13 year old just diagnosed

[jeriorleans]

My son, 13 years old has recently been diagnose with Wegener's. After always being a very healthy child, in Feb, 2009 he got the flu. We went to the pediatrician and he was diagnosed as viral, with fluid behind the ears, it will go away. After two more visits to the pediatrician we went to the ENT because he was losing his hearing. The ENT put tubes in his office (didn't take him into surgery). AFter four or five more visits with the same complaint (he can't hear) we changed ENTs. In June we went back to the pediatrician with pain while urination. Urinalysis was done, no infection, trace of blood. In July the new ENT put him in surgery and found granulation in his ears and nose. He had chronic sinus pain since FEbruary. THey biopsied his ears and nose and found it suspicious for Wegener's. Positive for c-ANCA put very low 1/35. Sed rate of 28. We ended up at Childrens Hospital in New Orleans where the rheumatoligist confirmed the diagnosis and put him on 60 mg Predisone and 400 mg of Plaquenil. I cannot find any research whatsoever for using Plaquenil for treatment. It does not appear that his kidneys or lungs are involved. Lung x-ray clear and no granulation in his kidneys. Any insight is appreciated.

[Doug]

My son, 13 years old has recently been diagnose with Wegener's. After always being a very healthy child, in Feb, 2009 he got the flu. We went to the pediatrician and he was diagnosed as viral, with fluid behind the ears, it will go away. After two more visits to the pediatrician we went to the ENT because he was losing his hearing. The ENT put tubes in his office (didn't take him into surgery). AFter four or five more visits with the same complaint (he can't hear) we changed ENTs. In June we went back to the pediatrician with pain while urination. Urinalysis was done, no infection, trace of blood. In July the new ENT put him in surgery and found granulation in his ears and nose. He had chronic sinus pain since FEbruary. THey biopsied his ears and nose and found it suspicious for Wegener's. Positive for c-ANCA put very low 1/35. Sed rate of 28. We ended up at Childrens Hospital in New Orleans where the rheumatoligist confirmed the diagnosis and put him on 60 mg Predisone
and 400 mg of Plaquenil. I cannot find any research whatsoever for using Plaquenil for treatment. It does not appear that his kidneys or lungs are involved. Lung x-ray clear and no granulation in his kidneys. Any insight is appreciated.

This is an odd one. The use of Prednisone with this one for juvenile rheumatoid arthritis apparently is typical, which makes me wonder if the rheumatologist actually is questioning the diagnosis in his/her mind, and trying what works for the JRA. In 12 weeks, I understand from reading the information on Plaquenil, the rheumatologist will evaluate your son's flexibility and pain. Makes no sense.

The problem with the scenario as I see it is that WG can take years to manifest itself or tear through your body like a tornado in a trailer court.
Where your son's symptoms seem not that severe, perhaps 8 in 100 people who develop WG die from it. It's good to keep that perspective, even though 92 in 100 survive, and return to reasonably to very active "new normal" lives.

Armor yourself with knowledge.

Start by contacting http://vasculitisfoundation.com/ the main support and vasculitis research-funding group in North America. They can recommend specialists in your area. "Your area" may be hundreds of miles away, but other members contacting Vasculitis Foundation have had positive results talking with the doctors listed in their website. They can guide you (then your son's doctors when you share what you learn) on the matter of the odd medication routine your son's on. It could be, too, that Cytoxan, the usual drug paired with Prednisone to bring WG into control is unsuitable for who's 13.

http://www.rxlist.com/cytoxan-drug.htm

The second website, above, is information on Cytoxan. Study it as it is possible your son will be put on this drug at some point, and this drug is pretty aggressive. You need to be prepared and informed, just in case.

The one below is on Methotrexate, another drug the doctors might use since Cytoxan generally is used now only for instances of severe WG (kidney involvement- but all cases of WG are serious, something that "severe" terminology tends to negate in many peoples' minds!).


http://www.nlm.nih.gov/medlineplus/druginfo/meds/a682019.html


Here's one on Prednisone:

http://vasculitis.med.jhu.edu/treatments/prednisone.html

You get a bonus with the Prednisone site because it is a Johns Hopkins website. JH is one of America's top centers for treatment and study of Vasculitis, and this attachment includes an excellent section on the forms of Vasculitis, including, of course, WG.

[jeriorleans]

actually, he appeared to be "GOD LIKE" with my question of what else could it be beside Wegeners. He said "What else could it be"?" He goes back in 6 weeks but in the meantime I am going to try to get an appointment at John Hopkins
'

[Sangye]

Welcome to the group!

In the past few weeks it seems like every few days we have a new member under 25. It's unbelievably sad.

I'm very glad your son got diagnosed quickly, though I bet it doesn't feel quickly to him or you. But if his lungs and kidneys are okay, then it's quickly.

Plaquenil is an anti-malarial drug. To be honest, I've never heard of it being used to treat Wegs once the diagnosis has been made. It's sometimes been used to treat an undiagnosed autoimmune disease. I have no idea if it's even effective in those cases.

You need to get him to a doctor who specializes in Wegs. Regular rheumys are just not skilled enough to handle it, and to understand the nuances of it. A member who goes by the screen name "Dumpy" lives in Baton Rouge, and a couple days ago posted that he'll be going to see a Wegs doc at UAB in a few weeks. I had suggested he contact the Vasculitis Foundation to find the closest specialist.

Due to the unpredictable nature of Wegs and its ability to go from not-so-bad to horrific almost overnight, it's important to get your son under the best care ASAP. It might spare him the more heavy-duty treatment, such as cytoxan, and certainly more extensive involvement/damage.

Also, the ANCA is famously unreliable. It correlates well with disease activity and response to treatment in very few people, but in the beginning of treatment, you can't tell if you're one of them. Most Wegs specialists don't rely on it at all after diagnosis, but use patient symptoms, lab findings and diagnostic test results to monitor the activity of the disease. Take-home message for you is that a low ANCA right now might mean his case is currently mild, or it might not be at all accurate.

[Sangye]

Just saw your post about going to JHU. That's where I go and I'm thrilled to hear you're checking them out. The process of getting in takes a bit, so start now.

[Doug]

Just saw your post about going to JHU. That's where I go and I'm thrilled to hear you're checking them out. The process of getting in takes a bit, so start now.

I can only add my "hosannahs" to that! Jeriorleans, you get Good Parent of the Month, maybe year, for jumping so fast into the correct lane to do what's best for your son! I award you 5 smilies, or would if we were allowed: + 1 (p.s. please go back up to my first note: it took forever to get the links located and pasted in, and everyone continued to post around me!)

[Sangye]

I'll add in my smilies,too. Way to go!

[jeriorleans]

I guess the hardest thing is he doesn't look sick at all anymore. He had several lesions purple in color on his ear and his face but the research I did on Wegener's mentions lesions on the lower extremities. His MRI of the brain was normal except for the ear problems. His hearing is mixed loss, mainly in the left ear. It happened within weeks. This doctor has treated Wegener's on three occassions but being that he is a pediatric rheumatologist, it hasn't shown up much. I am so frightened that they either misdiagnosed or that they are not treating it aggresively enough. I am getting much insight reading your posts however and appreciate all help. I have him on a low carb, low sugar, anti inflammatory food diet because I know how hard it can be on a teenager to gain so much weight. ANy insight on how not to blow up on Predisone?

[Sangye]

I can hear everyone's sighs when they read that first line.... It's the land we all live in. Even my Wegs doc sighed when another JHU rheumy (a great doc, just not a Wegs doc) recently told me I looked "too healthy" to be having a flare. It's part of Wegs-- unless you're super anemic, you usually look pretty good. Very frustrating and hard for others to take you seriously.

The lesions on the face are unusual. I have no idea if that rules out Wegs, though.

Three Wegs patients is definitely insufficient experience. You want the docs who only see Wegs all day every day. That's what you'll get at JHU for sure. You're right to be concerned. A Wegs dx is nothing to take lightly, and you're right to be concerned about it treated aggressively enough.

Pred and wt gain...again, you must hear the sighs. It's devastating. All I can say is for him to try his best not to eat fattier foods. The brain goes wild with cravings. You're on the right track with such a diet.

[Doug]

ANy insight on how not to blow up on Predisone?

That, Jeriorleans, is THE question! I'd hope a teenager's normal high energy needs to keep growing might stifle the normal response to this drug. That might be a good question to ask his pediatrician. Also, you get more "hosannahs" and smiley faces for the low carb, low sugar, anti-inflammatory diet you have him on. It's a good one for life, and certainly a good one for a weggie. I can't believe how together your response to his illness has been! +1

Your comment about how well he looks is classic: we weggies don't always look sick, but we have this disease for life. Seventy-five percent of use go into remission, but most of us can expect a flare (re-occurrence of symptoms and an active case of WG) once or many times the rest of our lives.