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.
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.