In the pilot of The Mary Dell Show, I explained how the name of the show is based on my brother's recording me as a kid on his reel-to-reel tape player as he interviewed me about newsworthy topics such as how to catch lightning bugs.
When seeing that gigantic tape player on the foyer floor as my parents said good-bye to my big brother on his way to Georgia Tech, the impact of his leaving hit me for the first time. That tape player defined my brother, and it defined the relationship I'd had with him my whole life.
That would mean no more Mary Dell shows, no more Walter Kronkite-depth interviews on the workings of my then eight-year-old mind.
And I mentioned that as my first heartbreak.
My second came when my baby was born two months early and was given a 50/50 chance for life in his first 24 hours, and a third came about nine months later when I learned he must've had a stroke while fighting for his life in the Isolette for 26 days, and his entire left side was partially paralyzed. And a simultanous one when learning, at the same time, that he had craniosynostosis, a malformation affecting the skull bones, which explained the odd shape his had developed into by then. By the time he was a year old, he'd had two neurosurgeries to chisel open the sutures between those bones, which had fused prematurely and forced his brain to grow in a long shape from front to back.
In a flash of nature's ironic fun with us, Jason had a third surgery to add plastic to those same sutures because, having been forced open with hammer and chisel, they refused to close again, as they do in most kids' heads well before then. And in yet another flash, nature took away his hair when he was in his early 20's, leaving his skull, with its scars and bone ridges exposed and no camouflage at all for it shape which, thankfully, had greatly improved after his surgeries but still draws attention until one gets to know the person he is, and of course it doesn't matter at all then. Nevertheless, life has been tough for him, and appearance matters in this superficial world. He's fortunate, though, that he is a very good looking guy, so any flaws in his appearance quickly disappear once you get to know him, as is true for all persons with unusual physical characteristics.
[N.B. June 27, 2014. In an interesting--no, groundbreaking, for me--twist, Jason's early baldness may have been for the exact same reason he was born with craniosynostosis--a genetic condition that I'm convinced runs in my family called myotonic dystrophy. The two conditions were just recently connected genetically by scientists. I'd been sure since this happened that there was a reason for my pregnancy problems and Jason's issues, something that would explain both--I was not satisfied with the explanation given at the time that his craniosynostosis was the result of a random gene mutation. Of course, doctors knew so much less than about our genes. The Genome Project was merely a dream then. It's taken me 35 years and a whole lot doctors' appointments, tests, medical advancements due to research, and personal sleuthing to finally come, I believe, to the answer. I'll know for sure soon, since a Hopkins neurologist has reviewed and agreed to take my case, and I'll see her on July 9.]
Oh, there's more, of course, but I as one critic in a poetry workshop told me after I'd read one of my poems aloud, "Sometimes too much is too much."
I have to admit the deepest, most shocking heartbreak came when my dad died while taking a shower the night after he and my mom had gone dancing while staying at their beach-side "cottage." Actually, it was a trailer, which always kind of embarrassed us siblings who'd sung along to Jimmy Buffett, but it was only a few blocks from the ocean and they did own the land. We really should have kept that thing in the family after all ....
And when my mom went through breast, then liver and pancreatic cancer a few years after that and died before I was thirty, well, heartbreak hardly expresses it. Psychic break, internal fissure from head to toe. More of a splintering, I suppose, as I'd already cracked in two with my dad's death.
And so much heartbreak does take its toll.
As does 13 years of out-of-control hypertension, endless medicine changes, and the havoc numbers close to the malignant range over many years does with one's heart.
Have I mentioned I had a hard time breathing in Europe this past Christmas, when I stayed with Sabine for about a month while she was dying?
No, I don't think I have. Obviously, that will be the topic of many upcoming episodes.
Until then, I'll just mention what the round doctors' visits and medical tests seems to have revealed. Not that the Wegener's is gone, I don't think--and happily I will see a nephrologist at Hopkins very soon who specializes in Wegener's and other renal vasculitis, so I'll know more then about my kidneys,. Tests do show that their function is--no pun intended--going down the tubes.
(This, by the way, is my latest theory about dreams: Our dreams are our lives and memories filtered (literally, perhaps in the kidneys!! ha) through our bodies' cells and tissues, and the messages they are sending have to do with our internal, er, architecture. And I thought of this recently after wondering why the heck I have so many dreams in bathrooms--and then it dawned on me that maybe my "plumbing" was telling me I needed to pay attention to it. And I have other examples to explore with you, viewers, on another episode.)
So, three cardiology tests had weird results, and when I research them they all seem to point to heart failure. That would certainly correlate with my inability to climb stairs without my legs wanting to give out from under me and me doubling over to try to catch my breath.
Sometimes things are so simple.
I'd done so much research I was convinced I have Chiari I, a compression of the brain stem that can be present from birth. Actually, I'm not convinced I don't have that as well, especially given craniosynostosis is apparently related to it in some way, and doctors could never explain why my firstborn had this malady. Spontaneous mutation, is all they could guess.
But considering I've had numerous health issues throughout my life, nearly all of which correlate with Chiari, I'm still thinking we can blame all of this bullshit through the years on a squooshed brain stem.
Update 6/27/14: As mentioned in my earlier update, the culprit now clearly seems to be myotonic dystrophy which also originates in the brain and affects the respiratory system and other autonomic functions (heart rate, wake/sleep cycle, etc.) Some research suggests the disease may also affect processes in the brain stem. I was sure whatever it was related to my shortness of breath and sleep problems, and research attributes these problems in myotonic dystrophites to muscle weakness in the diaphragm, weak cough due to smooth muscle weakness leading to fluids accumulating in the airways, leading then to hypoperfusion (lack of oxygen in the blood) through alveolars in the lungs filling with fluid, leading to hypercapnia (too much carbon dioxide in the blood, which I was shown to have when my job called an ambulance one day when I was having breathing troubles), which leads to--Guess what? Polycythemia. When the blood lacks oxygen, the body starts pumping out red blood cells, those little oxygen carriers. Which, at the end of a diagnostic maze lasting most of my adult life, are all common in persons with myotonic dystrophy. And I had to figure it out myself and more or less beg my skeptical primary care provider for a referral to a neurologist, and she even put "PR" on said referral, which must have meant "Patient Request."
So, you see, knowing how to do medical research (literature, I mean, not laboratory) is a skill that just might save your life, or at least help you step off the diagnostic roller-coaster at last. Knowing how to research the medical literature, however, is not the same as doing a Google search for a health condition. As I said earlier, it's taken me a lifetime to figure this out, and I am a Master's prepared medical librarian, a dying breed in these days of cookie-cutter medicine a la UpToDate--a wonderful tool, don't get me wrong, but not the end all and be all. Hell, if one of my doctors would just read UpToDate on my symptoms, they'd eventually come to myotonic dystrophy--but that's not how I found it. Someday I'll have to develop a course on doing your own medical research and put it on YouTube.
At this point, I guess the question is somewhat moot. If I'm in heart and kidney failure, I don't think I'm gonna be around for too terribly much longer. The damage has been done. My heart is sort of just sitting there in the back, refusing to pull its weight.
Slacker.
Guess that explains why I'm so tired all the time and sometimes sleep for 17, 20, 24 hours at a time--that lazy heart is just plumb exhausted.
But, hey, my fiance and I learned early on that we both loved the Steve Earle song "Fearless Heart," and I've always had one of those. So maybe this isn't a death sentence. And, as Dick so lovingly informed me when I told him, "We're all dying."
He's right, of course. Just another typical day.
__________________
Oh, my gosh, Steve Earle is so amazing and so cute ("hot," I suppose, is today's parlance)--do yourself a favor and clock on the link for the song and then just spend some time YouTubing Steve Earle. It'll be well worth your time! His first album (and all his albums) is one of the best of any kind ever made--WHAT a songwriter, singer, and human being, even if he apparently can be obnoxious. I hope he's still sober and happy with his new wife and baby--of course, that baby is probably 15 by now. the way time is flying!
When seeing that gigantic tape player on the foyer floor as my parents said good-bye to my big brother on his way to Georgia Tech, the impact of his leaving hit me for the first time. That tape player defined my brother, and it defined the relationship I'd had with him my whole life.
That would mean no more Mary Dell shows, no more Walter Kronkite-depth interviews on the workings of my then eight-year-old mind.
And I mentioned that as my first heartbreak.
My second came when my baby was born two months early and was given a 50/50 chance for life in his first 24 hours, and a third came about nine months later when I learned he must've had a stroke while fighting for his life in the Isolette for 26 days, and his entire left side was partially paralyzed. And a simultanous one when learning, at the same time, that he had craniosynostosis, a malformation affecting the skull bones, which explained the odd shape his had developed into by then. By the time he was a year old, he'd had two neurosurgeries to chisel open the sutures between those bones, which had fused prematurely and forced his brain to grow in a long shape from front to back.
In a flash of nature's ironic fun with us, Jason had a third surgery to add plastic to those same sutures because, having been forced open with hammer and chisel, they refused to close again, as they do in most kids' heads well before then. And in yet another flash, nature took away his hair when he was in his early 20's, leaving his skull, with its scars and bone ridges exposed and no camouflage at all for it shape which, thankfully, had greatly improved after his surgeries but still draws attention until one gets to know the person he is, and of course it doesn't matter at all then. Nevertheless, life has been tough for him, and appearance matters in this superficial world. He's fortunate, though, that he is a very good looking guy, so any flaws in his appearance quickly disappear once you get to know him, as is true for all persons with unusual physical characteristics.
[N.B. June 27, 2014. In an interesting--no, groundbreaking, for me--twist, Jason's early baldness may have been for the exact same reason he was born with craniosynostosis--a genetic condition that I'm convinced runs in my family called myotonic dystrophy. The two conditions were just recently connected genetically by scientists. I'd been sure since this happened that there was a reason for my pregnancy problems and Jason's issues, something that would explain both--I was not satisfied with the explanation given at the time that his craniosynostosis was the result of a random gene mutation. Of course, doctors knew so much less than about our genes. The Genome Project was merely a dream then. It's taken me 35 years and a whole lot doctors' appointments, tests, medical advancements due to research, and personal sleuthing to finally come, I believe, to the answer. I'll know for sure soon, since a Hopkins neurologist has reviewed and agreed to take my case, and I'll see her on July 9.]
Oh, there's more, of course, but I as one critic in a poetry workshop told me after I'd read one of my poems aloud, "Sometimes too much is too much."
I have to admit the deepest, most shocking heartbreak came when my dad died while taking a shower the night after he and my mom had gone dancing while staying at their beach-side "cottage." Actually, it was a trailer, which always kind of embarrassed us siblings who'd sung along to Jimmy Buffett, but it was only a few blocks from the ocean and they did own the land. We really should have kept that thing in the family after all ....
And when my mom went through breast, then liver and pancreatic cancer a few years after that and died before I was thirty, well, heartbreak hardly expresses it. Psychic break, internal fissure from head to toe. More of a splintering, I suppose, as I'd already cracked in two with my dad's death.
And so much heartbreak does take its toll.
As does 13 years of out-of-control hypertension, endless medicine changes, and the havoc numbers close to the malignant range over many years does with one's heart.
Have I mentioned I had a hard time breathing in Europe this past Christmas, when I stayed with Sabine for about a month while she was dying?
No, I don't think I have. Obviously, that will be the topic of many upcoming episodes.
Until then, I'll just mention what the round doctors' visits and medical tests seems to have revealed. Not that the Wegener's is gone, I don't think--and happily I will see a nephrologist at Hopkins very soon who specializes in Wegener's and other renal vasculitis, so I'll know more then about my kidneys,. Tests do show that their function is--no pun intended--going down the tubes.
(This, by the way, is my latest theory about dreams: Our dreams are our lives and memories filtered (literally, perhaps in the kidneys!! ha) through our bodies' cells and tissues, and the messages they are sending have to do with our internal, er, architecture. And I thought of this recently after wondering why the heck I have so many dreams in bathrooms--and then it dawned on me that maybe my "plumbing" was telling me I needed to pay attention to it. And I have other examples to explore with you, viewers, on another episode.)
So, three cardiology tests had weird results, and when I research them they all seem to point to heart failure. That would certainly correlate with my inability to climb stairs without my legs wanting to give out from under me and me doubling over to try to catch my breath.
Sometimes things are so simple.
I'd done so much research I was convinced I have Chiari I, a compression of the brain stem that can be present from birth. Actually, I'm not convinced I don't have that as well, especially given craniosynostosis is apparently related to it in some way, and doctors could never explain why my firstborn had this malady. Spontaneous mutation, is all they could guess.
But considering I've had numerous health issues throughout my life, nearly all of which correlate with Chiari, I'm still thinking we can blame all of this bullshit through the years on a squooshed brain stem.
Update 6/27/14: As mentioned in my earlier update, the culprit now clearly seems to be myotonic dystrophy which also originates in the brain and affects the respiratory system and other autonomic functions (heart rate, wake/sleep cycle, etc.) Some research suggests the disease may also affect processes in the brain stem. I was sure whatever it was related to my shortness of breath and sleep problems, and research attributes these problems in myotonic dystrophites to muscle weakness in the diaphragm, weak cough due to smooth muscle weakness leading to fluids accumulating in the airways, leading then to hypoperfusion (lack of oxygen in the blood) through alveolars in the lungs filling with fluid, leading to hypercapnia (too much carbon dioxide in the blood, which I was shown to have when my job called an ambulance one day when I was having breathing troubles), which leads to--Guess what? Polycythemia. When the blood lacks oxygen, the body starts pumping out red blood cells, those little oxygen carriers. Which, at the end of a diagnostic maze lasting most of my adult life, are all common in persons with myotonic dystrophy. And I had to figure it out myself and more or less beg my skeptical primary care provider for a referral to a neurologist, and she even put "PR" on said referral, which must have meant "Patient Request."
So, you see, knowing how to do medical research (literature, I mean, not laboratory) is a skill that just might save your life, or at least help you step off the diagnostic roller-coaster at last. Knowing how to research the medical literature, however, is not the same as doing a Google search for a health condition. As I said earlier, it's taken me a lifetime to figure this out, and I am a Master's prepared medical librarian, a dying breed in these days of cookie-cutter medicine a la UpToDate--a wonderful tool, don't get me wrong, but not the end all and be all. Hell, if one of my doctors would just read UpToDate on my symptoms, they'd eventually come to myotonic dystrophy--but that's not how I found it. Someday I'll have to develop a course on doing your own medical research and put it on YouTube.
At this point, I guess the question is somewhat moot. If I'm in heart and kidney failure, I don't think I'm gonna be around for too terribly much longer. The damage has been done. My heart is sort of just sitting there in the back, refusing to pull its weight.
Slacker.
Guess that explains why I'm so tired all the time and sometimes sleep for 17, 20, 24 hours at a time--that lazy heart is just plumb exhausted.
But, hey, my fiance and I learned early on that we both loved the Steve Earle song "Fearless Heart," and I've always had one of those. So maybe this isn't a death sentence. And, as Dick so lovingly informed me when I told him, "We're all dying."
He's right, of course. Just another typical day.
__________________
Oh, my gosh, Steve Earle is so amazing and so cute ("hot," I suppose, is today's parlance)--do yourself a favor and clock on the link for the song and then just spend some time YouTubing Steve Earle. It'll be well worth your time! His first album (and all his albums) is one of the best of any kind ever made--WHAT a songwriter, singer, and human being, even if he apparently can be obnoxious. I hope he's still sober and happy with his new wife and baby--of course, that baby is probably 15 by now. the way time is flying!
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