New WG Diagnosis

[norcalian]

Hi All,

I was diagnosed with WG about three weeks ago while in the hospital. Although, like many on this forum, I think it started much earlier. I've always had seasonal allergies, but a couple of years ago I became congested year round. Nothing really helped...and frankly, it wasn't bad enough that I really pressed it much. In January of this year I started having middle ear problems. Fullness and a lot of hearing loss...especially in my left ear. I started meeting with an ENT specialist, got a CT Scan of my ear and was told that I had a cyst in my middle ear. I was suppose to meet with a surgical specialist...but before I could I started getting sick. It started with a fever, cough and some pretty sever joint pain. My GP, who is really good, immediately referred me for tests and and a chest Xray. The Xray came back with some spots. So my GP ordered a CT scan on my chest which found multiple binary pulmonary nodules...one almost an inch in diameter.

I was immediately referred to a pulmonary specialist who diagnosed me with Valley Fever (I live in California - it's a lot more common out here and in the southwest). I was put on anti-fungals. After a week I just kept getting worse...loosing a lot weight, feeling miserable. At my follow-up, my pulmonologist took one look at me and admitted me to the hospital. At first they thought I had TB and put me into isolation (kind of funny to watch how these particular nurses reacted to me thinking I had TB). Finally, the attending pulmonologist came in to see me and said "You've got to have Wegener's". He ran the tests, did a bronch scope and it was confirmed with the rheumatologist. I spent a week in the hospital (I was lucky it was only a week).

At the time my Wegener's only involved my lungs, joints, sinuses and ears. I was put on high doses of prednisone in the hospital and started my first infusion on Rituxan. Out of the hospital I am still on a lot of prednisone (80mg/day - I'm a pretty big guy), finishing my Rituxan treatments (last one is next week) and am taking 150mg of Imuran a day (plus all the other stuff I take to manage the side-effects). When I was in the hospital there was no renal involvement...but since being home, my protein clearance levels have increased to indicate that there is now inflammation in my kidneys. My Creatinine levels are solid and stable and my CRP is back to normal. But my Rheumatologist (who I really like and have an immense amount of confidence in) says that if my protein clearance levels don't improve or get worse that he will take me off the Imuran and put my on oral cytoxin...which has me concerned.

A bit of personal info about me...just had my 36th birthday. I'm married and a father of 4. I work in education. This whole thing has really been a blow on me and my family. Recovery so far has been a bit rough...I feel weak all the time. I'm really glad that there is a community of us to talk and exchange information and vent with. I'm looking forward to participating. Any thoughts, comments, advice, warning, etc are most welcome. And if anyone else is from the San Francisco Bay Area - or knows of additional resources out here, please give a shout out. Thanks!

[Chris G]

Welcome Norcalian. You will find many people here with similar experiences, who will offer great advice. My wg is localized to head and neck, so I don't have a lot to offer where lungs and joints are concerned. But I'm happy to hear they started you on rtx. Many will be eager to hear how that works for you, as I believe that most of those here who had lung involvement were started first on cytoxan. Yes, feel free to vent all you want!

[Kathie28]

Welcome to the group!

I work in education as well which certainly adds on another battle. Now that I am on all those immune suppresent drugs it is so hard not to get sick. I had the Rituxin as well and now I am on methotrexate. My labs improved a lot after the Rituxin. I have both kidney and lung involvement. My kidneys have improved but I continue to have elevated protein and blood. I can't comment on the Cytoxin so far I haven't had to go there but I know it has helped a lot of people. I do fear it though. I wish you the all the best and keep the hand sanitizer handy at school. At least summer is almost here!!

[norcalian]

Welcome to the group!

I work in education as well which certainly adds on another battle. Now that I am on all those immune suppresent drugs it is so hard not to get sick. I had the Rituxin as well and now I am on methotrexate. My labs improved a lot after the Rituxin. I have both kidney and lung involvement. My kidneys have improved but I continue to have elevated protein and blood. I can't comment on the Cytoxin so far I haven't had to go there but I know it has helped a lot of people. I do fear it though. I wish you the all the best and keep the hand sanitizer handy at school. At least summer is almost here!!

I am away from work right now. it will be interesting to see how things will be in the Fall depending on what medication I'm on (or how much prednisone I am still taking). How long after you finished your rituxin treatments did you start to see improvements?

[Jack]

It sounds like your diagnosis was about as fast as could be expected and you should have avoided most of the permanent damage that is often caused in the early stages. If your doctors know what they are doing, you should now be on the path to making a reasonable recovery. Don't worry too much about switching to ctx, it is the drug most of us have experienced and the side effects and how to avoid them are well understood. It has proved itself to be a very effective treatment in most cases.

[Sangye]

Hi norcalian, welcome to the group. I'm so sorry to hear that you've been diagnosed with Wegs but I'm glad you found our group. The single most important factor is to be under the care of a Wegs specialist. I know you are happy with your doc right now. Things aren't too complicated at the moment, but with Wegs that can change on a dime and a regular doc will quickly find themselves in over their head. A regular rheumy is not skilled to deal with the complexities of Wegs.

There is a new Wegs specialist in San Francisco-- Dr Sharon Chung. You can find her info here: Vasculitis Foundation Medical Consultants | Vasculitis Foundation (http://www.vasculitisfoundation.org/vasculitismedicalconsultants)

Also, this thread has info that will help you: http://www.wegeners-granulomatosis.com/forum/general-wg-chat/986-what-newly-diagnosed-need-know.html

[norcalian]

Thanks for the welcoming messages. I will definitely be on here looking for information as my treatment progresses/changes. I'll probably find out this week whether or not I will need to switch from 150 mg of Imuran a day to CTX. With my protein clearance raising over the past couple of weeks, I think my Reumy wants to give it one more week to see if the full dose of Imruan will knock it back to a more normal level...but with the way the numbers were going I'm guessing that they'll be the same or a bit worse...so I'm preparing myself for starting oral CTX...which will replace the Imuran. The thing I'm concerned about is becoming even more open to infection while on CTX...any thoughts about this? I can't use Bactrim because I'm allergic to sulpha antibiotics, so I'm wondering if anyone out there has experience with all this? What other meds are out there to help you avoid nasty infections?

[Daggar]

hello norcalian,

Pentamidine is one alternative to Bactrim-sulpha antibiotics to avoid pneumonia if you don't have a choice due to allergies.

As for infections and such the doctors will be watching your blood counts closely and should be telling you when you really need to avoid risks outside the normal recommendations of avoiding anyone who has a cold or viral issue.

Our daughter Holly went back to school in January while on ctx and she did wear a medical mask for a bit until her numbers improved and fellow students/teachers were made aware of her condition and her need to avoid those who were sick.

Quite scarey at first but everything went well and the mask came off fairly quickly. She did experience one cold but her blood counts were fairly strong at that point and it ran its course pretty much the same as pre-WG.

She is now scheduled to switch to Imuran in a month.

One thing to remember if you do have to do ctx - drink, drink, drink.... water that is! The drug is very toxic if it sits in your bladder so make sure you have lots of water with you at all times.

[jmmilliorn]

Welcome Norcalian. You came to the right place. Good info here from many who have been there. I too am experiencing the weakness--even after the RTX infusions. From what I heard, you don't get your strength back right away, even if the RTX helps the WG symptoms. Do your thighs feel like jello? Are you light headed, too. I am. I have anemia with my WG. I also spent a week in isolation in the hospital when my symptoms sent me there the last time. The lab techs wouldn't do a CT scan of my lung until it was proven that I didn't have TB. I really had no symptoms of TB at all except a chronic cough. (mine was dry). They did discover a nodule in my lungs when they finally did the scan. I have finished my RTX infusions and will be taking Azathioprine for maintenance. I'm anxious to get my strength back and get off the prednisone. Gained 40 lbs since Jan.

[norcalian]

I also spent a week in isolation in the hospital when my symptoms sent me there the last time. The lab techs wouldn't do a CT scan of my lung until it was proven that I didn't have TB. I really had no symptoms of TB at all except a chronic cough. (mine was dry). They did discover a nodule in my lungs when they finally did the scan.

It's interesting to watch how people react to TB. When I was in the ER I had this nurse that would run in and out of the room. I would have to quickly yell questions at him as he was scurrying out of the room. On the plus side, when they moved me up to the hospital for the majority of the stay, they put me in an isolation room which was a really nice corner room looking at the green hills (Northern California's really green this time of year). So that was good.

I will look into contacting Dr. Chung at UCSF if the need arises. I have Kaiser (which might not be as familiar to most of you outside of California - but it's huge here). So she definitely would not be in my network...but my Doctor is sort of the main Rheumy guy here and has been working with Wegener's for over 30 years. And I think he's pretty good about consulting with other doctors as he's talked about doctors that he works with from UCLA to UCSF and Stanford. One thing about Kaiser is that they have been treating with Rituxin for the past 5 years (the organization is run by doctors rather than shareholders - which I think cuts through a lot of that red tape about payment). They were the only HMO that would cover it before it was approved by the FDA. It sounds like the rheumatology dept. pride themselves on being on the cutting edge of treatment. But of course if I have any doubts, I have this site and I have all of you and I can always try calling Dr. Chung. Especially if things get weird with this disease, as it sounds like it can (like I mentioned...my kidney involvement started after I was put on high doses on prednisone and Imuran). When I was told in the hospital that I had Wegener's my first thought was "thank heavens it's not cancer." I really didn't know anything about the disease. It's hard not knowing what to expect.

[Sangye]

That sounds good, norcalian. I hope it continues to work out with Kaiser. All I've ever heard are nightmares about their coverage!

[norcalian]

That sounds good, norcalian. I hope it continues to work out with Kaiser. All I've ever heard are nightmares about their coverage!

Yeah - Kaiser gets a pretty bad rap. I grew up on it and it was always fine. However, I never had a serious chronic condition. Trust me, I'll be on here ranting if it turns sour.

[norcalian]

hello norcalian,

Pentamidine is one alternative to Bactrim-sulpha antibiotics to avoid pneumonia if you don't have a choice due to allergies.

As for infections and such the doctors will be watching your blood counts closely and should be telling you when you really need to avoid risks outside the normal recommendations of avoiding anyone who has a cold or viral issue.

Our daughter Holly went back to school in January while on ctx and she did wear a medical mask for a bit until her numbers improved and fellow students/teachers were made aware of her condition and her need to avoid those who were sick.

Quite scarey at first but everything went well and the mask came off fairly quickly. She did experience one cold but her blood counts were fairly strong at that point and it ran its course pretty much the same as pre-WG.

She is now scheduled to switch to Imuran in a month.

One thing to remember if you do have to do ctx - drink, drink, drink.... water that is! The drug is very toxic if it sits in your bladder so make sure you have lots of water with you at all times.

Thanks for the information. I'm glad to hear that your daughter was able to resume school and some normalcy in her life.

If/when I start the cytoxin I'm sure I will have more questions about protection from infections. right now I'm practically OCD about my interaction with people and germs. but I'm also just a couple of weeks out of the hospital...so there seems to be a bit of figuring out what I can and can't do in terms of being around people and things.

[marta]

Hey norcalian,

Welcome to the gang ;)

I have a similar story to yours and am also allergic to Bactrim. I've been taking Dapsone to prevent the PCP and it hasn't given me any problems thus far. It's apparently hard on the liver and I have had liver issues, but I and the docs now don't think it has anything to do with the Dapsone (we tried eliminating it from the 'cocktail' for a bit but my liver function tests didn't change with that variant). My liver function tests are back to normal now, I'm still taking Dapsone and my disease isn't active, so I believe the liver flares up when the disease flares up.

It's funny that you say that you were relieved to hear that you have WG instead of cancer - not funny ha ha, but funny interesting. I was just thinking this the other day, about how lucky I am to have had the seemingly horrible experience on my first day in ER of being told I have secondary, metastasized lung cancer... it has given this whole Wegener's experience a whole different twist. On day two when they told me it might be something different, and then after the lung biopsy when it was confirmed I was actually relieved to have a diagnosis of Wegener's. I too didn't know what it was, but it offered a chance of life - regardless of the drugs and everything else - I got a second crack at watching my girl grow up and having her remember who her momma is and spending more time with the love of my life - my hubby.

Welcome to the group and I look forward to getting to know you and all of us moving forward and learning and laughing and crying (on those rare days) and getting better together.

[jmmilliorn]

Before i was diagnosed, my internist sent me to an oncologist because he thought i might have cancer. i spent the next six weeks being poked and having blood drawn. It all ended with a very painful bone marrow biopsy in the right hip. When the Onc. didn't find any cancer he said, "Too bad it isn't lymphoma or something. I could cure that. I'm afraid you have something worse." Next came the rheumatologist. The first one didn't figure it out either, so we went to the Cleveland Clinic and got my diagnosis. Some days I think the Onc. was right, I might be cured by now, but mostly I'm just glad I got diagnosed correctly.


Hey norcalian,

Welcome to the gang ;)

I have a similar story to yours and am also allergic to Bactrim. I've been taking Dapsone to prevent the PCP and it hasn't given me any problems thus far. It's apparently hard on the liver and I have had liver issues, but I and the docs now don't think it has anything to do with the Dapsone (we tried eliminating it from the 'cocktail' for a bit but my liver function tests didn't change with that variant). My liver function tests are back to normal now, I'm still taking Dapsone and my disease isn't active, so I believe the liver flares up when the disease flares up.

It's funny that you say that you were relieved to hear that you have WG instead of cancer - not funny ha ha, but funny interesting. I was just thinking this the other day, about how lucky I am to have had the seemingly horrible experience on my first day in ER of being told I have secondary, metastasized lung cancer... it has given this whole Wegener's experience a whole different twist. On day two when they told me it might be something different, and then after the lung biopsy when it was confirmed I was actually relieved to have a diagnosis of Wegener's. I too didn't know what it was, but it offered a chance of life - regardless of the drugs and everything else - I got a second crack at watching my girl grow up and having her remember who her momma is and spending more time with the love of my life - my hubby.

Welcome to the group and I look forward to getting to know you and all of us moving forward and learning and laughing and crying (on those rare days) and getting better together.

[Sangye]

I had no idea my lungs were hemorrhaging (for 3 months!) and was only going to holistic docs to treat what we thought was Lyme's disease and secondary pneumonia. The x-rays worsened and looked odd so the radiologist recommended a chest CT. That looked really bad-- lungs were solid gray. I got in to a pulmonologist within 24 hrs who did history/ exam then showed me the CT. Then he said, "I don't know what this is, but it's not cancer. There are 5 diseases that can cause this. They're all awful. One is easy to fix but it has a high emotional cost for you." Then he proceeded to describe in detail each of the diseases. Wegs was one of course.

There is a lung disease called "bird fancier's disease" which is a severe allergic reaction to birds--usually poultry. It causes lung hemorrhaging and is deadly. The cure is simple-- avoid exposure to birds. In the history I'd told him I have a cockatiel and he had picked up on how dear he is to me. It was quite touching that he knew it'd be painful if I had to give him up. Since the lab work would take a week to come back, he said I had to send my cockatiel to a friend's house while we waited. After 4 days I couldn't stand it and went to get him. I hadn't improved anyway! The dx of Wegs wasn't a relief for me, other than the huge relief of being able to keep my sweet bird.

[norcalian]

Finally some good news...I thought I would have to start CTX this week due to rising protein clearance levels indicating inflammation in my kidneys. I just finished my fourth infusion of Rituxan, I'm on 80 mg/d of prednisone and 150 mg/d of Imuran. My Rheumy said that if my protein levels didn't improve or got worse that he would have me stop the Imuran and start oral CTX. fortunately this week, my protein clearance stayed about the same and my overall protein levels dropped (also, my blood creatinine levels have been steady in the normal range and my CRP is back to normal)....so I get to hold off on CTX (at least for another week - hopefully indefinitely). I'm wondering if the Rituxan is starting to finally kick in. Whatever it is, it's the first piece of good news I've had since Dx.

[elephant]

That is great news and it might be the RTX kicking the wegs out of your system. When will your doc start to taper you off the pred?

[Sangye]

Very good to hear. Remember, rtx doesn't really start working until 6 weeks after the 1st infusion, so you have a little more time. It also takes a couple months after that to really get up to speed. You should just get better and better.

[norcalian]

When will your doc start to taper you off the pred?

I certainly hope soon. It's been a little more than three weeks and I'm getting all of the fun side-effects. I grew a beard to try to conceal my emerging moonface...I shutter to think what our food bill will be this month. While on disability I might eat my family out of house and home. Acid reflux, weakness, name the side-effect, it seems that I'm getting it. so the sooner I can begin what I'm told will be at least a nine-month process of tapering prednisone the better. My rheumy said that he would like for my cough to have significant improvement and for my protein clearance numbers to start coming down before he starts tapering the pred. So hopefully soon. I meet with him in a little over a week. We'll probably talk about it then.

[pberggren1]

I certainly hope soon. It's been a little more than three weeks and I'm getting all of the fun side-effects. I grew a beard to try to conceal my emerging moonface...I shutter to think what our food bill will be this month. While on disability I might eat my family out of house and home. Acid reflux, weakness, name the side-effect, it seems that I'm getting it. so the sooner I can begin what I'm told will be at least a nine-month process of tapering prednisone the better. My rheumy said that he would like for my cough to have significant improvement and for my protein clearance numbers to start coming down before he starts tapering the pred. So hopefully soon. I meet with him in a little over a week. We'll probably talk about it then.

I noticed you mention acid reflux. Are you on a stomach acid reducer. If not you should be because pred can do some nasty damage to the stomach lining.

[norcalian]

Very good to hear. Remember, rtx doesn't really start working until 6 weeks after the 1st infusion, so you have a little more time. It also takes a couple months after that to really get up to speed. You should just get better and better.

So maybe it's the Imuran...or just a good week. I'm anxiously waiting for the point for the RTX to start taking effect.

[norcalian]

I noticed you mention acid reflux. Are you on a stomach acid reducer. If not you should be because pred can do some nasty damage to the stomach lining.

Thanks for the note. The discharging physician at the hospital sent me home with 20mg of pepcid to take at night...which did nothing. My GP just put me on 40mg of pepcid twice a day...which seems to be doing the trick.

[elephant]

I know how you feel about the eating. When I was on 60 mg of prednisone..I consumed breakfast, lunch and dinner by 10am. It was insane!

[jmmilliorn]

I have felt "OK" since my first infusion 7 weeks ago and my 2nd infusion 10 days ago, but haven't really noticed any progress toward strength. Still have shaky legs and shortness of breath. (SOB) I'm down to 12.5 mg/day on the pred. I feel pretty good afternoons and evenings, but I have figured out that sometime during the night my dose of pred runs out and when I wake up--sometime between 3AM and 5AM most days, I feel really sick and shaky--a feeling that gets gradually better throughout the morning and early afternoon until another afternoon/evening of feeling better. I think this has become worse since I reduced the steroids the last two times--every other week by 2.5 mg. Tonight when I wake up at 3 AM, I'm going to take the 2.5 mg and then take the other 10mg after breakfast and see if my mornings are any more tolerable. I think I'm just "out of juice" in the mornings and suffering from withdrawal like all addicts. What do you guys think of my theory?


Very good to hear. Remember, rtx doesn't really start working until 6 weeks after the 1st infusion, so you have a little more time. It also takes a couple months after that to really get up to speed. You should just get better and better.

[RudiK]

Hello Mike, my pred dosis was also gradually reduced from 60 mg (I was flying while on 60 mg) to 10 mg, which brought me down to earth again. I experienced the same issues until I split the dosis to 5 mg in the morning and 5 mg at lunch/early afternoon. My energy level is more balanced now.

Good luck, I hope splitting helps you too.
Rudi K.

[norcalian]

I know how you feel about the eating. When I was on 60 mg of prednisone..I consumed breakfast, lunch and dinner by 10am. It was insane!

I feel like a Hobbit (for any LOTR fans out there)...eating first and second breakfast, elevensies...etc, etc. I take pred four times a day. So I'm always eating...of course I'd probably be eating even if I was taking it just once.

[Jack]

I think the theory may well be correct. I also seem to run out of Pred and my routine seems to be that I'm at my worst in the morning, but always recover by the afternoon.

For some special occasions I allow myself to take extra Pred and can then have a really good day. Not something I could recommend and not to be used as a regular way of life, but sometimes a little quality of life is more important. Pred has already ruined my body beyond repair so I don't have much to lose. :wink1:

[norcalian]

Mike - I'm with you on the "weak legs" thing. I have a good days and bad days with feeling week in the legs...but mostly I feel like my large leg muscles are wasting away. My Rheumy said that Prednisone has the opposite effect on muscles as anabolic steroids (the kind that Barry Bonds was so fond of) in that they can negatively effect muscle growth and strength. I don't know if anyone has heard of this. His suggestion was resistance training for my legs. I started doing some light Yoga in the morning and walking on an incline on my treadmill. It's helped some, but I still have days (and muscles) that are still feeling pretty week. So I've started using bands to do some additional resistance work. I'll see how that works. I have an appointment with my rheumy in a week. I plan on following up on all of this and see if I can get more information.

[Sangye]

Norcalian, pred is a catabolic steroid-- it causes muscle wasting and fat retention. (Anabolic steroids do the opposite). Those exercise suggestions are very good. Take care not to overexert or over strain--pred makes it easy to rupture tendons and ligaments. I would rather see you walking on an incline during only part of a treadmill routine. Otherwise you're overstressing the Achilles tendon-- an easy target for rupture.

[Sangye]

Mike, 12.5 mg is still a lot of pred. You really shouldn't be running out of steam like that. Are you overdoing it during the day?

[norcalian]

Norcalian, pred is a catabolic steroid-- it causes muscle wasting and fat retention. (Anabolic steroids do the opposite). Those exercise suggestions are very good. Take care not to overexert or over strain--pred makes it easy to rupture tendons and ligaments. I would rather see you walking on an incline during only part of a treadmill routine. Otherwise you're overstressing the Achilles tendon-- an easy target for rupture.

That is great advice! thank you Sangye. I will do.