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

[Tim wga]

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

I have glaucoma from the pred medicine.
My pressure has been over 50. safe levels should be under 20.
On top of Weg medicine I take 3 different eye drops 3 times a day.



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[richard052018]

Yeah mine has been in the low 30s, the drugs are getting it into the low 20s, but yes, before good ole' GPA, it was in the teens.

All the best!

[Pete]

Hi Richard,

Welcome to Weggie world. If you’re seeing Dr Villa Forte, you’re seeing one of the best. My first appointment (July 2012) with her lasted almost two hours. She’s given me the time I need every appointment since. She gives me complete answers to all my questions.

My current treatment regimen is one infusion (1 gram) of rituximab each year. I also take a bactrim DS three times a week. I just got off prednisone two weeks ago.

To give you some hope, I now feel very well. I walk 2-3 miles several times a week, play in a seniors golf league, do my own yard work, and enjoy playing with my grandkids. I’ll be 72 next month.

Hope your appointment with Dr Villa Forte goes well.

[richard052018]

Pete,

Thanks for the uplifting words. Happy to hear you are living what sounds to be a full and great life. My big issue in all this is ensuring my daughter has as normal of a childhood as possible. Anything beyond that is a bonus.

As for Dr.. Villa Forte, should I try to send her my questions in advance? I have much to ask her. At a minimum, I will certainly have it all written down.

I'll do my best to be more positive. It is all we can be.

Take Care

[drz]

You could ask one of your current doctors to consult with any experts listed on the VF page of experts in the mean time althogh it sounds like you are doing very well currently. If no one is pushing CTX and super high steroids for treating you, that is a good sign too. Learning to live with GPA is a challenge and it is normal i think to experience anxiety about the uncertainty of dealing with it. Finding a good health care team that you trust is essential i believe and you sound like you are up to that task.

Generally, once you get correctly diagnosed and treated correctly, the prognosis is favorable for improvement. And for avoiding serious damge to vital organs.

You can also buy pee sticks from a pharmacy to help monitor your kidney function. Kidney function can deteriorate quickly so it is good to be vigalant about it. A nephrologist can advise you on how often you need to test and how.

Best wishes for a long happy life.

[richard052018]

Thanks for the advice. I have bought SIEMENS Multistix 10 SG to test my urine (as recommended by Cleveland Clinic). Doing everything I can to be proactive with this disease. This community is amazing.

[Pete]

As for Dr.. Villa Forte, should I try to send her my questions in advance? I have much to ask her. At a minimum, I will certainly have it all written down.

I generally have the written list of questions and concerns with me. She takes the time to fully address them all.

Once you’re established as a patient at Cleveland Clinic, you should open a MyChart account there. You should also sign releases with your local doc’s and Dr Villa Forte so that they can see your entire record.

MyChart has an e-mail facility so you can message Dr Villa Forte directly. She generally responds in 2-3 business days. If something is urgent, you can call her. She’s called me in the evening or on a weekend in response to my concerns.

Let us know how you’re doing.

[Alysia]

Welcome to the forum, Richard. Thank you for sharing your story. The more you know about WG, the better you can fight it.

As for your occular pressure, do you know what causes it ? Wg can also affect the eyes and the best is to be checked by neuropthalmologist. My wg dr. wrote this article, in which they treated succesufully orbital manifestation of wg with rtx

https://www.ncbi.nlm.nih.gov/m/pubmed/19960854/#fft

As for your question ctx or rtx, from what I read here, both work good. Rtx should be easier on the body. I am on rtx since 2013 every 6 months, 2000mg. Mtx never worked from me. I know it does work well for many of us.

As for mortality, no one knows what awaits him. Wg or not. It is forbidden to discuss religion in the forum so I will just say that the best is to trust in God. Or like someone wise said: "pray, hope and don't worry".

[jeto]

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,
Richard052018hi Richard welcome to our group,
i wish you the best
here in this group you will find friends who will be with you all the time to get your information and help you need.

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[richard052018]

Alysia,

Not sure what is causing the pressure, but the docs believe it is the Pred and not disease. Nonetheless I'm going to squeak and make noise until I can get an MRI on the eyes. Overall though my optic nerves still look good. I pointed the doc to this article for context:

https://www.reviewofoptometry.com/article/ocular-hypertension-in-wegeners-granulomatosis

I am with you in terms of acquiring the most knowledge one can get on this disease. Every case is unique so there is an infinite amount to learn, but I'm doing my best. I'm big into home diagnostics as well: urine test strips, BP/Blood Sugar/SpO2/Pulse.

When I meet with Dr. Villa Forte I will certainly get her recommendation on a neuropthalmologist with GPA experience.

Excited at what this place will bring to my life. I don't do Facebook or social media so this is new to me.

[NatriceRomeo]

Hi Richard,
Sorry you have been diagnosed with this disease but happy you have found us! I just turned 40 & have had this disease for over half my life (diagnosed when I was 17). There is hope. I think it’s easier for me than a lot of people whom have lived most of their life without this disease. I honestly don’t remember how I felt before! The point is yes-you absolutely can live with this. The trick is to know your body & know when to rest (I push this envelope all the time). Some days are good and some not so much. I am on prednisone and rituxin. My ocular pressure goes up when I get the infusion because they also add 100 mg soul-medrol. I believe your high ocular pressure is from steroid use. Prednisone what a fabulous and horrifying drug! I hope everyday gets easier for you. Keep your head up! WG isn’t for the weak that’s for sure. Look at all the warriors here! You got this!


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[AngieB]

How long have you been diagnosed (for the 72 year old gentleman that plays golf) ? I am a "fnew member" too and this is a very encouraging picture you paint. So happy for you. I will see Dr. Villa Forte in August.

[Pete]

How long have you been diagnosed (for the 72 year old gentleman that plays golf) ? I am a "fnew member" too and this is a very encouraging picture you paint. So happy for you. I will see Dr. Villa Forte in August.

Hi Angie,

I'm the 72 year old hacker (bad golfer). I was diagnosed in January 2011. I had been sick for about a month with odd symptoms - persistent earache, loss of energy and appetite, coughing up a little blood, and night sweats. It took about 18 months before I began to approach being in remission. It took another 18 months before I felt normal all the time.

Hang in there. Take your meds. Get some exercise. Watch what you eat (especially when on high-dose prednisone). Enjoy life as much as you can.