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Thread: Recapitualtion

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    Default Recapitualtion

    [Note: A few days ago, drz gave us a moving personal narration. In a private message, he asked me to say a few words about myself. I did so. He suggested that I post my story for the whole forum. Now, on my very first post (http://www.wegeners-granulomatosis.c...conscript.html), I had given a synopsis, so was reluctant to repeat myself. However, we have since had many new members. On the assumption that personal narratives are sometimes helpful, I post below an expanded version of what I wrote to drz.]

    I read your post wherein you tell your dramatic story. It was, too, a beautiful story, especially considering the ugly facts. Those facts do overlap mine, but there are differences. First, I am a few years younger (63 now), with no diabetes (or, fortunately. asthma). I was in pretty good muscular shape before the first major flare, and I have never had major ear, eyes, or upper airway involvement (some minor issues, though--and pred-induced cataracts). Indeed, Though diagnosed with WG, from the evidence, a DX of MPA (microscopic polyangiitis) seems more in keeping with standard nomenclature. (The treatment is identical for both diagnoses.)

    Also, my DX came very fast--once I got the medics interested in taking me seriously. But the lead-up, alas, seems to have taken many years. I'll give you a taste. In 2008, I got a cold or flu, which eased in a couple of weeks, but the cough stayed on. This cough got to be quite a nuisance, personally and professionally, so I eventually hied myself to a pulmonologist. The results of going down this path were, in retrospect, a waste of time. Despite the cough, my PFT tests were "near-normal", and the bronchoscopy proved inconclusive (the provisional diagnosis was idiopathic hypersensitivity pneumonitis, which means: “huh?”). The next step was to see the hot-shot pulmonologist at the University, He took me seriously, and did go through my history with a fine-tooth comb. I liked him. Yet, his idea of a complete workup was to start{!) with an open-chest biopsy. To me, this seemed like an overly drastic (read: annoying, painful, and expensive) step, considering that no blood tests had yet been ordered (and, as it turns out, both pulmos missed the kidney stone on the CT scan). Though I knew nothing, then, about ANCA associted vasculitis, it seemed to me that any inflammatory process that could cause lung fibrosis could be hard on other useful organs as well. Why not start with much less invasive lab work? So, on my own, I got this done: CBC, lipid panel, renal panel, ANA, and UA. This was at the end of January, 2010. The results indicated some dicey kidney numbers, and both hematuria and proteinuria. Incidentally (or not), I was now just coming off a nasty bronchial infection.

    Next stop: Nephrology. At our first meeting, Drac (so-named because of the amount of my blood he wanted, then and now), pooh-poohed my lung-kidney theory, but ordered a battery of blood and urine tests, and: “Come back in a week”. (I vaguely remember his saying that there were some esoteric tests that could be employed if we didn't get the answer at this point, but he doubted these would be needed.) The results of that lab work: Creatinine was rising, and a bunch of stuff was fishy with the 24 hour UA, but nothing else tested was amiss.

    Another round of increasingly complex tests, and an ultrasound. Now something clearly scary was happening. Creatinine was now starting to spike, and the ultrasound was not encouraging--next we still had no diagnosis. On March 4, a Thursday, I had one more round of tests, including one for ANCA, though I was at that time told only that it was for a rare vasculitis. That night, I my left eye hemorrhaged subconjunctively (i.e., I had a red eye). I didn’t notice, but a colleague at a job pointed it out. On Friday night, still at the same job, I started getting edemas in both ankles. By Saturday, the edemas were very painful--and working that night was excruciating. I spent Sunday on the sofa with my feet up, nearly unable to hobble to the bathroom or bed.

    Monday, Drac called: "I think it is small-vessel vasculitis. But we need a biopsy. Normally, elective kidney biopsies take a few weeks to schedule, but I can get you in tomorrow. I advise you to be there." "Can I have until Wednesday in order to clear up some business?" Drac reluctantly agreed; "Just show up at 7:00 in the morning." By this time, the edema had subsided enough that I could walk in on my own--but the creatinine was higher still--dangerously high. (It eventually peaked at 6.7.) What I thought was "just a test" was, as it happened, the start of 12 days in the hospital.

    I still had not heard the term "ANCA", but the "autoimmune” term was being dropped by my numerous medical visitors, along with “vasculitis”. So, awaiting the pathology report, I was started on high-dose steroids and given a transfusion (anemia markers, including RBC and hematocrit, were very poor; this happens when kidneys are struggling). Then on Friday, we had the DX: Wegener’s Granulomatosis. CTX was scheduled for the next week. Then my lungs started hemorrhaging--bright, red blood, in ugly quantities. My third pulmo was called in, who did a BAL (bronchial alveolar lavage), producing a lot of bloody detritus, along with confirming evidence for the DX. Drac and the pulmo went into a huddle and decided on plasma exchange as the next step, which requires some additional plumbing, so I was next raced downstairs for the new pipes, then back up for the first PEX session. That was a busy day. (It only worked because my wife could be there all day with me. Because I was compromised by diazepam twice in the same day, I way was not allowed to consent to anything myself.) But the result was, from my point of view, near-miraculous: For the first time in many months, I could take in a full breath without incurring a massive cough reflex.

    The rest was somewhat anti-climactic: the CTX infusion, and more PEX, and tons of steroids. But I was restless: I hated the food and the confinement, being awakened in the middle of the night to have blood drawn and have my vitals taken (or only to ask if I was resting comfortably!), and the idea--promulgated by the hospital staff, presumably for their own convenience--that I should be in bed all the time. I started walking laps regularly--hundreds of laps, day and night, which annoyed the staff no end, though I think I did amuse them at the same time by my almost desperate need to get them off-script. (Toward the end of my stay, I toured other floors, I would go to the lobby, get some tea from the coffee shop, and walk around like a regular guy. (It is amazing how nervous hospital employees and visitors can get when you amble past the administrative offices in full patient costume.) So, after 12 days, I was sprung; I could do the remaining PEX sessions and CX infusions as a day patient.

    The other reason to blow that popcorn stand was that someone had to somehow pay for all that outrageous treatment: Even with insurance, all the various expenses, plus farming out work, is hideously expensive. Fortunately, I was still ambulatory--and even hyperactive (because of the pred). At least, I could be useful around the house: I love to cook, though I was truly concerned that, because of the steroids, I might slice my hand instead of the onions. In any case, I was on my way to a sort of recovery, and had a pretty good summer, mostly. Then, in August, the family weddings started, on top of a huge work month for me.

    Clearly, the events of that summer were important to me personally. But they also bear upon the theory that stress is challenging to to immune system--especially a compromised one. Therefore, I ask you to indulge a little side story here: It is thought that a son’s wedding is easier than a daughter’s. This is not necessarily true. In this case, the wedding was on our home turf--the bride being from Chicago, but she and our son wanted the public wedding to be on-island; the private church wedding was scheduled to be in Seattle, the previous day. I was working a lot at that time on Orcas, and spending every spare minute preparing the house for the various guests. My wife was in Seattle to meet and the in-laws and deal with the church details. On Tuesday, I worked a show, then caught the midnight ferry to the mainland. My daughter met me, and we rolled into Seattle around 3:00 AM. The Church wedding was at 10:30 in the morning. As I was dressing, I noticed a rash developing, but there was little to do about it then, considering the tight choreography. After a post-ceremony lunch, my daughter took me to catch the float plane back so that I could work the afternoon show. The rest of the party raced to the ferry. We all met at the hotel that evening for a wedding concert for the assembled guests (the bride and groom and both mothers being professional musicians). Then, the bus for the “rehearsal” dinner (we felt that, as many of the guests were from out of area, we couldn’t not invite the whole gang). Wedding day: The delayed rehearsal, then the wedding, then the reception till midnight. The rash was getting worse; though I did not yet know what it was. The Friday morning-after breakfast was at our house. We arose early to make the frittatas, scones, and such for about 40 people. Then I went to work--all day and evening. Saturday was a morning recording session and an evening concert. I was a horrible, sick mess.

    The stress was Brobdignagian, of course, and the rash turned out to be shingles (officially diagnosed a few days too late to start any anti-viral program), even though I had previously had the inoculation. I suspect the immunosuppression was to blame for this state of affairs. And I suspect the shingles played, in part, a major part in the return of the ANCA. The cough and rusty urine came back. I blame the shingles, and the new bronchial infection a few months later. The second big flare became obvious in March, 2011, incurring a new round of CTX, and a bump in the pred, and, now, azathioprine, but not, blessedly, any overnights in the hospital.

    So that is where things stand at the moment. I have since somehow managed to avoid any major new infections, but the the anemia will not go away. I can't seem to get my act together until mid-afternoon (on the lower pred dosages), and my brain doesn't fully engage until 9:00 PM or so (then again, it never has!). I have avoided dialysis so far, though that was a near-thing at the height of the crisis. Clearly, the kidney reserve is low; another major flare would be very bad. The lungs are in better shape, though the cough has come back slightly, which I will be keeping an eye on. This, the congestion, and full-body itching increased when the pred was dropped to 7.5 mg. But these symptoms have now abated some when the pred went back to 10. Also, the morning fatigue retreated after reverting to the higher dose of steroids. The “dog” is, I think, snoozing, but I hardly trust it. I know that if I get an infection, the ANCA (and the disease) will follow, so I am constantly on the lookout.

    In retrospect, I think the disease process started a long time earlier than the big flares. Indeed, I may have had an occult (“silent”) flare or two. This is, of course, a longer story. Around 2003-4, I had a lot of night sweats. Was this a symptom? Who knows? Later, urine became rusty and foamy (indicating, respectively, hematuria and proteinuria). There are other possible explanations for both, but if everything was taken in context....In the late 1990s, I suffered a series of nose bleeds (never having them before or since), At the time, these were blamed on ineffective blood pressure management. Since I also had a weird cough, I was taken off lisinopril and put on a calcium channel blocker. The nosebleeds stopped, but the cough only slowed down. A sign? Who knows?

    Perhaps the best chance to finger any rumbling vasculitis was in 2003. On a fine Sunday afternoon, I was badly rear-ended. Though the car was a mess, I thought I had survived without injury. But the next Friday, my back spasmed violently. I was in such supreme pain that I could almost not reach the phone to call in sick to my job. (My wife was then out of town.) By afternoon, I was able to drive to the clinic--barely. My doctor hand-carried my up to the next floor to see the rheumatologist, who gave me a pain patch and a prescription for physical therapy. The next Tuesday, my feet started “buzzing”. Eventually, I met with a neurologist--a supremely unsatisfying experience. The neurologist thought I was fibbing in order to get a settlement from the insurance company. (The insurance company did pay for for the physical therapy, and offered me--unrequested--a small amount and they were grateful for being let off the hook.) He did order an ANA test, on the grounds that maybe is was lupus or something. As it happens, ANA was slightly high, but not enough for lupus. Beyond this, he was not curious, and didn’t want to see me again. Was this a trauma-induced flare? Maybe, maybe not, though physical trauma can incite a cytokine storm, just like infection. Anyway, in retrospect, this may have been a lost opportunity.

    In the late 1990s. my left eustachian tube started to become periodically locked. Thankfully for my career, I have always been able to open it up, and my hearing is otherwise pretty good. The ENT who scoped me found no granulomas. But it is known that the cilia that clear the tube can be damaged by vascular inflammation. Did this happen in my case? Who knows?

    It is hard to prove, but my personal intuition is that the disease process began a few years earlier. In the early 1990s, I had a horrendous intestinal infection, which I now, after reviewing all the evidence, believe was due to a nasty bacterium--most likely one of the bad E. coli strains or something closely related. My primary care physician was not an internist, but, by specialty, a gastro-enterologist. Given his specialty, he kept looking for an answer he knew all about, like diverticulitis. He never found evidence in support of that hypothesized DX, and he eventually put it down as an infection of indeterminate origins. E. coli was not tested for, though no matter, perhaps, if it had been: Nowadays, the recommendation for treating such dastardly infections is to avoid antibiotics, on the grounds that killing off the bacteria that way just releases more toxins. But in the event, I was given some industrial-strength antibiotics. A mistake? Who knows? Certainly, my kidneys took a terrific hit at that time: Blood pressure hit the roof, and has never been normal since; urine turned opaque for several days. Yet, kidney function was not, then, checked. (Why not? Who knows?) Eventually, I did recover, though the much later kidney biopsy did show ancient injury.

    Should have my doctor have dug deeper? Perhaps, though it is hard to see, from his point of view, where else to go at that time. And I was just glad to put it behind me....

    Howbeit, many years later, a new theory about the etiology of ANCA disease now posits that certain bacteria, including some bad E. Coli strains, are, on a molecular basis, essentially identical to a naturally occurring human protein called LAMP-2. As it happens, most people with anti-MPO and anti-PR-3 ANCA also have anti-LAMP-2 ANCA. (I have never been tested for anti-LAMP-2 ANCA; no one, except subjects of a couple of research projects, has.) This is a controversial theory (called molecular mimicry), its contentiousness owing, in this case, to the dearth of confirming research. But it does have a ring of truth in my situation.

    The fact is that it is hard to assign “blame” to anyone for not finding my ailment earlier. All those manifestations that, in retrospect, may have been clues, individually have other, and more benign, explanations. They were “dots” that are not easy to connect without seeing the largest possible picture. Given the specialist system in medical care, connecting the dots is not so easy. And, in fairness to the doctors, I, as a patient, preferred to assume that the small complaints were not connected and, in any case, not worth bothering the doctor for--at least until they got too large to ignore. Yet, this delay was costly. Is there a lesson here?

    Then, too, diagnosis is an art form that not every doctor knows how to perform artfully, It really does require a big picture view that is not antipathetic to current medical training and practice.

    And what about treatment? This disease is “manageable”, but not “curable”. It it the best we can do? Perhaps--for now, On the other hand, I truly believe that global immune system suppression will seem utterly barbaric in the coming decades. For now, though, we soldier on. Steroids are yucky, but necessary. Immunosupression is I believe, is essentially the wrong way to go, as it is hard to be truly human without a fully working immune system, But no one knows, yet, a better way to manage the disease.

    You know, I am a lucky man, considering everything. Sure, I have my ailment, with its annoying symptoms and constraints. But I have an immensely supportive family; I am still able to work; I haven’t lost kidneys or had to suffer dialysis (so far, at least!). Many people have a much heavier load to carry--often, with little or no support. Constraints notwithstanding, I still envision a strong future, and I feel that my best work is still to come. Hope reigns.

    Al
    Last edited by Al; 03-23-2012 at 02:07 PM.

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    Thanks Al for sharing your well put story. A lot of it sounds all too familiar. I wish lots of good health in your future!

    Katrina
    "Never go to a doctor whose office plants have died." - Erma Bombeck

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    Yes Al, thanks for sharing your story. Like you I became ill following a severe gastrointestinal infection. From that day in September 1989 my life was changed for ever. Knowing what I know now, I am sure WG attacked my gastrointestinal system. I suffered severe debilitating gastrointestinal symptoms for many years and although all test were done (and many tests were done over the years) everything came back normal. My diagnosis was ME and I think for many doctors, hypochondria. My story is long and complex but one day I must write it as I dont think I have come across anyone on this site who had gastrointestinal involvement. In September 2007 the disease disseminated and went multi system and life threatening. Diagnosis was made in March 2008 and in a way it was a godsend as with treatment my gastrointestinal symptoms have improved and I no longer have the gut ache and severe fatique which plaqued me for such a long time.!
    Last edited by Rose; 03-23-2012 at 05:11 AM.

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    Wow, Al. Thanks for sharing your story in such detail. As an aside, I suspected you lived in the San Juans, and it's nice to have it confirmed. Those islands and I go back a long way, to when I was a teenager in the 1960's, and we used to spend summers at the UW marine labs in Friday Harbor, where my dad did research on cancers in fish. In fact, my parents met there as UW students in the late 1940's, I guess. My dad and his second wife have lived there at least half the year since the mid 1980's or so, though he passed away last year. I hope to be up there sometime this year to help go through his belongings, prior to her selling the house and moving to S. California full-time.

    I'm glad you feel lucky in spite of all you've been through!
    Anne, dx'ed April 2011

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    Thanks drz. This will be helpful for many.
    Phil Berggren, dx 2003

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    Thanks Al for sharing it here. I enjoyed reading it again and think many others will also find it interesting and helpful.

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    Quote Originally Posted by drz View Post
    Thanks Al for sharing it here. I enjoyed reading it again and think many others will also find it interesting and helpful.
    drz, both you and Al are excellent story tellers. Both of your stories are so sad and yet beautiful and full of hope at the same time. Thank you both for sharing so much of yourself with us so that we will find comfort in the commonalities and the uniqueness that identifies us all as weggies.

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    I read the whole thing.

    I had a follow up yesterday and it was the first time a doctor ever showed me or explained blood tests to me. He read them over and explained what it may mean to my treatment. First time in 12 years. Mind you, I have never asked any of them to explain it to me. I just go in and wait until they tell me what the next step is. Yesterday at my appointment the resident asked me how it started.

    "what do you mean?"
    "what were your first symptoms that lead to the diagnosis".
    "um my joints hurt a lot and i had a strange rash from my knees to my toes and it was hard to breath"...oh ya and i was coughing blood".

    I dont remember it anymore vividly than that.
    lightning crashes
    leigh

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    I hear you, Leigh. This is such a complicated disease and there is so much to explain. At my doc's appts. there is NEVER time to ask all the questions or get many explanations. I look forward someday to finding a doctor who will give me that time. Meanwhile, I'll depend on this forum to answer most of my questions.
    Anne, dx'ed April 2011

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    I dont usually ask but yesterday I realised how much I dont know or ask about. I think I am a difficult patient.
    lightning crashes
    leigh

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