Wegener's...what in the heck is it?

So, about that Wegener’s….I guess I should probably explain what the disease is, seeing how it’s what my entire blog is about. Before I go on, remember, I am NOT a medical professional; I am simply just another Wegener’s patient. My attempt is not to give anyone medical advice, but, maybe just some more information and personal experience. It’s so easy to look through the internet when you’re diagnosed with a new disease and come back with a whole bunch “information” that probably makes you more confused than you were in the beginning. It’s also easy to come back very frightened! The thing we need to remember is Wegener’s is an elusive little fella that shows up many different ways, so, you could have all of the symptoms, or some of the symptoms…..the only true way to know is to speak to your doctor and get tested. As it’s so rare, people need to be more informed. We need to find a cure! Anyways, I digress…….

What IS Wegener’s?

The very first time Wegener’s surfaced was in 1931 and was “founded” by a medical student named “Heinz Klinger”. A couple of years later a German pathologist came along and actually recognized the disease as a particular form of vasculitis. So, guess what the German guys name was? Yup, Wegener…..Friedrich Wegener. I betchya Klinger was awful P-O’d that the disease wasn’t named after him!!

Wegener’s Granulomatosis (WG) is a VERY rare type of vasculitis which effects approximately 1 in 300, 000 people (gee, I must be so lucky!) (http://www.vasculitisfoundation.org/book/export/html/40). Vasculitis is an inflammation of your little arteries and veins. The arteries and veins that WG likes to inflame are the ones which give blood to the lungs, sinuses and kidneys. Thus, why there are so many very odd symptoms which makes it very hard to diagnose.

Symptoms?

The classical symptoms of Wegener’s include, fatigue, weight loss (how come I couldn’t get that one?), fever, shortness of breath, coughing up bloody mucous, joint pain, sinusitis, ulcers in the nose, and nosebleeds. Other parts of your body can be affected as well; your eyes (mine turned a very bright shade of red and was easily confused with “pink eye”), your middle ear (for me, I had some serious ear infections, one of which lasted almost a month making it very difficult to hear, it kind of felt like “swimmer’s ear”) and the skin (Again, mine came out in a rash).

So, that’s why for me, the doctor’s just kept thinking I had chest infections or sinus infections or even just the flu. It was only until we really started looking in to the joint pain that we realized there was something bigger there. So, if you DO have any or all of these symptoms, don’t freak out just yet. It could very well be, “just the flu”. But, I’d get it checked out by your doctor, just in case!

How does the doctor know you have Wegener’s?

Well, like I said, it’s so hard to diagnose so, the doctor definitely can’t tell just by looking at you. More than likely, if your doctor thinks there’s something going on, he/she’ll order a bunch of blood work, urine tests and perhaps a chest X-Ray (depending if you have chest symptoms) to start you off. In people with Wegener’s there is normally blood and protein in the urine (it’s there, but can only be seen under a microscope). Luckily for me, I only had a little bit of blood and a little bit of protein.

For the X-Rays, they look for abnormalities that come from the inflammation. In my case, they found little nodes on my lungs, which were confused with pneumonia. I’m not a radiologist, but, I’m assuming everything on an X-Ray is all fuzzed together anyways, so, it’s probably hard to determine what exactly is floating around in there, but, then again I’m not a radiologist.

In blood tests they check a whole bunch of stuff. They look for any inflammation, which can be found in your sedimentation rate (ESR). My sed rate was pretty high and c-reactive protein. The actual “Wegener’s marker” however, is the ANCA test. This is the antineutrophil cytoplasmic antibody……fewf; say that 3 times fast……When your disease is active, this “ANCA” is elevated. It’s supposed to be between 0-5 but, mine was greater than 100….woo-hoo! I’m above average!!

Ultimately, the final diagnosis needs to be confirmed with a tissue sample….so; they can either do a kidney, lung or sinus biopsy. Yuck, yuck and yuck. As I mentioned before, I had to get the kidney biopsy. So, they stuck a few needles in to my skin to freeze it, then, they put a “biopsy gun” in my back. I told them not to use the word “biopsy gun” around me, and they were pretty good about that. I also asked them not to show me the needle, you know, given the length and thickness and such…..So, it wasn’t the most excruciating thing I’ve ever gone through, though it wasn’t the most pleasant either. I tell yah though, I was VERY happy when he said “we got two good samples! We’re done”…..hallelujah! The worst thing for me was the fact of the “extra fluff”. If you have any extra weight on at all, it makes a kidney biopsy harder to do, I guess because they have to go through more stuff to get to the organ. Anyways, in the biopsy they’re looking for vasculitis and little granulomas, or nodular inflammation….or, in layman’s terms little bumps on the tissue.

Now, how do I get rid of it all?

WG is quite serious and sometimes can be fatal. As my doctor said “the ultimate thing we’re trying to prevent is DEATH”….just like that, he threw in the D word and scared the beejeepers out of me. The point of the treatment is to suppress the immune system and stop the inflammation.

Currently I’m taking a very high dose of steroid called “prednisone”. Hand-in-hand with the prednisone, I need to take calcium and vitamin D (because of what steroids do to the bones). I am also taking something called septra; this apparently helps prevent a relapse. Finally, the scary drug…..the “chemotherapy drug”…..Cyclophosphamide or Cytoxan. The cyclophosphamide has some serious side effects thus; we needed to do some serious thinking about when to start the drug. The biggest side effect of our concern was the infertility thing. They told me I may not be able to have babies when I’m finished my treatment. This spun us in a brand new direction. We had to meet with a fertility specialist and start thinking about the options. However, the only option which existed was the “harvesting of my eggs”. This option is not always guaranteed and it’s quite, QUITE expensive. It would cost approximately 15, 000 for us to get it done. It involves taking a whack of drugs to make a whack of eggs, then; you need to fly to Calgary, Alberta. Then, they take a huge needle, stick it up in to your lady parts and remove your eggs. After that, they take your spouse’s sperm, fertilize your eggs, put it in a tube, pack it in a Ziploc and put it in the freezer until you’re ready to have your baby. So, this is not actually “harvesting your eggs” it’s more so, harvesting embryos. But, the egg harvesting option exists as well. Or then, there’s the option my best friend, V suggested , it involved a turkey baster and some Rubbermaid containers……though I’m thinking that’s probably NOT such a good idea……Anyways, that’s too much information for a newly diagnosed WG patient. Back to the cyclophosphamide. You need to drink lots and lots and lots and lots of water, stay away from people with colds/infection, be constantly monitored and so on and so forth. It CAN make your hair fall out and it CAN make you sick. However, they DO have some nice drugs to make you feel not-so-sick and there are some lovely wigs, hairpieces and hats out there for you. So, it’s not the end of the world. The best thing is, you’re getting the treatment you need.

So, how do you get rid of it? Unfortunately you don’t. It’s something you’re going to have forever (until they find a cure). With that being said, they have many wonderful treatments which can put it in to remission and make it stay there for a long, long, looooooooong time. So, they key is to have a positive attitude. I have a super “support team” and I know with their help I’m going to be beat living heck out of this thing. While there’s no cure yet, there’s hope. So, I hope I can do my part in helping find that…..even if it IS just a little tiny, tiny part.

**References for this blog, because it certainly didn’t come off the top of my head!

http://www.vasculitisfoundation.org/book/export/html/40
http://www.medicinenet.com/wegeners_granulomatosis
http://www.mayoclinic.com/health/wegeners-granulomatosis/DS00833
http://vasculitis.med.jhu.edu/typesof/wegeners.html