Mother of 13 year old daughter diagnosed with GPA in August

[MamaEndora]

Hello All!
I never pictured myself in a forum like this, but I bet none of us did. Here I am, or shall I say here we all are. I hate the fact that you all are fighting this ugly disease, but look forward to getting to know you and am asking for your experience and guidance as we battle this together. My daughter is 13 years old and has been dealing with autoimmune problems since the day she was born, but NO one knew what we were dealing with until August of this year. Initially we were told it's eczema, it's allergies, it's whatever. She did small doses of methotrexate from age 6 to age 11. She took herself off at 11 as she hated it so much. She continued to have problems, but this past summer I noticed her having fluid coming out of her nose and huge bloody scabs coming out of her nose. Took her to the ENT and he had a horrible look on his face before telling me what he thought he was seeing. 3 days later surgical biopsies, then he worked hard to get her into the Univ. of Iowa Children's hospital with in a week or two and our adventure began. My daughter has been home schooled because when she was little she always caught everything and school on the traditional level didn't work. She was scheduled to enter junior high this year and when she was diagnosed and spent a week a the children's hosp. shortly before school started she was devistated by this diagnosis and is presently depressed and angry. She is only doing school on a part time basis and is a jock as she puts it. She played, soccer, volleyball, did swimming and presently is in basketball. She just wants to be normal and is scared as a result she is angry and hasn't been cooperating since Dec, at which time we learned the disease isn't getting under control and that they want to do serious plastic surgery on her nose so that it doesn't collapse any more than it is already. My husband, son and I are scared too, but due to having had numerous health problems myself I learned a long time ago you can't wait for the storm to pass you have to dance in the rain. I want her to get to a point she can do this, but right now we're struggling. She refuses to see a psychologist and I'm at a loss. The plastic surgeon says she won't do anything until my daughter is ready, but without terrifying the kid how do I get across to her how serious things are and that she has to cooperate and help herself to get the best results for her health and her happiness. I know this is frightening! Any guidance is appreciated. Have a good night and peace to all.

[drz]

Hello All!
I never pictured myself in a forum like this, but I bet none of us did. Here I am, or shall I say here we all are. I hate the fact that you all are fighting this ugly disease, but look forward to getting to know you and am asking for your experience and guidance as we battle this together. My daughter is 13 years old and has been dealing with autoimmune problems since the day she was born, but NO one knew what we were dealing with until August of this year. Initially we were told it's eczema, it's allergies, it's whatever. She did small doses of methotrexate from age 6 to age 11. She took herself off at 11 as she hated it so much. She continued to have problems, but this past summer I noticed her having fluid coming out of her nose and huge bloody scabs coming out of her nose. Took her to the ENT and he had a horrible look on his face before telling me what he thought he was seeing. 3 days later surgical biopsies, then he worked hard to get her into the Univ. of Iowa Children's hospital with in a week or two and our adventure began. My daughter has been home schooled because when she was little she always caught everything and school on the traditional level didn't work. She was scheduled to enter junior high this year and when she was diagnosed and spent a week a the children's hosp. shortly before school started she was devistated by this diagnosis and is presently depressed and angry. She is only doing school on a part time basis and is a jock as she puts it. She played, soccer, volleyball, did swimming and presently is in basketball. She just wants to be normal and is scared as a result she is angry and hasn't been cooperating since Dec, at which time we learned the disease isn't getting under control and that they want to do serious plastic surgery on her nose so that it doesn't collapse any more than it is already. My husband, son and I are scared too, but due to having had numerous health problems myself I learned a long time ago you can't wait for the storm to pass you have to dance in the rain. I want her to get to a point she can do this, but right now we're struggling. She refuses to see a psychologist and I'm at a loss. The plastic surgeon says she won't do anything until my daughter is ready, but without terrifying the kid how do I get across to her how serious things are and that she has to cooperate and help herself to get the best results for her health and her happiness. I know this is frightening! Any guidance is appreciated. Have a good night and peace to all.

I am sorry you need to be here but glad you found us. There are some other parents who have shared your ordeal and hopefully some will contact you soon. Getting some expert consultation from doctors with experience treating children is good first step since there are some differences. As for gaining her acceptance for her disease, consult with the experts, like pediatrician, child psychologist, social workers, pediatric nurses etc. And some of these experts can see her for consultation as members of the care team and maybe advise you as they assess her and contribute to her care team. Being an inpatient for treatment of Wegs will normally involve being seen by a myriad of different doctors and care professionals so that will not be unusual. The hospital may have a way to determine who directs this team since some one needs to do that but as the parent you have an important role in approving the care plan so you can have great input too. Make sure you build in support for yourself and the rest of the family if it is not already sufficient.

Most of us have needed assistance accepting and adjusting to managing our wegs, the "gift that often keeps on giving", but one that can usually be adequately managed with proper care. Young people often tend to have a better prognosis too for a quicker recovery so be hopeful that things can get better soon as they usually do once the diagnosis is made and correct treatment begun.

[vdub]

I don't think the nose surgery is anything that needs to be pushed for. That is, if its purely for cosmetic reasons to correct a saddle nose, then that can be done most anytime. However, if the surgery is to open up sinus channels or something, then, yeah, it probably has to be fairly soon.

I'm sorry she has this disease, but as she'll find, she doesn't have to look very far to find someone much worse off than her. 13 is a bad age for a young lady to be going through this, but she'll manage. I would think that she will eventually, if not already, be put on high doses of prednisone. Unfortunately, pred has some bad side-effects like weight gain and erratic mood changes. On the plus side, though, she'll feel a lot better.

Keep us posted and you can tell her that she can jump on the forum and ask questions herself.

[Debbie C]

Hi MamaE. Its sad to hear that someone so young has this disease. I imagine since she has been sick all her life that this is kinda of the straw that broke the camels back !!! She is probably just wanting to be a normal teenager and hang with friends. The most important thing is now that she has been officially diagnosed that you find in expert in the field of wg. Usually a rheumatologist will manage things. You can look on the Vasculitis Foundation website to find one near to you. I think the best thing you can do is just be honest with her about how serious things can get if she refuses to get and take the meds she needs. Drz. made some good points about her seeing a therapist,its hard for adults to deal with this,I couldn't imagine having to go through it at her age. The prednisone will definitely cause alot of changes but that is what she needs to be on. Good luck to all of you and ask any questions or just vent whenever needed.

I like your saying by the way !!! :)

[Alysia]

Welcome to the forum Mama. Not sure I understand what treatment for WG is your daughter getting ? Does she still have nose bleeding ?

There are more parents of kids with WG around here. Please check more threads. Hang in there. When she will get the right treatment she will be soon much better in all ways. Kids are stronger then us.

vdub andrew maybe it can be helpful to have subforum of pediatric WG in order to help parents ? Is it possible to make it ?

[vdub]

maybe it can be helpful to have subforum of pediatric WG in order to help parents?
That's not a bad idea, Alysis. Is pediatric treatment so different that it needs to be discussed differently? That's an open question that I don't know the answer to. Maybe some pediatric parents could weigh in. I would hate to see something missed by a parent because they thought the pediatric forum was the limit of knowledge on the subject. But, I'm open either way and I'm sure Andrew will be, too.

[Jaha]

Mama, Welcome to the best most informative forum you will find it there. There will always be someone around to vent to or ask questions to. I am sorry that your child has to go thru such a nasty disease and wish you guys all the best in getting her to take care of herself and consider the treatment options given to her.

[andrew]

That's not a bad idea, Alysis. Is pediatric treatment so different that it needs to be discussed differently? That's an open question that I don't know the answer to. Maybe some pediatric parents could weigh in. I would hate to see something missed by a parent because they thought the pediatric forum was the limit of knowledge on the subject. But, I'm open either way and I'm sure Andrew will be, too.

Yep I think it's a good idea too. I'll make a separate thread to get feedback.

[whatthewhat]

Welcome, MamaE. I am so sorry you are going thru this. My daughter was diagnosed at age 14, 4 years ago. Pediatric- onset Wegeners can be much more aggressive and quick than adult-onset, and studies show it is more refractory (goes to flares / out of remission ) more frequently than adult-onset, so it's definitely time to use all your familiarity in working with the medical establishment to be an advocate for what your girl needs. Getting the disease under control is the top priority and it doesn't sound like that's been done yet. High doses of steroids can heighten already strong adolescent mood swings. I'm concerned her medical team isn't very experienced with pediatric-onset Wegeners (you can ask / insist they consult with pediatric rheumys experienced in WG,) that you don't mention a social worker (should have been assigned,) that maybe the whole family needs some type of counseling (definitely younger sibling in our situation was very much affected by sister's diagnosis etc.) Please private message me with any questions, and take care.

[Green Grass & High Tides]

Hi Mama Endora,

I'm sorry to hear about this illness with your daughter. It sounds like a long struggle but that you now have definitive diagnosis?
I was misdiagnosed for two years. It was such a relief to finally be able to know what I was fighting.

Being 13 is rough no matter what, imo, but the faith you've learned "dancing in the rain" is probably the best thing you can pass on...by example.

I hope the tx and pred gives her energy and a chance to play and be a "jock".
Fun and laughter has been a great pathway for me to accept, more and more, the severity of wegeners. I'm still trying.

Not sure I've added much to the great suggestions already but just wanted to chime in and say don't get discouraged!

Best wishes
Tom

[Mrs.M]

Hi MamaEndora,

First, I'm sorry to hear that your child has this diagnosis. It's a rough disease, but it's one that can be managed.

My husband, while not a child, lived in denial about the diagnosis for a couple of years. Once it got to see the difference between a flare and no flare, he became less agitated when it came to dealing with the disease.

My singular question is whether or not her WG is under control?

Sent from my SAMSUNG-SM-G935A using Tapatalk

[vdub]

I've been dx'ed with wegs for nearly 7 years now and I'm not sure what "under control" really means. In fact, just this morning, my pulmonologist asked me if my wegs was in remission. I really don't know, so I have put the question to my rheumy and he hasn't got back to me yet. Does remission mean you feel great and you're getting on with life or does it mean you aren't going downhill any further from where you are?

I hurt most all the time. Is that a normal consequence of the disease even if its under control? Or, does it mean I have active wegs? I really don't know. During the time I've had wegs, I've only felt really well for about a 6 month period a couple years ago. I'm not totally disabled, but I'm certainly not feeling well.

I am really tired of this disease. For 7 years I have thought "just after this next procedure, things will be better", or "just after this next surgery", or "maybe when summer comes", etc, etc. But, it doesn't get better. Very depressing.... I was told this afternoon that I have an unexplained enlarged lymph node between my heart and lung. I didn't even know I had lymph nodes there! :-)

[Mrs.M]

I've been dx'ed with wegs for nearly 7 years now and I'm not sure what "under control" really means. In fact, just this morning, my pulmonologist asked me if my wegs was in remission. I really don't know, so I have put the question to my rheumy and he hasn't got back to me yet. Does remission mean you feel great and you're getting on with life or does it mean you aren't going downhill any further from where you are?

I hurt most all the time. Is that a normal consequence of the disease even if its under control? Or, does it mean I have active wegs? I really don't know. During the time I've had wegs, I've only felt really well for about a 6 month period a couple years ago. I'm not totally disabled, but I'm certainly not feeling well.

I am really tired of this disease. For 7 years I have thought "just after this next procedure, things will be better", or "just after this next surgery", or "maybe when summer comes", etc, etc. But, it doesn't get better. Very depressing.... I was told this afternoon that I have an unexplained enlarged lymph node between my heart and lung. I didn't even know I had lymph nodes there! :-)
Vdub,

What is your B cell count? It should be zero. If it is higher than that, then your disease is active.

Sent from my SAMSUNG-SM-G935A using Tapatalk

[vdub]

What is your B cell count? It should be zero. If it is higher than that, then your disease is active.
I wish it was that simple, but its more involved than that. I'm hoping my rheumy can explain it to me and then I'll pass it on to everyone, but I don't think its a black and white issue. I suspect there are several components to dx'ing a flare. For that matter, there's probably several components to determining if you are in remission.

[Mrs.M]

There is. However, the most significant factors seem to be b cells and pr3. Best of luck to you.

Sent from my SAMSUNG-SM-G935A using Tapatalk

[Alysia]

Vdub,

What is your B cell count? It should be zero. If it is higher than that, then your disease is active.

Sent from my SAMSUNG-SM-G935A using Tapatalk

I agree with vdub. Its not that simple. My wg doc says that one can be flaring even with zero B cell count. Also not all of us are anca positive so PR3 is not allways an indication (although for me it is).

Vdub, I am sorry for the ongoing issues you have to handle. It is tough. I keep you in my prayers. With love ♡

[vdub]

I had time today to do some research on b cells. Its very complex, but as near as I can determine, b cell count has more to do with how effective the treatment is with respect to tamping down your immune system, than it does as a measure for a flare. But, you sure don't want to take my word for it. This seems to be a good article.
RBCS - Clinical: Relative B-Cell Subset Analysis Percentage (http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/36440)

I left a note for my PCP today to see if I should have a PR3 test done. I think that might be a better indication of a flare. I don't recall that I've ever been tested for PR3 ANCA, but surely I was. I do recall a test for cANCA early on and as I remember it was not positive. I probably should have left the note for my rheumy, but I decided to go with the PCP first since I already have an apt scheduled for a week from now.
PR3 - Clinical: Proteinase 3 Antibodies, IgG, Serum (http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/82965)

[Aneinu]

I heard that from doctors as well. B cells might not be a proof that the disease is active. It is more from the blood tests and the inflammation levels and ANCA.

Was there a thread made for parents?

Sent from my SM-G920V using Tapatalk