I must admit, that while I was pretty comprehensive in my wrap up of our last time with Dr. Day, I wasn't entirely forthcoming. I could rationalize that I was just overwhelmed with the wonderful news that Sam could come off oxygen for the day. I'd be more accurate in admitting that the other piece of the conversation with Dr. Day was met with a healthy dose of skepticism. After all, I had conditioned myself to believe and expect one immutable truth - Sam's condition would be fixed, but in doing so, we would never really know why the journey had to happen in the first place. From the beginning, we were told that Sam was special - one of the doctors even said "one in a billion". Fistulas like this happened as a fluke, and Sam had simply drawn the wrong numbers in life's lottery. I had accepted that. Early on, it drove me crazy. As time passed, I found a certain comfort about it. After all, if there was another cause, I was sure that there would be other problems...health concerns that we hadn't even considered. No, I was fine thinking that Sam was just that fluke. It made it easy to see how special he was. It made it easier to wonder why we were the ones having to go through it. But more than anything else, it made it infinity easiest to know that once the fistula was fixed, we would hopefully be in the clear.
Hence the skepticism, as Dr. Day started fishing. I must admit, the guy has impeccable timing. Only Dr. Doom could find a way, at the end of a fantastic visit, to throw a cold glass of ice water on the festivities. As we were wrapping up with our visit, and Dr. Day was doing one last cursory examination of Sam, his eyes stopped on Sam's knee. There, in plain sight, was a red birthmark. I watched as his eyes considered it. I heard as he asked if that had been there the whole time. I doubted when he started to unveil his next theory. But I also listened, as Dr. Day had been remarkable in his theories and ideas. Where would we be if he hadn't put a stethescope on Sam's head? As we stood, getting Sam ready to go home (and to start weaning him off of oxygen), Dr. Day mentioned that it might be a good idea to get the birthmark checked out. A few seconds later, he had scoured Sam's legs and found a couple more birthmarks. He wondered, out loud, if Sam might have something called HHT (Hereditary Hemorrhagic Telangiectasia). I had heard about this genetic disorder when we were living through our first Primary Children's visit. Do you remember me blogging about the Genetic Army coming through, and being thoroughly put out when our family history didn't match up with their preconceptions? So when Dr. Day mentioned it, I simply remembered the dejected looking counselors walking out of our room when they didn't find a bunch of people that happened to have bloody noses in their family history. He asked if we would mind him making a connection with the University Hospital HHT clinic, and at this point, I felt the most respectful thing would be to let him get on with his theory. The worst that could happen was to come back and tell him he was wrong, and to try again.
So we left and went on with our lives. After all, Sam's oxygen was dominating many of our thoughts for the next couple weeks. I vaguely remember Elisa telling me she had scheduled an appointment at the end of January at the HHT Clinic (they called us, after talking to Dr. Day). I was still surprised when we got our reminder in the mail. I wasn't the only skeptical one, either. Elisa and I had a drawn out conversation in our kitchen when the reminder came, questioning whether we needed to go at all. Why waste time off work to go up, explain our family history, and observe the dejection as it came into focus on their faces? But we agreed that, at the very least, we could just cross another thing off the list (a little more officially) as to why Sam had a fistula at all. In the end, we headed up to University Hospital and into the HHT Clinic.
It was weird to be back at University Hospital, since the last time I was there (except for the little trips to the cafeteria or Starbucks) was for Sam's first embolization. Going now to see specialists that would poke and prod at the little man to find a root cause gave me no further comfort or satisfaction. I know what you're thinking - wouldn't you want to know the cause? There's something scary about finding out more. When you have a sick child, you sit in hospital rooms or doctor's offices, hoping for good news but dreading the worst. In fact, it's the fear of what they'll find that dominates you, not the hope of what they won't. You're in a hospital, after all...there's usually a reason beyond treating the place like an Extended Stay hotel. So here we were, facing an office that exists for the sole reason of digging through your DNA to find out why things happen. I didn't get the warm fuzzies that we were here.
We started our consultation with Jamie MacDonald, a geneticist and the assistant to our eventual Dr. (Stevenson). I thought, hopefully, that the geneticists from Primary's would have talked to these guys, and we could avoid the whole family history diatribe. I was wrong - so we painstakingly walked through our family histories, as Jamie was looking for some very specific commonalities. HHT is known for capillary malformations that can range from red spots within the oral cavity, to recurrent nosebleeds, to...you guessed it...AVMs and AVFs. As we went through our histories, though, I was the only one that had even a small amount of nosebleeds, and the couldn't really be categorized as recurrent. Jamie's ears did perk up, however, when I mentioned that Sam's birthmarks weren't out of the ordinary. After all, I've had some of those very same birthmarks my entire life. No biggie, right? She wrote down a lot of notes as we talked, and openly said that Dr. Day seemed to be off on his initial thoughts of HHT. As I was physically exhaling from that bit of news, she added "but based on what we had heard, we weren't of the opinion that it was HHT anyway. We think it's most likely something else". Another theory. Another hypothesis. Another genetic problem that could be linked to little Sam.
We were then joined by Dr. Stevenson, who was a very nice man...and clearly knows his stuff. Jamie was a bit...rough around the edges. You could tell that Sam was almost a laboratory experiment that happened to show up for examination. I half expected her to pull out a banana and ask him to do a trick. Dr. Stevenson was much softer. He reviewed the family history and then began his examination of Sam. It was clear that he was focused on finding only one thing...red birthmarks. I knew Sam had a few, but I was kind of taken aback when they hadn't even left his legs and we were up to 6 already. Sure enough, Sam had some on his head, his arms, and his trunk (in addition to the legs). No, he's not a patchwork quilt. They aren't large and they're hardly noticeable, but by the time they were done, they had seen enough...and it seemed to be confirming their suspicions of whatever was rolling around in their heads. And then this exchange occurred.
You know, before I share, I must admit that this is one of those moments that I'll add to that blog I wrote some time back. Sam's birth. The September 30th Dr. Doom incident. December 10th embolization. I added January 28th to the pile. The date of the exchange. The day that Sam's journey took on a very different...and very personal new path.
Dr Stevenson: "So you said in the family history that you have red birthmarks on your body?"
Brad: "Yeah, I've always had them. I have a couple on my arms. A couple on my chest. So I wasn't surprised when Sam had them."
Dr. Stevenson: "So we don't believe that Sam has HHT. The distribution of the red marks isn't consistent, but we do think there is a high probability that he does have a genetic condition called Capillary Malformation - AVM, or CM-AVM. This is a condition that most commonly is inherited."
Brad: "..."
Dr. Stevenson: "Would you mind if you took your shirt off, so we can look at your birthmarks?"
Two realities were crashing upon me as I slowly unbuttoned my shirt. First, there was the very real possibility that Sam's fistula had a source...a reason beyond being a fluke or one in a billion. The second, more troubling, was that the reason could be me.
I've lived my entire life with a few random red birthmarks. There is one on my right arm, where my elbow bends in, that I remember for as long as I have memories. But it was harmless, worthless. Nothing more than an interesting dent in a car that had to have an interesting backstory. To know that something like that was going to be the eventual cause of Sam's struggles in life felt decidedly overwhelming. Like an out of body experience, I watched as I was examined, and Dr. Stevenson found multiple red birthmarks...now being referred to as capillary malformations...on me. I was in my own head, thinking of how to handle the impending guilt that was crashing upon me. I looked at Elisa, who almost had a sense of relief. I could tell that a different emotion was washing over her...realization that we did have an answer and a cause of Sam's story. It was strange to have heard the same news and yet react in such different ways.
I didn't blame Elisa. I could see the relief in her mind...sure, we weren't really positive about what CM-AVM might entail, but from the initial sounds of it, it was the genetic disorder you wanted to have if you had to choose! It appeared that the primary symptoms were red birthmarks, or areas of the skin where the capillaries are malformed (causing the red appearance). In only 30% of those with CM-AVM did an actual AVM or AVF appear. Later that night, I'd do my own internet research, and find myself quite shocked that this disorder read word for word like Sam's initial 5 months (when a fistula was large enough to cause problems). Even as the doctors talked about the disorder, it felt like the worst part was Sam's current experience. It gave rise to hope that perhaps if the fistula could be completely eradicated, Sam might be okay.
But that's not where I was at the moment. I was standing in a room with my shirt off, getting examined when I didn't expect to be. I was exposed, though not for the physical reasons you're thinking. It was just that the attention had turned to me. Sam was probably enjoying himself...he wasn't the center of attention for once. I was, and I didn't care for it. And as I was dealing with what I can only call a building sense of guilt that my blood...my genes...might be the culprit behind all of it, I hadn't made the mental leap that Dr. Stevenson was going to take for me. MRI. No, not Sam's. They already knew what they were dealing with in Sam. No, a MRI for me. Not only were they confident that Sam and I shared a genetic disorder called CM-AVM...now they were worried (and had to rule out) about me having an undiagnosed AVM or AVF. Sure, it wasn't likely to be as large as Sam's, or I would have encountered something much earlier in life. But the risk was there and had to be addressed. As Elisa took care of Sam and got him ready to go home, I walked through bare halls of the hospital, in the wake of Jamie MacDonald, as we tried to find someone to schedule the MRI with.
Before I knew it, I had a MRI scheduled for the following Monday. That would be preceded by my own official appointment at the HHT clinic, along with some really fun conversations that I got to have with my parents about the potential existence of red birthmarks on them. And then, I had to go home, strip my two older boys down, and desperately look for a lack of red birthmarks on them. And that's what I did. I stumbled home, nearly crying 2-3 times through my guilt, and now personal worry for what might be rattling around in my own brain. Once home, we ate dinner and then it was time to get the boys ready for a bath. In succession, each one stared at me while I looked over their arms, trunks, and legs...searching for the existence of little red birthmarks. Something so generic and seemingly unimportant that I had convinced myself for 35 years that they were nothing...just part of my genetics.
Well, I guess I was right about that one. Just wrong about it being 'normal'. Suddenly, Saving Sam was taking a whole new perspective in my head. If we had spent the last 5 months trying to save Sam, was it even remotely possible that the roles were reversing? Was Sam's condition and a chance trip to a genetics clinic going to be in the position to save me? And why did I even need saving in the first place?
Until next time.
Saturday, March 29, 2014
Wednesday, March 26, 2014
Saving Sam (Chapter 5.02): A long, deep breath
Elisa has been giving me grief for falling off of the blogging wagon for weeks, if not months. She told me multiple times that people were asking where the blog posts had gone - they wanted updates on Sam, and since I spread the news that Dr. Doom was not as dreary as previous, and Sam could come off of oxygen during the day. That was over 2 months ago. No blog posts. No extended Facebook posts. Nada. For a few weeks, I've wondered where the blog bug had gone. Why wasn't I writing? Why didn't I pull out my computer every night, as I had since September? Well, here I am, writing again, both because I actually saw someone (on Facebook) as Elisa where the blog had gone, and I think I also figured out why the writing stopped.
You can all thank Amie Corey, one of Elisa's friends, for the return of the blog. She was the one that publicly asked, and her post was the one that pushed me to start writing out an outline of a 6-blog series to catch everyone up on little Sam. Yet, it was the hours leading up to the writing that finally opened my eyes to why I had stopped. For awhile, I had convinced myself that happy thoughts weren't as easy to write about (in a strange, twisted way). I also wondered if one of my chief reasons for the blog (public therapy) had faded, as Sam was no longer under a danger watch, and I could probably manage my personal feelings without long winded blog posts. It turns out, both were wrong. The writing stopped because I had hit an emotionally exhausted wall. For nearly 4 months, Elisa and I lived at the edge of sanity. Every day, we would wonder what could happen...heart failure, neurological problems, or worse (thanks, Dr. Doom). I'd thank the universe before I went to bed that we (and Sam) had survived through another day. I'd wake up every morning, worried at what the next 16 hours would hold. Dr. visits were worse, as some of the most "benign" trips would turn into horrific nightmares. So when we left Dr. Day's office in early January with a diagnosis of improvement, as well as a reduction in Sam's intervention, my mind decided it needed a break.
Blogging is, for the reader, a (hopefully) interesting story that they've never heard before. For me, it was reliving pain, agony, fear, happiness, hope, and the lack there of. As we drove home from Dr. Day's, there was silence...a type that hadn't existed before Sam was born. A few nights later, after I had blogged about our visit, I brought my computer to the bedroom, intent on writing more. I stared at the screen for 30 minutes. No words came...and that's saying something from me. It was as if I was tapped out. I needed to not write. I needed to revel in the fact that things weren't as dire as they had been. I needed to embrace the silent comfort of hope.
Over two months later, however, it's time to go back. And as I look all the way back, I'm surprised at how much has transpired. So while I thank you all for letting me have a blogging breather, it's time to catch you up. Let's get started.
When you last left us, Elisa had headed home with the go ahead to take Sam off of oxygen. It was more than we had hoped for. We were crossing our fingers for a reduction from 1/4 L to 1/8 L. Now, we were okay to take him off for 14 hours a day. We were thrilled. We were shocked. We were...scared. That night, we both sat and stared at the oxygen tank. Were we to start to tomorrow? How would he do? It was funny to me that for four months, all I hoped for was to be rid of the oxygen, and now, I was clinging to it as though it was the only think keeping Sam here. It turns out that, while annoying, the oxygen was more of a security blanket than an albatross. And being given license to remove the security blanket, we felt exposed and scared.
I wish I could say we were brave - that we pulled the oxygen off during the days and never looked back, but that's not how it went. No, we focused on small oxygen trials before bed. 20 minutes, 30 minutes, 40 minutes. And then the oxygen would return and we'd get through the day. We had a million reasons why the oxygen should stay - did he have a cold? Was he breathing harder? Was it a Wednesday? Every reason we could find was considered. No, we didn't just leave it on all the time. We'd have mini breaks from the oxygen. We got really brave one night and did it up to a nap. As it turns out, though, it wasn't us that finally pulled the plug. It was my Mom.
By this point, Elisa was back to work. So my Mom started coming back around to watch the kids. While her love for Sam was equal to ours, her dependance on the oxygen was not. So we would both leave to work, and my Mom would drop the oxygen. Sure, we'd come home worried that he was breathing harder, but he'd survived a day and we started wondering if he was doing a little better than we were letting ourselves believe. Logical me, when I stepped back, just saw oxygen levels (on the oximeter) of 90-93. It didn't seem high enough, even though he wasn't dropping into dangerous territory. But my Mom's actions gave us a little more confidence to see how Sam was going to do by himself. Before we knew it, Sam was off of oxygen during the days, save some time where his naps would worry us back to the cannula. But then, something amazing happened. Sam's oxygen readings started to change.
It was a Saturday after a long day without oxygen when I decided to plug Sam into the oximeter before turning on the switch at the tank. Usually, I'd get him set, turn on the o2, and then put him in his room. Tonight, I was curious as to how he held up during a very long day. The oximeter went on, the numbers popped up, and I was immediately sure that we were getting a bad reading. Sam was sitting at 97%...without oxygen. I was sure it was going to change. I was right, though not about the result. Suddenly, he was 98%...still awake, cooing at me from his crib. At 98% oxygen. I'm now convinced that Sam was ready from the moment Dr. Day said we could move forward. But this is a baby, and I sometimes forget that he is learning to do everything from scratch - including how to breathe. He needed to learn a bit - how to handle this thing on his own. I'm immensely grateful to my Mom for trusting Sam more than we were ready to trust him.
Like a light switch clicking on, though, Sam getting off of oxygen changed the world for the better. For four months, every day, were hooked to a tank at the top of our stairs. Sure, the cannula was on Sam, but it's cord extended around our own hands and feet. It grounded us in our house, and was a reminder of the struggles Sam had been through. You may be scoffing. I'm sure someone out there is thinking "I see people at the grocery store with a little oxygen tank all the time!" So do I. But switching to the small tank, always making sure Sam was hooked to a good flow, and the cord...the dratted cord...the number of times I tripped on that thing, I tell you.
But off oxygen, we truly did feel free. Elisa brought Sam to the boys swimming lessons one Saturday morning. I started a mini habit of bringing Sam to the grocery store every Sunday morning. We started going out to stores again. We started feeling normal. And while this was all wonderful (and it was), that wasn't the best part of the experience. No, that was when we finally let go of the cannula during the day. When I saw his face...his beautiful, cannula free face, I nearly lost it. He looked so different. Almost like someone who wears glasses all the time might look if they finally take them off. It's more subtle than you think, but you realize that your perception of them includes the glasses...or the cannula. It feels foreign...wonderful foreign...when it's gone.
As for the rest of Sam, I'll admit that the adjustment off of oxygen made us hesitant and worried elsewhere. We had tried to start him on rice cereal, but his stomach and intestines seemed to react - he seemed to have a constant belly ache and a bout of constipation. He was still on three doses of Lasiks, 3 doses of Sildenafil, and 2 doses of Zantac. And from a couple visits to Dr. Pete, we were well aware that Sam's heart failure episode in December had really slowed down his weight gain. The boy, while long, was not a chunky baby. While other kids were spending time morphing into Michelin Men (and women), baby Sam was using every ounce of his energy to pump an overwhelmed heart.
Yet, for all of the struggles, it did remain that it really felt like Sam was on the road to recovery. A long road, still with perilous and sharp turns, but a road worth taking. As for the oxygen, it took me about a week to get used to a cannula free Sam. And for now...for that moment, it also felt okay to put the oxygen back on at night. It felt like were keeping that security blanket just within reach, hopefully allowing us to try to get some sleep.
Sleep...now that was something Sam wasn't willing to give to us just yet. And there was still that pesky little issue of still not officially knowing what had caused Sam's fistula in the first place. In the back of my mind, I was always expecting to never find that out...to view Sam as a fluke, and one in a billion. How very wrong I'd be.
Until next time.
You can all thank Amie Corey, one of Elisa's friends, for the return of the blog. She was the one that publicly asked, and her post was the one that pushed me to start writing out an outline of a 6-blog series to catch everyone up on little Sam. Yet, it was the hours leading up to the writing that finally opened my eyes to why I had stopped. For awhile, I had convinced myself that happy thoughts weren't as easy to write about (in a strange, twisted way). I also wondered if one of my chief reasons for the blog (public therapy) had faded, as Sam was no longer under a danger watch, and I could probably manage my personal feelings without long winded blog posts. It turns out, both were wrong. The writing stopped because I had hit an emotionally exhausted wall. For nearly 4 months, Elisa and I lived at the edge of sanity. Every day, we would wonder what could happen...heart failure, neurological problems, or worse (thanks, Dr. Doom). I'd thank the universe before I went to bed that we (and Sam) had survived through another day. I'd wake up every morning, worried at what the next 16 hours would hold. Dr. visits were worse, as some of the most "benign" trips would turn into horrific nightmares. So when we left Dr. Day's office in early January with a diagnosis of improvement, as well as a reduction in Sam's intervention, my mind decided it needed a break.
Blogging is, for the reader, a (hopefully) interesting story that they've never heard before. For me, it was reliving pain, agony, fear, happiness, hope, and the lack there of. As we drove home from Dr. Day's, there was silence...a type that hadn't existed before Sam was born. A few nights later, after I had blogged about our visit, I brought my computer to the bedroom, intent on writing more. I stared at the screen for 30 minutes. No words came...and that's saying something from me. It was as if I was tapped out. I needed to not write. I needed to revel in the fact that things weren't as dire as they had been. I needed to embrace the silent comfort of hope.
Over two months later, however, it's time to go back. And as I look all the way back, I'm surprised at how much has transpired. So while I thank you all for letting me have a blogging breather, it's time to catch you up. Let's get started.
When you last left us, Elisa had headed home with the go ahead to take Sam off of oxygen. It was more than we had hoped for. We were crossing our fingers for a reduction from 1/4 L to 1/8 L. Now, we were okay to take him off for 14 hours a day. We were thrilled. We were shocked. We were...scared. That night, we both sat and stared at the oxygen tank. Were we to start to tomorrow? How would he do? It was funny to me that for four months, all I hoped for was to be rid of the oxygen, and now, I was clinging to it as though it was the only think keeping Sam here. It turns out that, while annoying, the oxygen was more of a security blanket than an albatross. And being given license to remove the security blanket, we felt exposed and scared.
I wish I could say we were brave - that we pulled the oxygen off during the days and never looked back, but that's not how it went. No, we focused on small oxygen trials before bed. 20 minutes, 30 minutes, 40 minutes. And then the oxygen would return and we'd get through the day. We had a million reasons why the oxygen should stay - did he have a cold? Was he breathing harder? Was it a Wednesday? Every reason we could find was considered. No, we didn't just leave it on all the time. We'd have mini breaks from the oxygen. We got really brave one night and did it up to a nap. As it turns out, though, it wasn't us that finally pulled the plug. It was my Mom.
By this point, Elisa was back to work. So my Mom started coming back around to watch the kids. While her love for Sam was equal to ours, her dependance on the oxygen was not. So we would both leave to work, and my Mom would drop the oxygen. Sure, we'd come home worried that he was breathing harder, but he'd survived a day and we started wondering if he was doing a little better than we were letting ourselves believe. Logical me, when I stepped back, just saw oxygen levels (on the oximeter) of 90-93. It didn't seem high enough, even though he wasn't dropping into dangerous territory. But my Mom's actions gave us a little more confidence to see how Sam was going to do by himself. Before we knew it, Sam was off of oxygen during the days, save some time where his naps would worry us back to the cannula. But then, something amazing happened. Sam's oxygen readings started to change.
It was a Saturday after a long day without oxygen when I decided to plug Sam into the oximeter before turning on the switch at the tank. Usually, I'd get him set, turn on the o2, and then put him in his room. Tonight, I was curious as to how he held up during a very long day. The oximeter went on, the numbers popped up, and I was immediately sure that we were getting a bad reading. Sam was sitting at 97%...without oxygen. I was sure it was going to change. I was right, though not about the result. Suddenly, he was 98%...still awake, cooing at me from his crib. At 98% oxygen. I'm now convinced that Sam was ready from the moment Dr. Day said we could move forward. But this is a baby, and I sometimes forget that he is learning to do everything from scratch - including how to breathe. He needed to learn a bit - how to handle this thing on his own. I'm immensely grateful to my Mom for trusting Sam more than we were ready to trust him.
Like a light switch clicking on, though, Sam getting off of oxygen changed the world for the better. For four months, every day, were hooked to a tank at the top of our stairs. Sure, the cannula was on Sam, but it's cord extended around our own hands and feet. It grounded us in our house, and was a reminder of the struggles Sam had been through. You may be scoffing. I'm sure someone out there is thinking "I see people at the grocery store with a little oxygen tank all the time!" So do I. But switching to the small tank, always making sure Sam was hooked to a good flow, and the cord...the dratted cord...the number of times I tripped on that thing, I tell you.
But off oxygen, we truly did feel free. Elisa brought Sam to the boys swimming lessons one Saturday morning. I started a mini habit of bringing Sam to the grocery store every Sunday morning. We started going out to stores again. We started feeling normal. And while this was all wonderful (and it was), that wasn't the best part of the experience. No, that was when we finally let go of the cannula during the day. When I saw his face...his beautiful, cannula free face, I nearly lost it. He looked so different. Almost like someone who wears glasses all the time might look if they finally take them off. It's more subtle than you think, but you realize that your perception of them includes the glasses...or the cannula. It feels foreign...wonderful foreign...when it's gone.
As for the rest of Sam, I'll admit that the adjustment off of oxygen made us hesitant and worried elsewhere. We had tried to start him on rice cereal, but his stomach and intestines seemed to react - he seemed to have a constant belly ache and a bout of constipation. He was still on three doses of Lasiks, 3 doses of Sildenafil, and 2 doses of Zantac. And from a couple visits to Dr. Pete, we were well aware that Sam's heart failure episode in December had really slowed down his weight gain. The boy, while long, was not a chunky baby. While other kids were spending time morphing into Michelin Men (and women), baby Sam was using every ounce of his energy to pump an overwhelmed heart.
Yet, for all of the struggles, it did remain that it really felt like Sam was on the road to recovery. A long road, still with perilous and sharp turns, but a road worth taking. As for the oxygen, it took me about a week to get used to a cannula free Sam. And for now...for that moment, it also felt okay to put the oxygen back on at night. It felt like were keeping that security blanket just within reach, hopefully allowing us to try to get some sleep.
Sleep...now that was something Sam wasn't willing to give to us just yet. And there was still that pesky little issue of still not officially knowing what had caused Sam's fistula in the first place. In the back of my mind, I was always expecting to never find that out...to view Sam as a fluke, and one in a billion. How very wrong I'd be.
Until next time.
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