My first flare since diagnosis

[alicia_b]

Well it's official. I am having a flare. Its been almost 7 years since my extended hospital stay leading to the WG diagnosis. I can't help but think the flare is my fault and how am I going to tell my children. I guess I should start at the beginning...
I'm August we took a vacation to the ocean. It was wonderful, the sun, warm weather and family time. I didn't want to return to cold indiana. But we did. And the kids went back to school and we went back to work. A week later, my kids were sick. Then I became sick. Of course I brushed it off with every logical reason I could think of. My favorite was telling myself it's just allergies. It was 3 weeks later and I was getting worse. My hands, elbows and knee joints were swollen. my toes began to turn purple! So I went to the Dr. Everything sign points to a flare except for the labs. Out of the 10 tubes of blood and 3 ct's only 2 labs were slightly off. The drs said and I agree, this is a flare. My numbers dont change Much unless it's a major problem. I've doubled my Imuran added prednisone and the symptoms are decreasing. I was so excited about being down to 50 Imuran after almost 7 years and doing reasonably good. So the flare has been upsetting. What did I do? Was it caused by my taking probiotics? Maybe I pushed the drs too much bc I want off the Imuran to avoid the high cancer risk caused by it. I may never know what caused it.
In the next few months, I get to start the Rituxan treatment. I have not had since diagnosis. It didn't work then. So one of my drs questions it. But it's worth the try right? If not, we Will try the cytoxan.

[Arleta]

So sorry to hear this. I also take Imuran, but 150 mg per day. I have tried to lower it a couple times, but symptoms come back. Next month will be my 10 year anniversary of the on come of symptoms. I have not had a real flare yet...just worsening of symptoms when I overdo it. I am resigned to being on Imuran the rest of my life. I hope the treatment works and you don't have to go back on cytoxin or even prednisone. (I am a fellow Hoosier.)

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[drz]

Previous studies showed that most people relapse when they stop their maintenance meds. I believe some studies showed a 80% relapse rate within two years once they stopped their maintenance meds. That means though that many people can get off all meds and it is worth pursuing if you are not likely to have life threatening or serious damage from a flare. Like wise some people find they need to keep taking prednisone cause any time they go below a certain threshold their symptoms worsen or return. Their threshold can vary a great deal. Most people find this some where in the range between 2 and 20 mg daily to control their symptoms.

My doctors have told me I will be on my maintenance meds until they either find a better treatment or a cure for GPA. They have reassured me that many people take similar meds for other medical problems for many years. One example is people with a kidney or other organ transplant often take an immune-suppressant for ever. Many people with various arthritis take prednisone so we are not alone.

[John S]

I've been getting two rituxin infusions every 6 months for the last two years. My Dr. rcently told me that I'll be going to one treatment every six months next Spring. I have fear about this change to the protocol but trust my Dr.

[Alysia]

Sorry about your flare. Dont blame yourself. You have enough to deal with, so please be kind to yourself. It is what it is, Wg just comes and goes.
My labs also not allways indicate a flare. But my joints indicate for sure, with other symptoms.
I am on rtx since 2013, every 6 months, 1000mg x 2 IV.
Sending you prayers. Please update us.

[[email protected]]

Hopefully Rituxan will work for you. Both my son and myself were treated with Prednisone, Cytoxan, and Imuran. They kept us alive but each caused unacceptable side effects. The Rituxan has worked beautifully and it appears we each need the Rituxan to be repeated for once weekly treatments over four weeks, then repeated every four months thereafter to prevent the kind of extreme relapse I’m now enduring. I hope you have doctors who understand your disease and who can treat it successfully.

[carolynamu]

Your story sounds much like mine. I was doing great. Blood numbers looked wonderful in July. In August went on vacation to visit my daughter. I was bit by some bug (yes it was a bug bite) and it would not heal. Suddenly everything seem to go off. I had made it down to 2 mg of pred and really wanted to be off of it. I can really understand just trying and wanting to be off the meds. Sending some hugs your way. I just started rituxan myself. I have heard it works well for a lot of people.

[[email protected]]

Thanks for your response. Support makes a big difference. Yes, inflammatory markers showing up in blood tests, which many physicians rely on exclusively, are insufficient in identifying a Wegener’s/PAN relapse. Patients symptoms or clinical signs can be far more important. I realize now I may have had signs of a relapse as long as 7 months ago but allowed physicians to confuse the signs as something else. The first sign of a relapse occurred as few as 3 months following my last Rituximab and nearly ended my life in a month long emergency hospital stay with uncontrollable seizures and pneumonia. If I had had a high index of suspicion, or those around me had, this may well have been avoided.

[alicia_b]

Thank you everyone for the support and advice. I pushed the rituximab treatment to start next month. I didn't want to deal with that around the holidays.
I was brave And told my boss. I was panicking! The wg conversation is never an easy one, so I didn't go into all that. I kept it simple with, 'oh I maybe need time off work. Not sure how much though.' Given I work 2 full time jobs for them , I thought they may need some notice.

[gilders]

Alicia, so sorry to hear of your flare.
You can not blame yourself for this. I have Wegener's that affects multiple organs and I'm currently awaiting a kidney transplant due to Wegener's. Yet even though I have what could be considered an aggressive form of Wegener's, I've always tried to be drug free (after a long period of remission). I have had 4-5 relapses since I was diagnosed aged 17 (now 41) and perhaps if I'd of stayed on maintenance meds, maybe I wouldn't have had so many severe relapses and wouldn't be needing a kidney. Yet on the flip side, if I'd have stayed on maintenance meds, maybe I'd have died from cancer. We never know which is the best choice and neither do the drs, so don't blame yourself.

I do think if someone plans to stay on maintenance meds, then if possible change them every few years (azathioprine, methotrexate, Rituximab).
I had been on Azathioprine for many years and developed immature, odd-shaped, red blood cells. After stopping Aza and moving onto RTX, my red blood cells were checked 2 years later and had returned to normal shape.
My Wegener's specialist is happy for the kidney transplant to go ahead, but is concerned about me picking up a fatal infection. Usually transplant patients have an increased risk of fatal infection, but my risk is considerably more due to many years of immunosuppression. So coming of maintenance meds has been beneficial for this reason.

[[email protected]]

Alicia, so sorry to hear of your flare.
You can not blame yourself for this. I have Wegener's that affects multiple organs and I'm currently awaiting a kidney transplant due to Wegener's. Yet even though I have what could be considered an aggressive form of Wegener's, I've always tried to be drug free (after a long period of remission). I have had 4-5 relapses since I was diagnosed aged 17 (now 41) and perhaps if I'd of stayed on maintenance meds, maybe I wouldn't have had so many severe relapses and wouldn't be needing a kidney. Yet on the flip side, if I'd have stayed on maintenance meds, maybe I'd have died from cancer. We never know which is the best choice and neither do the drs, so don't blame yourself.

I do think if someone plans to stay on maintenance meds, then if possible change them every few years (azathioprine, methotrexate, Rituximab).
I had been on Azathioprine for many years and developed immature, odd-shaped, red blood cells. After stopping Aza and moving onto RTX, my red blood cells were checked 2 years later and had returned to normal shape.
My Wegener's specialist is happy for the kidney transplant to go ahead, but is concerned about me picking up a fatal infection. Usually transplant patients have an increased risk of fatal infection, but my risk is considerably more due to many years of immunosuppression. So coming of maintenance meds has been beneficial for this reason.

After both myself and my son being on azathioprine, methotrexate and Rituximab we would pick Rituximab every time of these three. So far it has proven to be far less toxic and more effective. This is backed up by solid research as well.

But goodness gracious Andrew, facing a Kidney transplant with your doctors concerns has got to be very stressful! Please let us know how this goes.Are your immunoglobulins running low?

You are right when you mention the concerns of cancer (for patients on Cyclophosphamide especially). From what little I can gather the risk is not increased for those taking Rituximab. Good luck on continuing off the maintenance meds.