New Weggie

[richard052018]

Age 42, male, father of 6 year old girl. Had the usual weight loss, night sweats, jumping/everywhere arthritis, primary care found elevated ESR/CRP as well as Anemia and referred me to rheumatologist, but appointment was 6 weeks away. A week later I started coughing blood, and I went to the ER, where they did a CT scan and found a hot mess in my lungs. ER doc sent me to pulmonologist (my guardian angel), who interviewed me on my symptoms for an hour, took 8 tubes of blood, cleared his schedule the next day to give me a bronchoscopy, diagnosed diffuse alveolar hemorrhage, admitted me to the hospital, and started IV steroids. Two days later I got the crushing bloodwork (positive C-ANCA and PR-3). Official diagnosis on May 12.

Despite intermittent blood/protein in urine, I have yet to have elevated BUN/Creatinine, and upon a negative kidney biopsy, the nephrologist in the hospital concluded I don't have kidney involvement, or if I do, it is minimal. Urine consistently comes back negative for blood/protein now (but I'm not convinced I'm out of the woods yet in terms of the kidneys).

I do have a few questions for the regulars:

1.) The rheumatologist opted to tread me with 4 weeks RTX infusion because of the lung activity. This confuses me because I believe the RAVE trial excluded patients with diffuse alveolar hemorrhage, and while there are very serious side effects, my readings tell me the gold standard to remission is CTX + PRED. I would hate to think I went the wrong route to achieve an initial remission. My OCD has led to me booking a flight to Cleveland and will see Dr. Alexandra Villa Forte in August (it seems she is the expert on remission). FWIW the pulmonologist felt my disease was "limited" enough to treat with MTX (again, another differing opinion). Any thoughts on the drug choice considering my disease presentation?

2.) The worst symptom I've had at this point is an increase in Ocular Pressure (both eyes). Eye doctor has me on Travatan Z drops, which work. The question is the pressure Pred related or Wegeners Related (I think the ophthalmologist is in over his head)? Any Weggies with Ocular Pressure issues on the forum? Does the pressure go away once the PRED taper begins? Current Pred is 60MG (was 80MG, as I'm a big guy).

3.) The drugs have pushed my CRP down to .5 and my ESR is 12. As long as these numbers are within reference values, am I at risk for a major attack (re: lungs/kidneys)? Is there a way serologically to know I am out of the "kill zone"? FWIW: I've been going out of pocket to get CMP tests through Labcorp direct (via requestatest) to check my kidneys two weeks after my monthly labs with the doctor (the end result is bi-weekly blood work). Do I need to be tested more than once a month?

4.) Lastly everything I consume on this disease (VF, Cleveland Clinic, Hopkins, Mayo, NIH), the same two words show up over and over again: morbidity and mortality. Where is the hope? I guess the fact that I am already back at work and I can mow my lawn I should have hope, but I'm convinced that is nothing more than Prednisone in action. It seems through my readings there are some that fight through this, but the statistics seem awful (at least many of the NIH studies). My dream is to see my 6 year old graduate from high school (I need 12 years).

Thank You for reading my post. I promise to pay it foward to other newbies as I get more educated.

All the best,
Richard052018