My introduction

[Trilabby]

Hi everyone,

I found this group a couple of weeks ago and kind of been lurking but I think I am ready to introduce myself and tell my story. I am 62 which sounds so old to me but I feel like I felt 20 years ago. I have always been pretty healthy. I rarely got sick. My experience with hospitals has been 35 and 32 years ago when my two boys were born. Other than that I had knee surgery a few years back.

I work at a boarding Kennel part time or should I say I worked at a Kennel. I haven’t been back since my diagnosis. Around Labor Day the beginning of September I was working extra shifts and felt extra tired. I figured it was just working the extra shifts but I also was dripping wet from sweat which just seemed so odd. I had also developed a bad cough and flu like symptoms seemed to be coming on too. So I called my boss and asked to be covered for the next couple of shifts. I made an appointment with my doctor for the next day. With my symptoms she had me go for a CT Scan of my abdominal and a chest X-ray. The chest X-ray showed a mass in my left lung. My doctor prescribed antibiotics thinking it was pneumonia. The mass did not seem like the normal mass for pneumonia but she went ahead and treated me with antibiotics anyway. I progressively got worse with the cough and weakness and asked my husband to take me to the emergency room at our local hospital. I was admitted after another Ct scan and another chest X-ray of my chest. The mass had grown from a couple of days before. I stayed in for 3 days and felt better so they sent me home. I was fine for about a day and a half and very quickly went down hill and needed assistance getting around the house. I went to get out of bed and slid to the ground and couldn’t get off the floor. I sat on the couch and couldn’t stand. It was the strangest thing! My legs and arms just was not working. I had my husband take me back to the doctors office and he wheeled me into the building. My doctor told me to go back to the hospital but to a different hospital. I was admitted again. This time I had a crew of doctors trying to figure out what was wrong with me. They thought maybe fungal because I worked with animals, or infection. I had another CT scan of my chest and another chest X-ray. Again the mass was bigger. I was still coughing like crazy. I would sweat like crazy and soak my gown and bed. I needed help getting to the bathroom. I have never been this sick in my life. My husband and two sons were freaking out not knowing what was going on. My oldest son is a third year pharmacy student and knows too much for his own good. He was trying to diagnose me along with the doctors. My younger son that is in the military took a weeks leave to be with me.

The doctors at the second hospital decided that for the tests they wanted me to have couldn’t be done there so after 4 days I was transferred to the main campus. After a 4 th set of CT scans and chest X-ray I settled into my third hospital. I saw a ton more doctors along with interns from all areas, infectious disease, cardiology, internal, rheumatologist, Pullmonary. They all had their own idea of what was wrong with me. After a ton of tests they decided to do a scope and get a biopsy of the mass. Through blood work and results from the biopsy and a 5 th CT scan this time of my head they settled on Wegener’s. So after three hospitals in three weeks I had my diagnosis which was pretty quick from the sound of others stories. It made me a little uncomfortable seeing the doctors get so excited about my diagnosis. They started to bring in students to interview me because the diagnosis was rare. They said that all doctors read about it in textbooks but rarely see it. So I was put on Prednisone and quickly felt better. They sent me home and by then it was the end of September.

I have been waiting for the insurance to approve my treatment of Rituximab and finally today the 5 of November I went in for my first treatments. Well I got as far as the Benadryl, Tylenol and steroid. When they started the Rituximab after about 20 minuets I broke out in hives and I started to have trouble breathing and coughing too. I called my nurse and three more nurses showed up. They took me off the Rituximab and gave me more Benadryl. They called my Rheumatologist but of course she is out of town for the week. So now I don’t know what they will do to treat me for the Wegener’s if they can’t use Rituximab. Has anyone experienced a bad reaction to Rituximab? What was used instead?

Well that is my story. Wouldn’t you know I would get something rare and the one drug that is used to treat it I can’t use. Lucky me!
thanks for letting me share
Ellen

[andrew]

Hi Ellen!
Welcome! You've had a relatively quick trip between initial sickness and diagnosis which is great. I was interviewed by some medical students after diagnosis as well. They do get excited by something different for sure :)

There are a bunch of other drugs that can be used such as Methotrexate infusions and Cyclophosphamide which is a 'sledgehammer' and probably out of scope for your treatment if they have you starting immediately on Ritux. There are oral drugs as well such as Imuran and Cellcept although initially the infusions will probably be used to bring things under control. I've never had Ritux myself but there are plenty here that have and I'm sure you'll get something more specific than I can provide :)

Thanks for posting your story! Please feel free to ask any questions at all :)

Andrew

[BenHornsby]

Hi and Welcome

I hope they find a treatment that agrees with you soon, for me it was prednisone and Ratix and mostly just infusions unless I have a flare up.

I did get very bad rashes at the start of this disorder before being diagnosed on my limbs but never in connection with the infusions as they did not start till some time after and was stable on 40mg of pred before they started the infusions.

Ben

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[NatriceRomeo]

Hi Ellen. I know Rituxin can cause severe reactions. I’m sorry that has happened to you. There are other drugs available, however they may not be as effective. There are also drug trials depending on the type of of rheumatologist you see. I know mine is involved in one. Good luck! I will be thinking of you!


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[Pete]

Hi Ellen, and welcome to the forum. I was diagnosed at age 64 (now 71) after a similar, but shorter onset. I was quite healthy before I got sick, and can now happily report that I’m back to near normal. I was initially treated with cyclophosphamide for 16 months (about 10 months too long according to my rheumatologist), had a brief (and scary encounter) with azathioprine, spent two years on methotrexate (until I got tired of the fatigue I had for
a couple days after my weekly dose, and have “graduated” to rituximab (on it uneventfully for four years).

There are other meds available to treat GPA, but most have side effects. Some, you can learn to tolerate, and others are dangerous. Ask your doctors about side effects no matter what they prescribe and what you should do if they appear.

Best of luck with your future treatments.

[Trilabby]

Thanks all for your response and encouragement. I will find out Monday what the doctors will put me on. Always a waiting game it seems like for me. Take care!

[BenHornsby]

Good luck for Monday, Weggie's are very skilled at waiting....

[jmq]

Hi Ellen,

Welcome. I had a similar introduction to this disease as yours. I had never been sick in my entire life before this hit me. Was also diagnosed as a pneumonia with masses in my lungs. Moved pretty quickly and I had to be on high doses of prednisone. Got to be as high as 80mg. Don't wish that amount of prednisone to my worst enemy. The prednisone slowed down the disease but I had all kinds of problems as a result of being on such high doses of prednisone.
Then I got a Rituxan infusion and nothing happened for about 4 months. That's how long it took to do make a difference on my symptoms and ANCA markers. The first time I had the infusion, I didn't get hives but my throat started to close up. They had to stop the infusion and give me more Benadryl. Then had to contact my doctor for how to proceed. They couldn't get a hold of my doctor but there was a registered nurse at the Infusion Center and she made the decision to proceed but at a slower pace. It worked in that I didn't have the reaction when they slowed it down and after an additional shot of Benadryl. They were able to finish the infusion, although it took like 8 hours to do so.
Once the infusion kicked in (4 months later), I started feeling much better. I am now undergoing my second infusion of the year and no reactions so far.
They need to try it again but change the dose per hour to be slower and give you more Benadryl.
Hang in there. Once you are treated appropriately, it will be fine. Be patient as it sometimes takes time to figure out what works. It's such a rare disease that sometimes it's hard to find doctors that are well-versed in the treatments.
Let me now if I can help.
Jeannette

[Trilabby]

Jeannette,

It does sound like you had a very similar experience with your diagnosis. I wish I could have continued with the Rituximab but the nurses didn’t feel they could without authorization plus it would have taken beyond 5:00 to complete the infusion. My doctor was supposed to call today to tell me what the plan is now but of course I didn’t hear from her. This is all very frustrating. Thank you and everyone for taking time to write me!
Ellen

[Alysia]

Welcome to the forum, Ellen. I am sorry that your rtx IV didnt go well. Indeed a slower pace might have helped. Also, how much steroids did they give you before starting ? There is a protocol how to give rtx and it includes 120mg IV steroids before, 30 min pause and then statrting the rtx very slowly. Too many times, the nurses dont give it as they should. Do you know if in your case it was according to the protocol ?

[jmq]

Yes, I agree with Alysia. That's what they did last week when I had my rituxan again (2nd time this year). I go for the 2nd round of the 2nd infusion this Thursday. I'll pay attention to what they are putting in the IV. For sure, the benadryl before. I'll pay attention to how much steroids they include with the rtx. But they stopped every 15 or 20 minutes before increasing the speed of the infustion to make sure everything is okay.
Like I said, I'll pay close attention on Thursday when I go.

[Alysia]

Yes, I agree with Alysia. That's what they did last week when I had my rituxan again (2nd time this year). I go for the 2nd round of the 2nd infusion this Thursday. I'll pay attention to what they are putting in the IV. For sure, the benadryl before. I'll pay attention to how much steroids they include with the rtx. But they stopped every 15 or 20 minutes before increasing the speed of the infustion to make sure everything is okay.
Like I said, I'll pay close attention on Thursday when I go.

Streoids in IV can be 100mg or 120mg, both are ok.
Sometimes they consider body's weight.

[little jerry]

Hello and welcome aboard our world.
About 6 yrs ago when my adventure started, I was given an IVIG at the normal speed. In around 5-10 min. pain began to grip my low back and quickly traveled up the "pain scale" and made me say hay, Hay, HAY!! My nurse ran in and turned pump off. This made the pain stop. She told me because of kidney damage this will happen to me. By turning the pump down the pain stopped. She said that sometime a product that is too cold will also hurt. Now when I get blood or RTX, saline, anything I will tell the nurse to go slow. Now no problem.
I know this is not hives or a allergy, but it is a reaction that my body does to me.
Now there is more than one drug to send you to remission and I think that each one has different side effects. I think that RTX is the newest "go to" drug with the least often side effect.
Best wishes on your newest journey.

[Trilabby]

Hi Alysia,

I don’t know how much steroids the nurses gave me to begin with but they gave me Benadryl and Tylenol too with time to let it all take affect. I’ve spoken with the doctor and she wants me to have the cyclophosphamide once a month for 4 months now. So I am waiting again for insurance approval. I’m not too thrilled about having to take this new medication with the side affects and plus having it stretch out over 4 months too! I’ll just be feeling better I’m sure and have to go in and do it all over again. I’m really feeling down about all of this right now. Trying to stay positive but have a real hard time doing that! I’m glad I can come here and see how others are dealing with GPA.
Ellen

[Trilabby]

Jerry,

The nurses asked me if I had pain in my back but I didn’t. My head started to itch like crazy and I broke out behind my ears and all along my neck and side of my face in the hives. When my throat started to make weird noises and it was hard to breathe I pretty much freaked out. Luckily the nurses were right on it to give me more Benadryl. But that shut it down for the day and now the doctor wants me to have cyclophosphamide and I hope I don’t have a reaction to it. I am not thrilled about having to take it in the first place because of the side affects but I want to get this treatment over with. I just wish the Rituximab had worked for me. My son is a third year Pharmacy student and he describes Rituximab as a sniper just taking out the B cells and leaving everything else alone while cyclophosphamide will just be like a hammer and take everything out. I just want to get things going!
Thanks for taking the time to respond and to listen to me.
Ellen

[Alysia]

Hi Ellen,
I understand your concerns.
What you had, was an allergic reaction. I think that Aneinu 's son had a similar one. Maybe he can consult more about it.
You have the right to get the info from the nurses and to check what was given to you at that day. If you will find out that they didnt give you 100 IV steroids before, I think you can try again the rtx, this time as it should be.
Hang in there. This way or another, you will soon fight back the WG beast.
We are with you. Please update us.
Sending prayers.

[Aneinu]

Hi Ellen,
I understand your concerns.
What you had, was an allergic reaction. I think that Aneinu 's son had a similar one. Maybe he can consult more about it.
You have the right to get the info from the nurses and to check what was given to you at that day. If you will find out that they didnt give you 100 IV steroids before, I think you can try again the rtx, this time as it should be.
Hang in there. This way or another, you will soon fight back the WG beast.
We are with you. Please update us.
Sending prayers.Hi Ellen,

Sorry to hear about your ordeal.

Yes, Alysia is correct. My son (22 now) had four bouts of rtx. Two of the times (and perhaps a third) they couldn't finish the dose because of his reaction. One time they needed to shoot him with 100 mgs of something in order to get him breathing again. He didn't get the hives, but they called it an allergic reaction to the rtx. Personally, I believe it's because of the speed that it's given. He did have an allergic reaction once a week afterwards, but they're not sure if it's related.

Bottom line, after four attempts and after the wegs returning each time, the doctor switched him to cyclophosmide (sp) for 90 days. It was something we wanted to avoid, but we had no choice. He's finishing it now and the plan is to begin maintenance with methotrexate. We will say how that goes. Thank God, he is feeling well now (chronic coughs and cold, some fatigue, no real hair loss besides his hereditary genes) and able to study in school.

Wishing you well.

And all my brothers and sisters here.

Take care.

A

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[Alysia]

Hi Ellen,

Sorry to hear about your ordeal.

Yes, @Alysia (https://www.wegeners-granulomatosis.com/forum/member.php?u=7230) is correct. My son (22 now) had four bouts of rtx. Two of the times (and perhaps a third) they couldn't finish the dose because of his reaction. One time they needed to shoot him with 100 mgs of something in order to get him breathing again. He didn't get the hives, but they called it an allergic reaction to the rtx. Personally, I believe it's because of the speed that it's given. He did have an allergic reaction once a week afterwards, but they're not sure if it's related.

Bottom line, after four attempts and after the wegs returning each time, the doctor switched him to cyclophosmide (sp) for 90 days. It was something we wanted to avoid, but we had no choice. He's finishing it now and the plan is to begin maintenance with methotrexate. We will say how that goes. Thank God, he is feeling well now (chronic coughs and cold, some fatigue, no real hair loss besides his hereditary genes) and able to study in school.

Wishing you well.

And all my brothers and sisters here.

Take care.

A

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Thank you, Aneinu. I am glad that your son is doing well with the ctx. It is a powerful drug. Sending prayers for him and your family from Israel. Shabat Shalom.

[Trilabby]

Alysia,

would the the amount of steroids be the same for Cyclophosphamide? That is what my doctor wants me on now instead of the rtx. Would everything be the same like giving me Benadryl, Tylenol and steroid and letting everything start working before giving me the cyclophosphamide? And again just a real slow drip?
All of you have been real helpful! Thank you!
Ellen

[Trilabby]

Aneinu,

Having an allergic reaction along with trouble breathing must have been really hard with each treatment for your son. I certainly freaked me out with my allergic reaction. I wasn’t totally surprised though, because I am allergic to everything. I just spent 5 years going in every other week for allergy shots. Sometimes I think that is why I am allergic to everything! I’m glad he is doing better and hopefully the cyclophosphamide was not too bad for him. I haven’t seen much said about cyclophosphamide from the group. I would love to see what people that has taken it reactions are for it.
Thanks again
Ellen

[Pete]

I haven’t seen much said about cyclophosphamide from the group. I would love to see what people that has taken it reactions are for it.
Thanks again
Ellen

Cyclophosphamide is still used as induction therapy. As such, it’s generally effective and relatively inexpensive. However, long term use can adversely affect the bone marrow, and bladder cancer is a somewhat frequent side effect (usually presenting several years following use. (I’ve been off it for five years and no issues so far. I watch for blood in my urinalysis very closely.) I was on it for 16 months (about 10 months too long according to my wegs doc) and it worked very well for me.

I briefly tried immuran (azathioprine), and that was a disaster. My next drug was methotrexate which worked pretty well until the side effect of fatigue for a day or two after my weekly doses became too bothersome. I added rtx in 2013 and came off mtx about a year later. I had a very mild flare in 2013, so I was on both for awhile. I’ve been on an annual course of rtx since 2013.