Saturday, July 9, 2016

9. Wegener's

 What is Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis, also known as Wegener’s Granulomatosis, is an inflammatory disorder that mainly affects very small blood vessels in the respiratory tract. Both the upper airway (sinuses, nasal cavity, windpipe) and lower airway (lungs) can be involved.

The disorder may also affect small blood vessels supplying the kidneysm and other tissues throughout the body. Reference

The word inflammation comes from the Latin word inflammare, which means to light on fire. You can think of it like this: in people with Granulomatosis with polyangiitis, small blood vessels in certain areas of the body are “on fire” and are eventually damaged. This can cut off blood supply to vital areas of the body.

So, my immune system starts working overtime to fight the "invading army"; Only problem is, there is no army, so all of the defending anti body are still actively swelling up the blood vessels and stopping blood flow to different areas of my body.

So Cyclophosphamide (Chemo) is there to slow down the defense system. 1200 units every three weeks, just enough to do the job. Not enough to damage bone marrow... they hope.

I am scheduled for 10 doses to start, as it has a cumulative effect, I am getting more drained from each does and it takes longer to recover each time. The upset stomach is getting worse too, but medication is helping with that.

Pain has started to settle down nicely... and then the spots start to come back after my 8th session. Things are moving very slowly though, so it is helping, just not quite working.

On my 9th session it is decided that we will discontinue Cyclophosphamide.  Wait, I thought this was the big sledge hammer tool against Wegners ?

Remember when I said I had great doctors? They've got another treatment up their sleeves.

Rituximab is an interesting drug, as it is a chimeric antibody. This means that it contains portions of both human and mouse antibodies mixed together.  So, here is my thinking, as long as it works I don’t care right?

But then I think, hey wait, if this is part mouse and part human does that make me a mouseketeer? And, if I am a mouseketeer, do I get any discounts a Disney Land?

If it actually works I may even get to go.   It has a great story behind it too:
So, I go for my first treatment and we are preparing all the information, reviewing my current health levels and I have a little infection in my mouth from all the Prednisone and other medication I take.

Treatment canceled, I feel terrible as a special infusion nurse has been booked for the whole day, rooms changed and rescheduled and now all changed again just because of a little infection.

Usually, if you have a bad case of thrush, they will give you 1 pill it will go away in short order.

I get 2 pills a day for 10 days and a liquid 4 times a days until treatment is complete.

Rituximab apparently will make thrush go ballistic, so get it gone before treatment.

Ok, when you  get the treatment they give you Benadryl, an allergy medication, the kind that makes you really tired, 100 Prednisone and 1000 Tylenol and start putting this stuff in very slowly while they monitor for adverse reactions. Seems it is fairly common.

It’s a long day and by the time I am done I am so tired I can’t even drive, to be honest standing is a real challenge and I can‘t wait to get home to bed. But you know what, I had two pretty good days before my next treatment.

Treatment 2 pretty much goes the same way, except I start to feel better the day after and it stays for 3 days or so.

Hey! First improvements in 8 months, things are looking up!

Treatment 3, other than the day of treatment feels pretty good the whole week, starting to feel like I am taking too much Prednisone and could start to cut back (currently at 50 mg day, been at this level for several months now).

Treatment 4, the last one of the set, and it seems to take a little more out of me than the other treatments; But after a few days can really feel an improvement.

It's hard to explain, but the downward decline seems to have stopped, got the fire out. I think now it's time to rebuild and I am starting to feel better, so much improvement that I actually go outside for a little while, I even visit a large art gallery and I show my art portfolio... but  that's for another time.

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