Diagnosed 11-year-old

[AnaMama]

I'm Ana's mom, my name is Carrie. Ana is my precious daughter. She was diagnosed with Wegener's on Sept 27th, 2016. She just had her birthday a couple of days ago, here on the hospital, and now she's 12. We've been living in the hospital since Sept 24th, so we're going on 5 weeks now.

The disease has taken 25% of her kidneys and we were fighting for another 35%. Protein in urine has gotten much worse since our arrival. Creatinine also hasn't improved. Tomorrow she will have another kidney biopsy to see if the disease is still actively attacking her kidneys.

She's had two rituximab infusions so far with decent results. Her MPO antibodies were off the charts and now they are 3 (I'm told 0.3 is normal).

If the disease is still attacking her kidneys they plan to start Cytoxan. This is very sad for us because she hasn't had a period yet and so they can't save any eggs in case of infertility (I'm told infertility is a possible side effect).

Is there another powerful drug that would be better than Cytoxan? Should we try IV steroids again? So many questions, so little time, we must move fast.

Thank you to all who are concerned for my girl. She is the biggest sweetheart you can imagine. So full of love for all. So ready to live life to its fullest.


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[MikeG-2012]

So sorry to hear about your daughter. The only major advise I can give you is before they pull the trigger on Cytoxin, find a Vasculitis Specialist and make SURE that Cytoxin is the only option left.I hope things continue to improve so that she can have an easier battle with this awful disease.

[drz]

Some of us found plasma exchanges crucial to our survival and helping save our kidneys too. Is that an option for her? RTX is usually the first choice treatment but it can take awhile to work and then CTX is usally given. But there are some other second line drugs often given for maintenance that might be helpful like AZA, MTX, and cellcept. Use the Vasculitis foundation list for a free consult for her.

PS Kidneys often can and do some times recover a lot> It took me several years but my kidney function was near normal at last check up.

[AnaMama]

Thank you for your replies! We have done a blood transfusion and an albumin transfusion. Drz, she was given a rituximab infusion & high dose steroids for 5 days via IV after diagnosis, & she's been on daily prenizone (sp?) since. Should she have also gotten Cytoxan at the beginning, since it takes so long for the rituximab to kick in, or maybe one of the other maintenance drugs? I'm thankful she was diagnosed when she was but I wonder how much experience her pediatric nephrologists have with Wegs. They are brilliant, no doubt, it's just Wegs in kids is so rare. My biggest fear, perhaps, is that they will not intervene enough, since this disease is so unpredictably beastly. Thank you again.


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[Pete]

Hi Carrie, Here's hoping you and Ana get the treatment she needs.

I have a few suggestions for you:

1. Ask the nephrologist how many GPA patients they see in a year. If it's a small number, ask them to consult with a Vasculitis specialist or refer you to one (if it's convenient). Cleveland Clinic (where I go), Mayo, Johns Hopkins, and some others in the northeast are good to great and have lots of experience.
2. Ask your doctors questions about their treatment plan - how do the drugs work, common side effects, how soon before improvement should occur, what to do if side effects occur.
3. Don't discount odd symptoms. This disease is sneaky and shows up in different ways. If something abnormal shows up, contact your doctor.
4. Keep records of doctor visits (get a written after visit summary), medications, symptoms (new or different), and lab results (I keep mine in an Excel spreadsheet). Take these with you when you see a doctor (especially a new one).
5. Before you visit a doctor, write a list of questions and concerns so you don't forget anything - and make sure the doc takes the time to address all your concerns during the appointment. (My docs seem to appreciate my preparation.)

I was on cytoxan for 16 months at disease onset. Make sure your daughter stays well-hydrated while on it. The metabolates are hard on the bladder. Also make sure she urinates whenever she feels the urge so the metabolates are expelled frequently.

There are some women of childbearing age that have been on cytoxan who are in this forum. A forum search of cytoxan and fertility should lead you to some useful info.

Cytoxan and rituximab are the two primary induction therapy immunosuppressants. It seems that azathioprine (Immuran) and methotrexate are better for maintenance. Prednisone is an anti-inflammatory. Its role is to help the patient to feel better. Ask your doctor about the impact of prednisone on your daughter's bones as long-term high dosages tend to cause the body to lose calcium which can lead to osteopenia or osteoporosis. I take daily calcium and vitamin D supplements and have maintained stable bone density.

Encourage your daughter to be as physically active as she can handle. I hope she doesn't forget to be a kid just because she's sick. With effective treatment and some willpower, she can lead a normal life. It may take some time to get there though.

Good luck!!

[AnaMama]

Thank you for the thoughtful response, Pete. I will truly appreciate your concern and plan to take action on your advice.


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[annekat]

Welcome to the forum, Carrie, though I'm truly sorry you have a reason to be here. It is especially heartbreaking to hear of one so young being diagnosed and especially having kidney involvement, too. There is someone else here with a young daughter, a little older now, who was dx'd and is doing pretty well. If she sees this she will no doubt respond to your post.
As for infertility, I have heard of some women who went on to have babies after being on Cytoxan. So take heart, though I know it is troubling.

Good advice has been given above. If you will tell us where you live or where Ana is being treated, maybe someone will know of a good pediatric nephrologist with WG experience in your area, or other WG docs who can suggest one. WG does seem to manifest a little differently in children, from what I've seen on this forum. Not to doubt your docs, they may be fine. They could have given her CTX initially along with the RTX, but probably had good reasons for not doing so, including the fertility issue and the overall harshness of CTX. If her lab numbers don't improve soon, I'd follow her doc's suggestion to start CTX, along with the RTX, and it might not have to be for a very long time before improvement is shown. Best wishes and hugs to both of you!

[AnaMama]

Thank you, Annekat. I'm getting the sense that we will need to try the Cytoxan, and do it soon. She's laying in the sedation room right now for her second biopsy, but I feel like I can already tell you the disease is advancing again. We will know for sure soon. Thank you again for your help.


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[whatthewhat]

Hey, I have a daughter with pediatric-onset Wegener's, and am connected with many other parents. I think we can be a good resource for you - I will PM you my contact info

[Gab122]

Hi Carrie,
I'm so very sorry your daughter has this disease!
I feel for you and her especially because I have a 12 year old daughter (but I'm the one with Weg in our family) I'm sure you would switch places with her in a heartbeat!
The only other thing I can think to add is to ask if she's on bactrim?
Its an antibiotic but I understand it can have a dual purpose in both fighting off lung infection in immune suppressed patients but also has some sort of role in fighting Weg GPA too. I don't know why that is.
Has anyone else ever heard or read about that?

Ill be praying for healing for your daughter and wisdom for her doctors.
Please let her know she is not alone. Neither are you.
Sending hugs from your new Weg family.

Gabrielle

[annekat]

The only other thing I can think to add is to ask if she's on bactrim?
Its an antibiotic but I understand it can have a dual purpose in both fighting off lung infection in immune suppressed patients but also has some sort of role in fighting Weg GPA too. I don't know why that is.
Has anyone else ever heard or read about that? I remember hearing more about that in my earlier days of reading the forum. It's been suggested that Bactrim can play a role in keeping people in remission. Not only because of the lung thing but because it may control other bacteria that reside in the nasal cavity and sinuses, etc. I would assume these other bacteria could get out of control and trigger a flare. At least that's my paraphrase of what I think I may have read. But I don't know to what extent this is a proven fact. Maybe someone else can shed more light.

[Gab122]

edit: I changed typo of word 'hate' to 'share'.
Ugh - I should have checked before posting.

Carrie, thought I'd Share this story about two girls that got dx with Wegener's - a good message.
https://m.youtube.com/watch?v=6jjrZj9a-Ns

Gab

[annekat]

That was a great share, Gab! It made me teary to think how much more these girls are going through than I ever have. It's so nice that they have each other!

[AnaMama]

Gab122, I completely identify with this story. Thank you for sharing.


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[mater]

My 15 year old daughter was diagnosed in December. Since she is older, they were able to give her shots to suppress ovulation before the CTX. I don't know how well that works to protect her fertility, but I felt we had no choice. Her kidney was also affected - 90% of the filtering units were blocked and creatinine etc got worse after that for about 7 days. She is near normal labwork now so I know that kidneys can recover amazingly. My daughter got 7 pheresis treatments and also the high dose IV steroid and then 60 mg (she is down to 3 now) She got RTX as well. They did not want to put her on Bactrim because of the kidney function issues. They did put her on about 5 months later when her kidneys were doing better. Is she having to take phosphorous binders? My daughter hated those! The pheresis treatments(albumin exchange is the same thing I think) seemed to be key in the short term. CTX and RTX were not given until the 13th day. We were only in for 17 days. The treatment sounds similar to what my daughter got. She got 1 transfusion. They didn't seem to want to do those. Her hemoglobin was at 6.6 at one point but they didn't do another transfusion. This is incredibly hard for you all, even more so than for us. Hang in there. The first 7 or 8 days all my daughter wanted to do was listen to me read a Jane Austen novel. Take care of yourself. I walked the basement halls to get some exercise.

My daughter also had 2 days of dialysis.
Sorry this post isn't well organized! I just wanted to quickly respond.

[AnaMama]

Thank you so much for your reply, mater. I love that your daughter just wanted you to read Jane Austen to her. We've been indulging in movies and frequent trips to the gift shop. So bad! ;)
I'll be asking her doc about Bactrim this morning. Also want to ask about why we didn't start with the CTX, though probably because of the risk of infertility and since she hasn't had her period we can't harvest an egg. Anyway, thank you. It's important to me to hear how others have been treated and are doing so I can gage whether we're getting quality care. Looks like our care is similar. I'm delighted to hear your daughter is doing well!


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[nagesh]

hi Carrie
Very sorry to know about your daughter's condition. my son was also diagnosed for wg at the same age, I can understand your feelings.
Be confident during jan this year my son had a flare in kidney he was also given rituximab, at that time we worried a lot and confused whether Rtx started working or not because protein in urine constantly rising during 1 st and 2 nd month, next 3 rd month onwards gradually it started reducing so for my son RTX started to work only after 8 weeks.

[AnaMama]

hi Carrie
Very sorry to know about your daughter's condition. my son was also diagnosed for wg at the same age, I can understand your feelings.
Be confident during jan this year my son had a flare in kidney he was also given rituximab, at that time we worried a lot and confused whether Rtx started working or not because protein in urine constantly rising during 1 st and 2 nd month, next 3 rd month onwards gradually it started reducing so for my son RTX started to work only after 8 weeks.

This is what is happening with my daughter. The protein in the urine had been steadily rising even though it's been over a month since her first rituximab. She's had two RTX infusions so far. Her MPO antibodies were off the charts and now they are at a 3, apparently normal is 0.3, so that's better at least. Her kidneys have not gotten any better even though we've been in hospitals for about 6 weeks now. We haven't started Cytoxan yet and Im thinking that's exactly what we need. Maybe we should have started that weeks ago, that's my fear at least. Can't look back, gotta look ahead.
I'm sorry your son is going through this. Thank you for your concern.


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[annekat]

Carrie, Cytoxan won't give instant results, either, but for most it seems to be a little faster than RTX to show results. That doesn't mean the RTX is not already working, just that you are not yet seeing results. I was never on RTX but only CTX and prednisone for my initial treatment, and it took maybe two weeks to a month for me to show some improvement, but I was still in pretty bad shape given the side effects, etc., which I did get used to. After a month and a half I was able to be out in public selling the pottery I make, but not without some help from friends in loading and unloading my vehicle. And of course I was careful to do a lot of hand washing and use sanitizer. This is the way WG goes no matter what med you take. So don't beat yourself up for not having started CTX earlier. It is a rough drug, but for me, worth it if it means getting better faster. Some people get fast results from RTX, too, and no doubt your docs were hoping for that. It is all very complicated and variable. BTW, my CTX was in the form of oral pills taken daily at home, which some say is more effective than a CTX infusion. Still, if Ana is in the hospital, it might be more likely they will give her an infusion of it there, perhaps followed by pills taken at home. I was only in the hospital overnight for a battery of tests, so am luckier than her in that respect, and I've never had kidney involvement, though I still could. I just hate to hear of such a young person having a much harder time than I did. More big hugs to you both, and prayers for Ana's improvement in the near future!

[AnaMama]

I appreciate your perspective, Annekat. Im feeling pretty good about our care at this point. I think the Cytoxan wasn't used right away because Bactrim must also be used if Cytoxan is used and, as I now understand it, Bactrim isn't good when there's kidney involvement. So they must have held off until necessary. I think now is the time though, and I have a feeling that's what I'm about to hear (scheduled to talk to the doc in 10 minutes). If my understanding is incorrect, you'll can correct me, BTW. I want to learn.


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[annekat]

I appreciate your perspective, Annekat. Im feeling pretty good about our care at this point. I think the Cytoxan wasn't used right away because Bactrim must also be used if Cytoxan is used and, as I now understand it, Bactrim isn't good when there's kidney involvement. So they must have held off until necessary. I think now is the time though, and I have a feeling that's what I'm about to hear (scheduled to talk to the doc in 10 minutes). If my understanding is incorrect, you'll can correct me, BTW. I want to learn.


Sent from my iPhone using Tapatalk I don't think there's anything wrong with your understanding, except possibly that Bactrim must be used with Cytoxan. I was not started on Bactrim until a couple weeks after starting CTX, and it seemed like a bit of an afterthought. My doctor isn't the most experienced with WG. I am now on methotrexate and still taking Bactrim. If it is not good with kidney involvement, then obviously Ana should not take it, unless the doc says it's OK, but I would guess she could still take the CTX without it. Bactrim is a safeguard and a prophylactic against certain lung infections, but I've never heard of it being mandatory with any particular drug. If your doc says it is, I guess you should listen to him, as I'm absolutely not an expert. I've found on this forum that different docs say different things and have different perspectives, even among good docs and WG experts. There is not always one right way. Ana's doc sounds like a good one, but the more info you have the better. Perhaps by the time you read this, he will already have explained things to your satisfaction and set your mind at ease. Best of luck in all of this, I know it isn't at all easy making sense of it, and I doubt if many of us ever really do. Most of what I know, or think I do, was learned right here on the forum, from people with good, well known WG docs, or from people with less well known docs. There will always be conflicting info and we just have to weed through it and see what rings true for us in our particular situation, with consideration of what our docs say. :smile1:

Also, don't hesitate to correct me if what your doc says goes against anything I've written. I need to learn, too.

I might add that I think many on the forum, with or without kidney involvement, have been put on RTX as the first line treatment, without CTX or any of the other standard immunosuppressants. So there is not necessarily a right or wrong here. RTX is a relatively new drug and doctors overall are still learning how to use it and which protocols will best fit certain kinds of patients.

[AnaMama]

Thank you Annekat. Our doc did put me at ease about the decision to wait on the Cytoxan.

The kidney biopsy results weren't good. She has only 10% of her kidneys with good function, 30% are in question, and 60% is completely not functional. She will need a transplant at some point in her life. But for now she'll be managing with what she's got. The good news is that the inflammation had gone away, so the disease is not actively attacking anymore. The disease took her kidneys as it was backing away though, like a truck on a highway...you can put the brakes on it but it will keep going for a bit before completely stopping.


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[annekat]

Thank you Annekat. Our doc did put me at ease about the decision to wait on the Cytoxan.

The kidney biopsy results weren't good. She has only 10% of her kidneys with good function, 30% are in question, and 60% is completely not functional. She will need a transplant at some point in her life. But for now she'll be managing with what she's got. The good news is that the inflammation had gone away, so the disease is not actively attacking anymore. The disease took her kidneys as it was backing away though, like a truck on a highway...you can put the brakes on it but it will keep going for a bit before completely stopping.


Sent from my iPhone using Tapatalk I only pressed "Like" because I like the way you described what is going on, the truck on the highway. I'm very sorry to hear about the extreme loss of kidney function and probability of a future transplant. I know some on here have come back from very severe kidney involvement and gotten most of their function back. But they could relate this much better than I. Starting another thread with kidneys mentioned in the title might get their attention. I'd have to look back to see if any of them already wrote on this thread. Also, you can always use the search function for specific topics discussed in the archives. At least I can "Like" that Ana's inflammation and disease activity are under control and shouldn't be causing much further damage for now. That is very good to hear. I hope she gets to leave the hospital soon and live life a little before facing the prospect of a transplant. Keep us posted. :hug1:

[drz]

I was never given Bactrim cause I am allergic to Sulfa but I was bothered with frequent bladder infections while on CTX for several months and it had to be held often when my WBC got too low. Extra protections like isolation and sterile precautions were often used too during those periods to try reduce the risk of infection. I was treated several times too with various antibiotics.

My kidneys and lungs were both hard hit and I needed a couple weeks intubation and umpteen plasma exchanges and both IV RTX and CTX and got very close to needing dialysis but things finally turned around (I survived) and in a few years I recovered to near normal kidney function labs even though I only have 40- 50% normal function. People have redundant kidney function built in as only one is needed to maintain normal kidney function labs.

Younger people also tend to rebound and recover quicker and better than us old seniors too. Youth is an asset in recovering from GPA (Wegs).

[Middle of no where]

Check out book by Sarah Ballentyne
The Paleo Approach, Reversing Auto immune
Changing my eating, which would be difficult for an 11 year old, has given normal blood results for me.
Worth a try, health, it all starts with food.

[mater]

Check out book by Sarah Ballentyne
The Paleo Approach, Reversing Auto immune
Changing my eating, which would be difficult for an 11 year old, has given normal blood results for me.
Worth a try, health, it all starts with food.

I have wondered about diet and after reading your post I started a thread in the general forum called How to tell if dietary changes are effective

[Alysia]

Check out book by Sarah Ballentyne
The Paleo Approach, Reversing Auto immune
Changing my eating, which would be difficult for an 11 year old, has given normal blood results for me.
Worth a try, health, it all starts with food.

We never saw any weggie here who was cured by diet. If I am wrong, anyone around, please let me know. Middle of no where - you can tell your story under new members introduction. If you just want to advertise this is not the place. Sorry.

[Alysia]

Dear AnaMama, my heart is going out to you and your daughter. I cant add much to what the others said. Plasma pheresis might help with the kidneys. You need to make sure that the docs have enough experience with treatment of kids with wg. Also ask to make consultations of as many experts as possible. It takes time for rtx to work. About 2-3 months. Meantime enough pred might "hold" the beast. I am ignorant about ctx. Sending many prayers ♡♡ please update us.

[whatthewhat]

Definitely echoing that kids often come back from kidney damage much better than adults do. As far as Cytoxan, we held off on it for a number of reasons until there was kidney involvement (100% involvement, 23 or 26% function, it was touch and go) and then she went on Cytoxan. The Cytoxan knocked the WG beast down pretty hard and fast, while the Rtx took more time to work.

[AnaMama]

Thank you for your care and warm welcome to this forum. I truly appreciate it. I know I'll be here asking questions as we learn how to live with this beast of a disease. This forum is such a rich resource and it's people are so caring.


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[annekat]

We never saw any weggie here who was cured by diet. If I am wrong, anyone around, please let me know. Middle of no where - you can tell your story under new members introduction. If you just want to advertise this is not the place. Sorry. I think you are right, Alysia. But there are some good diets out there that are better for us than some others or than just eating whatever we feel like. No diet should be considered a cure for Wegs, but I can understand someone who is doing very well having the feeling that a special diet played a big part. Many of us are not that lucky, even if we follow the best diet in the world, take the right meds, and get all the exercise we can. I don't follow anything special but do try to eat healthful stuff, and I should get more exercise and could if I tried harder. Yet I'm one of the lucky ones who is doing pretty well, as a result of the treatments I've been prescribed.

[Gab122]

Hi AnaMama Carrie, How is Ana doing?