13yr daughter has Wegeners

My 13yrs daughter was diagnosed with this disease 2yrs ago. It has always affected the one eye where it would swell. She currently takes 10mg of Prednisolone, 1000mg of Mycopohenlate, 20mg Omeprazole and has Rituximab infusions every 6 months. Last week her other eye started to swell and the doctors confirmed the disease is now affecting this eye also. I?m at my wits end with worry, we have the hospital this afternoon and I?m anxious about what they?ll say. I have lots of questions but can anyone tell me what to expect as she gets older, how this will affect her life and what other medication can be considered.

I am so sorry that your young daughter has this very awful unpredictable disease. Does she see a Wegeners specialist? It does get better with the proper treatments and really good doctors to guide your daughter through this. She will probably have to have multiple specialists along the way. It would be good for her to see an eye specialist who knows about Wegeners. The doctor today should be able to refer her to one. There are several other options of treatments.
I'm sorry that you have to join us, but you will find a lot of good information and friends on this wonderful site. If you use the search, you can read really good treads on just about any subject. This was my first place to join after my dx, over 8years ago. This is the best group of people with so much knowledge and compassion you will find. Welcome to our family.
I'm wishing her all the best for getting the best care.

Smytk,
I am sorry about your daughter, I can only imagine what it is like having a child with this. Please have faith! My WG started in my left eye lid then my right. I was not as young as your daughter-I was 17. I have had this disease most of my life. That being said-there are calm times and there are horrible times. Personally, during the 23 years I have had it-the longest I have been without medication Is 4 months. That being said I have not always felt terrible if my disease was under control with medication. There are going to be good times. The trick is to get the disease under control then your daughter will feel better. I hope you have some comfort knowing that you are not alone & we are all here for you! Take a deep breath. Your doing awesome! Tell your daughter I said hi!!
Natrice


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There is no way to predict how it will affect her life. Having a WG specialist to help respond to symptoms quickly is very important and it sounds like you are doing a great job with that. I was 19 when I became ill and I was very very sick the next two years. At that time Cytoxan was the main treatment and I was on it for two years. I then achieved remission and was drug free and in great health for the next twenty years. This was 42 years ago. I have had eye involvement and it certainly is really scary. I have no damage or impairment as a result.

How long has it been since her last infusion? Some people do not do well on the 6 month schedule. I myself repeatedly fell apart before my 6 month infusion and the time was shortened to 5 months. This has worked well for me. I have heard of people on as short a schedule as 4 months.

How long has she been getting Rituxan? Some people it takes longer to get good results. This was true for me. Initially I believed it wasn't going to work at all because it didn't seem to help much. Now, my disease is very well controlled with Rituxan , 12.5 mg of methotrexate and 10 mg of prednisone.

Welcome to the forum. I am so sorry that your daughter needs to fight WG at such a young age. Each one is different. So you cant predict in advance. Having good docs is very important.
I suggest you to send a private msg here on this site to @whatthewhat . I also tagged her here so she might come and write.
Sending you prayers. Please update us.

Thanks, Alysia, for the heads-up! Hello Smytk, I have sent you a private message. You can read about my daughter's story by clicking my profile name etc. Although my daughter has not had eye involvement, I know a couple other kids who have, and I know many, many pediatric-onset WG cases overall. In general pediatric-onset WG flares more easily, and seems to move more quickly and target certain organs more readily. So, besides the differences that come with a still-developing body, there are other things that make it helpful to have direction for your daughter's case from doctors who have experience in treating pediatric-onset WG specifically and not just WG in general. If you do not have access to very good pediatric rheumatologists (Seattle, Dallas, Los Angeles, San Diego, Stanford, Philadelphia, D.C., Cincinnati, maybe Toronto...) then your daughter's docs can consult with a team from one of the children's hospitals mentioned in the cities listed above.

To answer your specific questions, studies have shown that a combination of Rtx and Ctx is most effective in stopping the WG progression in children. She may reach a medically-controlled remission, and very much can possibly have a fairly normal young adulthood and adulthood.

I just realized you are probably from the UK. I think you might want to ask your docs to consult with ped rheumys from the US - I believe our treatments are a little more effective than yours (?)

whatthewhat - I had not heard that Rtx and Cytoxan in combination was more effective for pediatric cases. Can you tell me what study that was or point me in the right direction? Thanks

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Originally Posted by me2

whatthewhat - I had not heard that Rtx and Cytoxan in combination was more effective for pediatric cases. Can you tell me what study that was or point me in the right direction? Thanks

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Here's a study about the efficacy of Rtx and Ctx in refractory WG https://www.ncbi.nlm.nih.gov/pubmed/28134075/ and here's an article citing a study about how pediatric-onset WG is refractory https://www.medpagetoday.com/rheumat...matology/50745 I hope this helps me2; it's another parent of a ped Weggie who has done an amazing job of keeping track of studies - too bad none of us were med students, I tell ya!!

I don't have much to offer other than well wishes, and I also wanted to comment on the members of this forum being able to bring up studies and clinical trials at the drop of the hat. Unbelievable. On behalf of all of us in the GPA community, thank you for your unbelievable service. You are a treasure!!

It is undoubtedly scary having a child with a serious disease. We believe our son had Wegener’s/PAN from preschool age although it wasn’t diagnosed until he was 18. It was devastating as he has been a very talented musician/performer from the beginning. Additionally when he was 2 weeks old his 3 year old sister had to have open heart surgery and she is completely well now. For us the unknown was far scarier than the diagnosis. When he was 12 years old he was hospitalized with Guillain-Barre which didn’t go away & was finally rediagnosed 6 years later as CIDP. Many years of chronic illness W/O a diagnosis intervened. In 1998 both my son and I were diagnosed with CIDP & we were both treated with IVIG. This diagnosis was changed by Los Angeles area specialists as Wegener’s/PAN later that year and our treatment began. After 20 years of this diagnosis we have both done well considering. Our lives are free of many of the drugs we’ve been treated with earlier by choice: no pain meds, no prednisone or other immune suppressants other than a choice to resume Rituxan treatment as needed. We work hard to exercise as much as possible, maintain an anti inflammatory diet & healthy body weight.Hope this helps.