My wife got busy

[Empty bucket]

Hello everyone. My first post. I've been lurking for a month or so and decided to jump in. My name is Dusty. I live on the Mississippi River in Sunshine LA. This is just south of Baton Rouge. I am a 56 year old male. I was diagnoised in May 2008. Prior to my diagnoisis, I was wide open in everything I did. That is why my bucket is empty if you know what I mean.
Here we go. I started feeling bad and simply out of sorts in the summer of 2007. My sinuses were giving me trouble. I did not have allergies or sinus problems prior to this. I went to my GP several times until January 2008. During this period I was given meds for sinus infections. The problems not only persisted they actually worsened. My GP referred me to an ENT in Baton Rouge. The ENT ordered a CT of my sinuses, but scheduled surgery on my first appt. He said if by chance there was major improvement, he would cancel the surgery. The results were bad so I was prescribed heavy does of antibiotics; he wanted the sinuses as clean as possible before surgery. Things continued to go downhill. I had surgery on April 1, 2008. He said if ever anyone needed sinus surgery I sure did. After surgery he informed my wife that the surgery went well but something was growing so he had taken several cultures. I was back home that afternoon. I felt OK for a few days then things started going downhill again. When I went for my post-op visit, I could barely walk into the building, pain in my knees and my head felt like it was going to explode. This continues for six weeks, my ENT continues to say that we just did not realize how bad my sinuses were and it would take a while to heal. My wife knew that this was not normal and made an appointment with my GP for a physical. She insisted on going with me, she felt that this doctor knew me and would listen. She also told him that one of my sisters had lupus. He orders extensive bloodwork. The results showed markers of RA. I was then referred to Rheumy, which would be a couple of months.
Meanwhile back to ENT, by now my wife knows this is not just a SINUS INFECTION, we ask about the results of the culture that is pending, all others were negative. ENT said the pending culture was negative. By now, I cannot hear and I have an eye infection. I tell my ENT that I never understood how anyone could be in so much pain that they would take their own life, but I could now see how it might happen. He said he had a friend who was a psychiartrist and wanted to know if I wanted to see him. My wife had to control me, but that was coupe de grais. He sends me to an infusion center for a pic line. After insertion I had to go for an x-ray to be sure it was in the right place. It was. A few days later, I am back at the ENT. He wants to know if I am aware of the nodules on my lungs. Well no I wasn't so he ordered another CT. He was out of town for a week. His nurse called and wanted me to start prednisone,another ENT in the practice had ordered the prednisone after reviewing the CT report. My wife did not fill the script because regular ENT said to stop the prednisone.
Alarms are going off everywhere. Our niece who is a nurse is coming by and she is very concerned - she goes home and tells her mother that I will die if something is not done soon. She and her daughter, who is also a nurse come again. They want me to go to the emergency room, but I am too sick. My wife is doing a lot to research and has decided I should go to the Mayo Clinic . Our niece comes again and convinces me that I have to do something because I am definitely not getting better. She said they are going to make the arrangements. I am so sick, I said let me see ENT one more time and anyway I needed to get the pic line out. She informed me that I would need the pic line whereever I went. (Her father had just died and she was not going to let me die.)
Emotions are fragile by now, I can not hear, my eye is bulging and my head is in constant pain. Someone calls a friend of mine who is a doctor in Kansas. He calls my wife and after much discussion, he tells her to get ENT doc to send him a copy of my records. He reads the reports and discovered a note from the pathologist that reads "Wegeners needs to be considered" but my ENT didn't read that or missed it.
My ENT's nurse calls early the morning of my appt and wants to know if I have been taking the prednisone. My wife told her she had not filled the script because my regular ENT had told me to stop taking it. The nurse wants me to go for another CT and my wife blew up. She said I was in so much pain I could barely walk and we were coming to pick up my records and go somewhere to get a diagnosis. The nurse said to come in now, we would not have to wait. I see ENT and he agreed I needed to be hospitalized. I was admitted that afternoon and three days later I was diagnoised with WG. I spend 7 days in hospital, have another sinus surgery and tubes in my ears while in the hospital. I am prescribed IV preds and ctx. In addition to ENT, I am assigned a rheumy and a plumony. The journey begins.
Summary of treatment to date:
sent home on 50 mg preds and 50 mg ctx. Improvement begins in about two months. Begin tapering the preds to 0 in 4 months. Increased ctx to 200 mg. Bactrim 3X per week. Sinus problems continue but am basically feeling OK. Got pneumonia mid Dec. Treated and recovered.Back on ctx in Jan. 09. Feeling OK, no pain just sinus problems. Switch to Imuran Feb. 09. Allerigic reaction. Back on ctx 250 mg and 20 mg pred. March 09.Tapered off preds in 1 month. Been on ctx 100-250 to date.
According to my rhuemy my anca levels were off the chart when I was in the hospital. In four months time under his care it was negative. The positive anca levels returned after I was not able to take Imuran. They remained low and I felt OK. My "Limited Wegener's" has been managed with very little preds. Ctx seems to work, but it is getting the best of me now. I have taken it for almost 2 years. I should own stock in bottled water industry.
Here comes the big question. For the past month, I have been prepped for my first infusion of rtx. It is scheduled for 4/5/10. Do the benefits of rtx outweigh the risks? Should I be worried about PML, my rheumy isn't.
Thanks for listening.
One more thing, if it wasn't for my wife I would not be here.

[Cindy M]

Hi Empty Bucket, welcome to our group. I know this is not the place you want to be but there are some very informative people here. I rely on there advice and opinions. When I hear stories like yours I realize how lucky I was to be diagnosed so quickly. I would love to give you some advice but I am still struggling with trying to find the right meds. I know you will be getting some very good answers to your questions so I will leave that in the other's experienced hands. You have come to the right place. All the best.

[pberggren1]

Hi Dusty:

I'm glad you found us. It sure sounds like you went through the mill.

You mention "Limited Wegener's". I personally don't believe it exists as well as most other folks on here. Your case sounds quite similar to mine and my case sure wasn't limited.

What is PML?

If you have a good WG specialist, not just someone who treats a handful of patients, and the specialist is confident about using rtx I would definitely try it. It is much less toxic than ctx.

[Jack]

Your case sounds very much like my own before diagnosis, but it took renal failure on top of everything else before they realised that I had more than sinus trouble!

In my own case, the IV ctx and pred had an immediate effect and I felt better in spite of the permanent damage caused. I would have expected you to have few symptoms by now if the treatment was right for you, but in any case you should not still be taking ctx. The potential for long term consequences mean that you need to minimise its use.

I'm not too clear about who is in charge of your treatment now, but you must make sure that they have plenty of experience with the treatment of Wegener's. They need to be a specialist, a general rheumy will just not do.

[elephant]

Welcome ! Sangye will be coming by to nag too. I suggest like everyone else on this forum that you have Rheumatologist that specializes in Wegeners. I travel very far to see Dr Langford at the Cleveland Clinic in Ohio. It is worth it! They are on top of your case and all the research that is going on with Wegeners patients. Wishing you wellness soon.

[JanW]

Hi, Empty Bucket, and welcome. Sorry you had to end up here, but glad you made it.

Rtx is definitely much less toxic than ctx, and Jack is right, you really want to minimize the use of it over your lifetime. As I am sure that you know, it is a highly toxic drug that has been shown to increase the risk of bladder cancer. Some of us have to use it, but what I've read seems to indicate that the specialists like to do it for no more than 3-6 months before switching to something with lower toxicity. Rtx has been shown in studies to be very effective in the treatment of WG.

I am confused -- are you being treated at Mayo now?

You will find that people on this board don't like the term 'limited Wegeners' it happens to be what my own doctor told me I had an when I came on here and announced it, I got a little bit of an earful. I continue to remind people that it really is a 'term of art' that doctors use to refer to WG that doesn't have renal involvemnt -- it says nothing about whether you will get renal involvement, it doesn't mean that you aren't as sick or that you have a better prognosis than someone who doesn't have the limited version. Both Sangye and I have limited WG in that we have no renal involvement at this time -- the state of our health is very different, however.

[Sangye]

Hi Dusty,
I'm here to nag! Okay, first I'll welcome you and say that I'm glad you survived all that and found this group.

I'm with Jack on the ctx-- you've been on it way too long. It's too dangerous and also if it were the best drug for you it would have worked in the first 7 months before you got pneumonia. Your doc is using ctx when he probably could have used mtx or Cellcept.

I definitely agree that you need a Wegs specialist involved. The main centers in the US are Mayo ( in Rochester, Minn or Florida), Cleveland Clinic, Johns Hopkins (Baltimore) and Boston Univ. It's very much worth traveling to a center to see a Wegs doc in person. I wouldn't want to be without my JHU team, that's for sure.

I'm glad you're starting rtx meanwhile. PML is a concern for anyone on immunosuppressants--particularly rtx and Cellcept. It's a remote risk. There's nothing you can do about it to avoid it. My doc isn't overly concerned, either--just told me the symptoms to look for.

[Empty bucket]

Thanks to all for the warm welcome. I knew I would get nagged for not having a specialist and rightfully so. I have been treated by the same rheumy in Baton Rouge for the past 22 months. He is 15 minutes from my house along with my other docs. We just got comfortable with him. He always said my blood work looked good. He was always happy that I used very little preds after the 4th month. Plus I felt OK most of the time. I do realize now that I was on ctx to long. I'm going to have my first rtx infusion on Monday. We plan to have a long talk with my doc during or after. Thanks again for the welcome and all the great info.

[jola57]

Hi and welcome. Your story sounds so familiar. Misdiagnosis and more misdiagnosis until you are so far gone that the only option is hospitalization. Your rheumy seems to know what to do but getting some consultation from Mayo would be a good idea. I was on cytox for a year and half and am now on methotrexate. Personally I don't think that it is too long if needed but I think that maybe you have been tapering off the pred too fast and thus relapsing quickly.

[coffeelover]

aGAIN, i AGREE WITH jOLA as that happened to me. I tapered off the pred too fast and symtoms came back immediately. I see an ENT/WEgs Secialist at the Mayo in Rochester, MN. I also see my ENT/surgeon here at home, my GP, my Rheumy with WEG experience and who ever else can offer some insight. As they say "It takes a village" Welcome to this village, there are many experts here that will continue to guide (not nag) you.
Lisa Coffeelover