Episode 19: Wonderful (Not) Wegener's Granulomatosis

My health journey continues to lead me to places I've never dreamed or heard of.  I mentioned earlier that my ENT thought I might have Wegener's or a similar condition.  I saw a rheumatologist at Johns Hopkins on July 18, and she has initially diagnosed me with this oh-so-wonderful disease.  She then sent me for a CT scan of my chest--lots of pulmonary and airway issues, which are common with Wegener's--and a slew of blood tests.

Now, we may yet find out it's not Wegener's; perhaps it's cryoglobulinemia, which has many of the same effects.  Since I also have hepatitis C, the cryo is a good possibility--it is common with folks who've had hep C for many years, as have I. Wegs and cryo both affect the blood vessels, which then affect vital organs, such as the lungs and kidneys. When I say "affect," I mean more or less destroy.  It's true--my nose has necrosis (dead tissue) in it from the inflammatory damage. So far I have not tested positive for ANCA antibodies, which would have just about confirmed the Wegener's.  But that doesn't mean I don't have it, as a small percentage of "Weggies" tests negative and/or may develop the antibodies later.

From MoonDragon's Health & Wellness
http://www.moondragon.org/health/disorders/wegenersgranulomatosis.html

             
I'm not going to get into the insurance hassles I encountered--I'll just say that my insurance company suddenly changed my plan this year, and Johns Hopkins is out of network.  They wouldn't pay for ANY of my bloodwork--they have an exclusive contract with Lab Corp.  In other words, rich fat cats lining the pockets of other rich fat cats, one of the major problems with our health care system.  I thought when I got Blue Cross Blue Shield I'd have good insurance, and up until now everything has been fine--but they must have changed their policies with the new fiscal year.  I guess I'll be on the phone with them to try to change their minds about it--and I hear these decisions have now been outsourced to third parties in other countries, so I imagine the fight will not be fun.  Will I have to once again declare bankruptcy due to being sick?  Our healthcare system is truly broken, and while I have hope with Obamacare I have a feeling it's not going to get much better.

I live in a small rural area in Maryland.  I doubt most of the physicians around here have even heard of Wegener's granulomatosis, much less treated it.  I am not trusting myself, with this potentially life-threatening condition and treatments that can also be life-threatening, to any of the local docs.  I do love my primary care physician here, but she did take 13 years to diagnose my hyperaldosteronism, despite my having all the classic symptoms.  Besides, I live IN Maryland, and Hopkins is in Baltimore.  Why isn't it in my network?!

Here are my symptoms thus far:

• Extreme--and I mean extreme--fatigue and sleep problems
• Difficulty going to sleep and difficulty waking
• Chronic sinusitis with crusting and nosebleeds
• Nasal perforation
• Sinus thickening and polyps on MRI
• Sore throats
• Frequent productive cough with clear stuff coming up
• Cough spasms that lead to vomiting stomach contents
• Chest pain; difficulty breathing while lying down
• Sleep apnea (though I'm not overweight)
• Low fever in evenings
• Night sweats
• Quickly worsening eyesight
• Bloodshot eyes
• Frequent afternoon/evening headaches
• Migrating myalgias and/or rheumatoid arthritis, particularly in legs and hip/saddle area, with positive RA factor on lab tests
• Subcutaneous (under the skin) nodules (bumps)
• Morning stiffness 
• Base of skull is "cracking" loudly in a frightening way--Cervical nerves C1 and C2 are often affected by Wegs
• Lack of appetite--but thus far no weight loss, though about five years ago I suddenly lost 20 pounds with no explanation
• Distaste for foods/drinks I used to like
• Rapidly decreasing glomerular filtration rate (GFR); normal for my age is 93, and it's gone from 64 to 52 in one year.  This is a measure of the kidneys' ability to filter, which is their job.  If it drops too low, it's time for dialysis and/or kidney transplant, and I'm really not interested in the latter at all. 

I tend to make my posts WAY too long, so I'm not going to belabor this too much.  Let's just say I feel pretty awful--but I'm not in a lot of pain at this point.  The symptoms are annoying more than painful.  However, the big picture is quite serious.

I go back to Hopkins in a month to see Dr. Smith again.  I am also scheduled there in the Nephrology (kidney) Department, and that's the one I really want to know about, since death by Wegs usually happens through kidney failure.  I can't get in until October, and many sites say that Wegs can be fatal in months if untreated, so that is a bit frightening. I couldn't get in to see the nephrologist who specializes in Wegs until December, but the one I'll be seeing is also familiar with it.  I am also to see the Hepatology (liver) Department; we may decide to finally treat my hepatitis C, particularly if it turns out I have cryoglobulinemia.

If not, I think the treatment for Wegs is toxic enough--medically, I don't think I could do both at the same time even if I wanted to.  The drugs probably just would not work well together.  Cytoxan, the chemo agent used for Wegs, is extremely toxic. (It's kind of funny in a sick sort of way that the pharmaceutical companies practically named the drug as a toxin.)  I also don't look forward to large doses of prednisone.

When my mom was dying of cancer, she had large doses of prednisone that made her go crazy.  She pulled out all her IVs and went running to the front door screaming that the house was going to explode, and if she'd gotten out she would have gone flying to the bottom of the street on our big hill--there was an ice storm that night, and she would have slid off those steps, down the front yard hill, and on down our street.  On top of that, because of the prednisone she hallucinated about my boyfriend and me doing scientific tests on her in a lab--He was in the "nuclear camp" and I was in the "metabolic camp" and we were battling it out with her as our guinea pig.  This was and is very painful for me, since she lost trust in me as she was dying based on crazy images her head believed were real.  But I guess, to treat Wegs, I'll have to take the nasty stuff.

I really don't want this blog to be about nothing but illness, but I am where I am in life, so that's what you'll get.  Hopefully, something here will help someone else who is facing similar strange symptoms.

By the way, "Wegener's" now has a new name--granulomatosis with polyangiitis or "GPA"--because Dr. Wegener was apparently a Nazi sympathizer.  But "Wegener's" is still widely used ... changes like that take time.  I should really switch, since I hate everything about the Nazis, of course, with Anne Frank one of my lifelong heroes.  But for now I'll just go with the more commonly used name.

Peace.