Cleft Palate Clinic

When Kate was discharged from CHOP in Dec of 2006, her plastic surgeon told us he'd like her to be seen by the Cleft Palate Clinic when she was 2 1/2. I made that appointment, but ended up having to postpone because of the stoma closure surgery this past April. They fit us in in November and we went 2 weeks ago having really no idea what to expect. It was fine, but I'm not sure we'll be doing this every year as they would like us to be. It's certainly not that we are not taking Kate's diagnosis seriously - it's just that she is SOOO typical now. Plus the issues she does have remaining (she does have a Sticklers dx, hearing and vision issues) are being handled through her pediatrician, opthalmologist and ENT). I'm not 100% certain we need to do the all day appointment thing, because it really seemed like it was as much for their research purposes as it was for her.

Speech
The first person to see Kate was a speech therapist. She asked Kate to repeat several words and phrases to her, held a mirror under Kate's nose and told us Kate was doing well. (Really? well golly we had NO idea - insert eye roll emoticon here). She said it was important that no air escape through Kate's nose during speech --- but didn't say why. Ok then.

Plastics
Plastics was next. I have no ill will towards the CHOP plastics department (except for that one Fellow who really ticked me off after the palate repair but I'm not holding a grudge). They did a beautiful job on Kate's palate and her tongue and lip are no worse off for having a failed TLA way back in 2006. What irritated me about this day was their need to take a full medical history including asking about my pregnancy. Seriously guys? Kate's a got a chart there and all her procedures were done there -- why go through all this again?? Ok, I'll get over it. We saw Dr. Kirschner again and that was nice. He hadn't seen Kate since Dec. 07 when he repaired her palate. He came into the room, looked at her and said, "it's nice to see her without all the other acoutrements she used to carry around". Yes doc, it is isn't it? He asked her a bunch of questions, asked her to repeat certain words and phrases (during which she suffered performance anxiety and some words sounded very baby, and Kate doesn't sound baby at all) and held a mirror to her nose. He said her repair looked great and was working well. Apparently the palate is used as a "valve" during speech and hers was working perfectly - no air was escaping from her nose when she talked. He also told us there was a small chance she would need another repair at about age 13 - but since her repaire still looked good, that may not happen, just have to watch it.

ENT
Some kids with PRS don't have an ENT or maybe they only have one for ear tubes. That's not the case with us but we didn't see our regular ENT during clinic. I knew we wouldn't because he was doing a bronchoscopy on a Facebook friend's son that day. Anyway, we saw a Nurse Practitioner who we didn't know and who could NOT stop talking about how great Kate's speech was. She told us not only does she have no nasality at all to her voice (can be a very common side effect of formerly having a cleft palate) she verbalized and articulated more like a typical 4 or even 5 year old - not a 3 yr old with a repaired palate. That was nice to hear! She didn't keep us long (could see on her computer that we had history with Dr. Jacobs and continue to see him regularly) which was even nicer.

Audiology
A long wait to hear the same results we heard in June when Dr. Jacobs office did the exact same tests -- mild hearing loss, keep an eye on it. Will do. We know she has some loss based only on the tests. Her speech is impeccable (see above) and she can clearly hear most things just fine. The loss occurs with some tonal things. We'll keep checking it, and I let her preschool know just to be proactive.

Dental
This was the real reason we trekked to this appt. While Kate's baby teeth have come in fine (and all are present), some are crooked and we have some concerns about there being enough room in her mouth for her adult teeth. Overall, the dentist said her teeth look good, keep brushing and see a regular dentist who you've made aware of her medical history and who will keep them aprised as well -- when they do get xrays of Kate's mouth, CHOP wants a copy. The orthodontist said she will almost definitely need work but within the same time frame as any typical child. The final determination will be after she has lost her front top and bottom baby teeth and the adult teeth come it. Likely about 11 she will get a set of braces or the like. Fine by us.

Social Work
This one cracked me up. They didn't require us to see genetics (even though she has a known genetic syndrome) and they didn't require us to see nutrition (even though she had a rough time with that) -- but they made us see social work. I guess it was so they could give us resources for support if we need, just seemed silly to me. But the social worker was lovely and I could have sat there and talked with her for hours.

Developmental Pediatrician
The pediatrician said she was doing fine, but took measurements of her entire face. Fine.

That was pretty much it. We'll definitely keep in touch with the dental people and of course ENT - still out to lunch on the rest.

Updates...part 2

I've been remiss in posting lately. Some of it has to do with just life getting in the way, but mostly I wonder if people want to read about Kate anymore. Her life is really that of a typical 3 yr old. There is hardly anything of note to comment about. That's good - it's what we've been working towards since she was born and I'm really glad she's come so far.

She started preschool last month. There were tears at first, but she's muddled through and loves her school now. She's my 4th kid to go to this school. I trust the program and have never looked back once I put them in it. I'm not at all the mom who is hovering at the closed door, peering through the window getting all teary about their baby. No, I dump and run. But with Kate, I did linger more - especially when I saw them plop her right in the middle of the line and she has no idea what "a line" is for. They treat her as any kid, and I'm not even sure if the teacher knows her full medical history - but I do and just watching them treat her regular made me cringe a bit. I got over it fast but I couldn't help but want to shout out "don't you kow what she went through!!". It's fine, I maintained my dignity and Kate has found her place in the class so it's all good.

Potty training took longer than I wanted it to, but it's pretty much done. Who ever says girls are easier - lies.

Kate turned 3 in September and had her well check a few weeks ago. This was her first full year trach free and her first full year without a feeding tube. She did great, gained 5 lbs over the year and jumped from the 5% to the 20% in weight. Her appetite is typical for a toddler and she clearly is able to monitor her intake appropriately. Whew!

Next Thursday she has her first appointment with the Cleft Palate Clinic at CHOP. It's an all day affair which I'm not sure how I feel about. On the one hand, I'm looking forward to showing her off. On the other, it's a perfectly annoying to have an ALL DAY appointment at a hospital 2 hours away on a day my sons do not have school. I guess we'll deal.

And I'll post about that later on.

Updates...

Coming soon - Kate started preschool and it's a tough lesson to learn you aren't the center of everyone's universe!

"That's what we do for PRS kids..."

I saw that on a message board last night and it got me wondering - just what was your care plan for your PRS child? It became clear to me during the first year that what happens varies a great deal regionally and what is the typical course for one area, is vastly different from the typical course somewhere else. It also became clear that sometimes, a hospital will offer you choices based on their own protocol, and not tell you that there are other options available - so lots of parents are making decisions without knowing all there is to know. Seems like there should be a database, or at the very least, a website where you can see the choices available in different areas of the country, or world.

So I'm curious --- what were the options presented to you??

A picture is worth a thousand words

Kate's 3rd birthday and her 1st anniversary of decannulation are both rapidly approaching. In honor of that, I'm posting these pics of her, for all the "silently reading" parents out there so you will all know that you CAN hope that things will end up well for your baby. Things are ending up fantastically typical for Kate, and they sure didn't start that way!!




Here is Kate still in utero. This was taken the day of her birth and she was born a few hours later. Now that I know what I'm looking at, it's glaringly apparent that she has PRS.



Here she is a few hours later, the night of her birth. She's already in the NICU and NOT HAPPY about it. The nurse took the pic so I could see her. She's been whisked away immediately and I"d not seen or held her yet. I remember looking at this pic and saying, "good I'm glad she's crying that means she's pissed".




And here is Kate now. This was taken at the end of May this year. Clearly, Kate is completely typical looking and I can assure you, completely typical developmentally as well. Just at the bottom of her extended neck, you can see where her stoma scar is. Other than that, there is absolutely nothing to indicate that she ever "had anything wrong with her".

Post op checkup

Yesterday we took Kate for her post op checkup with Dr. Jacobs. Since she's no longer an "airway patient", we no longer have to be see in Airway Clinic at the main hospital. We took Kate to the annex facility in Princeton, which was only 50 minutes from our house as opposed to 2 + hours. Needless to say, we were pleased before we even stepped foot in the place. The appointment was fast: her site looks great, keep it covered with sunscreen and come back in 6 mos. Great doc, see ya then. But he did ask about her hearing and when it was revealed she hasn't had hearing test at CHOP in over a year, he decided to do one right then. Thank God the audiologist was available and it was a relatively quick and painfree 30 min. She did 3 tests: the first one Kate wasn't interested in doing at all, the second one she failed. They put probes in her ears and measured her ears response to sounds. It showed no response. YIKES! But the 3rd test was behavioral (put the block in the bucket when you hear the beep) and she passed. Left ear tested completely normal, right ear showed mild loss. So we'll continue to test her hearing every 6 mos until she's 6, then yearly after that. Remember, Kate has Sticklers Syndrome, and progressive hearing loss is a symptom of Sticklers.

Otherwise, Kate's perfectly fine.

Hey, hey, hey potty!

So, I've potty trained 3 kids already. I consider myself, not an expert by any stretch of the imagination, but all 3 kids were very different and had different things that worked for them, or didn't, so I've seen quite a bit. But as has been apparent since Kate's birth, she's like a first child ALL OVER AGAIN. I let the entire winter go by (even though the signs were there occasionally) without even attempting it with her. But May 1, I took her to pre school orientation and felt like I was hit by a truck when they announced, "all kids must be trained before they can start school". YIKES. Clearly we needed to get down to the business of peeing and pooping on the potty - fast. I got videos and books and talked incessantly about it to her. She had pretty much no interest until she saw Prudence and her potty on tv. Things started clicking and she's forging ahead to parts unknown now -- the public toilet. Tuesday night, she told me she had to pee at her brother's baseball game. What's a mom to do? I had to take her to the toilet and pray it wasn't a bacteria convention. It was suprisingly ok, I held her on the pot and guess what? She peed!!! It's been pretty consistent since then. She's wearing Pull Ups (which I franky don't believe in - just a diaper in the guise of underwear) but the Pull Up is dry and she's yanking it down to sit on her potty when she needs to. Now we just need to progress to wearing underwear, all day.

Movin' on up

This is video of Kate's first time in the pool. She's only ever been in the bathtub and only with inches of water. She had never been submerged in water, never had water over her head, never mind swimming. Because of that, she only lasts seconds in the pool but it's still a milestone nonetheless! Two days after this, we went to my husband's brother's house and she spent 4 HOURS in their hot tub. She was positively prunish but so so happy. I think the combination of warm water and being able to feel the bottom of the tub helped.

Stoma closure - part 3

Post op 2 weeks.

Stoma closure - part 2

So her stoma is finally closed. She no longer has a hole visible in her neck, that shows where that tiny piece of silicone kept her alive all those months. It's a new dawn, a new day, a new life. She can wear a "bathing soup" and actually go in the pool, instead of watching from the side with me frantically making sure no water goes anywhere near her neck. The pic on the left shows her stoma (and Kate) as it looked the morning of the procedure. The pic on the right shows Kate, matter of factly, coloring the morning after the procedure.

Honestly, this was the easiest trip to CHOP for a surgery, that we've even had - even with a few hiccups along the way. We arrived in plenty of time and while they took Kate into the peri op area almost immediately, we stayed there waiting for over 2 hours. It was definitely torturous -- Kate had been n.p.o since 11p the night before and was starving. This was her first procedure without a feeding tube and she talked incessantly, while in the tiny cubicle, about the chicken nuggets, french fries and ice cream she was getting once it was over. Poor girl, just like her mom -- all about the food. Anyway, the finally took her back at 1:40 and by 3 sent out a nurse to tell us they were closing her up for good. Yippeeeee!!! By 3:30 another nurse brought us to a private meeting room to wait for Dr. Jacobs to speak with us. Now, this was Kate's 7th time in the CHOP OR and in those 7 times we've NEVER been brought to a private room. In fact, the day of Kate's trach placement, Dr. Jacobs came out to show me the pics from her bronch and explain that he needed to trach her.....in front of at least 30 people in a tiny crowded little room. The next parent was literally sitting 6 inches from me. So to be brought to a different room certainly upped the ante of our mild anxiety. B. and I sat in that room barely speaking until Dr. Jacobs finally came in, beaming and saying how great Kate had done. Whew! I breathed an audible sigh of relief and said, "you're killing us having us put in this room, we thought something had happened!". No, no she was fine he said. Her stoma was practically invisible inside her and the whole thing ended up being mainly a cosmetic procedure. Whew - again. Ultimately, she was sent to the regular floor, after a miscommunication sent B and I up to the PICU (where we both exchanged looks and decided through mental telepathy that we were keeping her door closed the entire time because the kids up there are SICK). She was pretty grogged out by the time we got to her room, but not groggy enough. She overheard B and I discussing our dinner plans (CHOP has room service for patients and parents) and shouted out, "I want my chicken nuggets!". Ok then. She ate them all, plus the fries, and a huge cup of apple juice -- all that 3 hours after surgery. Overnight, she required NO pain meds and slept from 12 -5. Needless to say, she was sent home early the next morning without incident.

Oh, and just as a point of reference, CHOP has remodeled their parent waiting area and you are no longer in that tiny room, with a parent 6 inches from you. It's quite nice now, with a little kitchen, vending machines, tvs and computers...and lots of space.

Stoma closure

Kate's stoma was closed today. Dr. Jacobs said he could barely see it on the inside. Her incision is covered with a small bandaid and she's eating like a champ. More later.

Chatty Kate

I'll do a more complete post about Kate's speech, but for now, here's a video of her talking from a week ago:

Intubation

At the end of March, we went CHOP for a pre op visit with Dr. Jacobs. We assumed this was a formality and that we'd sign the consent forms for Kate's stoma closure procedure. During the visit, after the staff complimented us up and down about how great Kate was doing (immaculate stoma, on target speech with no evidence of a delay of any kind, typical 29mos old behavior) Dr. J tells us he will do a bronchoscopy on 4/24. If her airway has grown, THEN he will close the stoma. If it hasn't, well then he may want to leave it open a bit longer. HUH??? We were sideswiped, but recovered enough to agree with him. Still, the thought of him leaving her with an open hole in her neck for a long brutal summer horrifies us both, so we're chosing to just go with it and hope for the best. Her sleep study was normal, her breathing at home normal. She used a pulse ox while sleeping for close to 6 mos post decann with it alarming maybe 3 times total. She's fine -- but still. One never knows what he'll find in the OR.

Another thing...Kate hasn't been intubated for sedation since November of 2006. Intubation (or being on a ventilator post op) is what caused the stenosis in her airway that kept her trached so long. We were nervous to have a tube shoved down her airway again, so we asked for it to be noted on her surgical file. Not only did they note it, they had an anesthesiologist call us to discuss our concerns. He was nice, slightly patronizing, but nice. He assured us they'd do their best, blah, blah, blah. I also emailed Dr. Jacobs to let him know we'd spoken to anesthesiology, etc. His response was an email to me that simply stated:

"she was intubated for scope in July and did fine. I have routinely intubated her for scopes, and she hasn't had issues. She'll be fine."

Ok then.

Sleep Study results

Kate's sleep study results were normal. I'm not 100% clear what that means, but it means enough that Dr. Jacobs has scheduled a stoma closure surgery for the end of April. Without being completely vulgar, I cannot wait for that sucker to be closed up for good. I should just be grateful and I am, don't get me wrong. But a wee part of me is sick and tired of letting her bathe in an inch of water. This girl is a water baby and she wants to be full on under the water. I'm hoping she enjoys it as much as she seems to think she will!!

I managed to get the results of the study via email. I'm normally a relatively patient person, but when it comes to this stuff, not so much. I emailed the Nurse Practitioner in the office when I thought they had the report and she let me know they were good. This enabled me to cancel our mid Feb appointment (where we were intending to discuss the results) and go right to the pre op appointment in late March. One thing I've learned is not to wait on the doctor's timeline. If you push things through, usually they just go along.

So April 24 is the closure surgery, where Kate will be intubated for the first time since late October 2006.

Yikes.

Sleep Study

Kate had a sleep study on Wed Jan 21, 2009 at CHOP. Even though Kate was decanned in July the study was done as a precursor to having her stoma closed surgically. We hadn't had one done prior to decann so we had no idea what to expect. The drive to CHOP is 2 hours and we needed to check in around 6:30. Most definitely it was in our favor to keep her awake on that ride and I was able to do so by feeding her gummy bears one at a time. By the time we arrived, she was positively manic on a sugar high! Anyway, we checked in extra early, only because we got there early, but we were glad we did. We had a choice of rooms. Kate layed around in the crib from 6p until about 6:30. If we'd turned off the lights, she'd have gone to bed, but she got her second wind and was roaming the halls when the tech came to set her up. The set up consists of placing various probes and wires on her head, face, chest, neck, and legs. She was FANTASTIC during the set up. She helped choose the color wire and was completely fine with all the probes, the glue they use to keep them on, the compressed air they sprayed on her to dry the glue quickly -- even the mesh headband they put on her head to keep the wires in place and the gauze around her head (for the same reason) were ok by her. But once they put the nasal prongs in she lost it completely. Adding insult to injury, they had to cover her open stoma completely with Tegaderm (a clear type of tape used mainly to secure IV sites). Hysterical doesn't even begin to cover it. Neither Brad or myself have seen her that upset since probably her palate repair 13 months ago. And because she was so pissed, she was struggling in some ways to breathe. In retrospect, she was probably just re learning what it's like to not have an extra hole in which to take in air, but it sure looked like a moderate form of distress to us. She never changed color though and the techs told us she was satting in the high 90s even while upset. I was able to rock her to sleep and then place her in the crib. She woke up hysterical 3 more times but I got her back to sleep easily. She slept pretty peacefully from 12 to 6, with no major snoring or weird breathing.

So all in all it was an ok experience. Definitely keeping her awake during the day helped. In fact the lab told us we should, not because it produced better results per se, just that she would be tired enough to eventually sleep. I knew there would be a lot of probes, but I didn't really try and relate it for her and I wished I had. Hopefully she won't need to do it again.

Now we play the waiting game. It's been 8 days and I haven't heard anything. I think Monday I'll make some calls. No WAY I can wait until Feb 12. That's our next scheduled appointment in airway clinic. We're supposed to discuss the results and either plan a stoma closure, or not.

Life after trach

SO Kate was finally decannulated on July 4th, 2008. It really did feel like Independence Day.

My daughter was finally independent of the piece of silicone that had kept her alive.

She was finally independent of a wire on her toe while she slept.

She was free of the stares of strangers who couldn't understand what she'd been through.

Life after the trach was bittersweet too. After a very successful post decann check up with Dr. Jacobs, Kate was released from all equipment, supplies and nursing that were related to being a technology dependent child. I was thrilled to bits with all of this, but I won't lie -- life without nursing was very different. We immediately went from pretty much glorified babysitting 16 hrs a day to no babysitting. Kate's primary nurse was a constant in all our lives, and was missed by all of us right away. It was like having your girlfriend in your house for 8hrs a day for chatting and companionship, and then all of a sudden she's gone. Sigh...

Anyway, Kate made huge strides the first few months trach free. She'd already learned to talk around the trach, but she continued with speech therapy to get her up to par. This didn't last long and Kate was booted from Early Intervention Speech Therapy and Physical Therapy within 6 weeks of decannulation. Once the trach was gone, she had a language explosion. It took about 4 months, but by the winter she was talking clearly in full sentences. And boy can this girl talk!

Another big BIG thing was the feeding tube. I'm sure if you've read enough you get that I abhored the feeding tube with everything in my body. We went back and forth so much with it, but ultimately it served her well and kept her gaining weight while she learned to eat. Her ENT knew we struggled with it, but he asked that we keep it in through the LTP surgery and recovery. We agreed and I used it to pump her weight up in anticipation of the post op period. Of course there wasn't an operation, but I was too tense to just take it out even though the morning of her decannulation she ate her entire breakfast tray and then some. How's that for stupid? After all my whining and complaining about the feeding tube, it was me that insisted it be kept in! So for several weeks after the trach was out, we continued to feed her that 7pm slow gravity tube feeding. One Saturday (the first weekend in August) we took the tube out for a visit to some relatives who'd never met Kate. That night, we decided to jump into the abyss. We didn't put the feeding tube back in that night, or any night. She was eating and she continued to eat. I felt good about it, but I was leaving her weight in her tiny little hands. I encouraged her to eat whenever and wherever she wanted...that caused it's own tiny problem later on, but that's ok.

SO life without the trach meant:

*no nurses in our house
*no supplies, machines or emergency bag next to her bed
* she could finally move up to that cute pink room
*I could take her anywhere I wanted without a GO bag in tow

Ahh freedom...or was it?? We still had an open stoma where that piece of silicone used to be. What were we supposed to do with that??