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.