Everyone has a story, but this story is a great story. I know, that sounds just like a parent, doesn’t it? Truthfully the reason this story is such a great story is because there’s not an ending and there’s not a true beginning; that’s right there’s no ending and no beginning. We don’t exactly know why there is a story, or what caused the story to begin. More answers will be given in future additions to this part of the story. Truly it begins with genetics, something that most of us take for granted. I’m not going to go into that though … This is simply Jackson’s part in the great big picture …
Jackson Wade Riley was born on December 27th, 2007. The pregnancy was labeled ‘normal’, and what did we know? It was completely different from my first pregnancy, which yielded a smiling baby boy. The pregnancy was so different I thought for sure I was having a girl, but nope we were having a boy. No problem, wouldn’t know what to do with a girl anyways!
Jackson did seem to be the ultimate ‘hurry up and wait’ baby. I felt like the timer had dinged for my body the oven, two weeks at least before I finally saw the Dr. and not a nurse. There was a lot going on in the hospital that day. Guild and my best friend Kelly were both there to cheer me on. There was a birth that came in the door, and we were told the girl had had absolutely no prenatal care. I was shocked, and outraged, because I had ‘done everything right’. Little did I know … We’ll get back to that. Anyhow, they ruptured my membrane ‘manually’ and there was ‘record amounts of amniotic fluid’. I guess looking back that should have been my first clue this was going to be a whole different ball game than my first birth. I was given an epidural and about 10 hours later Jackson was ready to meet the world. Jackson arrived at 10.30 p.m. and in the blur of everything that was going on, all I can truly remember is that he wasn’t okay. He was kind of a purple-blue color and it looked like the umbilical chord was wrapped around his neck. Eventually, and yes it felt like eventually, I finally heard a tiny sound, just a whisper of a cry and that was it. All I remember was Guild telling me that he had to be fine, because he cried, and Kelly just repeated the sentiment, while snapping away with the camera, while they cleaned him and weighed him and whatever else it was they were doing. Then he was gone, the nurses whisked him away to the nursery to run tests, calling it respiratory distress. When Guild tried to look in the nursery window they closed the blinds. There was absolutely nothing we could do except wait. Because the hospital we were at was not equipped to care for Jackson, we were informed that he would be transferred to Baylor University Medical Center.
When I saw my son for the second time he was in this giant contraption that the nurses from Baylor NICU had nicknamed ‘The Limo’. It was basically this giant incubating box on a stretcher. He was all swaddled on his belly, eyes closed, looking content. They opened the ‘box’ and we started talking to him, and he opened his beautiful little eyes, and right then I knew he was going to be ‘okay’. After a whole lot of information about what they had done and where they were going, and what was happening, they left with our son. I don’t think much of that information besides ‘we’re taking him to Big Baylor, Dallas’ and the word ‘hypotonia’ and make a ‘list’ of people you want us to allow a visit with your son, got through to any of us. All I could think of was that I wanted out of that hospital, and at some point they were going to have to let me out or I was just going to get up and go. Jackson spent the first night of his life and many more after that in a hospital. I spent the first night of my second child’s life in the same room as the girl that had no prenatal care and gave birth to a perfectly ‘normal’ baby girl. To say that God has a plan is probably the understatement of the century. The next morning I was ‘released’ from the hospital and Jackson’s story continues.
I can’t really recall a lot of what happened in those first few months of Jackson’s life in any exact order. I do know that I’ve never heard my son cry, I know that Reid, our then 2 year old, didn’t get to see his brother for the first two months of his life, Guild couldn’t take much of the hospital at a time, and it felt like forever but the whole ordeal of the NICU only lasted a couple months. We were told he was hypotonic, and was having problems swallowing, and sucking. For those of you who don’t know what hypotonic means, it basically is defined by low muscle tone, which to me meant very weak. His palate was slightly high and arched, and he has very long fingers and toes, his ribs were thin and slightly bell-shaped, his lungs were weak, but his heart was healthy. I know that he was eventually doing so well that he was extubated, (not even sure if that’s the right word?), or taken off of the ventilator, put on a nasal cannula, and at two weeks old he crashed three times, twice in an hour, was brought back from death’s doorstep yet again, and reintubated.
Jackson was only intubated for 3 days, and this time they tried a cannula in his mouth to suck out all the secretions he was having such a hard time swallowing. First lesson with Jack, NEVER EVER EVER EVER take him off the monitor. The first time I held my son he turned blue on me. I didn’t even know, he was resting on my shoulder and I couldn’t see his face. His alarms never really sounded, Kelly turned to the nurse and aked if Jackson was supposed to be that color. He was whisked out of my arms, as fast as you can whisk an infant attatched to more tube and wires than a science experiment, to get suctioned and a little ambu bagging. At some point we met Jackson’s geneticist Dr. Angela Scheuerle. Dr. Scheuerle handed us a stack of information on all kinds of diseases, but the info that stuck out to me was the info on myotubular and centronuclear myopathy. I think reading it I realized that if he had this myotubular myopathy he at least had a chance, so I latched onto it. Bad choice … I recall getting through that particular information and getting on the internet and finding a website MTMRG.org. I was horrified. Don’t get me wrong I now know that this is a wonderful website, however, at the time it had not been updated in quite awhile and all the stories I read were about the deaths of these children and no one really knew why until after they died. Most of the research said that our son if he had MTM or CNM wouldn’t live past the age of 1, if he even made it that far. I was working myself up for a break down and Guild ever so patiently let me know I was on my way to crazy land if I didn’t just let it go until we knew more. He was right, what was the point in torturing myself until we really knew.
Next lesson in the medical world was that there was a certain ‘Lead’ doctor on for a two week period, but when the Dr changes so does your child’s prognosis. We still had no clue what was wrong with our son, and neither of us had any real experience with what we were up against. Jackson had an EEG, an EMG, MRI and a whole battery of blood tests ran on him, everything kept coming back normal. Again it was ‘Hurry up and Wait’. We were finally informed that we had a choice to make. The hospital could not continue to care for our son forever. I thought they were all crazy, I could see my son getting stronger every day, and they were what? Going to send us home? What I thought was vast improvement I found out had been labeled ‘questionable improvement’ by his doctors. We were then told our best chances of caring for our child was to let him have surgery. They wanted to go in and do everything all at once, a tracheostomy, gastric tube, Nissen fundoplication, and a muscle biopsy. At the time I was horrified, I just wanted to scream at them, and shake them and make them see what I was seeing. I didn’t understand why they would go in and and stick a tube in my son’s throat, and fold his insides up so he can’t gag or throw up. Shock and probably denial are the best words I could use to describe it now.
The key phrase was ‘then you can take him home’ and that became the bottom line. Now that I look back, I know without a shadow of a doubt that we made the correct choice for Jackson. We have been absolutely blessed from day one, we have been surrounded by the best of the best, and this is no over exaggeration. No one told us ‘in no uncertain terms, your child is going to die’ they just said it was up to him, and it was up to us to learn how to care for him in ways that we couldn’t even imagine. There were some harsh words of warning, we were to make sure and do everything exactly as we’re told so that Jackson had the best chances of survival. I have read stories of gloom and doom and hateful ugly doctors that have tried to put these boys in a little box and send them home ‘to die’. If anyone said something to that extent I was oblivious, and I don’t think I would have been able to forget words like those. We chose to have the surgery. We chose correctly. We wanted to bring our son home, and we wanted to find out what was wrong with him. The trach and g-tube gave him the option of home, and the biopsy gave us a stronger hope of finding out what the heck was going on.
February 1st, 2008 Jackson went in for surgery, all went spectacularly. We were just waiting for him to recover a bit and then it was on to Baylor Our Children’s House. OCH is a rehabilitation facility for children of all ages, where we learned everything there was to know about caring for our little monster. The people at OCH were an extention of the people at Big Baylor Dallas. Jackson’s major doctor’s went with us I guess you could say. His pulmonologist, his surgeon, his geneticist … and we see all these people to this day. Again we were blessed with people who truly knew what they were doing, and cared about what happened to our son. We were at OCH for 17 days, pretty much day and night taking classes, taking turns at the hospital, with the care of our Reid, juggling Guild’s job, and everything else. It was difficult then, but now when I look back and think about it, it could have been so much worse. I will say that 99.9999% of Baylor Our Children’s House is excellent. They are well trained, have fabulous bed-side manner, are knowledgable, are there to listen to you as a parent, and help you to take care of your child 100% of the time. Our insurance fought us every inch, they wanted us to have to travel to Ft Worth, to Cook Children’s. Baylor Medical Center, and Baylor Our Children’s House truly knew what was best for us, and they fought with us to get what we needed for Jackson.
March 25th, 2008 we finally had an answer about what was truly wrong. Jackson was diagnosed from his muscle biopsy and a blood test, he was officially diagnosed with X-linked myotubular myopathy. As usual it brought on more questions than answers. After reading some of the MTMRG.org stuff I simply couldn’t go back to it. I’m sure it was denial, but all the research I’d read, the so-called gloom and doom papers weighted heavily on me, so I refused to acknowledge them. I was busy entering this new strange reality and my plate was full, we would come back to that later. Jackson came home, and flourished. He got bigger, stronger, slowly oh so slowly, but every single day was something new and exciting. So, for the time being we ignored the diagnosis. I think we were really afraid of adding more to our plates than we could swallow. Reid has been an awesome big brother, a bit jealous, but no one can blame him. Jackson went 8 months without any complications. He has a continuos flow of appointments, clinics, machines, gizmos and gadgets. He has therapy 6 to sometimes 8 times a week. We started out with about 40 hours of nursing, so there was a nurse to take care of him at night so we could sleep. I put him on lists and interest lists, and programs that I still sometimes have a hard time figuring out what it is that some of these people do. He stayed healthy and we all stayed in our bubble.
Suddenly and when I mean suddenly I’m talkin in the span of an hour Jackson went from good to bad, his Oxygen level started to drop, his heart rate skyrocketed, and 911 was called. Our first ambulance ride … and for those of you who have never been in one, it is HORRIBLE!!! I held Jackson in my arms with his suction unit in between my feet. How was I to know that riding in an ambulance is like riding a bucking bronco in the rodeo? The last mile or so the EMT was helping me top hold my left arm up with Jack in my arms. Guild was on his way. I was petrified, I had no way of knowing this was to be a somewhat regular experience, he’d been home for a full 8 months with no problems. We were taken to Children’s Hospital Dallas. The only part of the day I looked forward to was when our pulmologist did rounds so he could tell us we could go home.
Our first hospital stay there was a joke, only we weren’t laughing and we still have horror stories of the ways they tried to ‘care’ for our son. We realized quickly that you NEVER EVER leave your child in the hospital without a parent present at ALL times. Two weeks, a lot of anger, frustration, idiotic nurses, respiratory therapist’s, med’s, crying, and juggling later Jack was almost well, and we were sent home. A couple return trips and February of 2009 Jackson was ventilated due to continued respiratory distress especially during sleep. Since then Jackson has been hospitalized one time for a nasty bit of the flu, and we’ve been to the ER once because he was playing and almost yanked his cuffed trach out balloon and all.
After our February 2009 visit to Baylor OCH, to have Jackson vented, I crawled back to the internet and started looking for chat groups online that had to do with MTM. I found a Yahoo chat group that eventually led me to Facebook. It’s funny the reactions you get about FB. I was mortified, for I am truly not a social butterfly, and after some pain with Myspace I was very wary about starting up on FB. I swallowed all this and got on, and suddenly I was finding people who were connected by the Yahoo group that moved to FB. So I started my profile and put out some friend requests and then forgot about it, for about a month. Around April I came back to FB, and I had a few requests, but then I had some friends ‘suggestions’ also. Slowly but surely I was pulled into FB full throttle, there was a HUGE network of people and families that were affected by MTM and CNM. I was amazed. I went on to meet all kinds of people, but I’ll never forget the first time I saw a picture of Joey Wells. I was looking through all these profiles looking for pictures of the boys, and I was really looking for someone who had defied the odds. I wanted to find someone who made it past his teen years, and I found Joey. The first picture I saw was of Joey in his Cowboys’ jersey. I thought ‘Awesome a Cowboys fan!!’ The next pics I saw were of Joey’s 21st birthday, and I’ve told Joey, but I’m telling you my mouth must’ve been hanging open. Here was Joey, obviously hampered by MTM, being a perfectly normal 21 year old, out drinking and carrying on with his friends. I think from then on I realized that I would make sure that Jack got to do whatever it was he wanted to do for the rest of his life. Don’t get me wrong I am still in parental denial that either one of my babies will ever be caught in a bar, but that was not the point. I wanted to make sure Jack had a life comparable to Reid’s and that they could both do whatever it was they wanted.
From there I got involved in going to the 2009 MTM-CNM Conference. I would like to add one thing. It is difficult for me to be on FB most of the time because there is a lot going on in our little community, and most of it is not pretty. For myself I get to the point where my glass is too full, and I simply cannot take on anymore. These parents and families are some of the strongest people and they all fight the fight that we do. This year and every year we lose some of our fights, and God takes his favored angels back to heaven sometimes without any warning at all. Two of these little guys were close to my heart and close to our home (in a manner of speaking, they live within a three to four hour radius). Benjamin, and Lane. After the loss of Ben I simply couldn’t handle, and now Lane, Lane put the fight back in me. At the 2009 Conference I sat next to the Brassfield’s and their son, Rebekkah, Jason, and Lane, for most of Saturday. I was shocked that Lane was so obviously affected by this disease on the outside. He looked so different, what did I know? I’d been unaffected by any kind of special needs my whole life. Lane scared me, but what I learned after sitting next to that little boy for a day, was that he was just a ‘normal’ kid. I couldn’t help but laugh because Lane was bored, he didn’t want to sit through a bunch of people talking, he was a kid, he wanted to play. Lane was throwing his spit towel on the floor, he was making this horrid clicking noise with his chair that was echoing throughout the conference room, and he was ramming both of his parents with his pwc. He had such life, and love, it just touched my heart. Lane has now gone to join Benjamin and all the other angels, he’s running straight and tall with a giant smile on his face and no tubes or spit towels. Lane was the final push for me to finish Jack’s part in the great big story. I can only be selfish for so long when someone like Lane reminds me that Jackson has a bigger part in this MTM story than I’m sometimes willing to admit. This story is in their honor, and to make sure these memories live on.
I would like to reiterate that we have all been so blessed, we have wonderful friends and family that stood by us. Jack’s doctor’s, nurses, respiratory therapists, speech, physical, and occupational therapists have all been excellent loving caring and nurturing people. That’s not to say that we haven’t fought hard, every day is a fight, every breath is a fight for Jackson, but God or the gods or whatever you believe, they have blessed us over and over again. Jack is the most laid back, loving, happy, and of course charming little man. He’s grown and grown and gained strength and lost it just to gain it all back again. We’ve had an excellent nursing company send us out some highly motivated and intelligent LVNs, and RNs that have helped in more ways than they will ever admit to. Jackson’s PCP calls him his miracle baby. He is more than a miracle, he’s perfect in every way, just like his brother (That’s right the Mommy in me is coming out again!!). He’s now sitting on his own for hours at a time, he uses a stander, a passy muir valve to make all kinds of funny noises so far. He loves to take baths and splash water everywhere!! His favorite game right now is ‘throw all the toys on the floor, and make my nurses and my parents clean them up’ similar to the game 52 card pick-up. He loves to make funny faces and can manipulate the pants off of you if he wants to. He’s just plain smart. Jackson uses some sign language. We’re fighting for a power wheel chair. At this time Jackson is mainly behind in gross motor skills, and speech, however, as far as cognition and fine motor skills, he is spot on what some would call ‘normal’. He’s not ‘normal’, Jackson is just Jackson, a great kid with a great attitude, a huge interest and love for the people, and everything in the world around him.
Our bubble is starting to stretch, 2009 we went to the MDA Christmas party, where we hung out with another MTM young man Jeffrey and his wonderful family, the Johnson’s, and of course Santa. 2010 we started off the year with Ride n Stride at the new Cowboy’s Stadium again for the MDA. We were blessed to have Joey and Cindy Wells come out and grace us with their presence at the stadium, and to sell Joey’s awesome book of poetry. The day after the Ride-n-Stride we met at Joey’s hotel with the Johnson’s and just hung out, and had lunch from Steak-n-Shake. I am so proud of Jackson and all of these boys and all the amazing things they do on a daily basis. Like I said this is just Jackson’s little part in what Toni calls ‘Our World’. There’s no beginning because no one is really sure when the first case of MTM or CNM was, and there’s no end because it’s a day to day minute to minute fight for our boys. The end will come when there’s a cure, but until then we just treasure every second of every day we have with our boys, regardless of the fact that one is a little closer to God’s doorstep than the other. One day at a time.