The wolves are in pursuit
Near the beginning of White Fang by Jack London, there is an extended scene where a man is alone by himself in the wilderness, pursued by a pack of wolves. He has settled down for the night and built a fire, which is the only thing keeping the starving wolves at bay. But he knows it is simply a matter of time before the fire dies down... and when it does, he will no longer have burning pieces of wood to thrust at the wolves for protection, and then they will kill him.
I read White Fang a lot when I was a kid. That image has stuck with me into adulthood and somewhere along the way, it became my mental analogy for the kind of anxiety you experience when you wake up in the middle of the night, everyone else sleeping, the room dark, and nothing to distract you from your fear.
It is like going to sleep next to a raging fire, and I know the wolves are there, but they are far away. But when I wake up the fire has burned down and I feel a sudden cramp of fear; and then I look up and see the wolves, packed shoulder-to-shoulder, staring at me from only a short distance away. I can see the firelight gleaming in their eyes and smell the gamy odor of their fur. They want to consume me, and I struggle desperately, scrabbling around myself for any little overlooked sticks that I can throw on the fire in my pathetic attempt to keep them at bay.
And I tell the wolves tonight, when I wake up around 4am, "I do not have Marfan's syndrome. I am tall but not unusually tall; my eyes are fine."
The wolves smile at me; the firelight glints off their saliva-slicked fangs. They say, Out of the long list of symptoms, those are the only ones you can find that do not fit. How do those stack up against all the many symptoms that do fit?
I say, "The last echocardiogram was taken a year or so after Moo's birth, and showed no significant aortic dilation. So even if I have Marfan's, I've already made it through one pregnancy just fine."
They say, That was the first ECG that was done. How do you know that there wasn't dilation? Perhaps if you'd had an ECG done two years prior, you would have seen a difference. And the fact that you made it through one does not guarantee that you would make it through another.
I say, "I can't die, I have a small child."
They laugh with tongues lolling and say, An appeal to sympathy never saved anyone from aortic dissection. You think your weakening heart cares that you have a small child?
I say, "If I have this, I understand it can be an early death sentence. Just let me live until I'm 45; my children would be 21 and 18 then."
The wolves tell me, Neither we nor you have any control over when you will die. If you are going to die early, none of us can change your fate.
And I lie there knowing the truth of this. I watch the fire flickering and I have to acknowledge the things that I try to escape away during the day; that people with Marfan's have a high risk of sudden death, and it can come very early. That if I have this syndrome, then I have a significantly increased risk of dying while my children are still little and need me. That I may be in danger, right this very moment, and I have no way of knowing what is going on inside me until I can get an appointment with somebody who has a machine that can see inside my body.
I acknowledge the other things that go along with this; that it is likely, based on the significant overlap between the major symptoms of Marfan's and the symptoms that I have (and most of these are fairly specialized symptoms), that I have this syndrome. That this means, if it is confirmed, that I will be living the rest of my life (however long that may be) in fear, seeing doctors and getting tests frequently, always wondering if today is the day my heart will give out. There is medicine to slow the degeneration of Marfan's; there is no medicine or treatment to stop the degeneration.
If this is confirmed, it means I will be going through the rest of my life trying to keep my heart rate down; I will have to avoid all contact sports and anything else that could place my heart at risk. Exercise will be limited to very gentle activities like walking. There will not be games of chase or tackle with my kids. I will never get to teach my son how to rock climb. I will be the mother made of china, fragile and constantly trying to avoid anything that could cause me to shatter.
And worst of all; the knowledge that it is genetic and I have a 50% chance of giving it to any of my children. If it's confirmed that I have this, then it's a coin toss as to whether I have given a life-threatening syndrome to my son Gavin. It's a coin toss as to whether I have given a life-threatening syndrome to the son I carry within me. How will I be lucky enough to win the coin toss twice? Make no mistake about this; if it turns out I have Marfan's, if I had known about this before I got pregnant, I would not have had my children. 50% is far too high a risk to take with someone else's life.
The wolves tell me, You already know you gave Gavin your pectus excavatum. That's a major symptom of Marfan's.
And all I can do is reply miserably, "I know. Believe me, I know."
I read White Fang a lot when I was a kid. That image has stuck with me into adulthood and somewhere along the way, it became my mental analogy for the kind of anxiety you experience when you wake up in the middle of the night, everyone else sleeping, the room dark, and nothing to distract you from your fear.
It is like going to sleep next to a raging fire, and I know the wolves are there, but they are far away. But when I wake up the fire has burned down and I feel a sudden cramp of fear; and then I look up and see the wolves, packed shoulder-to-shoulder, staring at me from only a short distance away. I can see the firelight gleaming in their eyes and smell the gamy odor of their fur. They want to consume me, and I struggle desperately, scrabbling around myself for any little overlooked sticks that I can throw on the fire in my pathetic attempt to keep them at bay.
And I tell the wolves tonight, when I wake up around 4am, "I do not have Marfan's syndrome. I am tall but not unusually tall; my eyes are fine."
The wolves smile at me; the firelight glints off their saliva-slicked fangs. They say, Out of the long list of symptoms, those are the only ones you can find that do not fit. How do those stack up against all the many symptoms that do fit?
I say, "The last echocardiogram was taken a year or so after Moo's birth, and showed no significant aortic dilation. So even if I have Marfan's, I've already made it through one pregnancy just fine."
They say, That was the first ECG that was done. How do you know that there wasn't dilation? Perhaps if you'd had an ECG done two years prior, you would have seen a difference. And the fact that you made it through one does not guarantee that you would make it through another.
I say, "I can't die, I have a small child."
They laugh with tongues lolling and say, An appeal to sympathy never saved anyone from aortic dissection. You think your weakening heart cares that you have a small child?
I say, "If I have this, I understand it can be an early death sentence. Just let me live until I'm 45; my children would be 21 and 18 then."
The wolves tell me, Neither we nor you have any control over when you will die. If you are going to die early, none of us can change your fate.
And I lie there knowing the truth of this. I watch the fire flickering and I have to acknowledge the things that I try to escape away during the day; that people with Marfan's have a high risk of sudden death, and it can come very early. That if I have this syndrome, then I have a significantly increased risk of dying while my children are still little and need me. That I may be in danger, right this very moment, and I have no way of knowing what is going on inside me until I can get an appointment with somebody who has a machine that can see inside my body.
I acknowledge the other things that go along with this; that it is likely, based on the significant overlap between the major symptoms of Marfan's and the symptoms that I have (and most of these are fairly specialized symptoms), that I have this syndrome. That this means, if it is confirmed, that I will be living the rest of my life (however long that may be) in fear, seeing doctors and getting tests frequently, always wondering if today is the day my heart will give out. There is medicine to slow the degeneration of Marfan's; there is no medicine or treatment to stop the degeneration.
If this is confirmed, it means I will be going through the rest of my life trying to keep my heart rate down; I will have to avoid all contact sports and anything else that could place my heart at risk. Exercise will be limited to very gentle activities like walking. There will not be games of chase or tackle with my kids. I will never get to teach my son how to rock climb. I will be the mother made of china, fragile and constantly trying to avoid anything that could cause me to shatter.
And worst of all; the knowledge that it is genetic and I have a 50% chance of giving it to any of my children. If it's confirmed that I have this, then it's a coin toss as to whether I have given a life-threatening syndrome to my son Gavin. It's a coin toss as to whether I have given a life-threatening syndrome to the son I carry within me. How will I be lucky enough to win the coin toss twice? Make no mistake about this; if it turns out I have Marfan's, if I had known about this before I got pregnant, I would not have had my children. 50% is far too high a risk to take with someone else's life.
The wolves tell me, You already know you gave Gavin your pectus excavatum. That's a major symptom of Marfan's.
And all I can do is reply miserably, "I know. Believe me, I know."