Daughter recently diagnosed-see specialist in 3 days

[Joy]

Hello all, I have been following this site for abt 3 wks. now after my daughters diagnosis in Nov. This is not my 1st post . I told a bit of her story in early Dec. on Mel's intro post. I decided to go ahead & enter here after reading Jeff's story tonight. His wife is the same age as my daughter & my daughter wants to connect w/ other women who are concerned w/ fertility. That was my initial reason for writing Mel. Since then you have all given me so much helpful advice& support in many areas. I am learning so much but have so much more to learn. I will go ahead & retell her story.
Our daughter is 26 yrs. old & had been dealing w/what she thought was sinus , drainage & thick mucous in her chest for at least 3 yrs. She has been married 2 1/2 yrs. & they were planning to begin a family soon. In early Oct she noticed that she was very tired, just did not feel right & began having intermittant chest pains. She had moved to a new town & started working on her Masters in Aug. The pain increased & she wound up in the emergency room early one morn. By late that afternoon they had mentioned the possibility of Sarcoidosis or Wegners. Her drs. have been great! Then the waiting began , the bronchioscope was inconclusive & she had to wait for a lung biopsy for a confirmation. Once it was determined she had WG(2wks. later) the 60 mg pred was started. This was early Nov. Her lungs were full of granulomas & very inflamed. There seems to be no other involvement so her pulm. says she has limited WG. They would wait for her to heal b/f b/g the cytoxin.
After learning the effects of CTX we began calling Drs. we knew back home abt. harvesting eggs. They decided not to follow through w/ that but to look at other protocol options & to find a specialist nearby. That is where we are now. She has met w/ a local oncologist who w/ her pulm. were ready to b/g the oral CTX. After talking they decided to wait for the specialist's recommendation. She meets w/ her on Tues. Her rheum(WG specialist) was 1 of the presentors of the new trial on RTX. We are very interested in that possibility. Some of you weighed in on that earlier, thanks! She called her ins. & was told they would cover RTX. I am afraid to believe it. We are also in the process of making an appt @ Cleveland Clinic. She has been emailing the rheum. abt. her concerns & we now get the impression that a lessor treatment such as MTX or Imuran may be tried 1st. We are so confused...once we go to Duke & meet w/ Dr. Allen we hope their decision will be clear.
I have read w/ interest your comments on activity. Even though she does not feel herself she is still active-ice skating, tubing , walking. With that being said she still gets winded walking up the stairs. She went off oxygen completely after T-giving. She still has a positive outlook & is beginning to learn more abt. Wg & the diverse symptoms & treatment. She is also realizing that she does want to go to the specialist & Cl.Clinic. She is getting past hurting her Dr's. feelings. Although I think he is absolutely fine w/ her going. He has been really on top of things but has not see Wg that often. He diagnosed her quickly & we feel blessed to have been somewhere that she was diagnosed so quickly. Reading so many stories have helped me realize how lucky she was that her dr(pulm) had experience w/ WG. Thanks again to all of you for such a great forum!

[elephant]

I am so happy to hear that she is going to Cleveland Clinic, I go there too. I know it is uncomfortable at first to tell your doctor you want a second opinion, but we only have one life to live and this disease is nasty. Getting proper treatment in the beginning will save her from damage that WG can inflict. Wishing your daughter all the best!

[Sangye]

It sounds like she's on a good track.

I got a little confused with all the docs.... What's the name of her rheumy who was involved with rtx? Is s/he at Duke? Is that the rheumy she's emailing?

I doubt that someone whose lungs were full of granulomas would only be treated with mtx or imuran. Those are not nearly strong enough. If CC tells her that, then I'd believe it.

[Joy]

Sangye, we are going to Duke to (Rheumy) Dr. Nancy Allen this week. My daughter emailed her 2 wks. ago after meeting w/ the oncologist & asked her if she could get the ball rolling to see if she would qualify for RTX. Dr. Allen replied that she wanted to see her 1st & study her case. She also talked abt the poss. of treating her w/ MTX. We originally got Dr. Allen's name off the Vasculitis website. She is listed on the article abt. RTX.
The Cl. Cl. is processing ins. They may not take her ins. & will not make an appt. until they clear that. We are also going to look into the other centers & try to figure out what to do. We will follow Dr. Allen's rec at this time but plan to establish a relationship w/ a center. Thanks for the advice...

[Joy]

Thanks Elephant! Before moving to NC my daughter lived in Greenville. I remember reading somewhere that is where you are. I am in Anderson, so we are neighbors. We are still waiting for acceptance to Cl. Cl. They would not make an appt. until Ins. cleared. We are traveling to Duke to meet w/ rheumy Dr. Nancy Allen this week. Hope to get many questions answered & b/g treatment.

[Sangye]

That's a great plan, Joy. :smile1:

[elephant]

Yes, I live in Greenville and I heard about Dr Allen too, but I wanted to get to a Weg specialist who saw many Weggies like us. Hope your insurance covers Cleveland CLinic! Glad you meet you neighbor!

[Palmyra]

Hi Joy,

It sounds like you and daughter have done a great job on learning about Weg, in a very short period of time. Knowledge is power with this disease, so the more you know, the better.

Very glad she is off to the Cleveland Clinic. Many on this site strongly advocate going to one of the major vasculitis centers for treatment (including me).

My daughter (soon to be 25yrs old) was diagnosed 4+years ago, and has never had to take cytoxin. She briefly tried Prednisone and methotrexate, but when that obviously failed, her specialist (at mayo) started her on Rituxin. She has had 3 rounds of RTX in the past 4 years, and is due for another in approx'ly one month.

She and her husband would also like to have children, so we are hopefull that this line of treatment has left that as an option.

Best wishes to your daughter, and pls keep us posted re: her progress.

[Joy]

Thanks so much for your encouragement, I was curious -is your daughter doing the 4 or 2 treatments in a round of the rtx? I read w/ interest when Sangye reported on those 2 protocols. I also wondered when she started @ Mayo. Our daughter is starting @ Duke but we are working on getting her to Cl. Cl. When you say she is due for another rd of rtx do you mean she has gone out of remission? Sorry so many questions-I just am so concerned abt the treatment decision that will be made tomorrow for our daughter & I want to be as informed as I can be...It sounds as though your daughter has done well-has she been on anything else? Thanks !

[jola57]

Hello Joy, I am glad that your daughter will get proper weg's doctors treating her. I am sure that she will be well on her way to remission soon. I do not take rtx but was on cyclo and now on mtx. I believe the rtx is taken a few times before it starts to work,

[Sangye]

Jolanta, I don't think that's true about rtx. Rtx takes about 6 weeks (from the time of the 1st infusion) to start working at full speed.

The RAVE and RITUXVAS studies used one round (2 infusions or 4) to achieve remission. I know of someone who had one round in 2006 and is still in remission without more rtx. However, it took 2 rounds (5 mos apart) to get me fully into remission. The first round worked well but wasn't quite enough to get it fully controlled. I have refractory Wegs with severe activity (ie, alveolar hemorrhage). We had to do some fancy talking to get that Wegs dog to sleep! Dr Seo anticipates having to repeat it in 1 year (next March), but wants to stretch it as long as possible to minimize using it.

[jola57]

That was based on hearing from most of us that they had more than one infusion to bring about remission. Sure beats 2 years of cytox and 2 years of mtx.

[JanW]

I think that the confusion is that one round=more than one infusion.

[Palmyra]

Thanks so much for your encouragement, I was curious -is your daughter doing the 4 or 2 treatments in a round of the rtx? I read w/ interest when Sangye reported on those 2 protocols. I also wondered when she started @ Mayo. Our daughter is starting @ Duke but we are working on getting her to Cl. Cl. When you say she is due for another rd of rtx do you mean she has gone out of remission? Sorry so many questions-I just am so concerned abt the treatment decision that will be made tomorrow for our daughter & I want to be as informed as I can be...It sounds as though your daughter has done well-has she been on anything else? Thanks !


The protocol for Rituxin has evolved a bit over the last 5 years from what I have seen. Her first infusion (delivered at mayo by a Weg expert,) was four treatments, at one week apart. ( Much discussion in previous posts about that first infusion, as it is managable but can be difficult.) Her second infusion (about 9 months later,) was also four infusions, at same interval, delivered by local docs. Her Mayo specialist is now recommending the same four infusions for initial treatment, but for subsequent maintenance (at approx'ly 6-9 mo's apart) two infusions, one or two weeks apart (I forgot which? In notes somewhere!)

Because Rituxin is officially "off lable" for treatment of WG, you may see or read about slightly different protocols coming from some of the Vasculitis experts. I would follow what any true Vasculitis expert recommends, as they are looking at each patient individually through the lens of their vast experience. I would hold suspect any treatment recommendations from a local doc...just ask questions and read.

When I say Alison is "due" for another infusion, it is because she tends to flare approx'ly 9 months after her previous infusion, when B cells are beginning to reconsitute. She is immature, reluctant to treat aggressively and tends to want to delay treatments. The result is she flares (pulmonary granulomas, sinus problems) and then has to redress the entire situation. Her Weg specialist has encouraged repeats of Rituxin BEFORE she is in a full flare.

Sangye and others can give you lots of information re: how to better predict outcomes, especially dependent on what system the Weg originally presented in. Alison's original presentation was pulmonary, and after a brief unsuccessful trial of Pred and methotrexate, she was given daily pred (60mg's) and the initial Rituxin. She responded almost immediately (one week!). Pulmonary lesions were gone within 3 months. That first visit to Mayo was summer of 2006.

She does have difficulties with opportunistic infection, some of which have been severe. All the drugs that address Weg deplete the immune system, so nasty bacteria, fungal or viral infections can be problematic.

LONG post, but hope that shares a little info regarding Alison's experience. Happy to share. Many on this site have much more experience and knowledge.

Best wishes and good luck getting into CC. Each journey is different, but much the same!:wink1:

PS...regarding past treatment, she was diagnosed with Crohn's disease in 1999, so she has been on many drugs to treat autoimmune disease, with exception of the 'Big Boys', meaning no: Plasmapherisis, cytoxin or other heavy hittters, which have been very necessary for those with advanced disease. The only drugs for WG were methotrexate (not enought to induce initial remission,) and then to the Pred/Rituxin. She weaned off pred as quickly as possible. The Rituxin knocks her Crohn's disease in the dirt. YEA!

[elephant]

Glad to hear that her Crohns disease is in remission.

[Sangye]

I think that the confusion is that one round=more than one infusion.
Oh! A round of rtx is either 2 infusions in one month, or 4 infusions in a month.

[JeffS77]

She does have difficulties with opportunistic infection, some of which have been severe. All the drugs that address Weg deplete the immune system, so nasty bacteria, fungal or viral infections can be problematic.



could bactrim help maybe??

[Joy]

I do not know where to begin...which is probably why it has taken me so long. We took our daughter to Duke on Dec 21 & met w/ Dr. Nancy Allen. We all liked her very much & felt good to be w/ a wg specialist. In spite of all my daughter did to have everything she needed (she even hand carried some things -just in case) the slides of her lung biopsy did not arrive. Another phone call & they still had not arrived Jan.3...Dr. Allen ran more tests & they show she still does not have a positive anca score & her kidneys are still unaffected. Her lung xray shows a huge improvement . Dr. A. said w/o the biopsy she could not make a rec yet as she did not have all the pieces of the puzzle, but that she was doing very well & could wait longer to b/g her treatment. This has made us very uncomfortable but our daughter has emailed her all the concerns & Dr.A always responds quickly & explains & reassures her. So after the visit Dr. A began reducing her pred 10 a wk , she is @ 30 now & will maintain @ 20. Her next chest xray is next wk. & Dr.A will determine her protocol after she sees that xray. She continues to feel ok but does not feel back to normal & is anxious to begin treatment. Dr. A does not think she needs to take a stronger drug & is leaning towards MTX.
On another note: 2 days b/f we went to Duke her pulm. called. He had just heard back from Mayo(Ariz) & they said the biopsy was inconclusive. He was very upset as I had requested he send it to Mayo & he chose to send it to Ariz. due to a relationship he has w/ the pulm. there. Our Dr. totally disagreeed & is very confident in his Wegs diag. Soooooo-more confusion.
We are definately in a holding pattern. My daughter started back to school today. She wants to go to Duke & work w/ Dr Allen b/c she can drive there,& work around her classes. She does not want to miss class so she hopes to schedule her Cl Cl. appt during her spring break. I guess we will know what happens next soon.
I have learned alot from reading this forum-I am so glad you're here!

[elephant]

I hope you guys make the trip to Cleveland Clinic soon and get a second opinion, you might just feel better. I am sure Dr Allen is just fine. Not to be harsh, but school can wait, need to be healthy first. I started late in College, and graduated with two Degree's ( it took me 10 years to get through it) . In her own time she will get there.

[Sangye]

Joy, I hope everything gets sorted out soon. Treatment decisions are very stressful!

[JanW]

Indeed they are. If it's any comfort, I was diagnosed with WG a year ago tomorrow, and I didn't start medical treatment of it until March, because they thought I was having a bad reaction to a test dose of mtx (that turned out to be wrong) and they didn't want to jeopardize impending surgery (I had a subglottic stenosis fixed last March). So, I went two months between diagnosis and even taking a pill for WG except for a one week test dose (no pred). All this is to say that even not beginning drug treatment immediately doesn't mean that you daughter's case is necessarily growing worse.

[Palmyra]

Thanks so much for your encouragement, I was curious -is your daughter doing the 4 or 2 treatments in a round of the rtx? I read w/ interest when Sangye reported on those 2 protocols. I also wondered when she started @ Mayo. Our daughter is starting @ Duke but we are working on getting her to Cl. Cl. When you say she is due for another rd of rtx do you mean she has gone out of remission? Sorry so many questions-I just am so concerned abt the treatment decision that will be made tomorrow for our daughter & I want to be as informed as I can be...It sounds as though your daughter has done well-has she been on anything else? Thanks !

Joy, my daughter did 4 rounds of Rituxin for induction/initial therapy (along with pred) and is now on no meds other than bactrumDS 3 X's weekly. Her initial appointment was at Mayo (Dr Ulrich Specks,) after her Austin, TX docs realized this condition would be better served at a facility that had expertise in the area. (She also has a history of Crohn's disease.)

She has been treated with Rituxin a total of 3 times in her 4 year history of Weg. Per Dr Specks, it is now recommended that AFTER initial induction treatment (4 infusions of RTX, usually one week apart,) that subsequent treatment is 2 infusions (at one or two week intervals). To my understanding, all of this has evolved within the previous 5 years or so, and will vary slightly from major center to major center. Each of the experts seems to have a slightly different spin on how to treat this disease, and it is highly variable on the individual being treated.

Maintenance therapy guidelines are not really in ink at this time. It has only been within the past year that RTX has been shown (in the RAVE trial,) to be of similar efficacy to CYX as an induction treatment. We had heard discouraging reports regarding harvesting eggs, and with Rituxin's early promise, we chose to go that route rather than start with cytoxin.

My daughter has had problems with secondary infections....many on this site have similar problems due to immunosuppresion, when treated with any immunosuppressive drug (she is on Bactrum, but her infections have been fungal and by the odd bacterium that is not addressed by bactrum)

And yes, she currently uses Rituxin after about a 9-12 month interval, as when her B cells repopulate she does tend to flare. She is looking at other maintenance drugs (the only available to her might be cell cept, as she cannot take imuran) to try to prolong that remission period without having to use Rituxin with frequency.

Good luck to you and your daughter! She is lucky to have a skilled advocate looking out for her:thumbsup:

[drz]

Differences in treatment recommendations can be stressful. That is one of he advantages of dealing with a large treatment team under one roof. They may have similar differences in assessment and recommendations but they thresh it out among themselves so you only get one recommendation and avoid the stress of knowing how it was decided. Therapeutic rapport is often important variable in efficacy of treatment so it might work to allow your daughter considerable latitude in how she wants to pursue treatment. Good luck. At least info so far suggests it is not urgent or life threatening at this point so you should be able to get it all worked out with something that makes you comfortable. Good luck!

[Joy]

I want to thank all of you for your posts-they help so much & I feel your support & it means alot. We are in a strange place right now as the doctors do not think that the weg diag was correct. They are now leaning towards sarcoidosis. This was the other poss. diag. back b/f her lung biopsy. Her ANCA was never positive but her pulm. split the anca(back in Oct) & did 2 more detailed tests w/ one showing a pos. indicator for WG. She had her 3rd CT of her lungs on Fri. & they see nothing there anymore. Amazing!.All other tests are still neg. Her steroids have been tapered since Dec. 21 & she is now down from 60 to 20. I wondered if any of you know where to go for sarcoid? I have seen that poss. Mayo(MN) might handle S & WG. She plans to go her spring break & wants to go 1 place that is a center of excellence for both. It is hard to think in a different direction as I always felt that wg fit her symptoms best due to her sinus issues & neck pain along w/ the lung granulomas. We would appreciate anything y'all know abt. sarcoidosis, have you heard of this confused diag happening to anyone else? She is feeling so much better but still gets winded on stairs or w/ alot of walking. She does not feel back to normal & her mucous has increased w/ the tapering of the pred. Sure would appreciate your insights.... Thanks! Joy

[Sangye]

Joy, I suggest you go to a major center and let them evaluate her. Mayo docs get patients with an uncertain dx every day. However, spring break is a long way off. If it were me I wouldn't wait. While it could be sarcoidosis it could still be Wegs and waiting that long is not good.

It's great that her lung CT looks better but lung involvement with Wegs is not always straightforward. The first CT I did showed my lungs completely full of blood. I saw a pulmy the next day and we repeated the CT the day after. My lungs still looked awful but there had been enough improvement that he didn't want to do a bronchoscopy while we waited for labs. I didn't even have a dx, much less any treatment and they looked better.

Also in my 2009 flare, my lungs were bleeding again and I was coughing up big blood clots. It was decreasing every day for a week, and by the time they did a bronchoscopy there was minimal dried blood in my lungs. The JHU rheumys and pulmys who were treating me while hospitalized concluded it couldn't be hemorrhage due to Wegs since it had seemingly resolved itself. They discharged me with a dx of viral bronchitis. Dr Seo found out the next day and was livid. It was clearly Wegs and my lungs would have started hemorrhaging again without treatment.

[Jaha]

Joy,

After having a lung biopsy and 3 places removed from my lung (after about 2mos.) I self referred to a Dr Culver ( a Pulmonologist) at Cleveland Clinic who specializes in sarcoidosis, he sent for my tissue samples and had a team review my case. I have never tested positive to the ANCA but that team was able to pin it down to WG's and then got me in to treatment with a wonderfull Doc there at the clinic. Good Luck!
Jana

[emg]

Joy,
This thread made me cry! First off, your family is in my prayers.
What a lucky girl she is to have you as a mother! I feel so alone not having anyone to talk to, vent toand fam n friends I want to talk to have no knowledge of anything autoimmune....barely know general CBC type lingo. Lol
You are a special kind of woman to not only be with your daughter but also to gain the knowlege to help her and allow her to lay her burdens on u to lighten her load.
She is truley blessed.