Wegeners/GPA for 41 years - Transplant for 25 - Having a Relapse

Hi everyone,
I am happy to have found this site and find all of you here. I am 54 and have had Wegeners for 41 years. I was originally misdiagnosed when I was 13 with Henoch Scholein Purpura. Once the WG/GPA was diagnosed, I took oral Cytoxan for a year and the WG/GAP went into remission. I have had a kidney transplant for 25 years and had one for 10 before that. Like many of us with a long-standing chronic illness, my medical history is super-complicated. That being said, I live a very full life and feel blessed to be living it!!!!!

One of the things that is so amazing is to see how medicine has changed for the better in those 41 years!! When I first got sick in 1978, the ANCA test, didn't even exist! Even Cytoxan wasn't being used much then and so the steroids, Imuran and 4 years of dialysis helped push me along long enough that I was able to be helped by the ANCA and Cytoxan in 1992. Now Rituxan seems like a miracle drug compared to Cytoxan.

Four years ago, 21 years in to my transplant, my nephrologist lowered my transplant drugs. I was so happy and felt great. Her thought was that I didn't need as much immunosuppression as I got older and less Neoral would be better for me in the long run. About a year later, I started having ongoing sinus and ear issues that have gotten progressively worse. I have always had sinus crusting and a deviated septum but it was all manageable. In the last 3 years I have had over 30 doctor appointments (with Primary Care, ENT, Rheum) about my sinuses and ears. I have had to be the one pointing out that this is a flare, demanding treatment and it has been a difficult long road. I was not seeing a Rheum regularly because, my old Neph used to managed everything. Nowadays, the new younger Nephs only do kidney stuff and won't hear much about my sinuses. My neph forced me to get a Primary Care doc and see my ENT, which I think only diluted how many issues I was having.

Anyway, I am likely starting Rituxan in 12 days. I wish I could get it tomorrow. My nose is so painful. My steroids have been increased to 20mg/day and I am pretty miserable, worrying that my nose will cave in while I sleep! Yikes! The bleeding has slowed but I have granulomas in my nose, it's completely blocked and super-painful. Just hoping I can go 12 days. Plan is to see a top Vasculitis Specialist in 10 days just to get a second opinion and then be ready for the infusion 2 days later.

I am worried about having an allergic reaction, simply because I am the type of person who is allergic to cats, horses, some meds, etc. Does that matter?? Praying I don't react and it works! Feeling grateful that I can take it instead of the Cytoxan. Thanks for listening everyone!

Be well,

Kim

Hi @Kim,

I guess we should congratulate you for surviving 41 years with wegs. Amazing!

You probably won’t have any reaction to rituximab. I have allergies to horses as well as hay fever. Since I was diagnosed, I’ve taken 10 mg of loratidiene and 10 mg of phenylephrine each day to keep my sinuses dry. Both are OTC and have worked well for me.

Do you rinse your sinuses to flush out the crusties? That may help soothe your sinuses as well.

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Originally Posted by Pete

Hi @Kim,

I guess we should congratulate you for surviving 41 years with wegs. Amazing!

You probably won’t have any reaction to rituximab. I have allergies to horses as well as hay fever. Since I was diagnosed, I’ve taken 10 mg of loratidiene and 10 mg of phenylephrine each day to keep my sinuses dry. Both are OTC and have worked well for me.

Do you rinse your sinuses to flush out the crusties? That may help soothe your sinuses as well.

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Hi Pete,
Thanks for your message! Good to hear words on encouragement on the Rituxan! I have only been doing nasal rinses to clear crusts for about the last 20 years. I started doing them to help clear a clogged tear duct in my left eye and really worked well. I have worked full time for 30 years and it could get a little embarrassing when I would tear up during a meeting or presentation. :biggrin1: With this relapse, which I think actually started after a severe cold in 2016, I haven't had much crusting. It's been more "bleedy" and raw escalating to super-inflammed granulomas. Over the past year, they have tried to treat it with a couple of intermuscular steroid shots, steroid tapers, antibiotics, adding a steroid solution to the nasal rinse, mupricin and even using steroid eye drops in my nose. It would seem to get a little better and then just run back to intense inflammation.

I am hoping that after the Rituxan, things will settle down and the crusts return like they had before. Compared to this, the crusting was great, which I am sure sounds strange! It's probably a long shot and I may have more ongoing maintenance issues but I am trying to keep a positive mindset. I know I have lost more of my septum but my ENT assures me you can live without it.
I usually take Allegra during allergy season but with all of the extra sinus rinses I was doing this year, my allergies weren't too bad. I'll have to see was my ENT recommends regarding ongoing maintenance.

Well thanks again for your note. It's good to know that a tendency towards regular allergies didn't significantly impact your treatment. Take care and be well!
Kim

Hi Kim,
Welcome. Wow, your Wegeners journey has taken you down a long hard road!
What you have already done for the nasal problems are all the things I would suggest.
As far as the Rituxan infusions I did not experience problems. My infusions also included Benedryl and prednisone. After the first series of infusions I did have some leg pain for a day or two, but nothing too bad. Since you are still working are you scheduling your infusions for Friday’s?
I do have a hole in my septum, but not yet saddle back. At first, my heart dropped to my stomach when I visualized my nose sinking. But there are others on this site who offered me comfort. They suggested wearing glasses to fake it out. I also talked to my ENT, a surgeon, who has performed some of those surgeries. I will be 70 on my next birthday, and I just don’t want to go down that road. So for now, I have just kind of put it out of my mind until when and if that happens.
You have been through so much. I am sorry you are going through more now. At least you know your way around doctors offices. Many go to rheumatologists who are not Wegeners experts, but I doubt that is the case with you. I wasted some time before I found my way to treatment at Duke. I hear great things about Cleveland Clinic too.
Sending prayers your way.
Masha

Thank you Masha for your prayers and words of encouragement. They mean a lot! Still counting the days (and hours) to my appointment at Hopkins Vasculitis Center next week and my infusion two days later in Washington DC (fingers crossed all goes as planned). My infusions are scheduled for Thursdays but I work from home so I should be ok.

I am still in a lot of pain with my nose and my Rheum said I could go in for another steroid shot but I am hoping I can just wait it out until the Rituxan, as I know I will get extra steroids at that time. Hope to make it for 1 more week! Just trying to get through each day as best as possible but I feel pretty miserable.

I already have a "saddle nose" but my ENT told me years ago that "people would pay money for that nose" - probably just trying to make me feel better :). Praying my nose does droop any more.

Thank you again for your note and prayers. I can feel them and they lift my spirits.
Kim

You have had WG for a year longer than I have. I think we probably have a lot in common from what you have shared so far. I have had allergies to many antibiotics and when I got my first Rituxan infusion it was very early and had not yet been approved for WG.
I have to say I was pretty scared of an allergic reaction because of things I had read. I had no allergic reaction. Even if I had a reaction I know now the infusion nurses are well trained and skilled in dealing with one. They will take good care of you.
I have had many infusions since then and tolerate it pretty well. You will "feel " some things though so don't panic. I think most of that is from the horse dose of solumedrol they give you with the Rituxan. Anybody would feel a bit off from a shock like that. Now- I often fall asleep during infusion. It's just boring and I ignore feeling a bit off.

It goes pretty smooth for most people- you can be one of those people.

Welcome to the family, Kim. Your story is amazing and can give hope to many. I tag @gilders who had kidney transplant few months ago. I guess he will be glad to read your story.

If you get rtx according to the protocol, ususlly there are no reactions. It means getting 100mg steroids before plus benadryl or similar (I get phenergan and 2 paracetamol). They wait 30 minutes after those meds and only then start. Also rtx should be delievered in a very slow rate at first and gradually increase the rate.

Drink a lot. The reaction that I get is very low blood pressure even 60/40. So I drink and eat something and walk a bit and then its better.

Please update us how it goes. Sending you prayers.

Welcome Kim (and thanks for tagging me in Alysia),

41 years is great, especially with kidney involvement!

It's very helpful to read that lowering your anti-rejection meds may have caused the relapse/flare. I'm only 4 1/2 months into my "transplant life" and we're still trying to get everything settled. I am on double the usual amount of antirejection meds (and at higher dosages) mainly because I do have some antibodies against the donor organ. I'd don't think the drs are likely to reduce my dosages too low due to the antibodies. But if they ever try to reduce the meds I'll make sure they're taking into consideration my W.G.

Since reducing your antirejection meds, has there been any decline in kidney function, or signs or rejection?

Best wishes and thank you for joining this forum. It's good to have someone to talk to who has more experience in kidney "transplant life". Alternatively, as your last transplant was 25 years ago, I may be able to give you advice on newer drugs and treatment.

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Originally Posted by me2

You have had WG for a year longer than I have. I think we probably have a lot in common from what you have shared so far. I have had allergies to many antibiotics and when I got my first Rituxan infusion it was very early and had not yet been approved for WG.
I have to say I was pretty scared of an allergic reaction because of things I had read. I had no allergic reaction. Even if I had a reaction I know now the infusion nurses are well trained and skilled in dealing with one. They will take good care of you.
I have had many infusions since then and tolerate it pretty well. You will "feel " some things though so don't panic. I think most of that is from the horse dose of solumedrol they give you with the Rituxan. Anybody would feel a bit off from a shock like that. Now- I often fall asleep during infusion. It's just boring and I ignore feeling a bit off.

It goes pretty smooth for most people- you can be one of those people.

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Hi Andrew,
Thank you so much for your message and kind words. They really give me a lot of comfort before my infusion tomorrow. I will prepare for the "feeling" of solumedrol. I think I remember it from the old days so I will expect that. I will let you know how it goes tomorrow. They put me on 40mg of steroids two days ago because my nose is such a mess and it sounds like they want to leave it at about 30 through these first two infusions and then taper (very slowly) back down to the 5mg I have been taking for 25 years. I just keep thinking that by the new year, I will be feeling a lot better.
Thanks again for the kind words - holding good thoughts that I will also have a boring experience :rolleyes1:! I will let you know. Kim

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Originally Posted by Alysia

Welcome to the family, Kim. Your story is amazing and can give hope to many. I tag @gilders who had kidney transplant few months ago. I guess he will be glad to read your story.

If you get rtx according to the protocol, ususlly there are no reactions. It means getting 100mg steroids before plus benadryl or similar (I get phenergan and 2 paracetamol). They wait 30 minutes after those meds and only then start. Also rtx should be delievered in a very slow rate at first and gradually increase the rate.

Drink a lot. The reaction that I get is very low blood pressure even 60/40. So I drink and eat something and walk a bit and then its better.

Please update us how it goes. Sending you prayers.

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Thank you Alysia for the welcome and kind words. I appreciate the reminder to drink a lot and the other details on the infusion. Many thanks also for the prayers - I feel them and they lift me up! I will let you know how it all goes. Thank so much!
Kim

Quote:

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Originally Posted by Alysia

Welcome to the family, Kim. Your story is amazing and can give hope to many. I tag @gilders who had kidney transplant few months ago. I guess he will be glad to read your story.

If you get rtx according to the protocol, ususlly there are no reactions. It means getting 100mg steroids before plus benadryl or similar (I get phenergan and 2 paracetamol). They wait 30 minutes after those meds and only then start. Also rtx should be delievered in a very slow rate at first and gradually increase the rate.

Drink a lot. The reaction that I get is very low blood pressure even 60/40. So I drink and eat something and walk a bit and then its better.

Please update us how it goes. Sending you prayers.

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Thank you Alysia for the welcome and kind words. I appreciate the reminder to drink a lot and the other details on the infusion. Many thanks also for the prayers - I feel them and they lift me up! I will let you know how it all goes. Thank so much! :smile1:
Kim

I'll be looking forward to hearing how your infusion goes. I usually take my computer to play around on because it takes so long. I think for a first time infusion they go extra slow if I remember right.
That sounds like a solid plan for your steroids. I know its not great to go through but you will be on the other side of it in better shape than before.

Kirk

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Originally Posted by gilders

Welcome Kim (and thanks for tagging me in Alysia),

41 years is great, especially with kidney involvement!

It's very helpful to read that lowering your anti-rejection meds may have caused the relapse/flare. I'm only 4 1/2 months into my "transplant life" and we're still trying to get everything settled. I am on double the usual amount of antirejection meds (and at higher dosages) mainly because I do have some antibodies against the donor organ. I'd don't think the drs are likely to reduce my dosages too low due to the antibodies. But if they ever try to reduce the meds I'll make sure they're taking into consideration my W.G.

Since reducing your antirejection meds, has there been any decline in kidney function, or signs or rejection?

Best wishes and thank you for joining this forum. It's good to have someone to talk to who has more experience in kidney "transplant life". Alternatively, as your last transplant was 25 years ago, I may be able to give you advice on newer drugs and treatment.

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Hi Gilders,
Congratulations on your transplant and thanks for the note! In the beginning the antirejection drug doses are always high and they take a few months to lower them to your baseline dose.

Just to clarify....My transplant drug (Neoral or Cyclosporin) was lowered after I had had my transplant for 21 years. The reason for that is two fold. 1) My old nephrologists had retired and so I went to a new (somewhat inexperienced) nephrologist. 2) There is a practice nowadays to try to get patients off of more immunosuppressants, namely prednisone. My body doesn't it make its own cortisone anymore (after so many years on steroids) so I have to take a little every day ie) 5mg. My nephrologist thought lowering the Neoral might be a good alternative. I was ALL FOR IT! I was so happy at first and my kidney didn't mind at all (my creatinine has always run around .9 or 1). A year later, I started having really bad sinus/ear issues. That was 3 years ago, they raised my Neoral back last year and tried a couple of shots and tapers of steroids but nothing has really helped. Now my creatinine is up to 1.14, my nose is a mess and I am really worried I will lose the bridge and they are finally going to start Rituxan. I have really had to advocate for myself but I still feel like this has taken forever, mostly because I wasn't being seen routinely by a Rheum until last year.

I have had this transplant for 25 years and had one for 10 before that. I was originally misdiagnosed with HSP Vasculitis so it wasn't until I rejected the last transplant that I was officially diagnosed with GPA/WG, even though looking back, all the docs agree, I probably had GPA since I got sick in 1978. Was also on dialysis for 4 years as well, waiting for that last transplant.

I know I sound like a worrier and I am a bit scared of the Rituxan but I am fortunately still functioning ok and living my life. I haven't missed much work and I try to get on the stationary bike a couple of times a week. Overall life is good but this setback has given me one more reminder of how fragile my health is living with GPA.

You are going to do great with your new kidney and I am sure they will get the meds stabilized! What drugs are you taking for it? These days, I think a lot of transplant recipients take Cell Cept and Tacrolimus, but I am not sure. Most don't take prednisone.

Great connecting with you and the other members of the site. Thanks again for your note and I will keep you posted on how I do with the Rituxan. -- Kim

It is great to read about people surviving decades of GPA even when the treatment was not as refined as it is today. This should be reassuring for people recently diagnosed.

Hi Kim,

Welcome! Have you had the rituxin infusion? How are you feeling? We’re there any problems? I find myself that I feel pretty good for a few days (that would be the solumedrol) my body sure does like steroids! But I really don’t feel at me peak for about 80 days. It’s frustrating. I hope this finds you well!
Natty


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Hi Natty,
Thanks for reaching out! I did have two infusions of Rituxan. I had some shortness of breath with the first one so they gave me extra steroids and benedryl and ran it over 9 hours - long day! The second one went a lot better and I got the infusion in just 4.5 hours. No major side effects. I have been tired since both infusions, still working from home but taking a couple of cat naps each day. I think it has to do with the steroids. They have me on a long taper starting at 40mg/day and I'm now at 35mg about to get to 30mg tomorrow. The pain and inflammation on the outside of my nose is almost gone but the inside is still very inflamed. I feel like there are some subtle positive changes happening in my nose and there is definitely a lot less bleeding! My nose seems to be running a lot and I'm hoping that will slow down as all of the internal inflammation calms down.

It's really helpful to think about the 80 days you mentioned. I have been thinking in terms of 2-3 months for the Riuxan to kick in but putting it in terms of days is also helpful. I only had the first infusion just 17 days ago so I still have a ways to go. The doctor said I will probably get another infusion in 6 mos. and then one in a year and my transplant drugs (cyclosporin and 5mg Pred) might hold me from there. I am not sure how they will make that determination but really feel like things are moving in a positive direction.

This has been an unbelievable experience. I didn't understand that I could ever have a relapse after being treated with Cytoxin in 1992. Feeling very grateful for the Rituxan and for this community which has been super supportive.

Thanks again,

Kim

Your post makes me feel bad that I even complain about my issues with GPA, you have it much worse than I do. My kidney functions seem to be fine at this point, I am sorry you are going through this, best wishes that Rituxan works, they may give you an injection of steroids before infusion, benadryl and Tylenol. Sherry

I am glad to read your update. It sounds like you are on the mend and sometimes that comes in baby steps. I am wishing you all the best for the RTX to kick in ASAP for you. Please take good care of yourself. Thanks for your update.

The same with me during the infusions, I just want to sleep, I think it is the Benadryl, lol

Thank you for all of the kind words. I feel like I am slowly feeling better. Still on 30mg of Prednisone which seems to still be giving me some insomnia as well as some weight gain and just general fatigue. Sometimes I feel like my heart is pounding and wondering if that’s the steroids too? It’s been about 27years since I have been on this high of a dose so it’s hard to remember.

Will taper down to 25mg in another week. The inflammation in my nose is resolving very slowly but still it has been only 1 month since my first of 2 Rituxan treatments. Grateful that I have gone the past month without another ear or sinus infection! Will have my labs done next week to make sure this hasn’t had an impact on my kidney.

Hanging in there, working (and trying to work out!) and thinking that I will be feeling so much better in a couple of months!!

Many thanks,

Kim




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