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. 

Third trachiversary

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

July 4, 2011

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

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

July 5, 2011

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

July 5, 2014

Happy 3rd birthday! (a few weeks late)

Little Miss Zadie turned 3 years old a few weeks ago.  Her birthday fell on a Saturday, so we had a great day.  We went to Jonathan's soccer game, out for breakfast, Build a Bear workshop, ice cream, and then home for cake and presents.  It's amazing to look back over the last three years and to see how far she's come.  More good stuff ahead, I'm sure!

Lots of updates!

Zadie had her bronchoscopy/sleep endoscopy in March at CHOP.  Dr. Javia said her airway looks great, with no obvious issues!  This is good news, but also bad news in a way.  At least if there was an obvious issue, there might be an explanation for her obstructive sleep apnea (OSA).  But, as it is, there are no clear reasons.  Dr. Javia did say that her tonsils were bigger than the last time he saw them (which was when he removed her adenoids.)  That's the last possibility for a cause for her OSA, other than just a hypotonia of her airway, which is something that can't be surgically corrected, she'd just have to grow out of it.  We are taking her to see him this coming week so he can look at her tonsils again and think about having them removed.  We'll be pretty upset if she has to go under for another surgery, when he could have taken her tonsils out when he was in taking out her adenoids in September, but at the time, he didn't think it was necessary.  Dr. Javia also said that there is the possibility of decannulating (removing the trach) and putting Zadie on CPAP.  We are very interested in this possibility.  I can't imagine it will be easy to get her to wear a mask on her face to bed, but I know other kids do it.

A couple of weeks after the CHOP visit, Zadie developed what looked like a pimple on her belly, right under her Gtube site.  It got bigger and redder and I ended up taking her to the doctor.  He took a swab of the pus inside and put her on antibiotics.  It turned out to be a staph infection.

Staph infection by Gtube

She ended up having a reaction to the antibiotic, Bactrim, so we stopped that, but her pediatrician felt that she'd been on it long enough and it was ok to stop.  

Red cheeks - reaction to Bactrim

Rash on chest that spread everywhere - reaction to Bactrim

Meanwhile, she was weighed when she went to the doctor for the staph infection, and she had gained weight since January, when she went to see the GI!  Between the weight gain and the fact that the Gtube site was getting yucky (and, I imagine, contributed to the infection), we decided to pull out the tube.  We waited until morning so that her stomach was empty, and just pulled it out.  It was a very exciting day.  I wasn't sure what we'd have to do to keep it from leaking, but it closed up very quickly, within a few hours, and we never had any leakage from her stomach.  Since she had some granuloma (scar tissue) built up around the site, it's not looking the prettiest, but I'm hoping as she grows that the scar tissue will lessen and be less noticeable.

One tube down, one to go!

In other news, Zadie has had two evaluations with the child study team from the school district so far.  One was with the speech pathologist and the other was with the learning consultant.  From what I understand, she did very well in the evaluations, which is great, but it also means she most likely won't qualify for the district pre-school.  It is pretty impressive that a kid who has had so many factors against her barely even has a speech delay.  Both Pierre Robin Sequence and having a trach are major causes of speech delays and issues.  But she is doing really well!  I'm back to work at the daycare now and when her nurse went on vacation last week, I took her to work with me.  Even though the class I have is a little younger than her, she wanted to stay with me and she did very well with the whole preschool/daycare thing.  She loved wearing her backpack and having a lunch box and she loved playing on the playground with the other kids.  One way or another, by the fall, I would like her in a preschool so that she gets out in the world!  She'll be turning 3 next month, so her speech therapy with Early Intervention will end at that point.

She's currently obsessed with Frozen, just like every other little girl in the world.  She's going to have breakfast with Elsa tomorrow morning, which should be a lot of fun!

I'll leave you with a picture of Zadie and her big brother and baby sister from a photo shoot we did about a month ago.  Super cute!

Jonathan, Beatrix and Zadie

ENT visit

This past Monday, we took Zadie to see her ENT, Dr. Javia.  Since she had failed her sleep study so horribly, we went to see what the next steps would be.  Unfortunately, Dr. Javia doesn't really know WHY she failed the study so badly.  It could be hypotonia of the base of her tongue/upper airway, so that it falls back and partially blocks her airway while she's sleeping.  There is a possibility of more granulation or scar tissue since the adenoidectomy in September.

So Zadie will be having a sleep endoscopy and a bronchoscopy in March.  The sleep endoscopy is similar to a bronchoscopy in that they're looking at her airway, but from what I understand, the anesthesia is a little different so that it mimics sleep so that they can see what is going on inside when she's sleeping.  If they find any scar tissue, they can take it out then.  If nothing needs to be done, she should be able to go home that day.

Unfortunately, if it's the hypotonia, nothing surgically can be done and we would just need to wait for her to grow out of it.  And there is no way to know how long it would take for her to outgrow it.

Meanwhile, we are waiting to hear from the insurance company about her nursing hours.  The ENT's office sent the company the notes from this week's visit.  Since there are no plans for decannulation at this time, I am hoping that we will be able to keep at least some of our hours.

On another note, we saw a few other trached kids in the waiting room.  And we met a little girl (not trached) with PRS!

Zadie turned 2!

Zadie turned 2 on Friday!  She had a fun day, spent some time at Jonathan's preschool, then we went to Chuck E. Cheese and then out to dinner.  For the first night in over a week, she didn't need oxygen while sleeping.  Yesterday, Grandpa and Uncle Sammy came over.  I made cupcakes and we attempted to sing happy birthday, but it was confirmed that she really doesn't like people singing happy birthday to her.  She gets very upset!

Just an update on her progress...she is talking a lot. She still gets speech therapy once a week, and is saying all sorts of words, including uh-oh, oh no, water, wow, mouse, and the old favorites mama, dada, baba and baby.  She's getting big, too.  She went to see her pulmonologist the other day and she's now 24 pounds, which is great.  She's moved up in percentiles.  She's eating more and more.  She's also getting very fresh, scuffling with Jonathan and throwing toys.  Typical 2 year old, I guess!

Fresh out of the oven May 31, 2011

May 31, 2013

You can really see the difference in Zadie's chin from her newborn picture to her current picture.  One of the things about Pierre Robin Sequence that made it difficult for Zadie to eat and breathe was the recessed chin (micrognathia).  If she was born at another hospital, she probably would have had a jaw distraction, but St. Barnabas doesn't really do them and they opted to let her chin grow out on its own.  A plastic surgeon at CHOP wanted to do the distraction later on in order for her to lose the trach, but we decided to wait and see what happened.  So far, I think we made the right decision.  Her chin has grown out a lot and her trach is capped all day long, meaning she's breathing through her mouth and nose.  We're hoping she is decannulated (loses the trach) this summer!

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!

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.