One year ago today, on October 4, 2011, the lives of my entire family changed forever.
Since this blog launched last November prior to the game against Oklahoma, I've been hesitant to reveal too many details about my life for fear that people simply wouldn't want to know them. This is a blog about Baylor Athletics first, foremost, and, to date, always. Because of that hesitance, most know me as simply a blogging automaton who gets a little angry on twitter sometimes and is obsessed with Baylor sports. Some know that off the blog I am husband to a fellow Baylor alum (undergraduate and Truett Seminary) who is the love of my life and father to two young children. One, our first, is a 3-year-old girl who is no less than the light of my life. The other, born the day after Robert Griffin III's win over TCU last year (September 3, 2011), is a young boy named William. After tweeting about him a little more the last few weeks, he's the one I'm going to talk about today. Be forewarned, this one may take more than one sitting, and I promise I'll get back to my regularly-scheduled blogging regimen later today.
Being a father has been and continues to be the greatest blessing of my life. When, in March 2011, my wife emailed me a picture of my daughter wearing a shirt saying she was going to be a big sister, I was absolutely ecstatic. (She emailed me because I was on the road for work). After waiting with bated breath for a few weeks, I peeked in the sonogram room when the technician wrote down the sex even though I wasn't supposed to know until the gender reveal party the next day. I couldn't help myself, and I was rewarded with the news I'd prayed for my entire life: I was going to have a son. Nothing against little girls at all -- I love my daughter more than anything in this world -- it's just the stereotypical thing; I wanted to be a father to my son just like mine was to me. We decided to call him William, a name from both families that seemed to fit our son perfectly before we ever really knew him.
About four weeks before his projected delivery date, we were rewarded with a baby every bit the healthy, happy boy that we hoped for and expected. He was large for being so early and spent only one night in the hospital with no problems. We took him home and started doing all the normal things parents of a newborn do like not sleeping, buying far too much baby stuff, and introducing him to our friends and family. It was during one of those visits that one of Beth's friends mentioned that William looked just like a little rag doll in her arms (I'm sure she meant it in a good way); a totally innocuous comment that we didn't think much of at the time. That was Friday, September 30. Over the weekend that comment started seeming less and less innocuous as we started quizzing ourselves about the fact that William wasn't moving much. We assumed that there was something we weren't doing to help him develop and decided to call a doctor the following week to have him looked at.
The first time our pediatrician could see us was Tuesday, October 4, a year ago today. I woke up late that morning after having spent most of the night with a crying baby sleeping fitfully on my chest. He'd never had problems sleeping before but seemed just ... uncomfortable. I was scheduled to leave town that day for work, so I headed south knowing he was headed to the pediatrician but thinking nothing of it. Maybe he was just growing too quickly, I had no idea. About two hours later I was in Colmesneil, Texas, just this side of the Big Thicket. I hadn't had cell phone reception for a few minutes and received a text message that is burned into my memory. "They're sending us to Children's," it said. "Something is wrong."
We got to Children's Hospital in Dallas that night around 7 PM. William's pediatrician in Tyler hadn't been able to tell us what was wrong but knew that something was; he had almost no reflexive muscle response and barely moved his arms and legs. They started running every test imaginable to figure it out, focusing at first on something gastrointestinal in the hope it was related to nutrition before turning to the neurological and neuromuscular side. We were talking to so many doctors and specialists every day that I started keeping their names in a spreadsheet with all the information I could find. I eventually stopped when it got over 100. I also started keeping a journal-- something I hadn't done since forced to in elementary school-- to organize my thoughts and write down things that were unfamiliar to me. My wife slept at the hospital in his room every night while I stayed with friends nearby, so the first thing I did every night was update my notes. I used the journal as a way to make sure I didn't miss anything and to say the things I couldn't when I was in his room. I wrote down everything doctors told me that I could remember, everything I thought about what they had said, and all my doubts and fears about what was happening to my son.
On Thursday of that week William underwent a test designed to check the connections between his nervous system and muscles. The name I wrote down at that time for the procedure is apparently incorrect, but it involved poking him with needles on either side of a nerve-muscle connection, sending an electric pulse through the junction, and cataloging the results against known patterns. They told us that night that the patterns were indicative of two possible diagnoses: botulism and a neuromuscular condition known as spinal muscular atrophy. I remember every second of that conversation, every word and movement of the doctor who came to speak with us. I'd heard of the first through various sources, primarily because of its use as a biological weapon, but the second was completely unknown to me. For those unfamiliar, botulism is a relatively rare (only about 110 cases in the US per year) but serious disease caused by spore-producing bacteria. The spores in turn produce a toxin that destroy the connections between nerves and muscle groups causing disruption in voluntary and autonomic muscle systems, respiratory difficulties, and even death in extremely serious cases. Most infants acquire the spores through eating organic, non-pasteurized honey or corn syrup. Spinal muscular atrophy (known as SMA) is similar up to the point of the prognosis; most people with botulism recover completely after hospitalization and have zero long-term effects. As the doctor told me that night, SMA is ... much much worse.
The next morning, a conference of about 20 neurologists and neuromuscularists took place on the campus of UT Southwestern Medical Center for the purpose of reviewing William's results from the day before. They didn't let me go to the conference itself, but William's doctors reported that it concluded about 60/40 in the direction of botulism. They decided that, since it would take 10-14 days to confirm SMA by genetic testing, they would treat for botulism in the meantime. That treatment, however, involves mountains of red tape and the approval of at least a half-dozen government agencies; like I said, you're dealing with something that can be used to make biological weapons. Negotiations between William's doctors, the Director of Children's, the Department of Homeland Security, and health organizations in both Texas and California (where the only lab allowed to produce the antitoxin is located) eventually yielded final approval for the treatment at around 6 PM. William's dose of BabyBIG (Botulism Immune Globulin Intravenous) came through the doors at Children's that night after being flown from California in a briefcase handcuffed to a man's wrist. This was serious stuff, and we were told the one dose William would receive cost about $46,000. The next morning they administered the golden liquid (seriously, it was gold in color) intravenously in the pediatric ICU and told us we should start to see improvement within a week with a child so young. We were overjoyed when we started seeing it almost immediately. More importantly, the doctors saw it, too, to the point that they moved him out of the ICU on October 10. Two days later they announced the suspension of further testing based on a clinical diagnosis of botulism. The head neurologist of the hospital told me while standing next to William's bed that he was 95% sure it was botulism and didn't think SMA was even a possibility anymore. I was so happy in that moment I had to leave the room before the doctor saw me burst into tears.
With the news that William was improving (and growing! He was already in 6-month outfits at just over a month), I went back to Tyler and work to try to occupy myself until he got to come home. To say I don't do well in hospitals is a tremendous understatement, and I'd missed enough time already. They told us it would be about a week before he was released but they weren't worried; they were just keeping him for observation at that point and to make sure he didn't react adversely to the BabyBIG, which is still somewhat experimental. Everything they saw from him indicated that he was doing better. I came to visit again that weekend before returning home on Sunday night for a short week at work. We were bringing him home that Thursday, October 20, if all went well.
I woke up early on Thursday morning after flying through work the previous three days. My plan was to pack a bag and head to Granbury, where my daughter was staying with my parents. I intended to pick her up and then swing back through Dallas to get my wife and son when he was released from the hospital. Because I had plugged it in to charge in the office, I didn't hear my phone ringing and had no idea until my wife's best friend nearly beat down the door that I had missed about a dozen calls. When I finally picked up, I heard the tears in my wife's voice before she ever spoke. It was the news no parent wants to hear: the genetic results came back and the doctors were wrong. William didn't have botulism; his improvement was a mirage, and he wouldn't be coming home that day. I wasn't even in the room when they told my wife that our son had SMA Type 1.
SMA is a degenerative, genetic neuromuscular condition characterized by extreme muscle weakness, a symptom known as hypotonia. As I understand it, a person with SMA does not create the spinal motor neurons necessary for development of the neuromuscular connections along the spinal column, so the impulses sent by the brain to the muscles do not reach their intended destination. Over time, this results in severe atrophy in every muscle system of the body, beginning typically in the legs before moving to the arms and eventually the core muscles like the diaphragm. There are 4 "types" of SMA with 1, known as Werdnig-Hoffman Disease, the most severe. Patients with Type 1 typically see onset of symptoms suddenly within weeks and/or months of birth and have an average lifespan of 18-24 months. As of this writing, there is no known effectual treatment for the disease. It is as close to a death sentence as I can imagine, and on October 20, 2011, my son was officially diagnosed.
That afternoon, after spending the longest, quietest 90 minutes of my life driving from Tyler to Dallas (a trip that normally takes a good while longer considering where we live), my wife and I, along with our extended families, sat down across a long conference room table with about a dozen doctors, respiratory therapists, and nurses, some of whom had been with William since he came to the hospital and had tears in their eyes. At that meeting, the doctor that would become his primary SMA specialist told us everything we already knew about SMA and laid out a series of extremely difficult decisions. They told us that William would eventually die what they called a "respiratory death" as his lungs became unable to expel carbon dioxide from his body, and that we would have to decide how we wanted to approach that process. We could choose, if we wanted, to do nothing and let William's condition run its course. We could wait to see how things developed and respond to his needs as they arose, a more risky approach since his condition could quickly deteriorate. Or we could be aggressive to combat known issues beforehand and start treatment immediately in an attempt to maximize the time he had left.
I hadn't planned to respond the way that I did, but I told them first and foremost that we were not upset about the way things turned out. As painful as the news was to hear, we knew it was painful to give, and we could see the genuine emotion from professionals who cared deeply about our son. I thanked them for that and for their diligence before telling them that we chose the option not offered: to maximize life. We would do everything in our power to make him comfortable, keep him happy, and give him the absolute best life it was within our power to give. That meant taking some immediate steps, but not all they recommended, and holding back on a few things that were a bit more invasive. If it didn't improve his qualify of life we weren't doing it. We weren't interested in just buying time at the expense of happiness.
That meeting was the first of numerous we had with William's doctors over the next two-and-a-half months until he was finally able to come home on December 28, 2011, after 85 days in the hospital. During the interim, William received a feeding tube directly into his stomach (because swallowing is difficult and aspiration a major concern), a surgical procedure called a "Nissen fundoplication" designed to eliminate reflux, and a tracheotomy. The attempts to extubate his breathing tube after surgery before Thanksgiving failed, so we decided to go ahead with the tracheotomy and put William on a ventilator. My wife slept just feet away from him on a stiff hospital bed every night but one during that time (they made her leave so I could do my overnight vent testing/certification), and never complained once about it. I'll believe until the day that I die that it was her strength that kept him going in the darkest days. And some of those days were extremely dark; we almost lost him half-a-dozen times due to one thing or another in the first two months of his diagnosis. We had quite a few heated arguments with doctors, disagreements with each other, and awkward conversations with people who thought they were helping but really weren't. Those two months were also full of questions to God about why He would this to our son, questions that have obviously and necessarily gone unanswered. Through it all I continued journaling, at home in Tyler during the week when I wasn't sleeping anyway, because it was something to do, and on the weekends when I visited as a way to compile information. I didn't like being at home alone while my wife and son basically lived in a hospital in Dallas and my daughter stayed Granbury, but it was my role. Every Friday afternoon I headed west only to return on Sunday night and do the whole thing over again. Sometimes I stayed at the Ronald McDonald House in Dallas but mostly with extremely generous friends who opened their home to me again and again. They didn't have to do that but they did, and I thank them for it. The launch of this blog in November also helped because it gave me something else to think about and obsess over.
I know what you're thinking: this is the saddest thing you've ever read in your life. I didn't mean for it to be. When I think back on it now after a year, I don't see it as a sad story at all. I don't see the painful moments, the worry, or the dreams I had for him that will go unfulfilled. Those things are still there, obviously, and more difficult to deal with sometimes than others, but they're not what I see in my mind's eye. We have new dreams now, new goals that mean far more than those other things did. Every day that we have William in our lives is a blessing. Every moment that we get to spend with him is one we were not guaranteed.
What I see in William is not a child damned by fate, but one blessed to have the time that he has who deserves as much as we can give him. I see my son, the perfect little boy I always wanted, perhaps packaged differently than we expected but mine all the same. I see the incredible support and generosity my wife and I received and continue to receive, the love people have shown every step of the way, and the kindness of even total strangers touched by William's story that has made this process so much easier than we had any right to expect. (The posters at Lone Star Ball deserve special praise for the support they've given us since the very beginning. They were the first people who really knew what was happening and gave of themselves repeatedly.) I also see the amazing doctors, nurses, and respiratory technicians who helped us get William home and keep him there. I see my wife, who spends every day at home with my son and daughter, playing with them, teaching them, managing every detail of William's life from nutrition to therapy, and advocating for him tirelessly and without complaint. I will never be able to thank her for the love she has shown my son.
William turned 1 year old a few weeks ago and has been back to the hospital only twice since we left in December. Overall, we've been extremely lucky that things have gone so well that he can go to his sister's soccer games, out to eat with his family in his wheelchair, and to visit his friends and family. He even got to go to my father's surprise 60th birthday party in Fort Worth. With the help of a rotating cast of nurses with him 24 hours a day, we've managed to let him do basically everything a normal 1-year-old does, and even though he will never walk, stand, or talk normally, and his life will be severely abridged, he doesn't know any of those things. All he does is smile basically non-stop and giggle like you would not believe. He never complains and rarely fusses; he takes just about everything that happens to him completely in stride. His bravery is an inspiration.
Like every child, he has favorite TV shows that he absolutely has to see every day as well as some he hates, a routine he follows from the moment he wakes up to the moment he goes back to bed, and days filled with games and activities in between. Even if it isn't always easy, I'd say so far we've been overwhelmingly successful in making every day count. Everyone, even our three-year-old, has simply adapted to manage his needs. Because his condition does not affect his neurological development at all, William has all the same emotions as a normal child and communicates as best he can through movements of his eyes and some sounds he can make from the back of his throat. He can call out for his "mama" so loudly that you hear it across the house. He's also quite simply gigantic (something his doctors don't really understand) and wears 4T clothes at 13 months.
It's an interesting feeling to know that you carry as a part of yourself the gene that will eventually rob your child of life. I know that it's not our fault because there's no way we could have known that both of were carriers of the abnormality-- and both have to for a child to be affected-- but you still feel responsible in a way. You shouldn't, but you do. The key for us is to always focus on William -- fulfilling his needs, enriching his life, and making sure that we keep sight of the ultimate goal: loving our child completely and with full devotion. And it's not like his is a situation completely devoid of hope, right now there are more treatment options in different stages of testing than ever before. From the literature I devour in my spare time, researchers understand fairly well what has to be done to "cure" SMA, they just haven't figured out exactly how to do it yet. Still, it feels like there is finally light at the end of the tunnel for the thousands of families affected by this disease (condition/syndrome, whatever you want to call it), we just have to reach it. That's why the word "incurable" rankles me like you would not believe. William's condition is not incurable, because that connotes impossibility and forlorn hope. It is uncured.
A year ago today my families' lives changed forever but not even mostly for the negative. Every day when I go home and see my son's smiling face, I am thankful for the time we have no matter how long it lasts. I didn't write this post to ask for sympathy or for anyone that reads it to really do anything. My only real purpose was to share with the community something that is obviously a big part of my life, and I figured that today, one year from the time this all began, was as good a time as any. If you want to, though, there are literally dozens of great organizations that could use your help in continuing the fight. You can view just a few of them on a blog my wife set up (we're a family of bloggers, I guess) called "Sharing William." She started it after realizing that not only do most people not know what being a parent of a special needs child is like, they don't know what being a special needs child is like. There's a lot of misinformation out there that we can combat, we just needed a platform from which we can fight. You can also find more information about William's day-to-day activities, links to resources about SMA, and, of course, a picture or two of William.