9 year old son just diagnosed

Good Evening everyone,

This evening I received confirmation that my son does have Wegeners. A little history- last year Thanksgiving 2013. My son broke his arm. He did not cry nor complain. I just saw that he wasn't using it. That next morning we went to get an x-ray which showed a very large tumor from the marrow out. His bone was paper thin. Initially it was thought to be osteosarcom. After seeing a specialist they believed it was benign. A simple bone cyst (horrible name). He was 7. After Christmas of 2013 they removed the tumor, oh and did a marrow transplant, bone graft and put in a telescopic rod.

Less then 12hrs after surgery my son, Caden, said he wanted to go home. Not once did he cry. Not once did he complain of pain. The dr. Laughed and said if you meet your discharge orders you can go home. 12 hrs after that he did. Not a single pain med at home either. I forced him to take tylenol.

Now it is 2 days before Thanksgiving this year, 2014. Caden complains of upper right abdominal pain. I google and decide he has appendicitis. We go to the hospital, he never complains so I believe him. A chest xray reveals "multiple metastatic masses in both lungs" That is what the Dr. Tells me. But his arm was benign how are these metastatic! We are transfered immediately to a Children's hospital.

At the childrens hospital they believed it to be fungal. After a bone scan, ct, and numerous lab test (none including the anca). Infectious disease, oncology and pulmonogy doctors couldn't pin it. None of the labs made sense! On Thanksgiving a needle biopsy was done. I was then informed that the nodules have grown in size, slightly but still grown. we are discharged the same day.

We wait and wait and wait. Its not benign yay! But what is it! Follow up xray now reveals a haze all over his lungs. Still he is asyptomatic. While waiting I decide I need sleep. To get that I need answers. I schedule an appointment with an amazing oncologist at Miami Children's, Dr. DeAngulo. The same day we go to see him the pathology is back from a pulmonology pathologist out of New York. She describes it as interesting....... and list everything that is seen. Still that means nothing to the dr's by our house.

I bring that to DeAngulo. He reviews all the labs and everything i brought with us. He says i dont think its me that you need to see. I have a gut that its Wegeners. That day he admitted us at Miami Childrens. Next morning a thorescopic biopsy is done on the largest lower right nodule. 1 chest tube followed by a pneumothorax we are now home. And I just received the confirmation phone call.

I appreciate Dr. DeAngulo for heading this. With it not even being his specialty. He truly is amazing.

Now to see the rheumatologist and pulmonolgist. Do we go straight for methotrexate?

I have had to kick and beg alot to get where we are now. We caught it early. No syptoms! I want to hit this right and do what we can. Any advice is appreciated!

Thank you, Erica.

Hi Erica,

Sad to hear in someone so young. Trust in your rheumatologist, they are the coordinators. I would hesitate to make suggestions for someone as young as Caden. We have a few people on this site who were diagnosed at a young age, perhaps they can offer their stories. Please feel free to express any concerns and fears you have, we all understand. This is a big weggie family and we are here for each other, and our families.

Terry

So sorry to read this story and this terrible disease attacking someone so young. I have always thought my illness was brought on by an infection and severe stress that i was under at the time...also probably a genetic predisposition and the infection and the stress were the triggers. Hearing that a child as young as 7 has this disease is making me think again.

I hope you are directed to a Rheumatologist who has experience in treating WG. I do believe there are Pediatric Rheumatologists around but the ones with WG experience will be a rare breed indeed. I am so glad you found this site. As Terry said, don t hesitate to ask questions or express your concerns. There is a wealth of information and experience here.

The decision as to what drugs will be used will be at the discretion of the treating doctors. Methotrexte is one that is commonly used but may not be as other factors may rule against it. I guess after all pertinent test are done and the degree of involvement will dictate which drugs will be used.

Wish you, your son and all the family best wishes with your journey with WG.

Rose

Hi Erica and welcome to this forum.

I am so sorry to hear that Caden was diagnosed with WG but I think I would rather WG over Cancer.

I'm not sure that methotrexate (MTX) is the answer straight away. MTX is classed as a medium to mild hitter and is also used as a maintenance drug once things are under control.

Never fear, things will definitely get under control. We have seen many children (or parents of) on this forum and I hope they come by and say hello soon.
Kids are so resilient. They take to the treatment and it seems to work quicker in them than it does in adults.

Keep strong and ask as many questions as you need too. No question is too small or too silly.
We are all here for you, Caden and your family.

Welcome Erica...sounds like you got a tough kid on your hands...good, it'll take some toughness to get thru the next phases. MTX is mild by comparison to the others, but may be the correct one for Caden. Tho the ages are distant, my initial flare/s had almost same lung symptoms, etc. as he did. I also added sinuses...anyway, docs went directly to mtx to see if it could hold the WG down; it did; I'm weaning off it now after 3+ years. I would suspect since Caden is pretty durable already that he will get thru this pretty well. IF the mtx doesn't work or he has kidney issues with it, the docs will go to RTX, but they're right in holding off. The 'idea', if done correctly, is to knock down the flares with prednisone initially (reduce inflammation quickly), then hope the milder drug will suppress the AI adequately. If not, the big guns come out next...don't go there until needed...docs and Caden's response will determine that. Stay with the protocols, keep Caden interested in the disease as it will not go away - only go into remission, and lots of fortitude on both your parts!! Best to you both.

Hi Erica,

I agree with what the others have said. Between Caden's toughness, a good wegs doc, and the right meds, he should do well dealing with our dumb disease.

Just one other suggestion: He needs to tell you when something in his body doesn't feel right instead of toughing it out.

Cytoxan and Rituximab are the 2 big guns used to treat organ threatening vasculitis from what I have read in the literature.
Mtx is normally used when the disease is not organ threatening.
Finding the best doctor can help you decide the best route.
Ask the drs how many weg's patients they have treated in their practice and this should help you find the right one.
Treating this disease as quickly as possible is key, so don't sit on it.

Also, if u have a dr that you are comfortable with, tell him that he can consult for FREE with some of the best vasculitis drs in the world associated with the Vasculitis Foundation here
VF Medical Consultants

Welcome to the forum, but I am so sorry you all are on this journey. I am the mom of a pediatric Wegener's patient, and am in contact with several others on Facebook. Please know you are not alone, but that pediatric Wegener's in general moves much more aggressively than in adults. Lung involvement equals major organ involvement, and would indicate something stronger than methotrexate is needed. ((Hugs))

Hi Mom, My heart bleeds for any parent going thru this. I was dxed at 16 and at the time my world revolved around ME. Never thought twice about the pain my own parents had to deal with while watching thier child fight. And now having kids of my own...i cannot imagine the pain. I'd much rather go thru it than watch them. So to you-make sure you have support for you. You will need it. As for your son-hes going to be ok!!! Get the right docs asap...and that will help so much !!! Come here when you feel alone. Ppl are so kind! Im praying for you and your son and family. Even tho i dont know you-now we are connected.

Hi everyone, happy Christmas Eve.

Received a call last night from a rheumatologist in Miami. Yesterday a.m. I emailed him all the labs and pathology report. He called rather frantic..... He was in disbelief as to why Caden was discharged without any medication. He hated that the pathology revealed necrotizing. .....

So he is letting us have Christmas and we have to be in Miami at 8 am on Friday for him to start treatment. He will be admitted for awhile.

I am a bit worried that the dr. Was frantic but at the same time a bit relieved to have someone who seems to care and as concerned as I am.

I asked specifically what the treatment will be. He stated he had to really review his medical history and images to make the decision.

Without even meeting him he has a plan. Which is something we haven't had in a while.

Thanks for all the support everyone!!

Hi again,

Don't worry, they will give him a slow intravenous feed to stun everything, then they will work out medication issues. He will be OK now, as he will get the proper treatment. Enjoy your Christmas, it is already Christmas morning here, so Merry Christmas

Terry

CadensMom, I suspect it's good to have a doctor on your side who's a bit frantic - at least you know your son's situation is being taken VERY seriously, and that they will be doing everything possible, ASAP. Best wishes, prayers and hugs in the days ahead -

Susan

Welcome to the forum, I'm glad you found us even though I wish you hadn't had a need to. Caden sounds like a brave young man who can get through this. Good advice has already been given as to what drugs might be used, so we will have to wait and see. Cytoxan worked great for me to clear up my lungs, and I might have gotten by with something milder like MTX because my involvement was not as serious as Caden's. Also, I am 62 years old, and the protocols for children might be different. I trust that the rheumatologist will make the right decision, but please keep us posted, as we are pretty good at spotting rheumies who are lacking in WG experience. Thank goodness for Dr. DeAngulo. Best of luck next week with getting started on a treatment plan.

I feel for your torment at this time, it is far worst to have a sick child than to be sick yourself. It sounds as though you have found a Dr who is switched on to what will be required to get this under control and allow Caden to live a normal life like all boys should. Good Luck with the treatment plan.