New blog from doc in Ark, USA regarding vasculitis

[Palmyra]

On random reading thru this vast and wonderful web, I found a Blog that was new to me, from a well informed doc that is located in of all places Arkansas. So glad there appears to be a good medical mind, challenging himself, from what is a geographically somewhat isolated position.

He has a recent post about Vasculitis, which mentions all types, but it is interesting stuff regarding pearls and myths.

Notes from Dr. RW: rheumatology (http://doctorrw.blogspot.com/search/label/rheumatology)

If any of you live in the state, he might prove to be a valuable resource.:thumbup:

Best of wishes to all!!!

~Palmyra J

PS...Haven't been posting much...the daughter is still having problems with sinus infection (fungal and bacterial). Not fun stuff, and I won't let the local ENT mess with her slightly deviated septum. I will pose questions to the group on that topic under separate topic heading.

[pberggren1]

Hello Palmyra:

I am sorry to hear that Allison is still fighting with a sinus infection. As far as a deviated septum goes, if she can live with it, I would not get surgery to correct it as the surgery can trigger Wegs to flare again. I hope she can get over this sinus infection soon. I know what it is like. I have had a sinus infection for a year.

[Jack]

I am inclined to think that a sinus infection that lasts for a year is in fact Wegener's. Have they been able to grow any cultures from it?

[pberggren1]

The 3 ENTs I have seen said a culture is useless but my new Wegs doc has done a couple of cultures and has come up with nothing. I am sure that the infection is gone now. I get the odd sinus pressure headache but nothing like I used to get months back. I still have the regular crusting and bleeding. And the sore where the one ENT did the unneccessary biopsy on the right nostril is doing better if I keep irrigating regularly and keep it moist. Right now I use sesame oil to keep things moist in the nose.

[WeggieParent]

Palmyra, this made me laugh. How did an intelligent doctor sneak into our little hillbilly state? He must've made a wrong turn at St.Louis and went south instead of north! :wub:

Actually we could use some specialists in Arkansas! I talked to the Vascular Foundation and none of the WG specialists within 10 hours of us treat patients under 18.

[Palmyra]

To Weggie parent....I am even further South of you, in the urban jungles of deepest, darkest Dallas, Texas.:wink1: We have large medical centers here, but no true Vasculitis experts, so when in need we make the long trek up to the Mayo Clinic, Rochester. It has been well worth the trip.

My daughter started with this at the age of 20....I certainly hope you have been able to find good pediatric care.

The Mayo clinic are the one's responsible for the positive culture of aspergillus (a fungal infectious mess,) that has been one of the organisms messing with my daughter's sinuses. Treatment is largely mechanical debridement, but I am wondering if there is an antifungal medication that needs to be involved....a question I need to ask the group under a different header.

And pberggren, I am so sorry you have been suffering with similar issues. "As far as a deviated septum goes, if she can live with it, I would not get surgery to correct it as the surgery can trigger Wegs to flare again."

I agree that a surgery on that septum is a dangerous idea...I worry about Weg activation and the possibility of saddle nose.

Thanks for the input!

[WeggieParent]

Speaking of aspergillus - my daughter is being treated for it in her lungs. She had many chest tubes back in Dec/Jan because of holes in her lungs (start of our WG diagnosis) that allowed aspergillus to get in. Our Infectious Disease doctor said it is very common for patients with chest tubes or trachs or any kind of long-term tubing. She started her with Voriconazole 200mg 3X daily. Her last blood work looked good so they lowered the dose to 2X daily. My daughter is being treated at Arkansas Childrens Hospital in Little Rock and we're very pleased with 99.99% of her team of doctors.

[Palmyra]

Well, nice to meet you Weggie Parent. Sorry you are here, but this is a great support group. Very informed.

My daughter now lives in another town (24yrs old,) and so that distance is healthy developmentally, but it does mean she is now managing more and more of her health decisions. It is very hard to cut that umbilical cord when your child has a disease condition like WG.

I wrote down the drug and dosing schedule for the antifungal your kiddo is on, and I certainly hope it is effective. I think that might be a logical next step for my daughter. At last check the infection had not affected her lungs (where her WG activity also first presented).

I am very glad you are getting good care in Little Rock. Wonderful, wonderful news. I am also a member of a 'parents' support forum, and good docs are always something nice to share. I may come back to find you in case someone from the other site is in need.

Keep me posted on your daughter's progress, and good luck.

[Sangye]

It seems odd to me that they don't have Allison on an anti-fungal medication. What's the point of just scraping it out if more grows back? :confused1:

[Palmyra]

That question will be asked today, as she has an appointment with her local ENT.

The vasculitis familiar ENT at Mayo did not put her on an antifungal, and he was the first to diagnose and treat. I believe the distribution of antifungals in the sinus cavity with systemic medication is limited, but we'll see. Alison plans on asking both her local Rheumy and ENT to consult with one of the vasculitis experts today.

I think she is over due for Rituxin, and is basically lacking a 'quarterback' to make the calls and direct treatment. I am more than a bit frustrated with her (grantedly, overworked) local Rhemy. She is back on pred (20mg's) which can make one more suseptible to a fungal infection...aghhhhh

[Sangye]

Oh, that is not good. They shouldn't be upping the pred instead of getting the Wegs under control.

The antifungal med explanation makes sense.

[Palmyra]

OK Sangye and any others with sinus experience. Alison is just home from local ENT consult, and he and the local rheumy are recommending correcting the deviated septum (its deviation is making flow into the left maxillary sinus blocked, therefore never allowing infection to clear) prior to infusing with Rituxin. They do not want to infuse while she has active infection, which makes sense.

My question and concern is, how sensitive is Weggie nasal cartilage? Do we risk septal collapse with surgery? We are trying to get the ENT to consult with VF experts. I think he will.


PS. I am half witted mode...how would I post this question to the group at large under a new thread? Thanks

[Sangye]

Wow, I have no idea. I don't have sinus experience, though. I would sure get input from the Mayo docs before proceeding. Also, I wonder if the Mayo docs would consult with Dr Lebovics about it considering that the risks are so high if they get it wrong.

[elephant]

Palmyr, my first sinus surgery was in Jan 2008 and I had surgery on my deviated septum ( left side) and cleaned out my left maxillary sinus. Then 6 months later it went back to being deviated. I would get several opinion's ENT who know Wegeners disease.
Most ENT don't even want to touch my nose, because they know I had four sugeries and they really did me no good. The last one I had was better, since he opened up my left maxillary hole, the ENT surgeon had to remove bone too.

[Jack]

how would I post this question to the group at large under a new thread? Thanks
Use the button at the top left of the Page covering the section you want to use. It is marked "+ Post New Thread"

[Palmyra]

Yes Elephant, I have been reading about similar issues from others for years. I am sorry you have had several surgeries, and I now know those delicate little septums can "re" deviate.
And Sangye, I wrote down Dr Lebovics as someone for her local doc to consult with. I think a young lady from the yahoo'parents group' actually saw him for saddle nose reconstructive surgery.
I think we will see what information trickles in over the next few days.

Thanks Jack...found it (my brain doesn't work when I get a bit nervous :-)

[JanW]

He is my doc as well, and he is pretty much the top ENT for WG -- trained at NIH with the Gary Hoffman and Carol Langford who are literally two of the people who have 'written the textbook' on the rheumy implications of this disease (so said the rheumatology fellow I sat next to at the conference).

I happen to have a deviated septum (more than half of the population does I think) and I have saddle nose. I think that the cartilage is extremely weak in a WG patient, and I'd never have surgery on mine except for a repair. Lebovics won't even repair the saddle nose of anyone who is still on immunosuppressants -- he only does them on non-medicated remissions, and only with a plastic surgeon/reconstruction specialists. It's tricky stuff.

He's easy to reach though, and he would certainly take a call about this stuff himself.

[Palmyra]

Thanks JanW we will proceed with caution, and consult with all our experts. Glad to hear Lebovics is easy to reach. Have you had to go see him repeatedly to keep things quiet?

[JanW]

I have to go every six weeks because I had surgery to repair subglottal stenosis (tissue scarring on windpipe). I had my surgery back in March, and should have had to have another already, but my repair has exceeded his expectations and my windpipe is still open, so I'm very lucky.

I wouldn't be seeing an ENT on a regular basis at all except for the stenosis. I don't really have any sinus problems at all, and a saddle nose is just an aesthetic issue, although, granted, quite a bad one to have!

[Palmyra]

Sorry you have to be seen so frequently, but I know that is the case with the stenosis. Really glad you have had such good results, as I hear differently from others with that issue. I am very glad you do not have issues with infection. She needs maintenance drugs, but more suppression with current infection is not appropriate. He did back her down to 10mg's of pred today. I think because it was not helping with the sinus issue anyway.

Our problem would be making that trip from Texas...I would love it if her local ENT would really engage and collaborate. I will see if we can encourage that exchange. Her local ENT seems to be a kind person, and said from the get go that he would be willing to consult.

[JanW]

I find that most of the people I have met on the forum who have issues with stenosis that are much worse than mine. Mine was quite severe, and, even so, I have spoken with people whose have not been as severe who ended up with a trach, or ended up coming out of a surgical repair with a trach (which was my big fear, and Lebovics assured me he hadn't had happen in over 20 years). People also die from the surgery -- he hasn't had a death, but many of his 'colleagues' have. He is a very down-to-earth guy but absolutely passionate in his belief that he is one of the top out there because he has worked with this disease for long -- it's his specialty. He told me that in one day he had 10 WG patients -- this was unbelievable for a guy not working at Mayo, or JHU or Cleveland, who are presumably seeing them all day long. This is a guy who is just chief partner in his NYC practice. I always say that I am incredibly lucky that he is a native New Yorker because he would never have ended up here -- I have to believe he could have gone to Cleveland with the rest of the gang--but he's really a local guy.

And he loves WG patients. He says he's never met one that was a jerk, although he used a much more colorful word.

[Palmyra]

JanW I am so happy for your success with Dr Lebovics. Isn't it amazing all the good things that happen when there is a collaboration between Dr and patient? The individuals that become experts in this field do not do so by accident. They choose to fully engage, and as we have seen, will fully invest in every patient they see.

So in the busy,busy world of all the local specialists that are in practice, wouldn't it be nice if all Docs behaved in a like manner? I do believe the choice to specialize results in superior results for those that have rare conditions. Thank you for sharing your own personal experience with me.

Yours,
Palmyra