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??

Decannulation video

Here's video of Kate's decannulation, July 4, 2008 at CHOP. You can see, after all the buildup, the actual process is very anti climactic.

Airway

Like most PRS kids, Kate's primary issues were eating and breathing. When we first started on the airway road, I assumed Kate's trach would be the hardest thing to deal with and get rid of. Again, and you'll take note of how often I was wrong throughout this process, I was incorrect. It took awhile, but I came to realize that airway and eating are 2 entirely different animals and while the eating issue could be helped along with therapy and time and patience and consistency, the airway issue could not.

The initial reason Kate was trached was for upper airway obstruction. Quite simply put, her tongue occluded her airway, and she couldn't breathe. All her anatomy was otherwise intact and perfectly fine. So she received a trach to help her through until she could control her tongue enough to keep it out of the way, or her jaw grew out enough to do the same. Kate's ENT told us to expect 2-3 years with the trach. Her plastic surgeon told us it could be as little as a year. We hung onto his words, but figured the ENT knew more and we were set to wait it out for up to 3 years.

Three and a half months later, Kate had her first bronchoscopy post tracheotomy. It was then her ENT found she had aquired subglottic stenosis grade 2. (I'll not bore you with all the medical details, but check the Favorite Sites area to the side for a very detailed article on this condition.) Basically, Kate's trachea was damaged during one of the times she was intubated. The damage caused her body to grow scar tissue which narrowed her airway considerably. A grade 2 stenosis means a 50-70% blockage of the airway. We were devastated especially when he started talking about her needing surgery in order to achieve decannulation, but we had heard the "D" word, so we were hopeful at the same time. He told us to expect a laryngotracheoplasty or laryngotracheal reconstruction (LTP or LTR) when she was between 18 & 24 mos old. (Again, check the Favorite Sites area for more details about this procedure) Pretty much, they harvest rib from her, and use that rib to open her airway. It's extensive and it's painful, but most times, it works. Kate being Kate, she would need the reconstruction in both the front (anterior) and back (posterior). Great.

So we came home and read up and got the pants scared off us. But we were truly hopeful underneath the disappointment that Kate would not travel the path most PRS kids do...and be decannulated within the year. Her next bronch would be in Dec. 2007, 9 mos down the road and we hoped to know something by then. But a routine visit to the ENT in October started the ball moving. Dr. Jacobs felt her jaw was growing out and it was time to start thinking about the LTP. The bronch 6 weeks later showed she would likely only need a posterior graft and an endoscopy showed no damage from reflux. So back to CHOP we went in January for a ph probe. Normally, a ph probe is done off reflux meds, to see if the person is doing alright without the meds and can discontinue use of them. Kate's was done with her meds to make sure they were controling the reflux well enough, so it would not damage her newly reconstructed airway post op. The meds were fine and the surgery was scheduled for May 1, 2008.

But, per usual for Kate, she caught a bad case of the flu, was sick for most of April and we were forced to postpone the surgery until July...which seemed like light years away. Still, July 3 came and we traveled to CHOP in the early morning hours, knowing that day was a turning point because it would put Kate on the road to decannulation...finally. During the late morning, Kate was wheeled into the OR and we settled into the waiting area, ready for a long wait. A bronchoscopy was first to double check, rule out any infection and determine finally if she'd need both anterior and posterior grafts or one or the other. Then a general surgeon would go in, harvest enough rib and Dr. Jacobs would then do the graft. We were told it would be an hour before they actually started, then another 5-6 hours from there. After an hours wait, Dr. Jacobs came out to see us. Pessimists that we are, we assumed something was wrong. Imagine our surprise when he told us Kate's stenosis had resolved to a grade 1, he didn't repair grade 1, that he would send her up to the PICU to wake up, put a cap on her (now downsized) trach, observe her overnight and decannulate THE NEXT MORNING, if she did ok.

HUH???

Never once in 18 mos of living with a trach did we think we'd get rid of it like this. It was now 16mos since he first told us she'd need the surgery, and we never ever thought she be decannulated without it. But guess what. She was. Without issue, without pain, without drama.

On July 4th, 2008 at 8:52am my baby girl was finally, finally trach free...after 601 days with an artificial airway. To say we were happy, doesn't even begin to cover it. Below are pictures of how Kate's airway looked at various points in her short life.





November 16 2006- day of trach placement. That's her n/g tube below and the base of her tongue above, blocking her airway almost 100%.



March 2 2007- ENT's notes are visible below the pic of my girl's very narrowed airway.

December 7 2007- 10 months later, you can see her airway larger in the front (bottom) and still narrowed in the back (top)

July 3 2008- Kate's LTP bronch. Her airway is open both at the back and the front.

A sense of normalcy

During all the drama that surrounded Kate's birth and eventual homecoming, there was never a sense of the normalcy we all expected with the birth of our 4th child. We thought I go to the hospital, have a baby girl, come home 3 days later and get on with the business of living. That wasn't to be, and when we did come home, there was absolutely nothing normal about it.

Still, we learned to live in our new "normal". Some days were harder than others. Some days just flew by. All in all, I'd say we did pretty good adjusting to life with a medically fragile child. My sons were princes. They had waited so long for their sister to finally be born...then come home, they were just happy to have her there. They dutifully Purelled their hands, changed their clothes, and stayed away when sick all in the effort to keep her well. But still they managed to treat her as one of them. It was harder in the beginning. She was an infant, so she just laid there, but the added presence of a nurse did have its own problems. Eventually, we insisted that the boys be able to be in her room and "visit" with her. She wasn't just their patient, she was our daughter and their sister. So they played with her, read to her, "fed" her and rocked her. As she got older and stronger the kids reveled in her achievements, applauding her when she finally learned to roll over, crawl and walk. All adored her, but let's be honest...boys are wild, and the wildness didn't take long to rub off on our little girl. Her favorite thing to do ws play rough with them. Of course we couldn't allow this...could we??? I mean, here she was, a "technology dependent" baby with an artificial airway, and a feeding tube. What if the trach got dislodged? What if her feeding tube came out...the mind could go lots of different places, but we chose to just let her be. The boys had been sufficiently cautioned and we knew they'd be careful.

And Kate couldn't have been happier when than when they were wrestling with her on the living room floor.

Eating (Part 2)

Teaching Kate to eat took far more effort than I initially thought. It's a natural thing to want to eat...to need to eat. But for a tube fed infant (and Kate was almost exclusively tube fed from birth) it's a difficult thing to learn and an even more daunting thing to teach. Around the time Kate was 11 mos old, I convinced her pediatrician to let us start weaning from the tube. Again, I could do whatever I liked, but the 16hrs of nursing we had in our house, couldn't. We needed orders to let them refrain from tube feedings during the day. Ped agreed to give it a shot. We took her from tube feeding every 4 hours (6x/day) to tube feeding only overnight (7p, 11p & 3a). It was a big difference for Kate and for all of us...and Kate fought it as best she could. We calculated every single ml she took in, to make sure she didn't dehydrate herself in the process of learning what hunger is like, and bumped her formula from a normal 20cal/oz to 27cal/oz. But she fought a good fight. By early September, I was thinking it was never going to happen. She ate, but she didn't take in nearly enough quantities to keep herself nourished, nevermind enough to grow. I called a feeding clinic and set up an evaluation for that week.

The feeding people were nice, but from the get go, I wasn't overly impressed. They didn't tell me anything I didn't know, or show me anything I wasn't already doing. They agreed she had issues (trouble coordinating her swallow, pocketing food, spitting food out) but said she wasn't nearly as bad off as some. She had no issues with textures, tastes or temperatures. The therapist they set us up with at first was awful. She was a nice girl, but she clearly had no real hands on experience, nevermind with a case as medically complex as Kate. I asked for a change of therapists, got a supervisor and at her first visit with Kate, she diagnosed dysphagia (an inability to swallow properly). Kate's issues were weakness in her mouth and jaw muscles, and a tendency to get tired and spit food out. She recommended a therapy called VitalStim. Essentially, they put electrodes on her cheeks to stimulate her muscles to contract and work properly. The rub is, she must be eating while the electrodes are on. Kate had VitalStim for 6 weeks, 2 times a week. She was having good results, but her palate repair in early December required us to stop the therapy while she recovered. We made plans to contact the therapist right before Christmas.

Unfortunately, Kate went on a severe hunger strike following her repair. She refused all food and most drink for 3 weeks post repair. I was twitching in the corner, thinking about all the progress she'd made and how far she was regressing with each passing day. I even offered her bottles, which she had been taking up to the night before the surgery with limited success...no dice. Christmas Day she ate a total of 5 olives and some water all day. Keep in mind, we tube fed her around the clock in order to avoid dehydration and loss of weight. December 26, Kate decided she'd had enough and ate an entire hot dog in 15 minutes...as if she'd been eating fine her entire life. She never went back to Vital Stim. She continued to improve and was discharged from the clinic in February 2008. We'd successfully weaned her from 6 tube feedings a day, to 1 tube feeding at 7p everynight. By this point it was Pediasure (30cal/oz) with Duocal. Even with only 1 tube feeding, she was gaining weight, and if her airway reconstruction wasn't looming on the horizon...we'd have pulled the tube and gone for full weaning right then and there.

Fortunately for us, we didn't because that spring was hard on Kate in similar ways to the previous year.

Here's Kate learning to use her Passy Muir Valve in July 2007: