Finally decided to reach out...

[hrunci79]

Hi. I have had Wegeners for 10 years now and have had 4 "relapses" since I was first diagnosed. I am 32 years old now, have taken cytoxan + pred 3 or 4 times (the last one was the double-blind RAVE study at Hopkins so I'm not sure if it was cytoxan or rituximab yet, although I should find out soon since the study is now over.) I have also taken rituxan once since the study treatment and now am on immuran as a long-term preventative measure.

I have recurring subglottic stenosis and have had 11 surgeries to open the airway along with one tracheotomy. The last one I had nearly killed me as I had a reaction to the mytomycin-C they injected which blocked my airway in the middle of the night. I could go on and on, but those are the highlights. :)

BUT, I made it and here I stand with a wonderful supportive family and two daughters, 19 months old and 5 months old. My husband and I were told we would never have children due to the cytoxan, went through IVF and AI with no success, so we adopted our first daughter, and then became pregnant 6 months later. Life is amazing.

I have a full-time job here in Texas and try to live life to it's fullest. For the longest time, I didn't want to reach out to other Wegeners patients or even subscribe to newsletters or message boards as I felt it was a daily reminder of the disease we are living with. I have since come around to the idea and realize what a good support group can do for you, and what my experiences could also do for someone else.

I have a blessed life and hope to live another 60 years to enjoy all life has to offer.

[elephant]

That is great Hrunci! You are really busy, and such a positive person. It is great to have family support, I could not do it without them. Both of my children are adopted too. Are you in remission? Are you off prednisone?

[Sangye]

Hi Hrunci, I'm glad you decided to join our group. You have a lot of experience with Wegs and living with it at a young age. I didn't want to be involved with other Weggies, etc... for my first 2.5 yrs. I understand your feelings.

Thank you for participating in the RAVE study. I'm a direct beneficiary of that work, having had 2 month-long round of ritux.

How wonderful that you have two little girls. Surprise! We can never tell how resilient the body is.

[coffeelover]

Hello Hrunci,
I too have the subglottic stenosis. I too have had numerous surgeries! I too have had a tracheheotomy! I too have a supportive family with a full time job. The difference I have is I am about 20 years older than you and my 3 children are now out of the house. I agree that a support group is the key to helping you and others through trying times. Although, I amnot on here as often any more, it became a true source of strength for me during my early diagnosis. I am so glad youjoined us!
coffeelover

[JanW]

Hi, Hrunci, I too have SS; had my first surgery in March and await the doc's word on when I willl need my second. So far so good...I am fully open...was down to a 2 and now I am a 9. I lived with it for five years misdiagnosed as asthma. I have a supportive husband and two kids, 12 and 8. Welcome to our group. It's wonderful!

[hrunci79]

Thank you for the wonderful responses. I am new at this so it will take me a bit to get used to talking with others about my journey, but I am thankful to have found you all (and my husband is really proud of me for opening up). :)

I am off of prednisone although every time I relapse I go back on and blow up like a balloon, but I am thankful for the times that I am off and always try real hard to get the weight back off as quickly as I can so I am back to ground zero when I relapse and start it all over again. Although I am now working on post-pregnancy weight. haha.

Speaking of which, if anyone is interested, I have felt better than I had in years while I was pregnant. The doctors weren't sure how it was going to go, but it was great!

Does anyone with SS have a particularly bad cough after having so many surgeries? I have tried EVERYTHING to help with the cough, but nothing seems to work...any suggestions? Things I've tried:

humidifiers
vicks
mucinex DM
Robitussin
cough drops
cough syrup with codiene
honey
asthma inhalers
reflux meds
tesslon perl

The only thing so far that has worked is being pregnant (cough went away almost completely, and the codiene cough syrup, which I hate to take very often as it knocks me out and I have a 19 month old and a 5 month old...not good to be so tired.) I think the issue is now the scaring and it's so sensitive after so many surgeries, so I really don't know what to do for it.

[elephant]

I am wondering if your wegeners is controlled if your coughing. What does your Wegeners Specialist think? I know codiene works, I had to use it after my lung surgery, but like you said I could not be on it long.

[hrunci79]

That's a good thought elephant, but I get labs done monthy (just had them done last week) and I fortunately never have had much lung involvement, and I can actually feel where the "tickle" in my throat is...right where the surgeries are always done. It has gotten progressively worse after each of my 11 SS surgeries and this last time it was a mess because the doctor had to go back in and vaccum out the membrane that grew across my airway in reaction to the mytomicin-C they injected. I have been coughing like crazy ever since.

[elephant]

It could be post nasal drip too. Sounds like there is something in there that is irritating your throat to cause you to cough. How are feeling otherwise? I didn't know I had lung involvement until I had a CT of my lungs....I had a couple normal Lung CT then six months later a good size lung nodule had to be removed ( they were not sure if it was cancer). It was confirmed a Wegener's nodule. Many Wegeners Specialist don't go by labs. Some people here on the forum have very active Wegeners and have normal labs. So everyone is different. I know how annoying the coughing can be. Hope you find your answer soon.

[Sangye]

Hrunci, I have a theory about why you did better during your pregnancy-- including the cough.

Most "healthy" people have subclinical adrenal weakness due to chronic stress, chemicals in environment, etc.... You can always tell a woman who has subclinical adrenal weakness because she felt much better during pregnancy than normal--emotionally and physically. This is because her body will draw on the baby's adrenal supplies. It's a horrible trick of nature but her body "steals" adrenaliine and other hormones from the baby in order to survive.

The baby is born with subclinical adrenal weakness and once it's born, the mom has major withdrawal symptoms. It's very common for babies in this situation to have frequent colds, fevers, viruses, etc... Sometimes it doesn't show up for several months. However, the mom usually can tell right away that her body is off. This is the cause of post-partum depression-- sudden withdrawal of adrenal hormones. It's a difficult situation, because you get a cranky baby and a depressed and exhausted mom. I saw this every day in my practice. Both mom and baby can be corrected, but it's far easier to prevent it by making sure a woman has strong adrenals before becoming pregnant. Tribal cultures knew this and fed potential mothers special diets to strengthen them.

The situation is much worse for someone who's taken pred. Pred depletes the adrenals terribly--beyond what medicine can measure. Having been on pred before, your adrenals were not fully restored when you got pregnant. So the same scenario happened. Why did the cough go away during pregnancy? I think it might be that your Wegs is barely controlled. So adding a bit of natural corticosteroid via the baby's adrenal glands actually worked like a small dose of pred.

[coffeelover]

I do not have problems with coughing and I certainly do not want to get pregnant to eliminate that type of problem.

If it is nasal drip I suggest cleaning out your nose two times a day with a salt/saline solution. You can buy them over the counter or make your own. I do and it has worked wonders for my nasal drip situations.

Just a thought!

[jola57]

Hruncie, welcome. I too have never thought that I would join any support group ever, yet here i am and I can't tell you how happy it has and is making me.

[Doug]

Hruncie, welcome. I too have never thought that I would join any support group ever, yet here i am and I can't tell you how happy it has and is making me.

Another benefit in the long term is you start to learn about new theories and new treatments for WG. One of the big benefits is sharing knowledge of WG specialists and treatment centers, I think.

[jola57]

You are so right Doug. With so many of us all over the world we are bound to pick up a lot of info and share with others. We live this stuff whereas our doctors only treat it. So who is going to better at gathering new treatments ?

[elephant]

The more we share our stories, will be able to to help each other out. I learned so much from everyone here that it feels like going to the doctors office without the exam. :)

[Sangye]

Me too! And, no exposure to germs! :)

[Jack]

Everyone on here has first hand experience - some good, some bad, but it all builds up into a huge knowledge base that easily exceeds that available to general doctors. You can soon become your own expert and only the medical specialists will know more.

I've recently spent time on an orthopedic ward where the doctors were clueless about my condition and the effects of my drugs. I spent a lot of time trying to educate them and stop them from changing my medication - many of my blood test results fall outside the normal limits and this upset them considerably! If I had not understood my own condition well and refused to let them mess with me who knows what they would have done. They eventually consulted with my Wegener's specialists and then they left me alone! :) But you need to build up this level of knowledge so that you have the confidence to stand up for yourself when you see doctors making mistakes. This site can supply much of the information you need to carry with you.

[DEE]

Jack you are so right
i had the same experiance recently with a stay in hospital ( blood clots on the lungs0 the doc kept going to look on the internet
i told them they could consult with GP or WG consult and they agreed to leave meds alone
i had to talk with a pharmist about my meds before they would let me carry on taking them and as you say it is only through the knowledge and advice that i get from this site that i have more confidence to be able to say what i need instead of agreeing with them all the time
I hope you get to feel alitle more comfortable soon Jack Take care of you DEE x

[Sangye]

I've had other docs try to change my Wegs drugs, too. Like they're gonna waltz in and make a big discovery after meeting me for 5 minutes....

My favorite example is from last year, when this Wegs flare was just beginning. It seemed like pneumonia and I paged the on-call rheumy at JHU. He said to stop the Cellcept and go to the ER. The Wegs rash I get on my legs went wild within a few hours, so we restarted the Cellcept the next day. When my Wegs doc found out I had stopped it, he said "Don't ever stop it unless you've talked to ME. No matter what." I said, "What if I'm on fire?" He replied, "Take it anyway." LOL

[Doug]

I've had other docs try to change my Wegs drugs, too. Like they're gonna waltz in and make a big discovery after meeting me for 5 minutes....

LOL

A large part of my issues with ENT doctors is everyone of them but one I've had experience with approached me this way. It totally shatters any confidence I might have in a doctor to have him or her have me figured out on the first consultation, from my medical history alone, without first talking with me, asking me questions, examinining me, ordering tests as needed, answering my questions: you know, being a proper doctor!

[JanW]

The only ENT I have ever really liked is the one I have now, Dr. Lebovics, who always says "I'm just the plumber." He really downplays the advances that he has brought forward in the study of weggies -- an incredibly humble man. I think that the doctors who try to hide behind the bravado and "I know ALL, I went to med school" are the scariest ones.

[Sangye]

My Wegs ENT at JHU is the same way-- very humble, calls himself "the plumber who answers to Dr Seo." I imagine the Wegs ENTs at the vasculitis centers are just like the Wegs rheumys there-- no big egos.

[Brooke]

Hello~
I too have SS but not very severe, I haven't had any surgeries to correct it. I used to cough and cough and cough. I also had tried everything that you mentioned. I was diagnosed in Sept 2009 and started on methotrexate and prednisone. I have mostly sinus involement and a little lung involvement. The methotrexate helped the lungs but didn't do anything for my sinuses so I had to start Rituximab. I do not cough nearly as much as I used to! I am still on 20 mg of pred, I hope I can wean off and stay healthy.
When I was pregnant in 2006, I too had the best pregnancy!! No problems and I felt great. A few months after having my son, my symptoms started again. (this was all before I knew I had weg)

[Sangye]

How are you feeling, Brooke? Do you notice any improvement since starting rtx?

[Brooke]

I am feeling good, thanks Sangye. I was feeling pretty good going into the infusions so now it is hard to tell. I don't think I will know for sure until I can lower my prednisone. I am not supposed to start lowering until next week (going from 20 to 15 mg) but I was getting so frustrated with my weight I tried 18 mg this week. So far so good, I still do my sinus rinses and my nose feels dry and crusty still so I don't know how it looks on the inside.
I walked another 3 miles on our treadmill on Tuesday and did great with no coughing or anything but then yesterday I felt very tired all day. Maybe I over did it walking 3 miles? Last night I did a 20 minute walk outside and I feel much better today.

[Sangye]

Brooke, please be careful lowering pred too soon. When I did my first round of rtx in October, I assumed I could start to taper the pred soon after. My Wegs doc said no way. It takes the rtx several weeks to really kick in. I was really impatient but I listened to his advice. I couldn't tolerate the taper until after the second round in March. Remember, "Slow but steady wins the race" with Wegs.

[Lola]

Yes, Brooke - slow and steady. I think you overdid it on the treadmill. Please, don't do that to yourself too soon. I was so anxious to be cured by the Rituxan that I overdid it, as well. I'd take one step forward, one step backward, on and on, for months. I finally feel like I'm taking 1 step forward at a time now...and that's after 4 months. I also did a long walk in April and injured my hip ligaments the next day - just by twisting my foot on the ground, no weight bearing.

Listen to Sangye about tapering prednisone. She is now the QUEEN OF THE TAPER. You must slowly sneak away from that crap before it wakes up, rears its' ugly head, and tries to snatch you back into its' ugly maw.

[Sangye]

LOL-- I didn't know I'd been crowned! :D

But really, who has tapered more slowly than me in the history of Wegs? Maybe there's someone else out there splitting 1mg pred pills into fourths, I don't know. I've questioned myself a number of times-- do I really need to go this slowly? But the answer is there in black and white in my daily log-- every single time I've dropped by 0.25 mg I have temporary symptoms. That Wegs dog can be a light sleeper.

[Brooke]

Thank you Lola and Sangye -
Sangye - What dose were you on in October? What dose did you drop to when you realized that you were not able to tolerate being lowered yet? Did your doctor tell you that since you couldn't tolerate being lowered that you need another round of Rituxan? Is that why you had another round in March?
What are your current symptoms?
I'm sorry Sangye I should know this, but what all Wegs involvement do you have again? I know you have lung involvement.

[Sangye]

My main involvement is lungs (hemorrhaging and a few small granulomas), joint pain, profound weakness and fatigue. In the past year I've developed a little nose involvement, but nothing like typical Wegs. I've also had major skin ulcers in the past. I've had a huge amount of complications that cause at least as many problems as the Wegs!

When I started rtx in October I was on 4mg pred and my lungs were hemorrhaging again. (I was still on ctx, even though we knew it was killing my bone marrow.) The infusions lasted all of October and since the rtx doesn't start working for 6 wks (mid-November) my doc said to wait to taper. I kept emailing "Now?" and he kept answering "No!" It was hilarious. By mid-December he said I could technically start tapering, but wanted me to wait until after the New Year in case I got into trouble over the holidays. I tried tapering in January, but no matter how slowly I went, I got sicker within a couple days--coughing up bits of blood, joint aches, etc.... Even without the taper I wasn't feeling that great.

We were going to do another round of rtx in April, but I could feel myself slipping and asked to do it in March. The first round had put out the major fire but the Wegs was still fairly active. The second round seems to have really contained it for the first time ever.

I started tapering pred from 4mg in April and am currently down to 1 mg. I should be off it within 3 weeks.

My Wegs is called "refractory" because it's been unresponsive to all other treatments. The lung hemorrhaging also categorizes it as "severe." I'm going for new labels: "Highly responsive" and "No evidence of disease."

[Jack]

I started tapering pred from 4mg in April and am currently down to 1 mg. I should be off it within 3 weeks.
Sounds like you are going to be the fittest person on the Forum!

[Sangye]

I've got a long way to go to win that prize! But I'm thrilled to finally be making progress~ :)

[Brooke]

Well I am very happy that the second round seems to be kicking wegs butt! I hope you continue to make progress :)
I look forward to the day I can get off of pred!! Or at least below 5 mg, anything to help get the extra weight off.

[hrunci79]

Sangye,
I live in Texas but I fly to JHU to see Dr. Seo as well, and before that, Dr. Stone. Can you tell me who your ENT up there is? I now see Dr. Simpson in San Antonio, but am still trying to figure out why I cough so bad, so I would like to explore some options. I saw Dr. Flint at one point up there, but I think he has moved.

Thanks!

[elephant]

Hey hrunce79 don't mean to butt in, but does Dr Seo know that you are coughing? Are you still on Prednisone, or other meds to control WGs? Just curious.

[hrunci79]

Yes, elephant, he knows that I am still coughing. He gave me suggestions such as nasal rinses, reflux meds, etc, but none of them have worked. He has given me the names of some other ENTs to try, but so far, no one has been able to help me. I've tried asthma inhalers, been scoped by an ENT to see what was causing the coughing, and still they can't figure it out. We know it's not my lungs, and it definitely has to do with the fact that I have had so many surgeries (11) for my SS with the last surgery causing an unexpected reaction that caused a blockage of my airway nearly completely. I think that it is because that area is now SUPER sensitive and anything that passes through sets off a lot of coughing. I have tried Mucinex DM to dry up the mucus, which works a little, but not a lot. I think post nasal drip might be the culprit that is "tickling" my windpipe, but not sure how to clear it up.

I am on azathioprine (immuran) long term. Since I have relapsed severly 4 times with kidney involvement the last time, the docs decided that instead of taking me off meds every time after I get better, to just keep me on azathioprine long term to try to prevent relapses.

[Brooke]

Hi hrunci79, butting in too!!
I have had a lot of coughing in the past, still do but not near as bad. I too have SS but not very bad and I have not had any surgeries to correct it. So maybe the coughing is not totally just from your surgeries?
I do a sinus rinse twice a day.

[elephant]

I remember Brooke when you were coughing so much....I think that the medicine RtX stopped your coughing. What do you think?

[Brooke]

Hi Elephant~
Yes, something is sure helping because I do not cough like I used to! I have said before that I felt really good right before I started the infusions so it is kind of hard to tell yet. It has been 6 weeks but I just started to taper the pred. I feel I am coughing a smidge more but I can't tell if I have a sinus infection or not because I have been getting bright green stuff out when I do my sinus rinses. This has been going on for many weeks though. No fever or anything else along with it, just the green gunk with my sinus rinse.

[elephant]

That green stuff is infection, been there done that. If you rinse your nose three times a day for a few days and still get green stuff...it is definitely infection. Are you feeling a little congested? I did not have a fever either. My Rheumy and EnT agreed that it was an infection and it went away after taking two different antibiotics...
Can you get in to see your ENT?

[Jack]

The green stuff needs treating with antibiotics. If they don't seem to work, get it analysed to see if they can identify an effective one.

[Sangye]

Brooke, I agree with Elephant and Jack. However, I suggest getting it cultured before starting antibiotics. Antibiotics are not benign drugs and just like with pred and our other drugs we have to consider the long-term effects of taking multiple types just to find the right one for an infection. Each time you take an antibiotic you're creating more superbugs in your body and making it less likely for that antibiotic to work again. And each time your body "sees" the drug, it's more likely to develop an allergy to it.

A culture will take 3 days. Unless your infection is growing much worse each day, it's best if you can wait for the results.

[marta]

The only ENT I have ever really liked is the one I have now, Dr. Lebovics, who always says "I'm just the plumber." He really downplays the advances that he has brought forward in the study of weggies -- an incredibly humble man. I think that the doctors who try to hide behind the bravado and "I know ALL, I went to med school" are the scariest ones.
I had one of those. Still have a sour taste in my mouth from that.