Miracle Walk 2019

Zadie is now 8 and about to enter 3rd grade!  She's doing so well.  We remain so grateful to St. Barnabas NICU and the doctors and nurses who do such wonderful work there.  We're walking in the Miracle Walk again this year, our 9th Walk!  The Walk raises money to make the NICU even better. 

Our family at the 2018 Miracle Walk

Mirace Walk 2018

Every year since Zadie was born, our family has participated in the Miracle Walk, which benefits the Saint Barnabas Medical Center Neonatal Intensive Care Unit (NICU), where Zadie was for 10 weeks following her birth in 2011.  The doctors, nurses and staff are incredible there, and the Walk helps ensure that wonderful care continues!

It's also a really great way to reunite with our nurses and doctors and fellow NICU families.  It's always a great day.  Please consider walking or donating by clicking on the following link!  Thank you!

My Miracle Walk Fundraising Page

Our family at the 2017 Miracle Walk

A cleft palate is not a cleft lip!

What do you think when you hear the words cleft palate?

If you have no personal experience with it, you might automatically think of a cleft lip, which is usually visible before it's repaired since it involves the actual lips of a person's mouth. You might see a scar in the space between the nose and the lip on someone who's had a cleft lip repaired.

However, cleft palate is different.  The palate is the roof of the mouth, which you can't see when casually interacting with someone.

Kids born with Pierre Robin Sequence (PRS), like Zadie, are often born with a cleft palate.  It's usually discovered at birth or soon after.  A cleft palate is difficult to diagnose in utero, which is why a diagnosis of PRS is often a surprise.  In Zadie's case, I had weekly ultrasounds leading up to her birth, and the cleft palate was not detected.  Cleft lip, which is more likely to be diagnosed in utero, is not usually seen in PRS babies.

I can't tell you how many times I've told someone about some of Zadie's issues and they say something like "wow, you can't even tell!" Hopefully, this info will help increase awareness and knowledge!

Facts about cleft lip and cleft palate:

https://www.cdc.gov/ncbddd/birthdefects/cleftlip.html

Update on Zadie

Zadie (on the right) with her brother,

Jonathan, and sister, Beatrix

It's close to a year since Zadie had surgery to close her trach stoma.  She's doing great!  She's in kindergarten now, and is in gymnastics and dance classes.  She loves to draw and play with her brother and sister.  Overall, she's a typical 5 year old.  Most people who just met her recently have no idea of everything she's been through, and I couldn't ask for more than that. 

In the fall, we started noticing that she wasn't hearing very well.  At first, we thought it was a "listening" problem, but then we realized it was more likely a hearing problem.  We took her to the doctor and found out that she had a terrible ear infection along with lots of fluid in her ears.  Interestingly enough, even with a raging ear infection, she never once complained about her ear.  She failed a hearing test and was put on some meds to help clear up the infection and fluid.  After a few more visits to the ENT, and the fluid wasn't clearing up very well, as well as another failed hearing test, we were able to get her an appointment with one of the pediatric ENTs from CHOP, but at their satellite office in Princeton.  Princeton is a whole lot closer than Philly! 

We were basically expecting to hear that the fluid was still there and that she'd need tubes again.  She's only had tubes put in once, at the same time as her palate repair at 11 months.  Kids with Pierre Robin Sequence often need tubes throughout their life.  Because of the way their anatomy is, they're more likely to have fluid buildup.  Zadie's been pretty lucky in that she has only had tubes put in once. 

The ENT looked in her ears and right away said they looked great.  He didn't see any fluid.  After we saw him, she was given a hearing test.  The test went great!  There were slight issues, but nothing of major concern, and the issues could have been just because she was on the tail end of an infection/fluid problem.   

Next up, some dental issues, but nothing major yet.  And she has a follow up appointment with her pulmonologist in a few weeks.  I'm going to ask him about a sleep study for her.  She hasn't had one since before she was decannulated and she does snore sometimes, so I want to make sure she's sleeping ok. 

And that, my friend, is what they call (stoma) closure.

Waiting with her Tubie Friends bear - who also got a hospital bracelet

Zadie is now blessed with only the holes God gave her.  She went in last week to have her trach stoma surgically closed.  At the same time, the surgeon cleaned up the scar tissue at her Gtube site and made sure that was fully closed.  I was able to go into the OR with her and be with her until she was asleep.  The surgery took about an hour and a half.  The anesthesiologist told me after that she was easy to intubate, which is excellent news, since a huge reason for her being trached was how difficult she was to intubate.  It's another sign that her lower jaw has grown out significantly and her upper airway is vastly improved.  A lot of Pierre Robin kids get jaw distractions, and it was suggested as a possibility for Zadie at one point.  We're very happy we elected to wait and see how her jaw grew out on its own.   


All ready to go!

When I first got to see her in recovery, she was still asleep.  They extubated her while still in a very deep sleep so that she didn't cough.  I saw Dr. Bergman right after surgery, he said everything went great.  I asked about the main possible complication, which is when air gets trapped between layers of skin.  It can be a very serious complication.  He was very confident that it wouldn't happen, since they had tested the closure in the OR by pushing air through. 


Once she was awake, she was pretty cranky, which is what she's typically like after anesthesia.  But, her oxygen sats were awesome.  Upper 90s.  I was so relieved to see that.  One of my worries going into this surgery was that she was relying on the open stoma to breathe and she would struggle once it was closed.  Seeing the good oxygen levels really reassured me. 




Great numbers!

She was to be admitted for the night just for observation, and it took forever to get a room in the PICU.  Unfortunately, she couldn't eat or drink anything for 6 hours after the surgery.  She was getting IV fluids all along, but she was hungry and thirsty.  Once we got past the 6 hours, she was allowed liquids, which included jello, yogurt, ice cream, juice, etc.  Then, the next morning, she was finally able to eat real food. 










Her new bear got bandaids in the same spot Zadie did. 

First meal, 24 hours after surgery

Feeling great and ready to bust out

 

She got released on Saturday afternoon.  We took the dressings off on Sunday.  She was a little freaked out by the sight of the incision on her belly.  She was asking a lot of questions; why was there blood, how did the doctor cut her, etc.  I wasn't expecting that!  But, it's amazing to see her neck without a hole!  And it's amazing to hear her talk without hearing the air rushing out of the stoma. 

Escaping!

Every day, she shows me her belly and her neck and says, "Look, it's healing up!" and asks me to take a picture.  She's definitely a strong kid and I would love it if this is her last surgery and she can just be a regular kid now. 

Some info on decannulation and stoma closure: http://www.chop.edu/treatments/tracheostomy-and-decannulation#.VvGl2W_mqM8

Dental visit

A couple of weeks ago, I took Zadie and her sister, Beatrix, to the dentist for regular check ups.  It had been about a year since Zadie's last dental visit.  She was such a cooperative kid for the dentist!  Considering how willful (to put it nicely) she can be, it was amusing to hear the dentist and the assistant gush about how great she was.  She sat where they wanted her to, opened her mouth when they wanted her, etc.  The dentist saw a small cavity, unfortunately.  They don't normally take x-rays this young (Zadie will be 4 this month), but the dentist wanted to confirm her belief that the cavity was not very deep.  Since it isn't very deep, and since she's so cooperative, they plan on taking care of the cavity using just novocaine.  They mentioned using laughing gas, but with her airway issues, it would be best if we could avoid anything like that. 

In looking at the x-rays, Dr. Emelie noticed that one of her permanent adult teeth is missing.  She said it's possible that it's there, but just isn't showing up, but it looks like it's not there.  From what we have heard from other parents on Pierre Robin Sequence groups we're in on Facebook, it seems like a fairly common occurrence.  I'm interested to find out just how common it is. 

Additionally, one of her teeth is loose already.  It's one of the front upper teeth. Last year, she had a bad fall which badly bruised her gum, and that could have contributed to this.  Coincidentally (or maybe ironically?) the loose tooth is in the spot where there is no adult tooth. 

The dentist office has an orthodontist in the office some days, and Dr. Emelie suggested that Zadie see him at one point, just to have him take a look.  He happened to be in the office that day and took a quick look.  He said there's nothing to do now, but we'll talk more down the road.  Kids with PRS often have lots of dental issues, so I guess we're already starting to see that. 

She's going back next week to get the cavity taken care of. 

6 months since decannulation!

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

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

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

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

 

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

Naked neck finally!!!

 

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