15yo Son diagnosed last week with WG

[ske2chief]

Hello everyone,

My 15 yo son was recently diagnosed with WG last week. He had been on medication for acne and developed ulcers on his back, neck and face. He also lost about 20 lbs and was extremely tired and aches and pains in his joints. He also had ulcers in his nose. We went to several doctors, pediatrican, dermatologist, Infectious disease, ENT and wound care without recieving a diagnosis. They all said that he was healthy and it would just take time to heal.
After about 2 months of dealing with this my wife and I decided to take him to the regional medical center which is a teaching center as well has having a childrens hospital. We were there for about 12 hours and finally saw a rheumatologist. She stated that there was an earlier blood test completed by another doctor called a C-ANCA which was positive. It had a titre of 1:80, normal was below a 1:20. She felt based on his clinical symptoms and this blood test that he most likey is suffering from WG. She did say that it was probably in the limited form since the kidney tests were normal. And the lungs did not appear to be invovled. She also did more testing. We were released that night and scheduled follow up appoitments.
The following day my son did a pulminary test which showed his lung function was 100%. The technician actually said that it was outstanding. Just yesterday we met with the Rhuematologist and pulminary Dr. to go over treatment and test results. The new C-ANCA test was still positive showing a result of 9.3 Eu/mL as opposed to normal being below 3.9 EU/mL. His sed rate was at 60. They also said that there may be a small nodule on his lung that was seen in a X-ray, however they are not concerned. They will re-take the x-ray in 1 months time. They are not concerned because they only see it on a frontal view and not a side view. We had 3 skin biopsies yesterday as well and will hopefully get results on Monday. This I guess is conclusive of WG.
The recommended treatment at this time is Predinisone and Methotextrate, which would begin next week. They tell us that with this treatment that we will fully recover. Sports are his life and he has a dream of someday playing baseball for the yankees.
I just don't know what to think. I am totally beside myself. I want him to get better and realize all his dreams. I am just not sure that it will happen. Is there any other possibilities for a cause of what he is going through. My fear is that this was a reaction to the acne medication that he was on and that if we treat it as WG and it is not that the medication that he takes will do more harm than good.
Just looking for positive re-inforcement and help interpreting the test reults. Thanks for your help.

Mike

[Sangye]

Hi Mike,
Welcome to the group. I'm sorry your son is sick. It sure does sound like Wegs--weight loss, fatigue, skin ulcers, nose ulcers, joint pain, (+) ANCA, lung nodule. With that combo it'd be hard to disprove Wegs. Don't put too much stock in the skin biopsies. There's a good chance of a false (-). I was lucky to have a single skin lesion give a (+) biopsy result. My other signs/symptoms were highly indicative of Wegs anyway, though.

I don't know if the acne med could produce these symptoms and still not be Wegs. Maybe it triggered Wegs or maybe it was all a coincidence.

I do recommend that you get a Wegs specialist to oversee your son's care. Regular rheumy's are not sufficient. We can help you find one if you need that.

[ske2chief]

Thanks for the reply. Any help that you or anyone can give with regards to a wegs specialist would be great. I was looking into JHU in Baltimore. But they are scheduling for april. My fear is that the condition can worsen without immediate care. However, his current doctor does not seem think the condition is immediatley life threatening judging soley by they way she is talking. Not that treatment should be delayed, but she isn't rushing into the hospital or anything.

[JanW]

Welcome to the group, Mike -- sorry you had to find us.

Sangye is right (and she goes to JHU too, so if she's crossposting with me she can tell you all about it!), the symptoms do sound like WG. C-Anca is only positive with very few other diseases (guys, help me out here), the primary is Wegs. My doc did c-ANCA as well as P3, and I also had a sed rate of 52 and high C-reactive protein and RA factor. I only came to this board after being diagnosed at 44 last month, and I am an African-American woman, so a rare Wegs patient indeed. I had a lung nodule that was seen on a CT scan about six months ago, but more importantly I have subglottal stenosis, which means I will have to have my windpipe surgically widened in a couple of weeks (it's being covered over by scar tissue). The bridge of my nose also collasped -- I'm happy that didn't happen to your son -- it would certainly have been devastating if I was a teen. Luckily I have lots of freckles on my face so they provide good cover. And of course it can be fixed.

In any case, enough about me. You will find a wealth of information on these boards from people all over the world. I encourage you (when you feel up to it) to read the stories of some of the people here. All of us are a little different -- but I have found people to be full of hope even though sometimes they feel so badly. My WG was diagnosed when I had an ankle problem that wasn't healing and that led to bloodwork. I had noticed the problem with my nose, but hadn't gotten around to addressing it (my nose is still functional -- this is an aesthetic problem for me right now). I was quickly plunged into the world of every other week rheumy visits, and since I have the ENT problems, I see him a lot too. I started on a trial dose of methotrexate but it was quickly determined that my liver couldn't take it, so I will go on another medication (not sure which one yet) after my surgery in March. I was really bummed about not being able to take the mtx, but people here assured me that it wasn't uncommon that it reacts poorly with your liver (which is one reason you want to make sure that you are getting regular blood checks -- my rheumy is doing 2x/month for at least a year.

As to recovery/remission/whatever you want to call it...my rheumy says that this is semantics. If you have this disease, you will always have this disease, although it may remain indolent for much of your life. You may need to be on medication for this to happen, or you may be able to stop medication and your condition will remain stable. It's not something that you can 'cure.' I too have a limited form of the disease (and those of us here like to add 'for now') but I still get urine tests every two weeks because kidney involvement could occur at any time. I was probably misdiagnosed starting in 2005 -- every doc though my wheezing was asthma, not a scarred windpipe, but I wasn't feeling sick.

The only other thing I would say as you are dealing with a young patient, and these immunosuppressant drugs are essentially chemotherapy, I would just ask about any impacts on potential fertility in the long term. Just so that you and your son know what you are looking at.

Good luck, stay strong. People here are living with, not victims of, this horrible disease.

[elephant]

I too agree, see a Wegener's specialist soon. The Vasculits Foundation has a list, it will make a world of difference. The know the lastest research and the right medicine to take for your son. I see Dr Carol Langford at the Cleveland Clinic in Ohio. She is great. Wish you the best.

[Sangye]

Mike, as Jan said I go to JHU in Baltimore. They're amazing. I've been to a lot of docs at this point. Started with local docs, then went to Mayo AZ. Mayo AZ was worlds better, but they don't specialize in Wegs like the other Mayo campuses do (I didn't know that at the time).

When I moved to Maryland and started at JHU, I couldn't believe the difference. I'm quite certain that without JHU, I never would have survived this current flare or at the very minimum, I would have been placed on extremely high doses of meds again.

To get into JHU you have to submit all your son's records. They take about a week to review them, then it takes about 6 weeks to be seen. During the week review, they determine if they need to see him sooner. At least one other new member on here got in way faster than 6 weeks because she was in serious condition.

[JanW]

I've always wondered, Sangye, why JHU needs a referral and to review your records before you are seen. I understand why it takes so long to be seen, especially since people are coming from all over the world, but I am wondering why they don't see people who are only suspected of having Wegs, or people who have very limited records of their disease (in other words, why you can't just deal with them directly). Is it sort of a combo research/clinical practice? Do they ever reject patients based on records?

[ske2chief]

Thank you all for responding. I am in the process of getting the referral and the records together. I am still hoping that the diagnosis changes for the better, but I don't think that it will. He has been taking an anti inflamatory drug which has started to reduce some of his symptoms and he does seem to be eating more and have more energy than he has in a while. Anyway, even if the diagnosis is correct I just want my boy back to normal and see him on a baseball field again. When that happens I know he will be fine and be able to live with the illness. thanks for your help and any other input is still welcomed.

[Sangye]

Jan, the week review actually works in the patient's favor. They do it because most Weggies (or possible Weggies) have very complicated histories.

My case is extremely complicated. When I saw my JH doc for the first time, he knew every detail and date inside out. He already had a treatment plan ready to go, along with a punch list of tests and procedures he wanted done and when. He also had a list of other specialists he wanted me to see and how urgent each was.

If you walk into an appt cold-turkey dragging all your records, they can't take action that efficiently or accurately.

They also use that week to review your case with the other Wegs specialists, the radiologists, pathologists, etc.... So you get a lot of docs working on it before you even show up. In fact, even though I had been treated at Mayo Arizona prior to JH, the JHU radiologists found things on my CTs that were missed by the Mayo team.

I don't know how/if they reject a patient based on records. I suspect if it's obvious they don't have vasculitis they might contact the patient to tell them that. I don't know that for sure, though.