Discouraged Mom

[Tmp521]

Hello everyone. I've been reading posts on here for a while but just now made up my mind to write my own. At the end of March, I took my daughter to the doctor for for a sinus infection. Just as the doctor was walking out the door, I glanced at my daughter and saw that her on again-off again rash was reappearing. I asked the doctor to look at it and long story short 6 days later she was admitted to the hospital for what we all thought was lupus. After a kidney, bone marrow and skin biopsy, it was determined that she doesn't have lupus but is positive for antiphospholipid antibodies. ANCA test was negative. There's alot more but too much to go into detail. Fast forward a month and here we are faced with so many questions that were unimaginable just a few moths ago. When we found out she doesn't have lupus, it was suggested it could be Wegener's but none of the test were conclusive. She had a sinus tissue biopsy last week and although she was negative for the particular vasculitis they were looking for, the path report said that what they did find was consistant with Wegener's and that it was highly suggestive of the disease. So now the docotor wants to do treatments. Cytoxan pill everyday for 6 months with 2yrs of maintenance or 2 infusion treatments of Rituxan with no maintenance. I've read that Rituxan can cause a rare brain disease that is fatal and that some people have died within 24hrs of treatment. With Cytoxan the doctor warned of various cancers and serious infections it can cause. It terrifies me to think that my child could die from treatment. It also terrifies me that she could die without the treatment. I question the fact that they want to give her the treatments and aren't 100% possitive it's Wegener's...only that all the testing is "highly suggestive" of Wegener's. There's really no one else I can take her to for a second opinion and as for the state of Georgia, she really does have some of the best doctors we could find. The doctor has left the decison up to us as to which treatment to administer. I guess what I am looking for from some of you is suggestions or someone else's input or thoughts on the different meds. Right now I feel so lost and confussed, I don't know what to do. My daughter is 15 years old and has been so incredibly strong through all of this. While I think she deserves to know what the risks for these treatments are, I don't want to unnecessarily scare her. I just don't know what to do. Please, give me some feedback on what some of you think. I know there's also great outcome for both the R and C but as you all know, with Wegener's, you're kind of limited on who you can talk to or turn to for advice. :unsure:

[mishb]

Hi Tmp521 and welcome to the forum.

I couldn't imagine one of my daughters having to go through this.

From reading your post (and I know you didn't want to go into much detail) but it appears that your daughter has what they like to label as limited WG.
In limited, I am only referring to, no kidney or lung involvement, just nose, maybe eyes, ears and possibly joints.......but not to downplay the involvement of these.

If this is the case, (and I also have lupus and RA), then I wonder why they haven't suggested Methotrexate (MTX) as a possible alternative combined with prednisolone instead of Cytoxan or Rituxan (which are the bigger hitters).
Cytoxan can cause sterility and also bladder cancer (from what I have read on here) so that is something to think about, but I guess all of the meds that we are taking, can cause some reaction.
I know most people on here would tell you that Rituxan is the way to go and they will swear by it. I sometimes wish that I could have it, but I think I'm doing fine on the MTX and pred.

However, if I am way of the mark, with the limited, and it is not the case, if it were me ....... I think I would go with the Rituxan 2 treatments possibly every six months, compared to a tablet every single day.

I hope someone else, who had tried these two meds, can help you out.

I wish a speedy, pain free remission for your daughter.

[Dirty Don]

Hello Tmp...your daughter is a tough person it appears...take that to heart in your frustration...no one wants such for their kids, but sometimes the kids take it better than the parents. As you're in GA, you are reasonably close to Mayo in Jacksonville...perhaps you could go there or at least have your docs consult with their vasculitis docs. Keep asking questions and venting when you need...we are always here. Best to you both.

[whatthewhat]

Dear Tmp,
I am so sorry for what you all have been through.
My 14.5 yr old daughter was diagnosed with Weg / GPA in March of this year, but we are fortunate to have an excellent team of pediatric rheumatologists in Los Angeles on the case.
Pediatric Wegener's has some major treatment & outlook differences than adult Wegener's, keep in mind.
The clinic we work with has now 60 ped GPA patients. The protocol they helped develop is 2 total infusions of Retuximab, given 2 weeks apart, right away after diagnosis, high steroids at first also, and weekly Methatrexate.

The brain thing you mention I do not believe is as high a risk for pediatrics. But the fertility & etc are considerations that help make Cytoxin a last resort for children with GPA.

As far as diagnosis, there is a process of eminination & identification that leads to diagnosising GPA. My daughter had weight loss, rashes, nose crusties, severe joint pain, and anemia. That all led to oodles of testing.

It is very frightening. I am glad you posted. You are not alone. Private message me anytime. I encourage you to find a pediatric rheumatologist who is willing to consult with ped rheums who have experience diagnosing & treating pediatric wegs. I believe the Vasculitis Foundation website keeps a list of docs who can consult. Our team is out of Children's Hospital Los Angeles and I believe they consult as well.



All my best to you and your daughter,
Wtw

[Pete]

Hi Tmp,

For what it's worth, my wegs diagnosis was also a process of elimination. When I asked if they had another possible diagnosis besides wegs, they said no. They went on to explain that if they treated me for wegs and I got better, they could assume the diagnosis was correct. If I didn't get better, the diagnosis was probably incorrect, but I probably wouldn't get any worse. I did get better, so they have since confirmed the wegs.

I don't think any of the drugs used to treat wegs are completely free of side effects. Your daughter's docs seem to be on the right track. I hope she tolerates her treatment well and is able to live a nearly normal life. Good luck!

[drz]

These decisions are tough so getting a second opinion is very desirable. The Vasculitis Foundation has experts in most areas and can offer long distance consultation free so I would contact them and get the name of an expert to review her case. They can fax or send all the info by internet and discuss the case by phone or video conference and give you and your doctor some more input to help you make a decision on how to proceed. Good luck. No decision is risk free so following your gut instincts is often the best way to proceed after you get the input of experts.

[Tmp521]

Hey yall, thanks for the replies. I should've mentioned in my post yesterday that my daughter does in fact have lung and kidney involvement. The lung biopsy report states she has "several ground glass nodules" and there's some damage to her kidneys as well as blood/protein in urine. When the ENT was looking to see if a sinus biopsy was needed, she found an ulcer in her nose. When this all came about in March, we found that she is anemic and has high bp. So to sum it up, I took her to the Dr. March 20th for a sinus infection. 2 months later she's on 2 bp meds, iron, 60mg prednisone, plus several other things and getting ready to start Rituxan. Everything just happened so fast. With 4 children, someone always has a runny nose, sore throat, cold...something minor. I keep thinking that she'll finish the meds she's on and this will all be over with. What if they're diagnosing her wrong? What if we choose the Rituxan and something goes wrong and we end up wishing we chose differently. This is all just hard to accept. The doubts that I have are mainly because they can't say for sure 100%, yes, this is Wegener's. I don't know. I guess most people usually think something like this won't happen to one of their children and curable or not, it's just hard when it does happen.
I will be keeping up with everyone and keep posting as we go along. Best of luck to ALL of you. So thankful I found this site!! :smile:

[mishb]

With all of these others symptoms, I think she certainly does have Wegeners.

I think the right choice has been made with the Rituxan and hopefully she will be on her way to feeling a lot better shortly.

Can I ask how old she is?
If you read on other threads, you will find our Gwen (yes, she's ours :biggrin1:) who was diagnosed at 14. She is now in her 30's and has three beautiful daughters.

Our thoughts and prayers will be with your daugther as she begins her infusions.

[carriej22]

Hi there,

I was terrified of getting Rituxan too - however my doctor reassured me that the chances of something severe happening in a young person such as myself (I was 22, am 23 now) was very very very low. Essentially; he very methodically and professionally told me my chances of survival beyond a few months if I chose not to get treatment. After he told me my other options (which wasn't much) I decided to take what at the time I thought was the "lesser" evil and go for the Rituxan. I cried; and cried and cried about it... Lost my mind about it. I also did not know if I "for sure" had wegener's either. I was waiting on biopsies - I had no choice though; my body was failing and something had to happen. So I signed the paper; and the next morning I got Rituxan. I got an itchy throat the first infusion; but other than being a bit sleepy and snoozy because I was all hopped up on benedryl; the infusions weren't too bad. They are more psychologically difficult to deal with than physically difficult.

Long story short; they need to tell you the risks but the chances of them happening are so so so minimal; most of us agree Rituxan is a godsend. I know for a fact; that I would have died last year if I did not take treatment. Rituxan "fixed" me as good as I can be fixed. You will not believe how quickly it works. This is also a good time of year to get it - infections are low since the weather is warming up. I received my first treatment on June 1 last year; and it's almost time for me to get more. I won't hesitate for a second to get it.

I opted away from Cytoxin because I was young - it's a much more toxic treatment.

[annekat]

Welcome, Mom. You have all the advice you need above, I think. I mainly want to say I'm glad you joined the forum and have started posting. It really is the best place to be for support, advice, and information. Everyone on here is just great. I took CTX, and now MTX, and have no experience with RTX, but am sure that it is correct that any serious adverse reactions are very rare. I'm sure you will make the right decision for your daughter and I wish her the speediest possible improvement in her symptoms, with the most minimal side effects, and a steady progress into remission.

[Tmp521]

Jordan is 15. She will be 16 in July. Thanks for letting me know about Gwen! Knowing what you've told me about her has given me a positive outlook on things for the first time in a while. THANK YOU!:smile1:

[Tmp521]

Thank you so much for your reply Carrie. Positive stories like yours mean so much and gives me so much hope for my daughter. I am curious though...you said its almost time for another treatment. Is that how it usually works? I was under the impression she would only do 2,2 weeks apart and as long as she goes into and stays in remission, she won't have to do it again.

[Dirty Don]

Thank you so much for your reply Carrie. Positive stories like yours mean so much and gives me so much hope for my daughter. I am curious though...you said its almost time for another treatment. Is that how it usually works? I was under the impression she would only do 2,2 weeks apart and as long as she goes into and stays in remission, she won't have to do it again.

Depends on how her body reacts to the initial infusions. Ordinarily, there is a maintenance mode...that seems to be about 6 months for many people. Better to be safe than sorry mode I guess. I'm in medical remission, and maybe nearing med free remission, but docs continue to keep me on mtx to be sure...maybe another year? Hope hope!

[JennZ1196]

Hi I was just curious were in Georgia you take your daughter for treatment. You said she had some of the best doctors. I just moved last July to North Carolina and because I am on the borderline of Ga Most of my doctors are there, I cant seem to find a good rheumatologist. I go to gainesville Ga but i didnt like the center for rheumatology, and in my small town there are no doctors. So I have to go to asheville Nc or Georgia. I have Wg and had it since 2007. Its been a nightmare and still cant get proper care or treatment. If I dont get a doc soon I am just going to go to the Mayo clinic, or duke. I will keep your daughter in my prayers and thoughts. Its a rough disease and I can barley handle it, I can just imagine how hard it has to be for your daughter. She seems strong willed and thats good. If I could help with anything please let me know :) God Bless

[Alysia]

Hi tmp521.
welcome to the forum. Im sorry to hear about your daughter.
I had RTX two months ago for the first time. I was very afraid and I wrote here, and the wonderful people here helps me to relax. It was not so bad. I felt tired, my blood presure was very low and no more. I brought good book with me, chocolate and tea. about 6 weeks later the improvement began and is continuing, eventhough Im not yet feeling well.
I believe it will be O.K. for your daughter. she is young and strong and have an amazing mom who takes care for her.

[BrianR]

Hi There.

You may be interested in visiting weggiesunite.blogspot.ca I also sent you a private message.

[carriej22]

Thank you so much for your reply Carrie. Positive stories like yours mean so much and gives me so much hope for my daughter. I am curious though...you said its almost time for another treatment. Is that how it usually works? I was under the impression she would only do 2,2 weeks apart and as long as she goes into and stays in remission, she won't have to do it again.

No problem, all of us have been there and want to help :D

Each and every one of us is different and all of us have different treatments; I know some weggies who get maintenance meds of rituxan every 4 months, some only had it once; some every year... The treatments will be as different as the individuals receiving them!

I had 4 infusions one week apart initially; and we were going to do another 4 at six months but I did not need them. Recently though, I have had elevated levels in my bloodwork that's been creeping up; so that's why I will have infusions again. I take Imuran now and it seems to work; and if I didn't need the rituxan I wouldn't get it. However; the imuran doesn't seem to be keeping it completely under control and my disease is sort of "smouldering" in there and nothing seems to be kicking it clean in the arse to get rid of it; so my doctor is opting for another round of rituxan probably.

If I had of been able to stay on MTX; it may have been not needed but I had a reaction to MTX and ended up in the hospital for a week. It seems like most people take that; but I can't have it.

[pberggren1]

Jean, Dr. Falk is at UNC and is one of the top Wegs specialists around.

[JennZ1196]

Hi Tmp521, I was just curious were in Georgia you take your daughter for treatment. You said she had some of the best doctors. I just moved last July to North Carolina and because I am on the borderline of Ga Most of my doctors are there, I cant seem to find a good rheumatologist. I go to gainesville Ga but i didnt like the center for rheumatology, and in my small town there are no doctors. So I have to go to asheville Nc or Georgia. I have Wg and had it since 2007. Its been a nightmare and still cant get proper care or treatment. If I dont get a doc soon I am just going to go to the Mayo clinic, or duke. I will keep your daughter in my prayers and thoughts. Its a rough disease and I can barley handle it, I can just imagine how hard it has to be for your daughter. She seems strong willed and thats good. If I could help with anything please let me know :smile: God Bless

[pberggren1]

Dr. Falk is a Wegs specialist at UNC. He is a Nephrologist though. He will be just as good as any Rheumy. He would be the best in your area by far.

[hmcollin]

Hey Tmp,
I was recently diagnosed in Jan. and have been going to Emory. I went to the Vasculitis home page and started looking at who was receiving grants for research studies since there were no specialty centers around here and there was a doctor at Emory that had been awarded 2 grants to study vasculitis. She has since moved to Calif. but the Rheumatology Dept. at Emory has quite a few WG patients. There comment was "even though it's a rare disease, it's not rare to us". I read extensively when i was diagnosed and found that my first rheumy was asking me what I thought we should do and her course of treatment had no research to back it up. My rheumy at Emory has been great. Thanks

[Tmp521]

Hello again everyone! It's been a while since my last post...a LOT going on. My daughter had 2 Rituxan treatments and did GREAT! She did have a slight reaction with the 1st infusion and it was very scary but once the nurse slowed everything down and took a few other measures, all of the side effects disappeared just as quickly as they came. Jordan is now on the taper from steroids and should be off by the end of September. We have already been told that she may have to have another treatment in December as her chest and back pains seem to be on a daily basis now where she use to have them only every so often (although episodes would last for days). My concern now is that not enough is being done about the chest pain. When she was hospitalized in March, a CT showed "several ground glass nodules in both lungs". A CT has not been done since March and none of her doctors seem interested in doing another one any time soon. Shouldn't I press the issue? Or do I need to give the treatments more time to work (received R in June)? Have any of you had the ground glass nodules with daily chest pain? If so did you do a repeat CT? How long after first one and how did it turn out? Thanks for your input everyone! Even when I don't respond immediately, I read posts on here and am so extremely grateful for each and every response! It's so encouraging hearing from others dealing with this instead of relying solely on the doctors for every bit of info. :)

[drz]

Hello again everyone! It's been a while since my last post...a LOT going on. My daughter had 2 Rituxan treatments and did GREAT! She did have a slight reaction with the 1st infusion and it was very scary but once the nurse slowed everything down and took a few other measures, all of the side effects disappeared just as quickly as they came. Jordan is now on the taper from steroids and should be off by the end of September. We have already been told that she may have to have another treatment in December as her chest and back pains seem to be on a daily basis now where she use to have them only every so often (although episodes would last for days). My concern now is that not enough is being done about the chest pain. When she was hospitalized in March, a CT showed "several ground glass nodules in both lungs". A CT has not been done since March and none of her doctors seem interested in doing another one any time soon. Shouldn't I press the issue? Or do I need to give the treatments more time to work (received R in June)? Have any of you had the ground glass nodules with daily chest pain? If so did you do a repeat CT? How long after first one and how did it turn out? Thanks for your input everyone! Even when I don't respond immediately, I read posts on here and am so extremely grateful for each and every response! It's so encouraging hearing from others dealing with this instead of relying solely on the doctors for every bit of info. :)

CT scans involve a lot more radiation exposure so they don't repeat them anymore than they feel is necessary. Has she had regular chest x-rays which can also tell you a lot or scopes to look inside her lungs? Is coughing up red blood? The main concern is if she is bleeding of if there are granulomas growing in lungs. Pain could persist long after healing has begun but they should address it some how and give you info on what is happening and what is there treatment plan?

[whatthewhat]

Our daughter (dx in March) got a CT scan & started Rtx & Methatrexate in March. Her ped rheumys (who see many ped weggies) don't do CT scans less than 6-8 months apart. In March they said her next CT scan would be Sept & that is still the schedule. It does take time for the Rtx to settle in, so to speak. All that being said, I encourage you to keep speaking up & advocating. If tests & treatments are not being ordered that you think might should be, the docs ought to be able to explain why not (i.e. radiation levels, as Don mentioned above. You have a right to be educated & spoken to like a partner on her treatment team, cause you are!!