New to forum, not new to Wegners

[samsgang]

Hello everyone,

We just found this site, and have been browsing it, and it is so helpful to find others who unfortunately, know the struggle. I work for an ENT, and we see several patients with this disease in our office. (back when we were originally diagnosed, it was suppose to be so rare? story to follow) however, in our lifetime, we know personally of 4 people with it, and that is besides the patients we see in our office, who have this disease.

Our story....My name is Candy (writing) and my husband is the one with Wegener's. (I guess it is GPA now?) He is part of this posting. Anyways, my husband was originally diagnosed with Wegener's back in 1997. It showed up for him in his kidney's at the time. We were still newleyweds, I was pregnant with my first child, (now he is 18 almost 19 years old) and thank God, two other children that have followed. We are believers, and find our support in God, and there are answers to prayer and miraculous stories, but understanding that not every one is a believer, that is all I will mention of that, unless asked.

Anyways, long story very short, we were diagnosed by a Kidney specialist in 1997 and his advice was "get treated NOW!" We were terrified, back 20 years ago, less was known, and it was rare, and we felt so alone. Needless to say, we got a second opinion at the UW. We were praying that my husband wouldn't have to be treated. (Okay, God is too big a part, I can't leave the fact that we prayed out.) We ended up seeing (by "accident") Dr. Kauser, the top doctor in the nation on this disease, we were later told. I was a 26 year old young women, who just picked the phone up and scheduled this appointment. We were able to get in fairly soon too. After a full day of work ups, (we prayed before we went in that God would give us direction through this doctor) the doctor set back in his chair and said, "well, if I were you, I wouldn't be treated right now." It ended up that Sam's numbers were very low. We felt sheepish like we went behind the kidney specialist back, (we were young, and he stated we didn't have a choice as to whether we would get treated) Later that week, we got a call from the Kidney specialist who called to let us know, he had the reports that we had gotten a second opinion, and that he was going to back Dr. Kauser up 100%! He said to us, "Do you know who you just saw?" to which we replied, "no" the Kidney specialist said, "you just saw the top doctor in this nation on this disease, he wrote text books that I studied out of, you had better do whatever he told you to do! I look to him as a mentor!" The kidney specialist was excited too, because he got to talk to the UW doctor on the phone.

So, we went on our merry way, 3 children and 21 years of marriage symptom free until, January of 2016. Symptoms flared off and on during this new season. (Again first time in 20 years) We honestly did not even think it could be Wegener's. It was off of our radar to be honest. We had since left that diagnoses behind. So by early April, my husband was limping at the end of a regular work day, all his joints through out his body were sore. We went to prompt care 3 different times in that time frame, and they always sent us away. Telling us they didn't know what it was. For the past month my husbands eyes had become blood shot, the doctors gave him eye drops for pink eye. That is when a light bulb went off in my head (finally:blink:, I know), because, I knew it wasn't pink eye, no one else in the family had gotten it, for one. So I got online and googled red eyes, what are causes? Boom, Wegener's pops up! We were in the doctors on Tuesday, and he was hospitalized Thursday! 3 days of massive doses of steroids. (I guess it is a common treatment, but we were completely new to this diagnoses all over again!) So Sam went through several months of 60 mg prednisone at release, then slowly weaned down, and is on a lower dose of a 1.5 year of chemo treatment)

Fast forward again to this past weekend.

Well, after a call from our Kidney specialist who is overseeing all of our labs and treatment about two weeks ago, when he stated, "he could not be happier with our progress and labs" we got a call this past weekend from a doctor we have never seen, (filling in while the other one is out), this doctor wanted us to be treated 3 days in a row again with the 1000 mg and then place him back on the 60 mg. This was all due to elevated anca titer. Our regualar doctor will back on this coming tuesday 12/27/2016. We went over tests, and results, and the only thing that went up was anca titer, all the other blood tests we monitor, especially the creatinine, is all within normal limits.
We have chosen to wait through the Christmas weekend, and we will be retested on Tuesday when the doctor comes back, and if by thursday, the results are still elevated, and if we are advised by our regular kidney specialist, we will of course, go and be treated again.

This is our story. Thank you for reading and your support.

Candy

[ThePowerCouple]

Welcome to the forum. We received a lot of great information and support on this website.
If you haven't done so yet, please check out the following website: Vasculitis Foundation (http://www.vasculitisfoundation.org).
Also, check out to see if Rituximab from www.gene.com will work for your husband.

[annekat]

Welcome, Candy. That is an unusual story, but shows how variable and sneaky this disease can be. With the joint pain and red eyes, Wegener's might be attacking different parts of the body besides the kidneys, and you might end up with a lot of trouble in the upper and lower respiratory tracts. You might consider consulting a rheumatologist with vasculitis experience (they don't all have it) who will oversee your case if things get worse in those areas and will be able to work with the other specialists such as nephrologists, ENTs, opthalmologists, etc. There are some excellent rheumies at UW and also at Virginia Mason. I'm in Olympia and am pursuing an appointment with a local one who may work for me. But if you are closer to Seattle, I'd go for those places. Good luck.

[vdub]

Had the disease for 20 years! Wow!

One of the top docs in the PNW now is Curry Koenig at U of U in SLC. He came from the Cleveland Clinic and started the U of U Vasculitis Center. He was my 2nd opinion when I was first dx'ed with the disease. He and his team are pretty amazing. When he took me on for a 2nd opinion, things happened real fast. I think I saw him 2 or 3 times in 2 days plus I saw about 4 other docs to include ENT and Neuro. After he confirmed the original dx, I had 2 or 3 follow-up apt's and then slowly weined myself from Curry and started seeing a local rheumy. I'm sure I could have stayed on with him, but I wanted to free him up for more complex patients. ( clicky for details (http://healthcare.utah.edu/fad/mddetail.php?physicianID=u0221591) )

PDX has a lot of direct flights to SLC for only $200 round trip -- such a deal!! Also, U of U has a lot of RV parking right at the hospital. As I recall, the RV parking has 30 amp, but no dump. We stayed at Hill AFB in Ogden, so I'm not all that familiar with the park at U of U.

[Alysia]

Welcome to the forum, Candy and Sam.
Wow, 20 years of "WG's sleeping" is something great ! Thank you for sharing your special story.
Is Sam only on pred ? Pred alone is in NO WAY enough to treat wg.

[samsgang]

Hello everyone. Thank you for all of your reply's. To answer the last question, yes, in addition to Pred. we were treated with Cytoxin (I honestly don't know the spelling) and the infusions, then the prednisone. Sam was completely weaned off of prednisone and switched to a "milder chemo" in October. He did fine, lab work wise and symptoms wise, until the week before Christmas. Then labs came back stating he was having a flare, (no symptoms yet.) Just the Anca Titer was going up from a past negative read. We were disappointed to already have a relapse so quickly. Ugh! I now realize even more so what a gift of 20 years of remission with no treatment was, now that we have had to be treated!

We will be starting Retuxamab (sp?) infusions this next week. And 60 mg of pred again! (Which was discouraging) The doctor said he is going to wean Sam off sooner than he did the last time from the high doses of Pred. this time around.

Does anyone know how long Retuxamab infusions take? Sam is just wondering with his work schedule how this will work out.

Sam does not like reading on here, because honestly, it freaks him out. I, however, do because I like the information, and we have already experienced the extraordinary miracle of 20 years of remission, so I know that anything can happen in each of our Weg. cases. Sam read the wrong thing I think when he got on here. I know to filter it all through a grain of salt. Not all experiences will be the same.

Some of this information can be so helpful. Thank you all for sharing, I'm learning alot.

I don't know what the future holds, but I do know who holds our future. Walked through enough valley's with Him to know I can trust Him.

(sorry, can't help it! :smile1: I can't not give credit where it is due in our case)

Candy

[vdub]

Not all experiences will be the same.
That is absolutely correct... I don't think I would be out of line to suggest that most of the regular people on the forum probably have a more active case of wegs than most. We have a lot of people who come to the forum, stay around for a month or two, then disappear for months or years. We have many, many more people who are lurkers and never join. I think the forum regulars represent a very small fraction of those diagnosed with wegs. Many wegs people are living relatively normal, pain free, symptom free lives.

[Pete]

Hi Candy,

The first rituximab infusion will probably last about five hours, assuming no complications. The flow rate is very slow at the beginning, but is gradually increased. Sam will also be given an IV steroid (solu medrol) and an antihistamine (Benadryl) before the rituximab starts. He may also get some Tylenol. If he has an allergic reaction, they will stop the infusion, treat the reaction, and resume the infusion at a slow rate.

For most of us, the side effects are pretty benign -- mild fatigue for 24-48 hours following. I haven't found the fatigue to be debilitating at all. The steroid may jazz him up for awhile (maybe 12-18 hours), and he may have trouble sleeping until it wears off.

Good luck!!

[samsgang]

Thanks every one! All of your comments really have encouraged me. So, it is good to hear from you Andrew that there are a lot of people doing well. And Pete, thank you for answering my Rituximab questions. I read both of the replies to Sam, and he appreciated hearing from you both. I honestly find this site invaluable for your information, and I said to Sam, "see, doesn't this make you feel good, that you are not the only one walking this road?" Community is where it is at!

[Gab122]

God has blessed me too. :)

[LJBrownyard]

Welcome! So many kind and knowledgeable people here. We have found this site to have the most information.

[Jaha]

Welcome to the forum,it is the best group of people that is out there for information and support. I am wishing Sam all the best for his Rituxan treatments. Please keep us updated on his progress. Every thing will be better soon.

[samsgang]

Hello my friends,

So, I want to update you on Sam and his progress. I guess also, we feel a bit bewildered in the medical field department. For starters, we have Group Health. That being said, we are kind of stuck with their providers they offer, if we want things to be paid. So our Kidney Specialist is the main treatment coordinator. I think he has done well up until around Christmas, and Sam's last flare. That is when we started to feel somewhat forgotten. Long story, being in the medical field and working for a specialist, I knew if we didn't hear from them in a timely manner, to call. (we were waiting to schedule the rituximab infusions) long story, they were waiting for us to call and set up the the appointments. (Joy) So we may have started a week later than we could have, to begin with. Second, the doctor put Sam on 60 mg Prednisone and he has been on that while waiting for his first infusion, (which he finally had on Jan.13th) so he has had 2 so far, with two more to go. He has them 1 x weekly with 4 total treatments in all this round. So it went fantastic the last 2 times, and his next one is tomorrow. I'm talking, "are you sure they didn't just give you water?" he felt so normal.

Onto the labs. The area for Sam that Wegner's likes to rear it's head is the kidneys. His Anca titer labs as of this past Monday the 23rd went from a ratio of 1:68 to 1:16. Happy about that, still not negative, but a bit better. His Creatinin went from 1.4 to 1.9. And his GFR went from 59 down to 38. We haven't heard from the Specialist. Luckily, we made an office visit with our regular provider, they said it could be dehydration.

So my questions for you experts, I told my husband, the true experts on this disease are the patients. In my opinion anyways.

1. Is it normal for Sam's labs not to have improved after two infusion treatments?
2. Should we be concerned about labs moving in a direction that seems worse after two infusions?
3. If Sam had been treated with Cytoxin for 6 months, and it worked, and they took him off for low blood counts, is it common to be placed back on that if rituximab does not work?
4. What is the protocol w/ Rituximab if it does start working? Do they normally do 4 treatments in a row, break for 4-6 months and then back at it?
5. Is this a big deal we have been on 60 mg prednisone with no end in site?

Do you ever feel like you've annoyed the providers with all of the questions? Anyways, our Kidney Specialist doesn't always get back to us, and the regular provider's main response was, "yeah, I'm not sure." and, "this disease is a mystery." Oh boy. They only positive we got is that the GFR is not yet near dialysis stage. (Great but when will this stuff kick in, and is it alright to wait another week?)

Finally, I know you are not Doctor's, so no worries if you tell me to go back to them and ask. I think our Kidney Specialist is doing his best. I also know, we are not his only patient. I tried to get a referral to a Rheumatologist by our Primary care doctor for this disease, her reply to us was, "no, they are usually joint doctors." I get that, but we had one on our case 20 years ago, she was great. Her name was Nancy Shasteen, but I'm not sure she is still in practice, and again it would be out of network.

Thank you for listening. Any insight would be appreciate. Feel free to offer advice too. Thanks you!

[drz]

The rheumatologists are often or maybe the usual doctors for directing treatment for GPA so I would question his knowledge on how to treat GPA. Those questions and the answers should be answered by some one with extensive knowledge and experience in treating GPA and there are a number of them listed on the Vasculitis foundation Web site, and I believe they consult for free on cases.

In my case I was treated with CTX almost a year and my treatment team said it would not be used again, just RTX if i had a big flare. I believe the risk of bad side effects increase the longer it is used. I think generally the goal is to get the Wegs under control and then reduce the pred to the lowest level that will help hold the disease in check. I was able to get down to 5 mg day plus my AZA but some can't get under 10 and a few people have great difficulty getting the GPA (Wegs) under control with any med routine but most people do with proper treatment. There is no one certain treatment that will be effective for everyone but the more experience the doctor has in treating this darn disease, the better the chances of success I think. I would push for that. I believe Ronald Falk is the expert my kidney team used and he is highly regarded as a expert in treating GPA. I am sure there are others too that your doctor should know so push him to do a consult with one. This would be reassuring to you.

[MikeG-2012]

So my questions for you experts, I told my husband, the true experts on this disease are the patients. In my opinion anyways. From all of us here, THANKS! (-8



1. Is it normal for Sam's labs not to have improved after two infusion treatments?

Yes, with this disease, there are no immediate reversal medications--well maybe with boat loads of pred, and that is because it decreases the inflammation pretty harshly and rapidly in higher doses.


2. Should we be concerned about labs moving in a direction that seems worse after two infusions?

Treatments take time to get going. It will probably be that way until the meds run their course and start to work. Until then, the disease is still active and probably getting worse until the meds have time to work.


4. What is the protocol w/ Rituximab if it does start working? Do they normally do 4 treatments in a row, break for 4-6 months and then back at it?

I had the four to start, and I have been on two, two weeks apart, every six months for almost 4 years now. My rheumy is managing my care, and as long as the RTX keeps the disease at bay, and my insurance keeps approving, then we'll keep going every 6 months.


5. Is this a big deal we have been on 60 mg prednisone with no end in site?

Not uncommon. I was on it for more than a month after my first significant flare-up.


Do you ever feel like you've annoyed the providers with all of the questions?

Yea, but let them be annoyed, they're getting paid to listen and take care of YOU and YOUR FAMILY!! If they stop listening, find ones that will.

[samsgang]

Thank you both for the responses! I feel better after seeing the list of answers! My heart goes out to Sam tonight, after being on Prednisone @ 60mg since Dec.23 and the dose of steroids they gave with the Rituximab today, Sam's face is so swollen. It makes me realize how blessed we were to begin with having a 20 year remission with out medicine. When he swells up, it makes it so much more real to me the battle that he is fighting in his body.

Your answers also helped me realize, I will be finding a Rheumatologist to get on board soon. (That didn't sound right when the Doctor said that to me.) I mean also, doesn't this affect the joints? I know Sam was sure limping around for 2 months back in April!

Well, he is snoring away on the couch, and I feel better after reading the answers. I will go cruise around on this forum while he sleeps.

Good night for now.

[Alysia]

Hi. I understand how tough it is to be the caretaker. Dont ever be too shy and dont stop to "annoy" the docs as much as needed. Your husband is blessed to have you as his advocate.

I was on pred 60mg for 5 months at the acute onset of my wg.

It takes time for rtx to work. Each case is diffrent. For me it reach its full impact after 3 months. For others - earlier. Meantime the pred is important.

Sorry for the rise of the creatinine from 1.4 to 1.9. I hope its temporary. Ask to check the creatinine every week. Please make sure that your husband is not dehydrated when he gives blood to check it. I dont have kidneys involvement but my numbers are up to the high border when my wg is active AND when I am dehydrated.

I get rtx since 2013 every 6 months 2000mg in 2 sessions.

Sending prayers.

[samsgang]

Thank you for the understanding and reply. You know what my husband is currently doing at this minute due to the steroid high? He is ripping the ceiling off of our old 1926 farm house kitchen.:crying::lol: He has always been a go-getter in general, so he has decided he better put the anxiety of the steroid to good use. I keep telling him, "if your going to do it, just take it easy in between, because you can still over do it!" (I on the other hand am the easy going, not so go-getter, so I could care less how long the kitchen takes.:razz:) But I think it helps to keep his mind off of it all! Thanks again for all of your quick replies and support. This group means so much. No fun walking this road, but it is easier together.

God bless you all my friends!

[MikeG-2012]

Thank you for the understanding and reply. You know what my husband is currently doing at this minute due to the steroid high? He is ripping the ceiling off of our old 1926 farm house kitchen.

Some times sheer exhaustion is the way to be able to sleep when on high doses of pred. Just have him be careful with his skin. Over time the pred will make it a little thin--mine did anyway.

[samsgang]

Hey everyone,

Just wanted to update. So Sam just got his labs back after his 3rd Rituximab, and all his labs have gone either down in the right direction, or back to normal even. We don't have the Anca results yet, because it takes a few days to send them to the UW. However, I hope this will encourage some one as, last week after 2 treatments we were not seeing improvement. But finally after 3 we are excited to say we have some good news.

Now we can't wait to at least wean down from the 60 mg prednisone.

I'm thanking God for this victory.

[Pete]

Great news about Sam!! Rituximab usually takes 4-6 weeks to reach full effect, so you should see more improvement over the next few weeks.

When he begins the pred taper, slower is better. He'll also need to be aware of vague symptoms such as roaming joint paid. They could indicate a flare or a too fast taper.

[drz]

Hey everyone,

Just wanted to update. So Sam just got his labs back after his 3rd Rituximab, and all his labs have gone either down in the right direction, or back to normal even. We don't have the Anca results yet, because it takes a few days to send them to the UW. However, I hope this will encourage some one as, last week after 2 treatments we were not seeing improvement. But finally after 3 we are excited to say we have some good news.

Now we can't wait to at least wean down from the 60 mg prednisone.

I'm thanking God for this victory.


Great news, stay the course and more improvement should come!:thumbsup:

[samsgang]

Hello everyone,

I just wanted to update, and also see if any of you have any input. So, long story short, after going to the primary care (also an internist), who told me "a Rheumatologist was not one who treated this disease", and hearing from you that it was indeed a doctor who we should see, I was able to self refer Sam. (We have Group Health, which requires an authorization to see any doctors outside of the plan.) The referral was approved, and we were able to get Sam in this past week to the Rheumatologist. The Kidney specialist, (who has been the only one overseeing treatment for Sam) had placed him on 60 mg of Prednisone after his last flare on 12/27/2017. That is the last we have heard from the specialist. The plan was to my knowledge, to place Sam on the Prednisone to help bring symptoms under control, then the 4 infusions of Rituximab, (completed in early Feb.), then I thought he was supposed to be weaned off of the Prednisone. Sam's blood levels are better, but not all the way. For instance, I think he is 3 weeks out from Rituximab, and he is still positive for Anca. His creatinine fluctuates, however, it is still not too concerning. His glucos was 190. Anyways, Sam is now down to 50 mg Prednisone because he was climbing the walls on the 60.

So back to the Rheumatologist that we saw on Friday, 02/24/2017. He seemed apalled that Sam had been left on 60 mg for so long. And, he mentioned Imuran (?). Then, he found out Sam has not been taking Phosphimax (sp?) for his bones, that upset him. Then, he also mentioned 1 baby aspirin everyday, (which Sam hasn't been doing either.) Luckily, Sam was taking Bacterium. (At this point the Rheumatologist said, "Please tell me you are taking Bacterium?")

The plan is, the Rheumatologist will be talking to the Kidney Specialist on Monday, to start a titrating plan for the Prednisone.

My questions for you if I may, again ;)
1. Is it strange to you, that we were left on Prednisone so long? Or, is that not unusual.
2. What is Imuran? And have any of you used it? Do you like it?
3. How about Phosphimax?

I'd love to hear your thoughts.

Thanks again, feel free to comment or advise.

Candy

[drz]

Generic Imuran or Azathioprine is often used as a maintenance med for Wegs. I have been on it for six years. Pred is usually tapered down as one's symptoms decrease since it has bad side effects like moon face and camel hump back if one takes a large dosage for a long time. But if the Wegs is active and causing problems or raising havoc with your body then pred is a miracle drug. Many of us went through this as a undesired side effect for our necessary treatment for Wegs but those symptoms generally fade as the pred dosage declines.

Fosomax or the generic version is often given with pred to try reduce or prevent loss of bone density which can be side effect of pred. I took it once a week for five years. It can also have some side effects as do most meds so the prescribing doctor will monitor for those.

Medication treatment for Wegs is best done by some one with lot experience in doing so. Or at least the treating doctor consults with a an expert from the list on the Vasculitis Foundation. I understand this consultation is free and I know my treating doctors consulted with three of the listed experts and I don't remember seeing any charges for their consultations.

[Pete]

Here's my two cents worth.

The prednisone taper/titer should be done slowly. Otherwise, he'll likely have withdrawal symptoms.

He should have a TMPT test before going on immuran/azathioprine to be sure his body can process it without difficulty.

I have been able to maintain adequate bone density by taking calcium supplements. You may want to ask about this option.

[timmy10]

timmy 10
Hi guys i had 2 infusions of Retuximab just before Xmas and the first one took about 5 hours done very slowly but the second one about a fortnight later took about 3 to 4 hours.They left me tired but apart from from that no effects.I was on 40mg Pred. but since then my consultant has been reducing them and now down to 15mg per day.Hope this helps

[samsgang]

Hi everyone,

Updating again. So my husband is now down to 15 mg of Predinisone. His P3 anca is not down to 3.9AI (from way above 80) Anca Titer is negative and used to be positive and anca MPO is neg. We have seen a steady decrease. He is supposed to continue 2.5 mg titrating every 2 weeks, until he is off. I want him to slow down to 1 mg once he hits 10mg, but the doctor thinks differently. I've read so much from all of the patients, which has lead me to think it is better to go super slow at that point.

My husband asked the doctor what constitutes remission? Anca scores? And according to the doctor, "no, it is not anca." So we are confused, because he didn't seem to answer that question. Sam's kidney function is staying in good range. The next thing we feel confused about, the doctor said to us, "before we do another round of rituximab, we will just watch his numbers and make sure he stays low, and if numbers start going up, we will do a round of rituximab then." I was under the impression it should be automatic for a few years, that we do a rituximab tx every six months as long as it's working and his lab scores are good. Not to wait for it to flare, and then treat it. (Why wait for the forest fire to start up again?) I feel like our doctor is head strong, and I hate to challenge him, but maybe I should. The joys of it all.

[samsgang]

Oh and another question, what is plasma tx? And also, what is B cell. Or more accurately, with Rituximab, what should we be aware of in that area. I've heard it mentioned but haven't figured out if it is good or bad?

[Alysia]

I am glad that your husband is getting better.

I think you are right that it is more safe to taper the pred by 1 mg for each step. I would do it even if the dr. said otherwise.

As for rtx, there is a controversy: some docs, like yours, say that one needs to wait for a change, indicating that wg is starting to be active and only then get rtx. Others, like mine, say that it is better not to allow the wg beast to raise its head, so they give rtx every 6 months or even every 4 months.

I think it is also a matter of maintenance meds. Will your husband take any, like methotraxat, Imuran, cellcept ?
I coouldn't bear Imuran and mtx did nothing, so my only wg med is rtx. It means that I have no other choice.

You asked about remission: check this thread, maybe it can answer your question...
http://www.wegeners-granulomatosis.com/forum/general-wg-chat/4056-you-remission-do-you-mind-answering-few-questions.html

Never be shy in challenging any dr. It is your right and your duty to make sure that your husband will get the best care possible.

[brew22]

I hAVE HAD GOOD LUCK WITH IMURAN!!!

[beverlyann]

Thanks sooo much for stating that many people with GPA live fairly normal lives followed treatment! I’m waiting (finally!!) for biopsy results from Thursday’s procedure following 16 months of horrific symptoms that no doctor here understood or treated. Finally got myself into Cleveland Clinic with a Pulmonary Specialist and a Vasculitis Specialist. A week inpatient couldn’t get me a confirmed dxs . Left almost hopeless. It was a University of VA ENT who pulled it together and realized the horrible ear symptoms of 16 months may be due to inflammation of artery and blood vessel above my ear. Now hoping for results before Thanksgiving !!
I needed to read that I do have a good chance of walking out of this nightmare world with treatment . Both CC specialists expressed optimism that this is very possible in my case. It just helped reading that here too!! Thanks again!You’be given me badly needed hope today.

[luan22]

Hi. I am also a new member and this forum is very useful for me