New, Daughter - 13 - just Dx with WG

[LynnZielinski]

HI, I am Lynn and I am new here.

I am here because my 13yo daughter Abby was just diagnosed with Wegner's. 6 years ago at 7yo she stopped being able to walk and move her arms so she was diagnosed with JRA which quickly became psoriatic arthritis. After 2 years of being for Psoriatic JRA she has cartilage breakdown and developed a subglotic stenosis, granulomas in her sinus and on her eye lids. Her ANCA was always negative and her kidneys were fine then. In the last 6 months she has had traces of blood in her urine so we (she) just had a biopsy 2 weeks ago. Her diagnosis has been revised to WG.

While recovering in Children's Hospital in Detroit MI, we left our now 13yo alone for 2 hours and she decided to look up her diagnosis on her own and scare herself with the bad and the ugly. So we have been doing our own learning by trying to find others who have the disease and can help her and us understand what she is going through and the treatment.
For now, she has been put on cytoxan infusions. They took her off methotrexate and folic acid that they had her on as well as celebrex. They left her on: Allegra, Qvar inhaler and Ventolin, Zantec, Tylenol, and have her on 80mg of prednisone.

Needless to say Abby (Abigail) has not had a typical childhood. But we are trying to keep it as normal as possible. This year will be her freshman year in high school and the doctors have told us to be prepared for a hard year to year and half. She has missed a lot of school even school years but we have managed to not lose a grade level. She is worried she will miss her freshman year and all the high school functions but we told her we will trying to keep it as normal as possible - hospital stays as limited as possible of course.

So that is what lead me to finding all of you.

Lynn ~ Mom to Abby

[Dryhill]

Lynn and Abby, welcome to the forum, while I am glad you found us I am sorry that you needed to. There are a number of folk here that have loads of experiance and can probably answer nearly any questions you have, so if you want any advice please do ask.

Abby, I guess one of the places you looked for info on WG is Wikipedia. Some of what they tell you is correct but quite a bit of it is out of date, and there are new treatments being tested. So remember while it is not a nice illness it is not all doom and gloom.

Jim

[Dirty Don]

Hi Lynn, welcome to our little group...it's a very special group with a lot of special knowledge and a great deal of compassion, albeit spiced with some dark humor sometimes. Good on Abby for confronting the disease even tho it may be scary at first...I thought I'd signed a death warrant when I began digging. But, as Jim points out, it is NOT all doom and gloom, and because Abby is younger than most of us, she stands a very good chance of getting into a remission. Some of the drugs, outside of the cytoxan, seem a bit much or not usual to what many of us take. For example, folic acid is far more effective than Zantec, 80 mg of pred seems awfully high (that alone can keep her from doing things), Allegra is for allergies and tho she may have sinus damage, as do I, it is not particularly effective for the sinus damage. So, while I don't know her particulars, I would be interested if you are in actual contact with a WG expert, a doc who has/does treat WG with experiential aptitude. So much is not known, that a regular doc, even a well meaning one, ends up reading out of a book, and frequently goes too far or too little. Please make sure you can or are using a WG expert. Best of luck. Go Abby! You can do this!

[vdub]

I feel so very sorry for this to happen to someone so young. But, as Jim and drz said, it is not all downhill. Many strides have been made with both wegener's and psoriatic arthritis. It's not going to be easy, but things will get better.

Something that will probably be hardest on her is the pred. She will have a weight gain and that could be pretty disheartening for a young lady going into high school. You might want to look at Marta's website ( WeggiesUnite: Home: Zazzle.com Store (http://www.zazzle.com/weggiesunite) ). She puts a humorous twist on the pred issues. Marta is one of our member's in the great white north of Canada, eh.

I'm glad you found us. Most of us have been around the block with the darned wegs thing and freely talk about all aspects of it. Remember that none of us are doctors, tho. We just have our own experiences and each of us is very different.

You can see in my sig that I also have wegs plus psoriatic arthritis.

[annekat]

Welcome, Lynn, and I hope Abby also feels welcome to get on here and say anything or ask questions. A lot can be learned here not just from the current posts but by looking at older ones under different headings, or doing searches on specific concerns. Without this forum, I know that many people with Wegener's would be a lot more in the dark, scared, and misled by those who don't know as much, or like Don said, are dispensing outdated information. So, it's a very good thing that you found us. There are some very smart and knowledgeable people here, along with some funny people and great storytellers. You will both feel very much less alone here and encouraged about the likelihood of getting through this and on to a more normal life.

It sounds like Abby suffered several years with various WG symptoms before being diagnosed. I agree with Don's advice about finding a Wegener's specialist, if possible, to see in person or have your doctor consult with. There are several specialist centers in the US and a list of specialists on the Vasculitis Foundation website. And depending on where you live, someone here may have a specific recommendation.

[vdub]

A lot of people have good things to say about the Cleveland Clinic which is probably the closest Vasculitis Center for you. It would be good to get a referral to them if possible. Just for peace of mind if nothing else. My specialist in Salt Lake came from the Cleveland Clinic and he is very impressive.

[mishb]

Hi Lynn and Abby and welcome to the forum.

We have many carers of children with WG on here and also some young teenagers/young adults that have received the diagnosis of WG.
I'm sorry that Abby has had to go through the pain of JRA and now WG.
I hope Abby can join us and see that even though things seem really bad at the moment (and they probably are), it will get better and hopefully she will find full remission like many of our members have.

Take care of each other and remember to ........

[Hopeful]

Welcome Lynn and Abby!

A few tips: I think the main thing I would like to say (and I think I read it in this forum) is get to know how your body responds to different drugs, foods etc. This can help you know when you are having a flare (if you have more subtle symptoms like me) or when something is just not "right". And be sure to tell the doctor when this happens.

Also a good thing to do is have a list of your meds: when you started a drug/treatment; how much/often; when and why treatment was discontinued. It's much easier to whip out this piece of paper than to have to try to remember everything when you see a new doctor (or even the same doctor!). By giving a typed copy to your doctor it becomes a part of your medical file. Doctors and nurses have told me how much they appreciated the list.

Everybody responds to treatments differently. My lifesaver for the chemo period of treatment was olivene tea. It helped soothe my irritated liver and eliminate some of the water-weight gain.

This is your own personal journey and I hope we can help smooth out the rough spots. Much love and hugs to you both.

[vdub]

What Hopeful said is sooooo true! Everyone reacts differently to the various drugs. Just over the past week, I had several visits to the ER due to an unknown sensitivity to a particular class of drugs. Absolutely no fun.

I also keep a list of all my drugs (15 at the moment), dosage, and who prescribed them. At the top of my list is the name of the diseases I have. If you have to go to the ER or a different doctor for some reason, you would be surprised how many don't know what Wegener's is. On the same list, I also have all my surgeries (6), and the phone number/fax number for each of my doctors (5 different docs) and the phone number for my drug store. I carry that list with me everywhere. It is indespensible.

Some people on the forum also keep a journal of how they feel from day-to-day, activities they did, drugs they took, and things like that. It helps to be able to go back and look for patterns.

[LynnZielinski]

Thank you everyone. You give me great hope for understand more. You knowledge will be a great help. As for Abs, I am not sure she will "join" and post on her own. She may use my acct from time to time. She is really shy, she has tried to hide her disabilities for a long time. She will be reading what you post to me. Some of my questions are actually hers.

I will figure out the signatures. I will also give a much more detailed history of what she has gone through. This has been a long journey so far and as a child she just wants to be normal, what ever that is. She has never been able to play sport/cheerleader/or just run...and it doesn;t look like she will being playing soccer with her big sister this year either (she had a really good year last year and was hopping to be in remission with everything..

I need to start a journal and go back to the start of her illness. Thanks for the idea.

Lynn

[Al]

Thank you everyone. You give me great hope for understand more. You knowledge will be a great help. As for Abs, I am not sure she will "join" and post on her own. She may use my acct from time to time. She is really shy, she has tried to hide her disabilities for a long time. She will be reading what you post to me. Some of my questions are actually hers.

I will figure out the signatures. I will also give a much more detailed history of what she has gone through. This has been a long journey so far and as a child she just wants to be normal, what ever that is. She has never been able to play sport/cheerleader/or just run...and it doesn;t look like she will being playing soccer with her big sister this year either (she had a really good year last year and was hopping to be in remission with everything..

I need to start a journal and go back to the start of her illness. Thanks for the idea.

LynnLynn, I would, for many reasons, encourage Abby to write in, both with her own personal narrative and with as many questions as she can come up with. Every journey is indeed different; yet we are all travelling together. The web is a good place to start the researches, but it also helps to talk to others who have been there. While most of us would love to "hide our disabilities", for social, personal, or professional reasons, this forum is one place where judgments based on such little things are definitely out of place. Life throws us curve balls, even at young ages. The trick is to figure some way to hit 'em out of the park. In this, we all coach each other.

I also encourage you, strongly, to make contact with a vasculitis specialist. Cytoxan is a somewhat surprising therapeutic choice, as the preferred "heavy hitter" these days, especially for adolescent females, is rituxan. But many non-specialist docs have limited experience with RTX. This is worth some discussion, I think.

Al

[annekat]

It's entirely possible that Abby will be able to play soccer next year. Also, I don't blame her for being shy about posting, since most of us are so much older. There have been people around 15-18 posting on the forum, though I haven't seen them lately. If she ever does feel like it, we'd love to hear from her!

[annekat]

I also encourage you, strongly, to make contact with a vasculitis specialist. Cytoxan is a somewhat surprising therapeutic choice, as the preferred "heavy hitter" these days, especially for adolescent females, is rituxan. But many non-specialist docs have limited experience with RTX. This is worth some discussion, I think.

Al Al, isn't it still true that RTX is very, very expensive and not always approved by insurance? Cytoxan worked very well for me, though of course there are the risks with long term use. (I'm now on methotrexate.)

[Al]

Al, isn't it still true that RTX is very, very expensive and not always approved by insurance? Cytoxan worked very well for me, though of course there are the risks with long term use. (I'm now on methotrexate.)Yes, Anne, RTX is expensive. So is life. Anyway, it, in most countries, is officially approved for use with Wegeners and associated vasculidites. So insurance is obiged to cover it if the doctor orders it.

Al

[LynnZielinski]

The plan is to more her to rituxan after the 6 months of cytoxan. the inflammation is severe in her kidneys atm. Once they have it under control they will step down to it.

[annekat]

Yes, Anne, RTX is expensive. So is life. Anyway, it, in most countries, is officially approved for use with Wegeners and associated vasculidites. So insurance is obiged to cover it if the doctor orders it.

AlI am now on Medicare and don't know what to expect if a doctor were to order it sometime in the future. Do you think they would cover it? Has anyone else on Medicare gotten it covered easily?

[vdub]

Apologies in advance. I really hate it when someone chimes in and basically says "well I've never had this done, I have no experience with it, and I have no first-hand knowledge about it, but I do have an opinion". Unfortunately, that's exactly what I'm about to do..... <sigh>

I am on TriCare which is the very close cousin to MediCare. I have not had RTX, but I have had some outlandishly expensive procedures and TriCare hasn't even blinked. Authorization routinely comes through in 24 hrs. MY gut belief is that they would cover it.

[annekat]

Apologies in advance. I really hate it when someone chimes in and basically says "well I've never had this done, I have no experience with it, and I have no first-hand knowledge about it, but I do have an opinion". Unfortunately, that's exactly what I'm about to do..... <sigh>

I am on TriCare which is the very close cousin to MediCare. I have not had RTX, but I have had some outlandishly expensive procedures and TriCare hasn't even blinked. Authorization routinely comes through in 24 hrs. MY gut belief is that they would cover it. Thanks, Vdub, and no apology needed. I hope you are right, but I'm thinking there'd be some sort of copay involved, and any typical percentage of copay on $40,000 isn't affordable by me or a lot of other people. Or has the cost of RTX gone down from what I remember reading on here? Of course being on Medicare, I need to pick a drug plan and pay for it separately, which I haven't done yet. I'm thinking to get very expensive drugs covered at all I'd need to pick one of the more expensive drug plans. In reality, I'm looking for one of the cheaper ones with no deductible, given my financial situation. All of this is why I was questioning Al's implication that nowadays, Rituxan, not Cytoxan, would be the automatic drug of choice for hitting severe cases of Wegs hard in the beginning stages. In my beginning stages, I was on Washington's Basic Health plan for low income, and my doc automatically put me on CTX, with the implication that the more expensive drugs wouldn't be covered, and that he'd run into the problem with people on Medicare. Of course, I think that was right before RTX was fully approved by the FDA or whatever. In any case, the copay with Basic Health would have been 50%, assuming there was no generic available and there was another alternative, CTX. Or IS there a generic RTX, or will there be? These are all rhetorical questions at this point, as I don't see myself needing RTX or more CTX anytime soon, if ever.

[Dryhill]

Also a good thing to do is have a list of your meds: when you started a drug/treatment; how much/often; when and why treatment was discontinued. It's much easier to whip out this piece of paper than to have to try to remember everything when you see a new doctor (or even the same doctor!). By giving a typed copy to your doctor it becomes a part of your medical file. Doctors and nurses have told me how much they appreciated the list.This is your own personal journey and I hope we can help smooth out the rough spots. Much love and hugs to you both.

Every time I see my doctor he asks what meds I am on, it is there in the file and on the computer screen, but he knows I can supply the information more quickly than looking it up. Also remember to tell your dentist abouit Abby's meds, especilaly Pred.

Jim

[vdub]

Also remember to tell your dentist abouit Abby's meds, especilaly Pred.
Good tip! I'd never considered that.

[Al]

Thanks, Vdub, and no apology needed. I hope you are right, but I'm thinking there'd be some sort of copay involved, and any typical percentage of copay on $40,000 isn't affordable by me or a lot of other people. Or has the cost of RTX gone down from what I remember reading on here? Of course being on Medicare, I need to pick a drug plan and pay for it separately, which I haven't done yet. I'm thinking to get very expensive drugs covered at all I'd need to pick one of the more expensive drug plans. In reality, I'm looking for one of the cheaper ones with no deductible, given my financial situation. All of this is why I was questioning Al's implication that nowadays, Rituxan, not Cytoxan, would be the automatic drug of choice for hitting severe cases of Wegs hard in the beginning stages. In my beginning stages, I was on Washington's Basic Health plan for low income, and my doc automatically put me on CTX, with the implication that the more expensive drugs wouldn't be covered, and that he'd run into the problem with people on Medicare. Of course, I think that was right before RTX was fully approved by the FDA or whatever. In any case, the copay with Basic Health would have been 50%, assuming there was no generic available and there was another alternative, CTX. Or IS there a generic RTX, or will there be? These are all rhetorical questions at this point, as I don't see myself needing RTX or more CTX anytime soon, if ever.Anne, they should cover it. But if there is any problem, there is also a program to subsidize copays. Just check with the the Rituxan website.

Al

[annekat]

Anne, they should cover it. But if there is any problem, there is also a program to subsidize copays. Just check with the the Rituxan website.

Al Thanks, Al. I don't even know if I'll ever need it, but if I ever have a bad Wegs relapse, I suppose I will, since I may have already had my "quota" of Cytoxan. I can also ask any drug plans that I'm considering signing up with what their position is on it. I had heard about the subsidizing program, too. Will visit the RTX website just to get the lay of the land.

[Sangye]

I am now on Medicare and don't know what to expect if a doctor were to order it sometime in the future. Do you think they would cover it? Has anyone else on Medicare gotten it covered easily?
I'm on Medicare. I started rtx before it was FDA-approved for Wegs and Dr Seo just had to submit an explanation that it was my only drug option. Once it was FDA-approved we haven't had to appeal anything.

[Sangye]

Thanks, Vdub, and no apology needed. I hope you are right, but I'm thinking there'd be some sort of copay involved, and any typical percentage of copay on $40,000 isn't affordable by me or a lot of other people. Or has the cost of RTX gone down from what I remember reading on here? Of course being on Medicare, I need to pick a drug plan and pay for it separately, which I haven't done yet. I'm thinking to get very expensive drugs covered at all I'd need to pick one of the more expensive drug plans. In reality, I'm looking for one of the cheaper ones with no deductible, given my financial situation. All of this is why I was questioning Al's implication that nowadays, Rituxan, not Cytoxan, would be the automatic drug of choice for hitting severe cases of Wegs hard in the beginning stages. In my beginning stages, I was on Washington's Basic Health plan for low income, and my doc automatically put me on CTX, with the implication that the more expensive drugs wouldn't be covered, and that he'd run into the problem with people on Medicare. Of course, I think that was right before RTX was fully approved by the FDA or whatever. In any case, the copay with Basic Health would have been 50%, assuming there was no generic available and there was another alternative, CTX. Or IS there a generic RTX, or will there be? These are all rhetorical questions at this point, as I don't see myself needing RTX or more CTX anytime soon, if ever.
Since rtx is administered in an infusion clinic, it should go through Part B, not Part D. Don't go to an independent infusion clinic, go to one in a hospital.

[Sangye]

Lynn, welcome to the group. I'm so sad to hear what Abby has gone through for her entire life. Does she have PA or JRA? Wegs will not cause cartilage destruction, so something else is definitely going on.

Please get Abby to either Mayo in Rochester or Cleveland Clinic (not sure which is closest to you). You cannot lose time with doctors who aren't specialists in Wegs, especially not with Abby's complicated history and severity of kidney involvement. Get the best, even if you have to travel for it. You can use a local doctor in between occasional visits to the Wegs specialist.

[annekat]

Since rtx is administered in an infusion clinic, it should go through Part B, not Part D. Don't go to an independent infusion clinic, go to one in a hospital. Thanks so much, Sangye! What you said occurred to me later, that it would be Part B and not subject to the Part D regulations and routines. That is great news, and I can feel more secure in the event of needing it in the future. I think I've had as much CTX as anyone should have, so RTX could be considered my only option for a major flare. (I'm now on MTX as a maintenance drug, more or less, with AZA in the background as an alternative, though the MTX seems to be working fine.) In any case, it's great to hear from someone who's been there and understands how Medicare works.

[Sangye]

I didn't realize it would go that way. We had gotten the Part D approval and never needed it!

[annekat]

I didn't realize it would go that way. We had gotten the Part D approval and never needed it! Hmm... well, of course I didn't really know, but had seen that in-hospital chemotherapy was covered under Part B so thought this could be similar. I'm thinking there could still be some copays on Part B stuff, but you didn't mention one in your case, so it all sounds good. I'm still thinking it might be hard to get RTX approval right after diagnosis of severe Wegs as long as CTX was a feasible alternative, which in my case it was. (My Wegs wasn't that severe, but there was significant lung involvement which cleared up well with CTX.)

[Jaha]

Lynn&Abby,

I am sorry that Abby has to go thru this at such a young age. I'm glad you found us and I hope you and Abby will find all the positive information and insite you will need to get thru this shocking dx, from this good group of people. I was dxed in Sept 2010 at CC and don't know what I would have done at times without this site. I wish Abby the best of care and hope she will read some of the posts here for inspiration and hope.
Jana

[KathyB]

Welcome Lynn & Abby! I've been at this for a year and found the forum to be enlightening and supportive. (Just reading this post, I've learned about Medicare Part B vs. D!) As an educator (teacher & administrator), I hope you will feel safe in contacting your school counselors, administrators, nurse and each of Abby's teachers. Their assistance and support should be invaluable to you in planning for Abby's courses, absences, and any difficulties or stumbling blocks which may occur. I wish you all well!

KB:cool: