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:

Eating (Part 1)

Back whe Kate was 3 weeks old, she had what's called a tongue/lip adhesion to try and solve her airway difficulties. The procedure basically pulls her tongue forward and stitches it to the inside of her lower lip in the hopes of protecting her airway. It didn't work, and Kate ended up trached, but the plastic surgeon didn't want to take her back into the OR just to take it down, so we waited. The original date for the takedown was May 6, but the illness that was April made that impossible, so we rescheduled for June 6. Kate had her tongue released on 6/07/07 and B and I waited patiently to see her tongue in action. As an infant, her tongue was quite recessed and when it was stitched she looked normal, but we knew better. We couldn't even tell if her teeth had started to come in yet. We stayed one night at CHOP for the surgery, which Kate came through with flying colors. She slept most of that day, was slightly swollen, but no worse for the wear. We came home the next day, Kate with several stitches across the edge of her tongue. You can see them in this picture.

In spite of all the hospital stays, Kate continued to thrive at home and was everything we hoped for in a daughter: beautiful & smart. To our eyes, she looked normal, like the boys but still her own person. You can see here, her jaw doesn't really look any different than a typical child, and I doubt very much anyone would be able to tell she had PRS, if not for the accessories that accompanied her everwhere.

I've said before that Kate's trach proved to be a far less formidible force than the feeding tube, and over the course of Kate's first year at home, it became readily apparent that getting rid of the feeding tube would take far longer than we anticipated. Her first forays into eating were right after birth, just as any baby. She did ok, but the cleft in her palate was an obstacle that prevented her from having the strong suck that a typical infant does. A child with PRS works really hard to get a fraction of the liquid a typical newborn does, and because of this, they get tired more easily. Forcing them to eat will only cause them to lose the calories they are working so hard to get in. Some pick it up right away, some eventually, and others take longer. Kate was doing alright initially, but when her airway started to become an issue, feeding took a back seat. Kate spent 5 weeks in CHOP being npo (no food by mouth). When we offered her a bottle finally, after she got her trach, she ate from it, but it was clear she'd need to work hard, and be fed more often in order to get the calories in. Coming home, we were told of course Kate could eat normally, things would be different at home, more normal, blah, blah, blah. Well things were more normal home, just not Kate's eating.

It's hard to think back on that time and not want to reach back into time and strangle myself, and every doctor and nurse who stuck to procedure. If I was to pinpoint an emotion that explained my behavior, it would be fear. I was afraid to go outside the box and trust myself, and my daughter. Instead, I followed the orders that read, "allow to po (feed by mouth) feed 3x/day 45mls". Kate took to the 45mls and sucked them down almost everytime we offered it...but we never hooked into that, and we simply stopped the feeding after that amount and pushed the rest through her n/g tube. One of my greatest and most profound regrets is that we didn't try harder, because what ended up happening was Kate got used to that, and later on when we tried to push her, she refused. I'm a firm believer that we did wrong by her, and though I can't change it, I'd never want another parent to make the same mistakes we did. It all comes down to this:

IF THEY WANT TO EAT, LET THEM EAT!!!

Here's Kate, June 07 eating her first cereal. The snorty sound you hear is classic trach baby noises.

It takes a village

While Kate was in the hospital, I focused all my energy on getting her home. After she was trached, when I thought I realized what I was in for, I took classes and learned what I needed to all in the mistaken belief that somehow, all would be normal at home. As I've said before, I was very wrong. Not only was Kate not in the perfect pink room I decorated for her, not only was she forced to live in what was formerly a toyroom, not only was she trached and had a feeding tube...she came with another unknown...the private duty nurse. Cause ya know...we couldn't take care of her ourselves. I remember really thinking we would not need the nursing, or want the nursing after we got settled. Clearly, I had never done this exactly this way before, but I'd done it 3 times before and the boys survived just fine. Again, like so many times during this journey, I was sorely mistaken. The insurance company agreed to pay for 16hrs per day, everyday, of private duty nursing. B researched, and we hired the agency who promised us a nurse with experience in the care of an infant with a trach. The afternoon we came home with Kate, S. met us at the house for the first time. We both liked her immediately, and counted our blessings that she seemed nice and easy to get along with. Ultimately, she and I became friends, doing a 'dance' in perfect unison, taking care of my daughter. She ended up working at our house 6 days a week, for the entire 18 mos that Kate was trached.

Fumbling through life with a technology dependent child is like walking through a fog...every so often there are pockets of vision, where everything seems clear, only to be shrouded in a muddy blur the next instant. We did our best, that's all we could do. While a 'typical' child has only 1 doctor, Kate had 3 from the start: pediatrician, plastic surgeon and ENT. Within a month of coming home, we visited a cardiologist to make sure Kate's PDA had closed (it had), and an opthalmologist to check her vision. I also contacted Early Intervention for an evaluation. Kate was diagnosed with hypotonia or low-tone. Essentially, that means that while she isn't 'floppy' (a really really bad thing for an infant which can indicate brain damage), she wasn't as strong as a typical baby. That plus the airway issues that kept her hospitalized for 80 days qualified Kate for physical therapy 2 times a week. A week after that came through, I harassed the EI coordinator to approve speech therapy as well. They denied at first, claiming Kate wouldn't be missing milestones at this point because of her young age. I countered with, she needed all the help she could get. The trach, plus having a cleft palate, put her a deficit in comparison with typical kids, and why should she be even further behind?? They agreed and speech therapy started several months later.

Starting out, Kate was at least 3-6 mos behind in her milestones. I didn't even realize what low tone was, nevermind how it could affect her in so many ways, including eating. She looked normal, seemed normal to us. But we deferred, as we would so many more times, to the higher ups and a physical therapist came to our house 2 times a week. Kate started to get stronger almost right away. Her legs were her strongest area, her upper torso her weakest. With time, she met all the appropriate milestones. I remember the day she finally rolled over. S. and I were so excited we almost squealed! Kate was 7mos old.

Keeping Kate well that first year proved to be a challenge. She qualified for Synagis shots once a month, to try and prevent RSV. We forced our boys and everyone who came into the house to wash up and use Purell religiously before touching her. If you were sick, you couldn't visit. Again, I remember not realizing how awful her being sick could be, but I deferred and we all "Purelled" the life out of our hands. All the nurses who came to work in our house, complimented us on keeping her well...no small feat in a house with 3 school age boys. Early that spring, all that good luck came back to bite us in the butt. Most of the month of April, Kate was sick with "respiratory infections", colds and the like. Her nose didn't run, but her trach would pour secretions, she have a bad cough, maybe a fever, lower sats and higher heart rates and breathing rates. The antibiotics were doled out like candy. We just couldn't get her well. Mid month, we consulted with a pulmonologist. At this point we were thinking maybe it was allergies. He told me it might be, but his suspicion was that once she was decannulated, all these issues would likely go away...that infections just went to her lungs because of the trach. I hoped, and I hung on that word...decannulation.

Unfortunately, after tons of running around, Kate was admitted through the ER, to a local hospital...who promptly said they wouldn't take her because of the trach. So off we went to yet another hospital, with a PICU (Pediatric Intensive Care Unit), more antibiotics, more xrays, more docs in the loop. She stayed for 3 days, improved and was sent home. Less than 24 hours later, we called 911 because she was lethargic and unresponsive. She'd had persistent diarrhea that day, downed an entire bottle of formula (absolutely unheard of for Kate) and then just passed out. Turns out she had c diff, a fungal infection of her intestines, caused by all those antibiotics running roughshod over the flora in her belly. She recovered nicely, we learned a few things about dehydration, antibiotics and PICUs.

So that makes the count 5 docs, 2 therapists, and nursing 16 hrs a day. Yep, it sure did take a village.

Home

Coming home with a "medically fragile" infant wasn't easy. Even though we were fully prepared for anything that might happen, it still was a difficult transition. She wasn't even a newborn anymore.
We showed up at home, after being away for 3 months, with a baby sister the boys had only met once. They rose to the occasion and worshiped her from the moment she came home. They learned better hygiene and "Purell" became a verb in our house...as in "did you Purell when you walked in the door????".

We came home the week before Christmas, which had been our goal. Our insurance company agreed to pay for 16 hrs a day of private duty nursing, but no agency can staff you like that from the beginning and they're lying if they say they can. The weekend of Christmas we were alone with Kate for 72 hours. Needless to say, B and I barely slept and we most certainly didn't leave the house. Kate needed to be watched 24 hours a day, so we slept in shifts and still managed for Santa to come and the boys to enjoy their holidays. Exhaustion became the norm.

When we realized Kate was coming home with a trach and a feeding tube, we also realized there was no way she could be in the tiny pink room we'd set up for her. There just wasn't enough space for Kate, the machines, the supplies and the nurse. So we moved her to the sunroom next to our main living space. It fit everything from her room, plus the equipment, a computer and a couch and rocker. Here's what turned into Kate's room. The machines in the lower right corner are the air compressor and her pulse ox monitor. They are sitting on one of those plastic 3 drawers on wheels, which turned out to be a fabulous space for supplies. The blue tubing is part of her humidification system which blew warm humified air into her trach every night. Beyond the head of the crib was a dresser on which stood the suction machine, and yet more supplies.

And the pretty tiny pink room we'd set up for her...agonizing about the color and the valances and the bedding. Well that became the prettiest pink closet. All her supplies, and there were many, ended up stacked in that room. Sad.

But Kate thrived at home. She got fatter and bigger and healthier. Even with 3 school age kids. No small feat considering she had an open hole directly into her lungs.

Out of the NICU and into the fire

While Kate was at CHOP, my youngest son and I lived at the Ronald McDonald House. I remember our first night there, when we thought it would only be a few days, looking around at the faces of people who had clearly been there longer. Honestly, any parent who comes to PRMH, thinks it's only going to be a few days, but in actuality, it turns out to be much longer. CHOP is a great hospital, but in an effort to be completely thorough, things seem to drag on and on. I spent many nights in the kitchen, with other mothers of sick kids, wondering when we'd get to go home. Any child's discharge, and their parents getting to check out of the house, was a thrill for all of us, because it meant that someday, we too could leave. That December, Kate's brother was chosen to flip the switch on the thousands of lights the house puts up for the Christmas holiday. B. was asked to say a few words about Kate and he did us all proud, there wasn't a dry eye in the house...especially my son's, since he developed a full blown fear of Ronald McDonald!

Once Kate's trach was placed, we moved fast to get her home. It was important to me that she and I be home, for good, by Christmas. We'd spent 4 weeks already away from home and it was getting hard on everyone. B. and I made our intentions known to the most important part of the hospital team...the nurses. They'd seen the toll this was taking on our family, the constant traveling back and forth for B, the tedium of all day hospital stays for my son and they knew we had 2 others at home as well. We were lucky that Kate had nurses who cared about her and who wanted her to be with her family. They pushed through classes for us, designated "training times" where there weren't any, allowed us to combine trach changes, so we'd both get credit for doing them...all the while making sure we were 100% comfortable with caring for Kate. At one point, the NICU was just about ready to kick Kate out because she finally didn't require a high level of care anymore. As good as that sounds, it terrified us, because we were used to the NICU. Plain and simple, I'd spent enough time with these people, and they with me, that I felt paralyzed at the thought of being without them...and with Kate, alone on another floor. That, plus the NICU attending had already said they would discharge us in a week. The nursing staff made sure she went to a unit equipped to deal with a trached baby. So on 12/11/06, Kate was transfered to the Progressive Care Unit, to prep for transition to home care. Here's Kate and I, the evening she was brought there. My thoughts that night were a jumble of anxiety, fear, and genuine nervousness...we were actually close to bringing her home, for the very first time.

Progessive Care ended up being the best thing for Kate and I. I sent my son home that final week, so I could concentrate on taking care of Kate, and take care I did. I was in the hospital from 9-5 everyday and I stayed in her room, doing everything for her the entire time. By the time B came for our mandatory 24 hour stay, I was more than ready and more than confident we could take care of her at home. (Every parent of a newly trached child must stay with them in the hospital prior to discharge and care for them for 24 hrs to prove they can do it.) See her here, so big and healthy looking. She was a dream. As soon as the trach was placed, she was a different baby and she started to develop by leaps and bounds. We even caught her smiling a few times, which thrilled me to bits. I couldn't wait to bring her home and finally be normal.

Kate was scheduled for discharge the week of 12/21, but an attending in the PCU took up her cause, and was determined she go home. He wouldn't take no for an answer and he didn't want to hear lame excuses. During rounds one morning, he told the team, "I'm interested in what's best for the patient, not your rules and procedures, and what's best for this patient is to be home and that's what we're going to do." And he did. Kate was discharged from CHOP after spending 80 days in 3 different hospitals. She was 11 weeks old and weighed 10lbs 6oz. To say it was a journey is putting it mildly. Here's Kate, on her second day home, 12/20/06.

Normal, of course, was now far from what I'd expected when I gave birth to Kate. Normal was now a strict routine of feeding, meds, breathing treatments, suctioning. Kate required constant care, and needed to be supervised 24 hours a day. A child with an artificial airway is a lot of work, and it quickly became apparent that although Kate was now home, things would never be as they are with a typical infant. I knew in my head they wouldn't be, but I guess in my heart I was still harboring some hope that it would be different for us. I naively listened and believed when nurses and doctors told us, "oh when you get home, she'll eat for you, things could be different." I'm here to tell you that's a load of crap. Once a feeding tube is inserted, it's extremely difficult to get out, and though I didn't know it at the time, the feeding tube would prove to be a far greater challenge for us than the trach ever would.

The Children's Hospital of Philadelphia

This is one of the final pics we took of Kate, while still in her second hospital. To me, she looks tired...tired of working so hard to breathe, nevermind eat. You can see she has an o/g tube and tape in place on her cheeks for the O2 cannula. Yup, by this time we knew it was time to go...onward and upward...to the best Children's hospital in the country. At least that's what the magazines all told us. Kate left for CHOP the night of October 23, 2006. B and I followed the next day. We assumed she would be a candidate for a tongue/lip adhesion and once she was recovered from that, we wanted to bring her back to hospital #2. It was 25 minutes from our house and sons, versus the 2 + hours CHOP was. So, with a tremendous amount of hope, we followed our youngest child to Philadelphia & checked into the Ronald McDonald House fully expecting to be home with the boys for Halloween. That didn't happen.

Within 24 hours of her arrival, we met with a plastic surgeon who scheduled Kate for a tongue/lip adhesion that Thursday. Dr. Richard Kirschner did the surgery on Kate, and apparently it went beautifully although Kate looked a tiny bit worse for the wear following it. She was kept completely still, in a medically induced paralyzed state that was torture to watch. Her tiny body puffed up from all the drugs, she was pale and most importantly she was on a ventilator. They kept her this way over that weekend, discontinuing the drugs Sunday afternoon, with the intention of her waking by Monday and being taken off the vent. But she had so many meds in her system, it took until Tuesday morning before she was sufficiently awake and breathing over the vent. Halloween morning, my youngest son and I walked into the NICU and saw Kate lying in her bed with no tubes on her face...and breathing on her own. I felt a tremendous relief. She appeared to be taking well to the procedure and it appeared to be working.

By Friday, Kate was still doing well. I was annoyed by now they wouldn't let us try to feed her, but I was just getting used to the slow gears of a major children's hospital...no one does anything without consulting the entire team. So it wasn't as simple as getting me a bottle with some formula. Dr. Kirschner had assured us we'd be able to feed her as soon as she woke up. That didn't happen, but here she is, post tongue/lip adhesion day 7 and looking ok. The next day, I left to come home for a few days and pick up our oldest boys. Our youngest was already with us, a concession to the grandparents when we realized we'd be here much longer than expected. Our older 2 hadn't met Kate yet, and at this point we thought she was in the clear. By the time we arrived, Kate was on a downward spiral she wouldn't recover from. She was stridorous and retracting and turning frightening shades of gray and blue. Again, lots more confering amongst the team which by this point included Dr. Ian Jacobs, the pediatric airway specialist. He performed a bronchoscopy on her, determined the buttons from the tongue/lip were too big and occluding her airway and sent her back to her room on O2 cannulas. More waiting, more discussion, more drugs and then they told me this:

"Mrs. _____ we think there is an 80/20 chance Kate will need to be trached. 80% she won't, 20% she will."

I held onto the that 80%, all the while watching my daughter grow more and more weary. The only place she was comfortable and stable was in my arms, but even I recognized the futility of that. There was no way I could keep her in my arms this way at home, though if anyone had given us that choice, I guarantee we'd have taken it. I'd already been in Philadelphia for 3 weeks, B had long since returned to home and work and I was needing a resolution to all this. Kate was 6 weeks old and we were no closer to bringing her home now than we were on day of life 1. It was time to get moving.

All that resolve meant absolutely nothing when they made the decision to trach her and asked for my permission. I cried buckets in the NICU. How could I do it? How could I not? B. and I consulted the plastic surgeon about a jaw distraction. CHOP would not do it on her, said she was too young. We got online and learned just how much our lives would change with a trached and "medically fragile" child. We agonized, but everytime we saw her struggling we had to wonder what damage this was doing to her. Ultimately, the choice was clear. I signed the consent, and Kate was trached on November 16, 2006. She was 7 weeks old. When Dr. Jacobs came to show me the pictures from the bronch he did that day, her tongue was almost 100% occluding her airway.


This is the first picture we took of her, post tracheotomy. To my eyes, she finally looks somewhat healthy. Still has the feeding tube, but more on that later. Much more. The plastic thing jutting from her neck is an HME. Essentially, that's an artificial nose, providing the heat and moisture she couldn't get from just a simple opening in her neck. I guess this is about a week, maybe 10 days after she got the trach. She recovered from the placement in record time, breathing over the ventilator and getting off the machine completely within days. I didn't realize it at the time, but some kids take weeks, months, even longer to be able to wear an HME. Kate was lucky and it made life a lot easier, since she was much more portable this way, rather than attached to a humidification system 24 hrs a day. She was stellar. The only thing worth noting was her breathing rate was much faster than a "typical" infant her age. She was tachypnic from the trach, but she was breathing and that's all we cared about. Now we could take her home.

It took another 3 weeks for that to finally happen. Turns out, again, it wasn't just about putting the trach in and sending her home. B and I had to learn how to take care of her, had to alert our insurance carrier to this new development, and we had to find nursing care for Kate...all while watching her, now able to breathe, get stronger each and every day.

She eats with what???

On Kate's third day of life, I was released from the hospital, still smarting from the c section, but anxious to see her and hold her and find out what the hell was wrong. I couldn't fathom it really. None of us could. This is what I saw, when I went into the NICU that morning. Nothing extremely out of the ordinary, just a newborn babe with chubby cheeks, yet hooked up to machines and with a tube through her nose and an IV in her arm. I was shocked to see her like this, even though I knew she was sent to a NICU, I didn't expect to see this. Nevertheless, I asked them to show me how to feed her, because in our minds, that is what was holding her back from coming home.

The Haberman was the oddest looking bottle I'd ever seen and a far cry from the Dr. Brown's that awaited Kate at home. I couldn't have known they were only the beginning of odd for us. Nothing was as it should have been, and it wouldn't be for a very, very long time to come. But everyday I went to the hospital, and persisted in feeding her. Her breathing was ok, and she was on oxygen through nasal cannulas only while eating. I kept vicious track of how much she ate, and stalked the nurses taking care of her to feed her as much as possible. I kept thinking, "as long as she eats, she can come home" and I resigned myself to feeding her almost around the clock if need be. She did ok eating, picking up at some points and refusing at others. I remember one night as B. and I were coming for our nightly visit, a nurse we knew and trusted told us Kate ate 48mls from her bottle. We were ecstatic! Her goal was 60mls so we weren't far off.

Eventually, her breathing issues took precedence. A nasal trumpet, possibly the ugliest instrument in all of medicine, was inserted into my baby's nose. It relieved her breathing and I was satisfied for awhile that this would help. After all, if she could breathe, she could eat...right? About week 2 of twice daily visits to the hospital, as well as caring for my 3 sons, my nerves were fried. I was crying to everyone and anyone who would listen. It wasn't getting better, it was getting worse. A nurse told me they'd had a kid "like this" once, who stayed in the hospital for 9 months. Devastated doesn't come close to how I felt...hopeless was more accurate. All I wanted was a baby, and here I was losing out on all the joy of a new baby. I was home and going about my life as if she wasn't here. Doctors started talking about other options for her: a tongue/lip adhesion, an orthotic device to help her get a good suck. Some nights her breathing was fine without the trumpet. Once she went all night without it, and the nurse caring for her was calling the doc to get an order to discontinue use when Kate started to desat. Typical of Kate, I was yet to realize, was for her to look fine, but breathe horribly. She just couldn't be positioned. She needed more help.

On Kate's day of life 24, we had a meeting with the attending. She advised us to seek specialized care for Kate. The hospital simply couldn't do anything more to help her. She told us, "go into the city, or go to CHOP". When she told us she had contacts with CHOP, we opted for that choice and she called them right then and there and asked them to come and get her and evaluate her for a tongue/lip adhesion. It took less than 24 hours for the CHOP transport team to come. By a stroke of good luck, B and I were just arriving at the hospital when the team arrived, early, to pick her up. There she was, in the tiny space of a transport isolette for the second time in her short life. Still, we both felt hopeful. I mean, CHOP was one of the best and we knew whatever hardship this might cause us, it would be better long term for Kate.

It took awhile for me to see it, but ultimately, we were right.

But she looks ok to us...

Here's Kate, day of life 2. We had no clue what lay ahead, just that "they" wouldn't let us bring her home. She looks slightly worse for the wear, and the band-aid on her finger is a battle wound from the c section. All those wires and tubes were only the beginning.






You can see her jaw is slightly small and she definitely has signs of a weak chin. Her eye is puffy from the delivery. In this shot, she's just over 24 hours old, and had already been transfered to the NICU at another hospital. A good friend of mine is a nurse at the hospital Kate went to and she took these shots for me as well as helped my husband get there. Kate already has a feeding tube in her nose...if I knew then what I know now, maybe that could have been avoided, but at the time, we were willing to do whatever for the chance to bring her home.


When I look at all the pictures from the beginning, it's hard to put myself back there. Those days were fraught with anxiety and worry and sadness. It was so hard to just be happy our little girl was here. It seemed as if she would never get home. And we had no idea the journey we'd embarked on. At this point, Kate was breathing fine on her own, even on her back no less!! The hospital was still telling us she just had to learn to eat from the Haberman, and then she could come home. We believed them of course. We had no choice.

Pierre Robin? What's that?

Pierre Robin Sequence (PRS) is the name given to a combination of physical birth defects. In order for PRS to be diagnosed, the baby must have a small or recessed chin and a tongue that appears too large for the mouth and affects breathing. A cleft of the soft palate is often present as well. It's estimated that PRS occurs in anywhere from 1 in 8,000 to 1 in 30,000 live births.

Our own story with PRS begins with our decision to have a 4th child. Believe me, we didn't take it lightly. We already had 3 healthy sons, but had lost a daughter who died in utero at 24 weeks gestation. For us, along with wanting another child, we wanted to try for the girl. My pregnancy was uneventful. At 37, I had an amnio and the results came back normal. Several ultrasounds showed everything in place and "normal". The fun started the morning of 9/28/06.

My OB wanted an ultrasound to determine the size of my daughter. My belly was HUGE. And when I say HUGE, I mean HUGE. We figured if she was very big, we'd have a c section and be done with it. SO off I went, no bag in hand, to the hospital for an ultrasound. During the procedure, the tech commented that I was "poly". I had no clue what that meant so I asked. She said, "oh just a lot of fluid"...then she commented on how chubby my baby's cheeks were.

Since I'd never been in this office before, the staff asked me to wait for the perinatalogist to arrive. I still didn't think anything was up, so when he finally got to the office (wearing a motorcycle jacket and big black boots and looking suspiciously young and a lot like Chris Daughtry), I asked him what was up. He said this to me:

"Mrs. _____. Your u/s shows an abnormal amount of amniotic fluid. Normal is 14, + or - 7, and yours is 38. This can mean 1 of 4 things: you have gestational diabetes (which we know you don't), a chromosonal problem (which we can also rule out because of your amnio results), nothing, or it could mean something is structurally wrong with the baby. In any case, your baby is about 7lbs 11 oz so big enough and I'm recommending you be delivered today. If you go home and your water breaks you are risking a cord prolapse and that's not good. So let's get you upstairs and speak with your doctor. "

So up I went. I remember feeling more and more apprehensive the closer I got to delivery and I remember saying to the perinatalogist "everything's going to be ok, right?" to which he slyly responded, "you're going to be fine".

The section was bloody and I recall hearing my doctor comment that whoever said my baby was head down was wrong, that my baby was frank breech. I remember the tugging and wondering how long it was going to take. When Kate was born, I could see she had all her arms and legs and she was crying. In that blissful moment, I thought all was well and I fell asleep.

Later, my husband came to tell me she had a cleft palate. I can still see the look on his face...half bliss that she was here, and yet a slight tinge of panic that all was not 100% well. Still, he said, "she has a cleft palate and they're going to transfer her to another hospital where they can fix it." I was groggy from the section and said, "ok, but she's fine though?" and he said "she looks great, perfectly normal". I fell asleep, B went home and the next morning I was awake in my room wondering to myself how women actually choose a c section, when a tall nurse came in followed by 2 EMTs and a transport isolette which held my newborn daughter. The nurse was from the hospital Kate was being transfered to and had heard I hadn't held my baby yet. She plopped her in my arms, I marveled at how positively gorgeous she was, breathed an audible sigh of relief she didn't have a cleft lip, and handed her back at which point the nurse said, "By the way, I'm pretty sure she has something called the Pierre Robin Sequence, but the doctor will call you later after someone examines her. It's not that big a deal, we see it a lot."

Pierre Robin Sequence....huh? What's that?

It took less than 12 hours for someone to call and confirm the diagnosis...and that's when the fun really started.