Quinn Fowlie
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How many ways can we tell our children's stories? Have you ever stopped to count the number of variations and versions of your child's history that you have created? From the day they are born we begin. At first they are sad stories, full of tears, reaching for and forgetting all those huge medical terms everyone is throwing at us. But we phone our families and friends and try to make sense of it all for them. "Part of his esophagus is missing", "No, I didn't know that was possible either", "He'll need surgery", "They're moving him to Children's". "Yes, please pray."
Right away the editing process begins. You pick and choose who you will tell what version to. Maybe Grandma won't understand that part, maybe Aunt Karen would get too upset. What about the neighbors? Though you wish they just wouldn't ask, they do, and you need a version for them too.
So I thought about this process as I sat to write this article. Finally, an audience that needs to translation, no editing, no softening. They've been there, they know. Some of you will read our story and say, "wow, we're so lucky we never went through half of that!" Others will say "they sure got off easy, they don't have half the things to deal with that we have." I know, because I've read so many of your stories and said both of these things. And then there're the people I'm most anxious to tell this story to, the parents who have just had their baby diagnosed with EA/TEF. I remember. I remember the fear, the pain, the anger and then the joy at finding the EA/TEF Child and Family Support Connection and this newsletter. You're not alone.
- Frequently Asked Question #1:
Did you know before he was born?
I had a relatively uneventful pregnancy. We were very excited since we'd been married for 10 years and finally felt "ready" to have a baby. Ah the arrogance! I developed a bit of hypertension in the last months of pregnancy and was on bed rest for 6 weeks. Other than that all was normal. Every practitioner I saw said "oh this is going to be a big baby". Comforting words to a woman soon to deliver! But after a long, tiring, induced labor I had a C-section due to failure to progress and delivered a 6 lbs. 10 oz. baby boy. In retrospect my "big baby" was likely undiagnosed polyhydramnios.
When the delivery nurse handed me the baby I knew something was worrying them. I held him for about one minute before they said that he needed to go to the Special Care Nursery because he had a lot of fluid in his lungs. Everyone was calm and caring, and while I was upset at not being able to hold or nurse my newborn I never for a moment thought it was anything serious.
It wasn't until the next day that the attending pediatrician came to tell us that he suspected Quinn had EA/TEF. "EA what?" Esophageal Atresia with Tracheo Esophageal Fistula. I lost count of the number of times I made my husband repeat those words to me. "Honey, what is it called again?"
That was when we saw the first of hundreds of quickly drawn diagrams of the inside of our precious boy. I still have the paper towel that contains that first drawing, along with the words Esophageal Atresia with Tracheo Esophageal Fistula.
Nothing could be confirmed that day, it was the weekend and the x-ray equipment at our local hospital wasn't strong enough to get a good picture. But when they tried to pass an NG tube it curled around and came back up. They were pretty sure. They told us Quinn's esophagus didn't reach to his stomach, it ended in a pouch, and that there was a connection from this stomach to his windpipe. He'd likely need surgery. He'd be transferred to British Columbia's Children's Hospital (about 45 minutes away in Vancouver) where they'd confirm the diagnosis and do the surgery.
It all sounded so simple. Heck, I thought, we'll be out of the hospital in a couple weeks with a perfectly sound baby. There's that arrogance again! The very first email I sent to the EA/TEF Support Connection must have made them laugh: "My son Quinn was born with EA/TEF, but everyone assures me he'll be perfectly normal. I promise that even when Quinn is all repaired I'll stay in touch with this group, it's been so great to find you."
So at the age of 3 days I sent my son into the operating room. That was the longest day of my life. But my husband and mom were with me, and the nervousness and lack of sleep caused us to laugh as much as we cried, and the day passed. Our surgeon is a gentle, quiet man, Dr. James Murphy. When he came out of the OR he looked like you could ring him out and you'd be left with nothing but a puddle. The news was not what we wanted to hear. Quinn's gap was too long to repair. They had ligated (closed off) the fistula and put in a G-tube for feeding and a replogle tube to remove secretions and they'd try again in 3 or 4 months.
- Frequently Asked Question #2:
How long will he have to be in hospital?
"Wait, you're saying he has to stay in hospital for 3 or 4 months?" That's what he was saying. "Be patient Mrs. Fowlie, we're going to develop quite a relationship over the next 7 or 8 years." 7 or 8 years? He said 7 or 8 years! Patience was not a virtue I possessed, but I learned quickly. In those early days if one more person had said to me, "you have to take one day at a time", I was going to give them a black eye! But in time I came to see the wisdom in that, in fact it became my primary coping philosophy. Though I'm still very careful not to say it near newly diagnosed families, I don't need a black eye.
So we settled into hospital life. After a few days Quinn was extubated and was breathing on his own. Everything seemed to be going well. We adjusted to the tubes and wires. We learned to love and loathe that darn replogle with its nonstop sucking noises. And I became very close friends with a breast pump. Everything was going along fairly well for about 3 weeks. So well in fact that the plan was to transfer us back to our local hospital to wait out our 3 or 4 months. We were set for transfer on Monday and on Friday night we left Quinn in the intermediate care room, happy and sleeping. Saturday morning we arrived to find Quinn in the acute care room, reintubated and in an overhead bed! He had unexpectedly aspirated. They weren't sure why, but they thought it best not to transfer him after all. So we settled in once again, to a life of long drives and hospital food.
After that episode we got another couple of weeks of relative peace and then Quinn started to have quite violent coughing spells. Suctioning certainly helped but the dusky color he turned was not comforting to anyone, least of all his mother!
Well it was early December, so they immediately suspected RSV (the next of our lesson in acronyms). Quinn was moved to an isolation room, where we were required to wear gowns, masks and gloves. Great for mom and baby bonding! They intubated him again the next morning because his breathing was so labored. We had no idea that he would end up being intubated from that moment until late February!
All the viral and bacterial tests came back negative. No RSV. Thank God. But what was causing the breathing problems, the huge coughing fits? Enter the ENT team. "We want to do a bronchoscopy to see what's going on in there". Okay sure, I said. I'd read a few EA/TEF newsletters by then and said confidently to the ENT resident that I thought it was likely tracheomalacia. Recognize that arrogance again?
- Frequently Asked Question #3:
What is it your child has?
Our first meeting with ENT Surgeon Dr. Fred Kozac was after that first bronch. He looked grave and hurried us into a private room. "Quinn has a hole between his trachea and esophagus, this is causing his secretions to pour over from his esophagus pouch into his windpipe and stop him from breathing." Okay, we asked, what the heck is that called? "Well it's a laryngotreacheaoesohphageal cleft (LTEC), type II, but let's just call it a post-laryngeal cleft for now".
Debates ensued between ENT and general surgery about who was going to have to do their repair first in order for the other to have a chance at success. In the meantime we got more and more confused about who we should be hassling to get things moving. Eventually it was decided that ENT would attempt an endoscopic repair of the cleft because without that Quinn could not be extubated. We were told repeatedly that the odds of successful LTEC repairs was very low and it often took several, even many, attempts before it would "take". We were hopeful, but after a week of medically induced, post-operative paralysis, another bronch showed that the repair had broken apart. Our only option now was a tracheostomy so that Quinn could get on with life and development without being attached to a ventilator by his nose. But it was Christmas by this time and Quinn's tissues needed time to rest before that could happen.
Time passed, Quinn learned how to extubate himself, and did so on a regular basis (twice in one memorable 24 hour period). The only answer was that whenever he wasn't being held or directly observed by someone he had to wear mittens and be bundled in blankets. I was frantic with worry about how that would affect his development. So by mid February when we still had no date for his tracheostomy I developed a strong case of "Angry Mother Syndrome". I took two of the attending physicians into a room and told them just how I felt about sitting and waiting! Words like that should never be heard from the mouth of a lady, but it got action. Miraculously the next day I was in the ENT surgeon's office making a date for my son's tracheostomy.
- Frequently Asked Question #4:
Refer back to Question #2
Wait, haven't those 3 or 4 months passed by now, you are asking. So were we. But with all that was going on with ENT and a fresh trach etc., surgery was in no hurry to do anything about the esophagus.
Along with the tracheostomy came a move from the Special Care Nursery to the Special Needs Unit, also known officially as the Technology Dependent Unit. After four months in the SCN it was a bit daunting to think of meeting and training a whole new set of staff to do things our way. But it wasn't long before we felt very at home in SNU. The unit is a subset of the PICU and is specifically set up for kid with trachs and vents who are often long-termers. For the first time in Quinn's life we had a window to the outside and with Spring quickly approaching the sunshine was a welcome addition to our life. The philosophy in SNU was refreshing too. Their attitude is that if these kids have to live here it should be as much like normal life as it can be. For the first time in his life Quinn was dressed in clothes during the day, played on a mat on the floor, was surrounded by toys and music, had daily physio and occupational therapy, and was only in bed to sleep!
Despite the fact that Quinn still had a replogle tube on continuous suction and had to be attached to the wall 24 hours a day, we once again settled into a pretty nice routine. The staff quickly fell in love with our charismatic, grey eyed beauty and he had them all wrapped around his finger in no time. His easy laugh and continuous curiosity were infectious. Tubing could be extended so Quinn could reach the playroom and still be attached to suction, eventually portable suction units were found for us, so we could even go for short trips outside! SNU became home as we realized we were in for a much longer stay than we had ever anticipated.
- Frequently Asked Question #5:
When is Quinn's next surgery going to be?
As we settled into the routine of daily living in the hospital my job became to chase and harass our surgeon. I was anxious to know what the next step would be. I knew that Dr. Murphy's goal was to be able to do a primary anastomosis. He was adamant about how much better Quinn's future would be if he could avoid an interposition. So, every time I approached him the answer was the same let's wait and see a bit longer.
Finally in early April, Dr. Murphy ordered a "gap-o-gram" to measure how far apart the two ends of Quinn's esophagus now were. The answer was encouraging but not cause for celebration. There was still a significant gap. This meant Quinn would be going back into the OR, this time for a Rehbein Anastomosis. If his esophagus was so determined to fistulize then surely it would fistulize to itself!
All we could do was wait. Wait. And wait. No one could tell us how long it might take for the two ends of Quinn's esophagus to decide they wanted to spend the rest of Quinn's life together. Nearly three more months passed before they did another "gap-o-gram". Sadly the Rehbein was a failure. For whatever reason the two ends did not want to grow together. If Quinn's esophagus was going to marry it was going to have to be a shotgun wedding.
Summer in the technology dependent unit was fun in an odd way. Several older trached children were in for varying length stays over their summer vacations. So there was always a lot going on. One teenage girl liked to paint everyone's nails, and even painted Quinn's toenails for fun. We did a lot of arts and crafts, played a lot of games, watched a lot of videos. Never a dull moment. The nurses and child life specialists even bought a little baby pool for Quinn to play in out on the patio. Quite an exercise to hook his replogle up to a portable suction machine and haul him, two suction machines, emergency trach equipment and the pool all outside.
Summer turned to fall and still the surgeons seemed reluctant to proceed. They just needed to give the esophagus "a bit more time" to grow. All hopes of being discharged before Quinn's first birthday went out the window. But I begged the doctors to get the next surgery out of the way so he could at least enjoy his birthday party. October 5 is the day we'll never forget. Primary anastomosis day! Going in we knew Quinn would come out with one of two outcomes. Either he would have a successful primary anastomosis or he would come out of the OR with a cervical esophagostomy. Either way, we'd be going home after this.
The surgery lasted most of the day. And when the tired, sweaty surgeon finally emerged from the OR I braced myself to hear that they had been unsuccessful. So few things had ever gone Quinn's way. So when Dr. Murphy said "it's done, his esophagus is together", my reaction was "no way". After 11 months my baby's esophagus finally went all the way from his throat to his stomach. No more replogle tube. I had not seen my baby without a tube taped to his face since the day he was born.
After a week of medical paralysis to keep him totally still to heal, they did a swallow test to check for leaks. All of us thought it was a foregone conclusion that there would be leaks. They had had to stretch the ends so far! Leaks and stricture were inevitable. But the nurse who brought him back from radiology said they'd found no leaks. No leaks? She must not know what she's talking about. I'm sure she's wrong. Later that day the surgical fellow stopped by to see me. I knew he'd know the real story. "No leaks", he said. "I saw it with my own eyes or I would not have believed it." I wanted to cry. Then I wanted to run out and get a T-shirt printed that said NO LEAKS! I immediately ran to the hospital library and sent out an email to everyone I knew with the subject line NO LEAKS!
On October 30th I took my son to the airport to pick up his grandma who was arriving for the big birthday celebration. Never in my life was I so joyful. The look on my mother's face when she saw her grandson, with no tubes on his face, "out in the world" for all to see, was the best gift I've ever received. The posters I made for Quinn's party said "he's got style, he's got grace, no more replogle on his face!" And never was there a more joyous first birthday party. We filled the hospital cafeteria with nurses, RTs , relatives, friends, other hospital families and more. A celebration of love for a gorgeous, special boy who'd had one tough year.
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