Friday, June 22, 2018

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:

Friday, January 27, 2017

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. 

Wednesday, March 30, 2016

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. 

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:

Friday, May 8, 2015

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. 

Wednesday, May 6, 2015

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. 

Monday, September 8, 2014

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.

Wednesday, August 13, 2014

I was and am a successful breastfeeding mother!

I'm very excited that Zadie and I were included in a 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!