Diagnosed in Decemember 2009

[Lightwarrior]

Hi, I'm happy to find a forum to help make sense of WG. I am a Clinical Nurse Expert in an ICU and I also teach Bachelors and Masters level nurses in an academic online environment.(not always a help, sometimes a little knowledge can be dangerous :-) )I have two daughters, one lives with me and five grandchildren who all live me. The oldest grandchild is 17 and the youngest 2. My oldest grandsons Boxer puppy who is almost a year old and a cat who the babies call bunny.

In July I started noticing that I went into anaroebic metabolism much quicker than I should be (started getting really tired from walking from my carinto the hospital). Prior to July I had a string of odd things which I chalked up to my 100,000 mile check up at age 54. In Februrary I broke my foot, in March I had a retina spontanesouly detach and in a CT that was done in July for a kidney stone it picked up an odd wedge shaped infiltrate on my lower lung. SInce this was odd, they did a repeat CT with contrast which also showed a Thyroid mass (benign after investigation, mulit-nodular goiter) one Doc thought I had an unusual pnx and another one thought I had Valley Fever, one of the Intensivists I worked with thought there was more too it and urged me to keep looking. Then I progressed to joint stiffness that was acutely increasing, started with my hands and then my knees, my ankles and finally it got so bad I couldn't walk from my office to the front of the unit. I have a great Nurse Practiotioner as my primary and she ran some tests, my ANA was negative, my Rheumtoid Factor was negative so she sent me to a Rheumatologist, meanwhile on Thanksgiving day I broke out in a rash that looked like petichiae/purpura (sometimes being a nurse helps, sometimes I think it makes things more scary). This guy took me seriously, out of a long list of blood test the only positive test was the C-ANCA. I hurt so badly that they started me on 60mg of Predisone on December 9th and 250mg Cytoxan on December 14th. The joint relief occured within 2 daysk. I now have a pulmonologist, nephrogolist, Rheumotologist along with my primary. So far all of my numbers for kidney function are normal and my last CT showed a lot of the infiltrates clearing.

I understand from reading and the Docs that I will need to be on steroids high dose for another month or so and then start tapering down to 40mg, and that I will need to be on the Cytoxan for about a year, at which time I can switch to methotrexate. At first I was unable to sleep and spent all of December and part of January cleaning and organizing every inch of my house while my family slept. My rheumotolgist starte me on 5mg amotryptoline for sleep and I have been sleeping three to four hours at a time. The last two weeks have been much tougher. I am tired all of the time.

Today it hurts my back and hips to stand for more than a few minutes, I seem to be okay, just sleepy when I am lying down or sitting in my recliner.

How do I know when I am in remission?? I want to go back to work on Feb 1. How immunocomprised will I be? If my WBC is within normal limits can I be in the unit?

I'm just starting this journey my overwhelming feeling about all of it is gratitutde. We figured it out in time, my friends and family have been amazing, the drugs have backed off the horrible joint pain. i'm confused about what is normal and when I should worry.

[Jack]

Hi Lightwarrior and welcome to the Forum.

Glad you got a quick diagnosis before too much harm was done. It is good to see that your medics take you seriously and your own knowledge will help a lot. Does your Rheumatologist have much experience of vasculitis and Wegener's in particular? It is an odd disease where experiece counts for a gread deal. It is not all written down in the books as I'm sure you will soon find from reading some of the posts on here.

12 months on Cytoxan is quite a long time because the drug has serious long term side effects. It is perhaps more usual to switch to something less aggressive such as Azathioprine as soon as the disease seems to be coming under control. The best judge of this is probably yourself and how you feel. The blood tests available don't give a yes/no answer to your state of remission.

I'm sure plenty more will be along to offer their advice.

Jack.

[elephant]

Hi Lightwarrior, I agree with Jack..being on cytoxan for a year is not good. I know if your on high doses of prednisone it can destroy bone and cause necrosis esp the hips.
If your feeling tired and not well, I recommend you don't work. What does your Rheumy think? Do you go to a WG specialist? I go to Cleveland Clinic in Ohio and drive 10 hours one way. It is worth the trip. I have a local Rheumy and see this one when I feel the need or it will be once a year.
There could be many reasons your tired...one of them still active Wg....secondly the medicine for sleep may be too much...
Let your Rheumy know how you are feeling esp if these symptoms are new. They may need to do CT scans and blood work. I am a RN too. Last job Case Management.

[Sangye]

Hi Lightwarrior,
Welcome to the group! Good to have another health care professional on board!

Jack and Elephant are old hands at this Wegs business. I'm usually the one who does the major nagging about getting a Wegs specialist no matter how your treatment is going. Regular specialists are not skilled to handle Wegs or to understand the nuances of this sneaky disease. The Wegs specialists aren't fond of the "1-yr on ctx" plan that was the standard protocol until a few years ago. Like Jack said, they usually transition you onto a milder drug like imuran, methotrexate or Cellcept after 3-6 months of ctx.

My original docs told me the 1-yr ctx plan, too. They seemed okay at first--willing to research, etc... But their ignorance about Wegs nearly killed me and wound up doing permanent damage. I went to Mayo AZ and got better care but not a Wegs specialist. I limped along, experiencing numerous life-threatening complications and never getting into remission. Now I'm at JHU, a major Wegs center, and finally getting excellent care. I'm still in pretty bad shape, 3.5 yrs post-dx (4.5 yrs after first major symptoms). This is why I really nag on this issue!

[Lightwarrior]

Thanks Jack, I felt like a year was a long time also. I am only the second Wegeners that my Rheumatologist has seen, my pulmonologist has only read about it and my nephrologist manages winter vistors when they are her with Wegeners so he has the most experience. He is managing my meds but is used to me asking for I want for patients so I have to remind him that I am the novice and he has to be the expert. Since I write most of the protocols and order sets based on the latest evidence for the pulmonologist and nephrologist they are very open to suggestions I have so I will bring up the time frames for meds. My rheumologist seems to know more about it and he was the most panicked at my first visit and pushed the hardest to get me started on treatment. Those last few days before I started the prednisone I honestly would have almost welcomed death to escape the pain (I wasn't sucidal).

I live in a rural border community three hours from Phoenix and San Diego. I wonder if it would be possible to arrange for consults with Cleveland Clinic or one of the Docs I work with has connections with the Boston medical community??

I am impressed with the level of support evident on this forum and add it to the many positive things that have happened since I was diagnosed.

[Lightwarrior]

Thanks elephant, I have had hip pain the last three days that prevents me from standing for more than two or three minutes at a time. I am a lightweight when it comes to medication, I may try to take the amitryptiline every other night, thanks for the suggestion. Nice to meet another RN, I love case managers they make patient care flow and keep people from falling through the cracks.

[Lightwarrior]

Sangye,
Thanks for the welcome it feels so good to find a group of people going through this. You really put a voice to my fears, that well intentioned, lack of knowledge my kill me or cause more damage. My Rheumy told me my last visit that he was afraid I was too far into the pulmonary symptons and that he was really scared for me when he first saw me. I do credit him for nagging my other Docs to move more quickly. Which they did when he scared them. Oh, by the way I value naggers. :-)

[elephant]

Lightwarrior- love the name. It is best to see a Rheumatologist ( WG specialist) because that way they get to know you and they do a great job head to toe assesement and check out every symtom/medicines and so forth. You could fly to one of the four WG specialist in the country. Look into the Vasculitis Foundation and see the references. I see Carol Langford at the Cleveland Clinic, Ohio. Love her! I know people on this website see other WG specialist and are pleased with them too. Get that hip checked out. You probably should call your doctor today about your symptoms...
It could be other things going on... drug reaction and such...not to alarm you....

[Lightwarrior]

I do seem to remember that femoral head necrosis is either part of WG or a side effect of Pred or Cytoxan. I'll make the call, since this is all new ground I'm walking on I'd rather be safe than sorry. Thanks.

[Sangye]

Yes, take it from me, you really can't manage your own care with Wegs. It's WAY too complicated and bizarre. The best thing is as Elephant says-- travel to a major Wegs center and see a Wegs doc in person. Then s/he can work with your local docs at home. You'd have a personal connection to the doc and could travel there every few months. I can't emphasize the importance of this enough. It's costly and time-consuming. But I haven't been able to work in 3.5 yrs because of improper care.

The major Wegs centers are JHU (Baltimore), Cleveland Clinic, Mayo Rochester and Boston University.

The Vasculitis Foundation has a list of these consultant docs who specialize in Wegs. They'll work with your docs for free, even if you've never met with them. However, this means your only communication with them is through your local doc-- no way to ask questions or engage in the very useful conversations about Wegs.