Okay, so maybe he was a Nazi and maybe he wasn't, but based on some research that he WAS, the doctor whose name stands for granulomatosis with polyangiitis, today's "preferred," non-epynomous name for what used to be called Wegener's Granulomatosis, I'll try to use the newer name, or GPA for short.
Whatever the hell it's called, it's hell.
Okay, I don't even have a firm diagnosis at this point--or, rather, I don't have the labs to back up the clinical diagnosis I was given by my Johns Hopkins rheumatologist, but given the fact that this is the place where the best specialists in most fields hang their pointy hats, I have to accept that she may well be right.
When I saw the kidney/vasculitis specialist for Wegener's a few months ago, she told me I needed to see the rheumatologist on an ongoing basis. I'd told her I wanted to cancel those appointments because my symptoms weren't bad at all--I don't enjoy these visits to Baltimore physicians and would like to minimize the pain in the ass of having to go there.
But today I just want to bitch a little about GPA or whichever vasculitis is apparently destroying my body one tiny bit of artery at a time. So, to update all my loyal fans (I think my only readers are what's known as pirate or vampire sites that click up your viewings though no one is actually viewing anything), these are the symptoms of GPA I've had and that are currently emerging.
I'm not going into all the other damned conditions I have; in one of my earlier episodes I list them, and it's just too depressing to go through again.
So here's the timeline, more or less, for JUST GPA in my life:
As long as I can remember, I've had nose problems. Fun things like nasty crusty bloody mucous inside that blocks my ability to breathe. I never thought much about it; I just suffered with it. Then, one day I figured out that my miserable headaches that made even my face hurt might be caused by sinus problems, and for a good many years I relied on Aleve Cold and Sinus, the best damned pill out there to make a gal feel better, but one I can no longer take because it's an NSAID.
An NSAID, if you don't know, is a certain type of pain/anti-inflammation medicine that is commonly found over the counter. Unfortunately, I can no longer turn to them because of my slowly (but more rapidly than normal) failing kidneys.
A few years ago, 2010 I believe, shortly after I was diagnosed with polycythemia (too many red blood cells; the cause of mine is still undetermined despite the best efforts of Hopkins Hematology, but I'm sure it's related to my continuing shortness of breath and poor oxygen saturation), I also had an MRI that showed sinus changes, specifically thickening of the ethmoid and maxillary sinuses.
These MRI findings are consistent with many years of inflammation. The Ear Nose and Throat (ENT) guy I saw in town noted that my nasal septum was perforated and I also had polycythemia; he said this could be due to Wegener's, the first time I'd heard of this horror story.
Actually, I've done the research (Mary Dell, MD -- Medical Detective! (and bona fide medical librarian), and an early case or two of Wegener's noted polycythemia, but it's not really that common.
This happens a lot, I fear--medical students read some of the seminal literature but while practicing often DO miss updates.
And this guy wasn't my favorite anyway; he'd attributed my dear friend's severe shortness of breath to exercise-induced asthma from training for a marathon--she'd run many in the past--and delayed the discovery of her ovarian cancer which killed her a year after diagnosis.
My primary care doc ran tests that she said showed I didn't have Wegener's--but I didn't know very much then and didn't realize there IS no blood test that can say for sure whether or not you have it. So I went another couple of years without pursuing it.
I think I remember when my nasal septum perforated. I had noticed a whistling sound and had looked inside my nose and saw a tiny hole in the divider between the nostrils--the septum. The whistling was very annoying--there was no way to stop it. I'd also noticed a weird feeling one day where something just shifted inside my nose, and I began snoring like a drunk sailor.
Fortunately, both the tiny hole (which is now apparently a huge hole) and the snoring went away, pretty much, but my breathing was never the same. [Though my polysomnography results for a different fun symptom noted "loud snoring." Ugh. Both my parents snored and sounded like buzz saws echoing in a cave.]
My nasal and sinus problems kept getting worse and worse, so I found a highly recommended ENT in another town with good recommendations. My PCP had already told me my nose was a mess after looking inside it with her light.
The ENT took one look, then sat down on his stool with a very solemn look, shook his head, and said, "Not good, Mary Dell, not good."
As a patient, not the happiest thing to hear. He also told me he'd be seeing a lot of him, but in fact he referred me on because he feared it was Wegener's ACK! GPA, so I went to my Hopkins, where I go for all my serious stuff except for a clinical study at NIH for my primary aldosteronism, a whole other topic, but one that also illustrates the failure of many physicians to adequately diagnose a serious disorder.]
So, sometime in early 2012, I was clinically diagnosed at Hopkins with GPA. "Clinically" means based on the doctor's judgment of clinical symptoms, but does not include confirmatory blood or other lab tests. I do not have ANCA antibodies in my blood, though, which something like 90-95 percent of GPA sufferers do have.
I won't get into what ANCA antibodies are--look them up on MedlinePlus or Mayo Clinic (not just any old Web site for your medical information!), but they can develop in GPA patients along the way even if they are negative initially.
Unfortunately, I've been researching like a monkey masturbates (credit to my friend Maggie), and I've read that ANCA-negative GPA has the most central nervous system (CNS--including the brain) involvement and the worst outcomes. I'll look for the citation and add it soon.
Since the disease seemed limited to my nose and sinuses, my Hopkins rheumatologist referred me to the Wegener's specialist nephrologist to check my kidneys, which can't really be checked without a biopsy, and started me on a daily dose of Bactrim, a heavy-duty antibiotic that is supposed to have prophylactic effects on the progression of the disease. Unfortunately, I ran out of it about three months ago, and since I'd cancelled my rheumatology appointment and my doctor had actually left Hopkins (she'd been a fellow, but her diagnosis had been confirmed by one of the regular physicians there as well) I'd never had it refilled.
I'm also supposed to be irrigating my nose and sinuses daily--THREE times a day!--which I've never done. I know I should, but I can barely get myself up and out of bed and to work, and even things like showers and brushing my hair have been increasingly difficult, so sticking my Sinugator (I kid you not) up my nose and turning on its jets is really low on my priority list.
Unfortunately, a couple of weeks ago I was touching the side of my nose and I felt an electric impulse, decidedly weird but not exactly painful, run from that spot just under my eye, down my face and across the top of my teeth. It happened once and I knew it wasn't right--it's not something that felt normal at all.
I immediately worried that the bony destruction of GPA that had started with my nasal septum might have spread. Then it happened again, and a third time, and a fourth, and a fifth, and now I avoid touching anywhere near that part of my face because it scares the bejesus out of me.
Wegener's is confused with and sometimes included in a group of conditions known as midline destructive diseases--and sometimes "fatal midline destructive disease."
It figures I'd get something like that--something that can eat away at your tissues and leave you with a giant hole where your face used to be. There's a photo of a poor guy in the medical literature--literally, his face is just a giant hole of sick-looking tissue surrounded by a little intact skin.
He didn't have health insurance so just ignored it--Ah, you compassionate Americans who don't believe the poor deserve proper medical treatment! There but for the grace of God go you!--and the poor guy died a week after the photo was taken.
I can't even imagine living like that. I'll try to find that citation, too, though I don't recommend anyone with GPA actually look at it--it's very frightening. Fortunately, there is treatment for those who actually have health insurance or who are rich enough not to need it but have it anyway, such as our fine legislators in Congress.
Treatment, however, is not fun, either. You start with an old-school chemotherapy agent, the kind that makes you really sick and makes your hair fall out. I don't even know if that's an option in my case; with my liver disease acting up (enzymes have been high lately) and my kidneys tanking (GFR--their filtration rate--falling precipitously over the past two years), chemo may not even be an option here, and I'll be damned if I let this disease eat my face away.
I'll take matters into my own hands first. I'm not even sure I'm up for the chemo and all the other horrible procedures in my future if this disease is spreading--along with all the other diagnoses I have and the fun each one of them promises; if it weren't for my two dear sons, I know I wouldn't go there at all. There are better ways out of this world.
So, of course, I had to put on my MD (Mary Dell, Medical Detective) hat and start sleuthing. I have no idea whether or not I have bony destruction other than in my septum, but it feels as if something is missing when I touch that spot near my nose, and I seem to be directly touching the nerve instead of the wall of my sinus which is supposed to be there.
I tracked down the nerve--it's the maxillary nerve, one of the three branches of the trigeminal nerve, a big honker that comes out of your brain where?? You guessed it, sports fans! Right around that pesky hypothalamus. (For those of you who don't know why I'm all about the hypothalamus, you'll have to read recent posts.)
As I think I mentioned, the maxillary bone between the nose and the cheek is often the first bone after the nasal septum to get destroyed by Wegener's, so now I'm beginning to believe that I might actually have Wegener's or, rather, GPA.
I called my Wegener's specialist--the kidney one--because she's a vasculitis expert and figured this must be some kind of vasculitis thing. [I would later learn it is called "neuropathy"--so, you se AND a recent MRI of my brain showed periventricular white matter changes consistent with vasculitis-type damage.
AND I've been having some weird things happening with my expressive language (a type of aphasia)--more than a little disconcerting for a former professor and someone who loves words--I will substitute a related word for one that I mean even though I don't mean to; usually I realize it right away but not always. That's another future episode. Some of the word substitutions are actually quite funny, but the underlying brain damage they suggest not so much so.
So, this wonderful doctor fit me in, and I'll be seeing her next week. Stay tuned for the latest on this oh-so-wonderful malady--one that makes our heads QUITE ugly, referring back to my title.
AND, referring back to my title, I'll just say this: From here on out I'm not using the term Wegener's, though I'll keep it in my labels so that sufferers out there might find a kindred soul.
Ever since I picked up a picture book at the local Dart Drug when I was about 12 years old and saw photos of newly freed Nazi concentration camp victims, starved and emaciated in their broadly striped, filthy prison uniforms, and I first understood that true evil exists in this world in a way I never, ever could have imagined, I have hated Nazis and any kind of jingoism, exclusivism, apartheid (God bless you, Nelson Mandela, and may you rest in peace at last, the peace you so dearly deserve), genocide, et al.
In fact, I hate any kind of cruelty or unfairness. It's one of the saddest things I have to face now that my life is sort of on the decline--the fact that these evil things exist and are probably endemic to the human condition.
Peace on earth--what a beautiful dream today's most vocal Jesus followers are working hard to ensure doesn't happen in our lifetimes--and a dream I fear may, in fact, never, ever happen. People are just too damned selfish and too damned cruel.
Leaving this world, I sometimes think, won't be such a bad thing.
Whatever the hell it's called, it's hell.
Okay, I don't even have a firm diagnosis at this point--or, rather, I don't have the labs to back up the clinical diagnosis I was given by my Johns Hopkins rheumatologist, but given the fact that this is the place where the best specialists in most fields hang their pointy hats, I have to accept that she may well be right.
When I saw the kidney/vasculitis specialist for Wegener's a few months ago, she told me I needed to see the rheumatologist on an ongoing basis. I'd told her I wanted to cancel those appointments because my symptoms weren't bad at all--I don't enjoy these visits to Baltimore physicians and would like to minimize the pain in the ass of having to go there.
But today I just want to bitch a little about GPA or whichever vasculitis is apparently destroying my body one tiny bit of artery at a time. So, to update all my loyal fans (I think my only readers are what's known as pirate or vampire sites that click up your viewings though no one is actually viewing anything), these are the symptoms of GPA I've had and that are currently emerging.
I'm not going into all the other damned conditions I have; in one of my earlier episodes I list them, and it's just too depressing to go through again.
So here's the timeline, more or less, for JUST GPA in my life:
As long as I can remember, I've had nose problems. Fun things like nasty crusty bloody mucous inside that blocks my ability to breathe. I never thought much about it; I just suffered with it. Then, one day I figured out that my miserable headaches that made even my face hurt might be caused by sinus problems, and for a good many years I relied on Aleve Cold and Sinus, the best damned pill out there to make a gal feel better, but one I can no longer take because it's an NSAID.
An NSAID, if you don't know, is a certain type of pain/anti-inflammation medicine that is commonly found over the counter. Unfortunately, I can no longer turn to them because of my slowly (but more rapidly than normal) failing kidneys.
A few years ago, 2010 I believe, shortly after I was diagnosed with polycythemia (too many red blood cells; the cause of mine is still undetermined despite the best efforts of Hopkins Hematology, but I'm sure it's related to my continuing shortness of breath and poor oxygen saturation), I also had an MRI that showed sinus changes, specifically thickening of the ethmoid and maxillary sinuses.
These MRI findings are consistent with many years of inflammation. The Ear Nose and Throat (ENT) guy I saw in town noted that my nasal septum was perforated and I also had polycythemia; he said this could be due to Wegener's, the first time I'd heard of this horror story.
Actually, I've done the research (Mary Dell, MD -- Medical Detective! (and bona fide medical librarian), and an early case or two of Wegener's noted polycythemia, but it's not really that common.
This happens a lot, I fear--medical students read some of the seminal literature but while practicing often DO miss updates.
And this guy wasn't my favorite anyway; he'd attributed my dear friend's severe shortness of breath to exercise-induced asthma from training for a marathon--she'd run many in the past--and delayed the discovery of her ovarian cancer which killed her a year after diagnosis.
My primary care doc ran tests that she said showed I didn't have Wegener's--but I didn't know very much then and didn't realize there IS no blood test that can say for sure whether or not you have it. So I went another couple of years without pursuing it.
I think I remember when my nasal septum perforated. I had noticed a whistling sound and had looked inside my nose and saw a tiny hole in the divider between the nostrils--the septum. The whistling was very annoying--there was no way to stop it. I'd also noticed a weird feeling one day where something just shifted inside my nose, and I began snoring like a drunk sailor.
Fortunately, both the tiny hole (which is now apparently a huge hole) and the snoring went away, pretty much, but my breathing was never the same. [Though my polysomnography results for a different fun symptom noted "loud snoring." Ugh. Both my parents snored and sounded like buzz saws echoing in a cave.]
My nasal and sinus problems kept getting worse and worse, so I found a highly recommended ENT in another town with good recommendations. My PCP had already told me my nose was a mess after looking inside it with her light.
The ENT took one look, then sat down on his stool with a very solemn look, shook his head, and said, "Not good, Mary Dell, not good."
As a patient, not the happiest thing to hear. He also told me he'd be seeing a lot of him, but in fact he referred me on because he feared it was Wegener's ACK! GPA, so I went to my Hopkins, where I go for all my serious stuff except for a clinical study at NIH for my primary aldosteronism, a whole other topic, but one that also illustrates the failure of many physicians to adequately diagnose a serious disorder.]
So, sometime in early 2012, I was clinically diagnosed at Hopkins with GPA. "Clinically" means based on the doctor's judgment of clinical symptoms, but does not include confirmatory blood or other lab tests. I do not have ANCA antibodies in my blood, though, which something like 90-95 percent of GPA sufferers do have.
I won't get into what ANCA antibodies are--look them up on MedlinePlus or Mayo Clinic (not just any old Web site for your medical information!), but they can develop in GPA patients along the way even if they are negative initially.
Unfortunately, I've been researching like a monkey masturbates (credit to my friend Maggie), and I've read that ANCA-negative GPA has the most central nervous system (CNS--including the brain) involvement and the worst outcomes. I'll look for the citation and add it soon.
Since the disease seemed limited to my nose and sinuses, my Hopkins rheumatologist referred me to the Wegener's specialist nephrologist to check my kidneys, which can't really be checked without a biopsy, and started me on a daily dose of Bactrim, a heavy-duty antibiotic that is supposed to have prophylactic effects on the progression of the disease. Unfortunately, I ran out of it about three months ago, and since I'd cancelled my rheumatology appointment and my doctor had actually left Hopkins (she'd been a fellow, but her diagnosis had been confirmed by one of the regular physicians there as well) I'd never had it refilled.
I'm also supposed to be irrigating my nose and sinuses daily--THREE times a day!--which I've never done. I know I should, but I can barely get myself up and out of bed and to work, and even things like showers and brushing my hair have been increasingly difficult, so sticking my Sinugator (I kid you not) up my nose and turning on its jets is really low on my priority list.
Unfortunately, a couple of weeks ago I was touching the side of my nose and I felt an electric impulse, decidedly weird but not exactly painful, run from that spot just under my eye, down my face and across the top of my teeth. It happened once and I knew it wasn't right--it's not something that felt normal at all.
I immediately worried that the bony destruction of GPA that had started with my nasal septum might have spread. Then it happened again, and a third time, and a fourth, and a fifth, and now I avoid touching anywhere near that part of my face because it scares the bejesus out of me.
Wegener's is confused with and sometimes included in a group of conditions known as midline destructive diseases--and sometimes "fatal midline destructive disease."
It figures I'd get something like that--something that can eat away at your tissues and leave you with a giant hole where your face used to be. There's a photo of a poor guy in the medical literature--literally, his face is just a giant hole of sick-looking tissue surrounded by a little intact skin.
He didn't have health insurance so just ignored it--Ah, you compassionate Americans who don't believe the poor deserve proper medical treatment! There but for the grace of God go you!--and the poor guy died a week after the photo was taken.
I can't even imagine living like that. I'll try to find that citation, too, though I don't recommend anyone with GPA actually look at it--it's very frightening. Fortunately, there is treatment for those who actually have health insurance or who are rich enough not to need it but have it anyway, such as our fine legislators in Congress.
Treatment, however, is not fun, either. You start with an old-school chemotherapy agent, the kind that makes you really sick and makes your hair fall out. I don't even know if that's an option in my case; with my liver disease acting up (enzymes have been high lately) and my kidneys tanking (GFR--their filtration rate--falling precipitously over the past two years), chemo may not even be an option here, and I'll be damned if I let this disease eat my face away.
I'll take matters into my own hands first. I'm not even sure I'm up for the chemo and all the other horrible procedures in my future if this disease is spreading--along with all the other diagnoses I have and the fun each one of them promises; if it weren't for my two dear sons, I know I wouldn't go there at all. There are better ways out of this world.
So, of course, I had to put on my MD (Mary Dell, Medical Detective) hat and start sleuthing. I have no idea whether or not I have bony destruction other than in my septum, but it feels as if something is missing when I touch that spot near my nose, and I seem to be directly touching the nerve instead of the wall of my sinus which is supposed to be there.
I tracked down the nerve--it's the maxillary nerve, one of the three branches of the trigeminal nerve, a big honker that comes out of your brain where?? You guessed it, sports fans! Right around that pesky hypothalamus. (For those of you who don't know why I'm all about the hypothalamus, you'll have to read recent posts.)
As I think I mentioned, the maxillary bone between the nose and the cheek is often the first bone after the nasal septum to get destroyed by Wegener's, so now I'm beginning to believe that I might actually have Wegener's or, rather, GPA.
I called my Wegener's specialist--the kidney one--because she's a vasculitis expert and figured this must be some kind of vasculitis thing. [I would later learn it is called "neuropathy"--so, you se AND a recent MRI of my brain showed periventricular white matter changes consistent with vasculitis-type damage.
AND I've been having some weird things happening with my expressive language (a type of aphasia)--more than a little disconcerting for a former professor and someone who loves words--I will substitute a related word for one that I mean even though I don't mean to; usually I realize it right away but not always. That's another future episode. Some of the word substitutions are actually quite funny, but the underlying brain damage they suggest not so much so.
So, this wonderful doctor fit me in, and I'll be seeing her next week. Stay tuned for the latest on this oh-so-wonderful malady--one that makes our heads QUITE ugly, referring back to my title.
AND, referring back to my title, I'll just say this: From here on out I'm not using the term Wegener's, though I'll keep it in my labels so that sufferers out there might find a kindred soul.
Ever since I picked up a picture book at the local Dart Drug when I was about 12 years old and saw photos of newly freed Nazi concentration camp victims, starved and emaciated in their broadly striped, filthy prison uniforms, and I first understood that true evil exists in this world in a way I never, ever could have imagined, I have hated Nazis and any kind of jingoism, exclusivism, apartheid (God bless you, Nelson Mandela, and may you rest in peace at last, the peace you so dearly deserve), genocide, et al.
In fact, I hate any kind of cruelty or unfairness. It's one of the saddest things I have to face now that my life is sort of on the decline--the fact that these evil things exist and are probably endemic to the human condition.
Peace on earth--what a beautiful dream today's most vocal Jesus followers are working hard to ensure doesn't happen in our lifetimes--and a dream I fear may, in fact, never, ever happen. People are just too damned selfish and too damned cruel.
Leaving this world, I sometimes think, won't be such a bad thing.
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