"I do!"

Little Miss Independent wants to do EVERYTHING.  Every step of the pottying process (lifting the lid, flushing, turning off the light, etc.), pouring her own drinks, opening the door for the dogs. Her latest thing she wants to do is her own suctioning. She doesn't need suctioning too often anymore, but she usually needs it first thing in the morning. I do let her help a little bit. She also wants to be the one to pull her gauze out from under her trach every morning and night. She gets very upset if we don't let her do things, and says, "I do!"  Just a typical headstrong 2 year old with some atypical things she's learning to do!

Follow up on Sleep Study

We went to Philly on Monday to see Zadie's ENT to discuss the results of the sleep study and to talk about the plan going forward.  The nurse on the phone had told me that Zadie had moderate obstructive sleep apnea, but Dr. Javia told us it was actually severe.  He thinks the culprit might be her adenoids, so she is scheduled for an adenoidectomy on September 30.  Her tonsils have always looked fairly small, but there is a possibility that they will be taken out at the same time.  While she's under for that procedure (or procedures) she will also get a bronchoscopy to really look at the airway.  About 4-6 weeks after her surgery, she will get another sleep study.  Even though we'll already be in November by that point, Dr. Javia didn't totally shoot down the possibility of decannulation (losing the trach) this year, although he usually doesn't do it in the cold and flu season.  I'm not getting my hopes up for decannulation this year, though.  I think chances are pretty good it will have to wait until the spring.  It would have been nice for the trach to be out before the new baby comes, but it is what it is.

Zadie showing off her boo boo from getting blood drawn.

Sleep study...

Zadie had her sleep study on Sunday night.  It started off pretty eventfully, as her Gtube balloon (the tube is held in by a water filled balloon - read more about it here) burst and the tube fell out.  I didn't have a replacement with me and the sleep study staff insisted that we go to the ER to get a replacement.  I was pretty upset, because I didn't know how long it would take for the replacement, and I really wanted the sleep study to happen.  But, the CHOP ER staff was wonderful and we had a new tube in within 2 hours and we were back to the sleep lab.  I have learned a lesson: always take a replacement tube!

Zadie showing off her new tube

So now that we were back in the sleep lab, we started to get ready for the study.  Since she didn't get a good nap that day (she slept for a total of about an hour, in two increments, in the car) and we already had to mess with her to get the new tube in, I was worried she'd be very uncooperative.  She did great while the respiratory therapist (RT) started putting the electrodes on her head and body.  She got to pick which color wire was put on next.  I said to the RT, wow, she's being really good.  She said, yeah, this part isn't the hard part.  We found out what was.  The nasal cannula.

All smiles for first electrodes

Wrap my head up in gauze?  Ok!

A gauze hat with bunny ears?  Great!

Nasal cannula...not cool.

Once the nasal cannula was in (it measured the carbon dioxide she was breathing - it had a little attachment for her mouth in case she was mouth breathing), she was very unhappy.  She cried on and off until she fell asleep.  The RT brought her a coloring book and crayons and she would be happy with that for a short time but then start crying again.  We really tried to keep her from crying too much, because the cannula couldn't get too wet and neither could the tape.  I really didn't want her to have to replace the cannula.  Zadie would just think she was getting it off and then to have to put it back on would be awful.

Occupied with a new coloring book and crayons..

Zadie didn't know what to do with herself.  She was crying on and off, she wanted to be off the bed (but she couldn't be, since she was all wired up and connected already), she wanted me holding her, she wanted me on my own bed, she wanted her milk, she didn't want her milk.  I finally got her to lay down by telling her I'd come back to her bed if she put her head on the pillow.  She did, and I did, and she fell asleep soon after that.  The RT told me when it was safe to move (she could see from her readings when she was in a deep sleep).  She slept fairly well, but she did snore a lot (just like Daddy!).  I knew that wasn't a good sign.  But she would sort of wake up every so often, crying.  I finally got to sleep, and then she woke up about 4 am and puked.  Her cup of milk was still on the bed, and I think she drank it and it wasn't very fresh.  We got her cleaned her up and she went back to sleep pretty quickly.

Despite it all, a smile at 4 am!

We were woken up at 6 am.  The RT cleaned up the wax out of her hair as best as she could and we were sent on our way.  Zadie was so happy!

So happy to be heading home!

I got a call from the ENT's office today and she briefly went over the results.  Zadie did have some apnea and that has to be addressed.  We have an appointment to see the ENT on August 12 to discuss the plan, but there's no way I'm going to be able to wait that long to find out what the plan is, so I'm going to call tomorrow and try to get more information.

The sleep study is two days away!!!

I'm getting very anxious as Zadie's capped sleep study is practically here!  I'm worrying about every aspect.  I want her to be 100% healthy so that something as simple as a cold doesn't hurt her results.  I want her to be tired enough to sleep easily with a million wires all over her and in a strange bed, but with a two hour ride to get to the hospital (CHOP), it's pretty inevitable that she will nap that day.

I am really excited that this will possibly be the beginning of the end of trached life for Zadie, but I'm really trying not to get my hopes up.  I am trying to stay positive, though, and not think about any negative possibilities.

Please keep Zadie in your thoughts on Sunday night for a peaceful and easy sleep with good numbers!

Persistence is key!

Since I made the appointment for Zadie's sleep study a few weeks ago, I've been calling every other day to check for cancellations.  Today, my persistence (or pestiness?) paid off!  A lovely woman by the name of Mary set Zadie up with an appointment on July 21, a full month earlier than the original appointment (and less than two weeks away!).  I'm very excited and hopeful that this will go very well.

The sleep study is scheduled!

The sleep study is scheduled.  Unfortunately, it's not until August 21!  I guess the doctor wasn't kidding when he said it would be about two months away.  The lady I talked to said that there are always cancellations and that I can call every other day to check.  So I guess that's what I'll be doing for the next two months!  Hopefully we'll be able to get in sooner.

CHOP update

We all took a ride down to Philly today. It was a crazy rainy day which made the trip even longer.  Because of the weather, I guess a lot of the appointments before us were running late.  Zadie's appointment was at 11:20 and we didn't see the doctor until after 2!

Dr. Javia looked at Zadie's ears.  She has an infection in her left ear, which we suspected.  Since she has a tube in there, it didn't seem to be hurting her, it was just draining a lot and she kept picking at it.  She was prescribed antibiotic drops for that.  The tube in her right ear fell out and is still sitting in her ear canal.  He said it will eventually fall out of her ear.  Even though she didn't pass her hearing test in that ear in April, possibly because the tube is out, we will just monitor it for now and will not take any action.

Since Zadie is doing so well with the capping, we are now cleared to get scheduled for a sleep study.  Unfortunately, Dr. Javia said it might be a couple of months until we can get in.  But, he recommended that we keep calling to ask about cancellations, etc.  If the sleep study goes well, then Zadie will be scheduled for decannulation.  When she had her bronchoscopy in September, Dr. Javia noticed that her adenoids were rather large, and they will possibly be a problem.  We won't know that until the sleep study, though.

I'm waiting for the sleep study department to call me with a date.  I'm very anxious about all of this.  They will not attempt decannulation in the winter, so we have a limited amount of time to get everything done.  It will all happen eventually, though, so if it doesn't happen this year, all is not lost.  I'm just really hoping it does!

As all good trips to Philly end, we stopped at Pat's for some cheesesteaks.

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!

A year ago today...

A year ago today, Zadie had her palate repaired.  I can hardly believe that much time has gone by!  That surgery was what we were looking towards her first 11 months, and here it is, a whole year after it.  It was not an easy surgery, but she bounced back remarkably well.

Right after surgery 5/1/12

Before her palate repair, she could barely suck from a bottle.  Now she drinks like a champ and is really improving with eating solid food.  And her food doesn't come out her nose anymore!

Eating!  4/2013

Hearing test

I took Zadie to get a hearing test on Friday.  Her ENT at CHOP wanted it done, and rather than trekking all the way to Philadelphia, we were able to visit their specialty center in Princeton.  

The audiologist checked the placement of Zadie's ear tubes first.  The left one was in place and unblocked.  She could see the right one in her ear, but it's either blocked or not in place.  Therefore, she has some hearing loss in that ear (which is hopefully temporary).  

I talked to the ENT's office, and we are going to try to unblock the tube by dropping hydrogen peroxide into her ear 2-3 times a day for up to a week (or if she starts showing signs of pain, we will stop sooner).  Once we are done with that, we will be dropping ofloxacin drops in her ear for 5 days.  Hopefully this will unblock the tube.  We are seeing the ENT in June anyway, so by then, he'll be able to look in her ear and see what needs to be done.  

In other news, Zadie is doing great wearing her Passy Muir valve.  She wears it all day while she's awake (we take it off for naptime) and she's even building up the time that she wears a cap (which completely blocks the trach and forces her to breathe only through her mouth and nose.)  Since she's been wearing the Passy Muir so much, she's been vocalizing a lot, and is really building up her vocabulary!  I'm hoping that by the time we see the ENT in June, she is wearing the cap all day and we can schedule a sleep study to see if we can get rid of the trach!

Questionable urgent care & the response

Last Wednesday, Zadie's fever just wouldn't quit, even with regular doses of alternating Tylenol and Motrin.  I called her pediatrician and the soonest appointment was 6:45 that night.  I didn't want to wait for her to be seen, and since there was a good chance that her ped was going to want her to get an xray, I decided just to take her to the new urgent care place that I had been to twice recently, once for Zadie and once for Jonathan.  It's a really nice place, and they can do xrays there, so we can avoid trips to the ER.  I really liked the place the other two times we'd been there.

However, I'm not so sure about the care Zadie received there this time.  I pointed out Zadie's gunky eyes to the doctor, but she didn't say anything about them, and didn't prescribe anything for them.  I figured that meant they were junky just from her congestion.  That night, after the bloody eye incident, her pediatrician immediately diagnosed her with conjunctivitis and prescribed drops.  And, two days later, the eye doctor confirmed the infection.

Then, later on Wednesday night, Zadie's nurse questioned the prescribed dose for the oral steroid.  The doctor at urgent care had prescribed 15ml every day for five days.  It seemed like a lot.  When we looked back at the same medicine she was prescribed about a month earlier, the dose at that time was 3.5ml every day for five days.  Very big difference!

On Thursday, I called both the pediatrician's office and Zadie's pulmonologist's office to ask them about the dosing, since the urgent care wasn't open yet.  The pediatrician said 15 ml was way too high a dose for her, and said it should be 4 ml.  The pulmonologist said she could have a dose of 7.5 ml, but she also said the 15 ml was way too high.  Later on, I spoke to the doctor at urgent care who saw Zadie.  I asked her about the dose and she said, oh, that's way too high.  She apologized profusely and said that the correct dose should be 3.5 ml.  Pretty concerning that such a big mistake was made.  I then asked her about the conjunctivitis and she said she did look at her eyes, and didn't feel that it was conjunctivitis.  She continued to profusely apologize for the mistake in dosing.

Yesterday (Monday), the medical director of the urgent care called me.  He asked how Zadie was feeling, and then he told me he was made aware of the dosing mixup and he apologized profusely.  He said because of this mistake, a new policy was in effect at the office to avoid this happening again.  Apparently, they generally go by kilograms for weight, but someone wrote down Zadie's weight in pounds, and that's where it went wrong.  Zadie's only 10 kg, but that's 22 lbs.  So someone read her chart as if she weighed 22 kg.  Their new policy is to only record weights in kg so there are no more mixups.

What's scary about this is that there's no real way to know, as a lay person, whether a dose you've been prescribed is correct.  I'm not sure if the pharmacist should have picked up on the mistake, but it wasn't picked up there.  If Zadie's nurse wasn't the one giving the med, I'm not sure it would have been picked up at all.

Good news is, Zadie is feeling much better!

Eye doctor visit

We took Zadie to see the eye doctor today to follow up on the bloody eye incident.  He checked out her eye, looked at the picture we took, and was still a little puzzled.  He said it was probably just from the infection (conjunctivitis) in her eyes.  He was puzzled especially because her eye wasn't bloodshot at all.  But, the good news is that her eyes look great.  Since Stickler Syndrome, a condition that involves eye problems, is often associated with Pierre Robin Sequence, she was due to have an eye exam.  Like all of the other doctors who have looked at her, the eye doctor didn't suspect Stickler Syndrome for her, just based on her physical appearance.  He dilated her pupils and checked her vision, and it's good!  She's feeling much better and her pink eye is improving!

Zadie's bloody eye

Zadie likes to keep things interesting.  After visiting urgent care with her yesterday afternoon for her respiratory issues and fever that wouldn't quit, we went home.  Her eyes were gunky and I mentioned it (and it was obvious) while talking to the doctor at urgent care.  She didn't say anything about the eyes, though, and didn't prescribe any drops.  Later on in the evening, while we were eating dinner, Zadie must have gagged on something and started throwing up.  For some reason the milk/Carnation she had been drinking for about an hour was a huge chunky disgusting mess that was practically choking Zadie as it was coming up.  As she was struggling to vomit it up. a blood tinged tear ran down her face.  Very shocking, to say the least.  I wiped it away and more blood appeared.  By the time she was done, she had a bunch of blood all around her bottom lashes and she had bloody tear stains down her face.

Sad baby crying bloody tears

I called her pediatrician, and even though it was 8 pm and I expected to get the answering service, they were still in the office!  After talking to her ped, he said he wanted to see her.  Meanwhile, I was frantically paging Dr. Google, looking for something to quickly tell me this was normal.  I couldn't even find anything close to what just happened to Zadie.

The pediatrician called a pediatric ophthalmologist, who was pretty puzzled himself.  He even said that it was odd and he'd never seen it before.  He wanted to see her in her office, and we're going tomorrow morning.  Zadie's pediatrician also said she had conjunctivitis (pink eye).  I'm not sure why the doctor at  urgent care didn't diagnose that.  He prescribed drops and said to irrigate the eyes really well before using the drops.  When I did it later, Zadie was really upset, crying very hard, and she had some more bloody tears.  Both times it happened, it was only from her left eye.  It hasn't happened again, thankfully.

After a lot of googling, BJ found the term haemolacria.  Most of the pages that come up when you search that term are only talking about how rare this condition is.  Wikipedia mentioned that it's sometimes caused by bacterial conjunctivitis.  So we'll see what the doctor says.  Never a dull moment, that's for sure!

Airway Clinic

Zadie and I drove down to CHOP today to see Dr. Javia, her ENT.  He only sees airway patients once a month, and he's not seeing patients in March, so even though I knew we weren't really prepared to go back and see him, I didn't want to have to wait another two months to see him.

There were a LOT of trach babies in the waiting room!  It was very exciting for me.  Zadie didn't really care.  We've seen one or two in the waiting room at other appointments, but today was like a convention.  I've still never seen a trach baby in the real world!

Since the last time Zadie saw Dr. Javia in November, we were supposed to have been working on getting her to wear the Passy Muir valve all day.  She's been sick on and off since then, so we weren't working on it while she was sick.  And she's just really a little stinker when it comes to wearing it.  I've gotten her to keep it on for 15 or 20 minutes at a time, a few times.  But most of the time, she pulls it off after a few minutes.  It's frustrating because we need to get her tolerating the PMV all day and then we can move on to the cap.  She needs to be tolerating the cap all day and pass a sleep study before she can lose the trach.

So today, Dr. Javia said to come back and see him in six months.  My heart dropped, because since the last appointment, I've had spring in my head as a possible time for decannulation.  But, since we haven't really made any progress with the PMV, we're not really any closer.  I am disappointed in myself for not pushing her harder to wear it.  But all I can do is look forward and be tougher with her.  I keep reminding myself that she WILL get decanned one day.

Early Intervention Annual Review

Zadie had her annual review yesterday for Early Intervention.  Early Intervention is a state program that is designed to help kids with developmental delays catch up in time for school.  She's been receiving services through EI for over a year now.  She gets speech therapy and occupational therapy.  We had two meetings to update things yesterday.  During the first one, a test called BDI (Battelle Developmental Inventory) was administered.  The therapist asked me a bunch of questions about different things that Zadie could or couldn't do and gave Z tasks to try to do, like stacking blocks and using nesting cups.

Little Z did great on the test!  She improved greatly since the first test was given over a year ago.  All of her scores improved.  In fact, based on that test alone, she does not qualify for EI.  However, since she has the trach and the related speech delay, they used a clinical assessment to determine that she is still eligible to receive services.  She'll continue receiving speech therapy once a week and occupational therapy will be reduced to once a month.  She babbles a lot and she is starting to say words.  The three big ones right now are mama, dada and baba.  She is starting to mimic sounds we make, and she said "duck" yesterday with the therapist while looking at a book with ducks.  She's still signing pretty regularly and still picking up new signs, so all in all, she communicates pretty well.

Plastic surgeon follow up

Zadie had another follow up with the surgeon who repaired her palate. It's hard to believe that it's been more than 8 months since she had the surgery!  He said she looks great, and in particular, he remarked on how good her lower jaw is looking. He said that he's glad we never did anything surgically to it.  Her lower jaw was recessed when she was born (a hallmark of Pierre Robin Sequence), and it was a major reason she had trouble eating and breathing. If she had been born in another hospital, she might have had jaw distraction right away, and she might have avoided the trach. But, she was born where she was and they don't really do jaw distractions at St. Barnabas. So, she ended up with the trach. A plastic surgeon at CHOP later suggested a jaw distraction in order to get rid of the trach, but we weren't positive that it was completely necessary, and we decided to wait and see what her jaw did on its own.

It's possible that she will still need some kind of jaw surgery in the future, but right now, she's looking good! We're hoping to get rid of the trach in a few months and just keep moving forward.

Knocking on wood....

Zadie's been really healthy!  (knocking on wood).  Her brother has been feverish on and off for the past two days, so I'm afraid of her catching whatever it is he's fighting.  But meanwhile, she's been great.  I've been trying to work on the Passy Muir Valve (speaking valve), but she's such a little stinker that she doesn't want to keep ANYTHING on her trach at all.  When we're out of the house, I'll manage to get her to keep the HME on most of the time, but she still pretty much takes it off constantly.  And when we're home, she pretty much downright refuses to wear one.  So when I try to put the PMV on her, it's the same issue.  She'll keep it on for a minute or two and then pull it off and hand it to me.  I talked to the ENT's office today to get suggestions on how to get her to wear it longer, and they just suggested distractions (like TV, iPhone, etc).  Any trach moms have any other suggestions?

Busted balloon

I really look forward to the day when Zadie doesn't need any foreign objects in her body to help her live. There's always the concern of her trach or Gtube coming out. Today, her Gtube popped out as we were leaving soon. It's held in her body with a water filled balloon. Occasionally, the balloon will pop or get a leak. I realized that was the case this time since the balloon was almost completely deflated.  This is an illustration of the type of feeding tube Zadie has, a Mic-key button.

We have a backup tube, but it was at home. I decided to pop the broken one back in and tape it up. The closest thing I could find was Scotch tape. Not ideal, but fine for the two minute drive home. Somewhere between school and home, the tube fell out again and I haven't found it yet! When we got home, I put the new tube in. By now, this is old hat for both me and Zadie. I took a picture of her belly with the button in to share here. 

Cute Zadie belly

As I said, I look forward to the day when we don't have to worry about this stuff!

Happy New Year!

Zadie's 2012 consisted of too many ER visits, five bouts of pneumonia, one very nasty case of RSV, two surgeries and a lot of learning and growing and fun!  We got great news when I checked the mail on Christmas Day (mail that had been delivered the day before) and got the letter from the insurance company that our second level appeal had been approved and Zadie was now back to 16 hours a day, 7 days a week of private duty nursing.  We are in the process of trying to get the shifts staffed.  I look forward to sleeping in a real bed, since my air mattress seems to be losing air quicker and quicker every night.  It might have to do with the 90 pound dog that likes to join me sometimes or the cats that like to jump around on it.  I also look forward to Zadie being able to be home during the day, rather than coming to daycare with me and Jonathan.  It's nice for her to get some socialization, and it's really nice to see her all day, but her health is more important right now, and I'm afraid that she will have a very rough winter if she continues to come with us.  

Zadie learned all sorts of stuff in 2012.  She learned to walk and sign and she even gained back her voice!  It's not 100%, but she can get very LOUD when she wants to express herself.  She can also say mama and baba meaningfully.  She got her palate repaired back in May, and can drink like a champion.  

By 2014, I would love it if Zadie could lose her trach and her Gtube.  I won't get my hopes up or put too much pressure on anyone, but it would be great.  For now, she is happy and healthy and that's all we can ask for.