention. They all loved her (of course!) and were really impressed with where she is right now. Zadie showed off all her best moves to the OT, including her almost-crawling and pulling up to stand. She also showed off how dexterous she is.

For the speech pathologist/feeding expert, Zadie did really well with a spoon with some applesauce on it. That was the most exciting part for me. It worries me that she takes 100% of her milk by feeding tube. I don't want her to have long term feeding issues. Seeing how excited she was to get that spoon in her mouth gave me real hope that we're getting somewhere! The therapists also said that they can tell Zadie is trying to vocalize. Hopefully soon we'll be able to try the speaking valve out on her.

Next week, we have an appointment with the surgeon at St. Joseph's to talk about a plan for palate repair!

Thursday, February 2, 2012

No real direction yet...

I've been wanting to start blogging about Zadie's condition and continuing progress.  I'm finally doing it!

A quick recap - Zadie is now 8 months old and was diagnosed with Pierre Robin Sequence the day she was born.  She has a cleft palate and micrognathia (an underdeveloped lower jaw), which makes her tongue fall back into her airway, making both breathing and eating difficult.  She spent 10 weeks in the NICU, and has a G-tube (feeding tube) and a trach.  She used to take some milk by bottle, but now takes 100% by the tube.  We're working on spoon feeding now!  Because of the trach, she doesn't make any sounds.  I never imagined that I would wish to be able to hear my baby screaming her lungs out, but I can't wait for that day!

Other than her main limitations (which are temporary!), Zadie is a very typical 8 month old.  She's almost crawling, and she just started pulling herself up yesterday.  She loves her new skill, but we're still encouraging crawling!  She's also very healthy.  She's only been hospitalized once since she came home (that's good for a baby with a trach!) and hasn't had any ear infections (knock on wood).   She does have fluid in her ears, which is almost expected with the cleft palate, but it is affecting her hearing.  She'll probably be getting tubes soon.

As for the title of this post, we're still kind of up in the air as to the course of action for Zadie, and it makes me more and more nervous as she gets older.  She will definitely need her palate repaired, and that will be done somewhere around her first birthday.  But, there are other things in play and different opinions about the best thing to do.  We've seen three craniofacial teams, St. Barnabas, St. Joseph's, and CHOP.  They all pretty much have a different idea of what to do, and none of them seem to have stepped up to say, we're the best ones to take care of your daughter.  So we're still trying to figure out what to do.  It's a scary situation to have to decide for your little baby which direction to go in, when our decisions will likely have a huge impact on her life.  Right now, we're just trying to keep on top of everything and hope that the right course will be revealed.