What is the standard treatment NOW when you have a flare?

[daystarr8]

Since my last post. Pretty sure I'm having a flare. First one in 11 years so I guess my body got a rest from it. I think this is relapse #5 or perhaps 6. I was hoping that it was just the really bad air here that everyone would say but NOT! My last treatment didn't end so well that's when they found the huge tumor that cytoxin had created from all the past treatments so I am full of anxiety to have to start another treatment. I've never had anything but cytoxin and prednisone and I've read all about the new drugs. So please tell me what your doctors start you on. I'm having upper respiratory and airway issues like always. Do far I've been on prednisone 30mg for 4 days and it's responding but I know it's only temporary if used alone. I haven't been in to see my doctor yet we spoke over the phone. She is new to me for my dr of 34 years retired last year go figure! Do most of you start on rutuximab ? And prednisone IV ? If so please tell me your side effects and for how long are you typically on the high dose of prednisone till the taper starts? I believe my rest will come to an end next week. Any info would be greatly appreciated.

[Donna-from-Philly]

My insurance wanted me on cytoxin before rutuximab. We fought it and won and the rtx worked well for awhile. I had fatigue the day after but nothing horrible. I had tried methotrexate and was really sick when I took that.

[Pierre42]

Earlier this year I had a flare (due to Immune System freaking out after surgery to reattach Achilles tendon, said freakout is _apparently_ fairly common/par-for-the-course).

This was detected early - "technically the numbers say you're having a flare, but only just", I was about to tell my rheumatologist how awesome my health was - no visible signs, no "health issues" of any sort (aside from the obvious pain/inconvenience/etc recovering from said surgery) and I Feel Great.

I had been on zero Prednisolone for over 6 months, and 10mg Methotrexate weekly. They put me on 60mg Pred, discontinued my MTX and added 150mg Imuran (Azathioprine) daily. (tablets only, no IV required this time around). The specialist also talked about perhaps using Rituximab should a larger hammer become necessary.

I was only on the high dose of Pred for ... 6 weeks, my numbers were not terribly far past normal when we started, and I responded well so we started tapering pretty quickly. Which means my body didn't have much time to initiate side-effects (YAY).

That was July: I'm now down to 10mg pred , zero by the end of the year and after that we plan to taper the Imuran to 75mg possibly less.

I do like a challenge: I'm also in the middle of losing weight (yes _while_ I'm on Pred) because middle-age+overweight=blood pressure issues and "I have enough crap going on without _that_ AS WELL".

Since the start of August I've lost almost 20kg, should be in normal BMI range by the end of the year. While I was on 60mg I visibly bloated a little, once I got below half that everything pretty much returned to normal.


The answer to "how long until you begin the taper" is "as soon as your numbers look good enough". If you respond well to treatment they'll taper you sooner than if you fail to respond.


If you like surfing the bleeding edge ....

RITAZAREM: An international, open label, randomized controlled trial comparing rituximab with azathioprine as maintenance therapy in relapsing ANCA-associated vasculitis - VCRC (http://www.rarediseasesnetwork.org/vcrc/research/5525.htm)
Rituximab Vasculitis Maintenance Study - Full Text View - ClinicalTrials.gov (http://clinicaltrials.gov/ct2/show/NCT01697267)

[mrtmeo]

I have heard that the accumulation of too much cytoxan can be a problem, but hoping someone can chime in on that.
The RAVE trial showed an improvement over cytoxan when used as a maintenance for flares.
I wish I knew which on was better for lungs and kidney issues, but I don't.

[Pierre42]

I have heard that the accumulation of too much cytoxan can be a problem, but hoping someone can chime in on that.
The RAVE trial showed an improvement over cytoxan when used as a maintenance for flares.
I wish I knew which on was better for lungs and kidney issues, but I don't.

Cytoxan = Cyclophospmamide.

Amongst other URLs, Wikipedia is your friend:

"Cyclophosphamide is itself carcinogenic (file:///wiki/Carcinogen) and may increase the risk of developing lymphomas (file:///wiki/Lymphoma), leukemia (file:///wiki/Leukemia), skin cancer (file:///wiki/Skin_cancer), transitional cell carcinoma (file:///wiki/Transitional_cell_carcinoma) of the bladder or other malignancies."

Oh the irony!

Specifically the Docs warn of ~30% increased risk of bladder cancer, so drink until you're urinating pretty much constantly, then drink some more....

Being treated with with cyclophosphamide (either high doses or long time) is only a good thing because the alternative is so ... final.

It is really (really) nasty stuff and the sooner you can move on to less toxic treatments the better.

Which is why there's lots of research into alternatives, eg Rituxan - so far studies are indicating it may be applicable for inducing remission instead of (ie equally as effectively as) cyclo, with less toxicity/side-effects. Though it has its own issues they're (a) mostly rare (b) more immediate eg infusion reaction and (c) not cancer ... On the other hand they are _severe_.

Apparently if you can tolerate treatment by Rituxan it comes close to being "a wonder drug" for many people.

[annekat]

For someone who has had enough Cytoxan in the past to cause a tumor in the bladder, I seriously doubt they would use it on you again. You could go on RTX. But if your flare is only in the nasal and sinus area, they might choose AZA or MTX instead, depending on the severity of the flare. Since you had problems with MTX, I guess that leaves AZA if not RTX, or maybe Cellcept (mycophenolate mofetil, sp?). MTX seems to be a more common choice than AZA, and many of us have gotten over the nauseous feeling and had it work well for us. Good luck!

[Wegetarian]

Guess it really depends on how severe the condition is. Like when I was first diagnosed we only used Pred tablets + MTX since I only had wegs in the upper airways. When I flared this summer I had it in my kidneys and lungs, so they gave me pred IV's and started me on cyclophosphamide IV's. My understanding is that CTX (cyclophosphamide) is the most efficient way to stop wegs when its threatening, but its more toxic than the other drugs. RTX takes a longer time to take effect, I remember some people from a FB support group saying that it took a few months before it became in full effect.

I don't know how much CTX you received, but I guess they keep track of how much you get since they don't want to give that much due to the risks increasing.

Did MTX cause you nausea or how did you feel bad? I know some people take some meds for the nausea. Some take it as an injection, not sure if that would help. I was also on MTX for almost a year, and I really don't miss the nausea that I got from it. I never asked for any meds for that, since I was popping so many pills already that I didn't want any extra meds unless I absolutely need them.

[Gamewell]

Hello, I am new on this site - I have had Wgs since 1988 - Effecting most organs. Kidney down to 15%. Was very sick. On Life support and all. Have been in remission for over 20 years. My labs are all great. Still have sinus problems at time, some light nose bleeding. Still on a low dose of pred. 2.5 every other day. Wondering if I sometime flare? Doctors seem to think it is from the damage that it did years ago. Anyone else have Wgs that long and what problems they have over the years. Thanks!

[annekat]

Nice to meet you, Gamewell.... I know some on here have had Wegs that long, and eventually relapsed and are now dealing with it like the rest of us.... others may not have relapsed, every case is different. One of them will see this, no doubt, or if you post in New Members Introductions and tell your story there, more may take notice. True, some of the permanent damage can cause symptoms even without a relapse or flare. I'm glad you found us.

[annekat]

Did MTX cause you nausea or how did you feel bad? I know some people take some meds for the nausea. Some take it as an injection, not sure if that would help. I was also on MTX for almost a year, and I really don't miss the nausea that I got from it. I never asked for any meds for that, since I was popping so many pills already that I didn't want any extra meds unless I absolutely need them. I've been on MTX a couple years or more and have gotten over the nausea. From the beginning, I split the dose, taking half at night and half the next day, and found that to really minimize the side effects. For me it was more of an overall yucky feeling than actual nausea. But then, I'm on a pretty low dose, only 15mg., and I started with 10mg. for several months, which really wasn't enough. Anyway, now, I usually don't feel it except when I don't get enough sleep or something. I've seen splitting the dose recommended on here quite a bit.

[Wegetarian]

Effecting most organs. Kidney down to 15%.

Did your kidney function improve over the years, and how has it affected your daily life?

[daystarr8]

Yes 34 yrs and counting since diagnosis. I'm on my 11th year of remission now. I am currently struggling with upper airway damage. I am in need of airway dilation and my sinuses are greatly scared. I have a consultation on Nov. 17th with a airway/throat and voice surgeon and I'm very excited about it. I'm tired of being so short of breath. My biggest problems from the damage is crusting and them drying up in my airways including that area where the sinus drain into the back of the throat. I also have a hole in my septum and I'm going to see about a repair of that as well. For what I have researched about air flow in the nose it can make certain areas more dry if it's altered in any way For these symptoms I use gel in my nose and water to help. Saline spray tends to dry me out more. Recently been using mucinex and it works well to loosen things up.

[miracleshappen08]

Wow! Daystarr8, I have only had it for 6 years and I have the same airway problems. I will be having my 7th throat surgery soon. I have not been able to get my nose fixed yet. When I had my first dilation my airway was the size of a straw. Now, I know why I couldn't breathe.
I hope all goes well on the 17th!!!