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Lila
02-10-2010, 01:53 PM
Hello All!

I'm having trouble finding my old thread!
My sister had her visit at JHU today! All I can say is WOW!! She saw Dr. Levine and his associate. In all these years of her suffering she has NEVER met doctors so knowledgeable and KIND. When we walked out of there today it was like a weight had been lifted!
Dr. Levine confirmed that my sister DOES NOT have Rheumatoid Arthritis. She does have Wegener's. She has been getting weekly injections to treat RA, and she does not even have it. Dr. Levine upped her Pred to 40mg (Oh God, I dread this!) and also upped her Methotrexate (I forgot the dosage) and I think he gave her Plaquenil. I'm not positive about the last one. I will find out tomorrow. He was so positive. He said that she should only need to be on high doses HOPEFULLY for a few months. Once the inflammation has gone down she will need to get the trache out. We set up another appt. for 1 month and she will be seeing the ENT surgeon (Dr. Kim?) and Dr. Levine on the same day. Dr. Levine also took pictures of her nose. He says that she really needs to get the nose surgery ASAP because her nose has completely caved in.
Although we're not exactly thrilled that she definatly has Wegener's we ARE thrilled that she is being treated properly by the BEST doctors! :)
It was a very expensive trip up there!
We left yesterday, got a room at the Clarion (very nice there) and between that, tolls, gas, food, parking...we spent over $300. But it was def worth every penny. We scheduled the next appointment for later in the day so that we would not have to stay overnight.
BIG Thanks and HUGS to Sangye and everyone else who encouraged me during this whole process of getting her into JHU! Words cannot explain how thankful I am, and I KNOW my sis is too! :)

AnnaG
02-10-2010, 02:13 PM
That's wonderful news that she will now have appropriate treatment! Now hopefully it will kick in fairly soon and she'll be feeling better. :)

Lila
02-10-2010, 02:15 PM
Yes, thank you! This has been a great day! :)

Sangye
02-10-2010, 03:07 PM
I am so relieved that she finally got there. And while it sucks she has Wegs, we already pretty much knew that. I'm really glad she doesn't ALSO have RA! That's huge!

Dr Kim is the ENT who sees all the Weggies. Very sweet doc, spends time with patients, very thorough. He runs really late unless you have an early morning appointment (no later than 9:30). You might wait 2-3 hours otherwise. Just be prepared.

Regarding the higher pred, I really think she should consider seeing a psychiatrist about a mood stabilizer. It's common to need one with pred. There's no need for her to suffer with trying to contain out of control brain hormones.

jola57
02-10-2010, 04:46 PM
Lila, while Wegs is not a good news, not having RA is. I am so glad that you had a good visit. Hopefuly now Wegs will be controlled and you sis is well on her way to feeling better.

elephant
02-10-2010, 10:31 PM
Lila, that is great news for the both of you. I am so glad she is on her way to great care and recovery!

moyan
02-11-2010, 01:56 AM
Yahoo! The good news about WG is that it will now be treated properly! So glad you had that visit and sending a good luck hug to both you and sis!

JanW
02-11-2010, 02:27 AM
Lila -- that's great news. I was worried about your sister and think about her often. I'm sure too that she will be happy to get her nose fixed, and obviously it's not just for the aesthethics -- it sounds like a pretty serious medical condition on its own. My ENT said that for some saddle noses they must fix them -- I don't fall into that category yet, anyway.

Did she get her trach because she has a stenosis? Or did she have some other trauma that made her unable to breathe?

onatreetop
02-11-2010, 04:20 AM
I am so glad you got her there!!!! They are very nice and knowledgable. It is good to hear from you too.Was getting worried with all this weather and everything that is happening. My sinus/septum repair/ biopsy was yesturday and went well.Now I get to recover! Ha ! HA!
Again I am glad she has her meds adjusted. Theydid tell me when Iwent that they only use cytx when the kidneys are involved so her testing for that must have been good. Keep us near your heart!! We are all here for you.Hope the pred helps her feel much better but not to moody.

elephant
02-11-2010, 04:29 AM
Glad you biopsy went well onatreetop. Hope you feeling better today.

JanW
02-11-2010, 04:30 AM
That's an interesting comment about cytx, onatreetop. When I said that my rheumo said the same thing about it, Sangye said that was a red flag. Sangye I know that you go to JHU as well -- has this been a recent change, do you know? Not trying to needle you ;-), but I was assuming that they used cytx even without renal involvement at JHU because it seemed unusual to you that my rheumy would even make that comment. Maybe cytx is somewhat "going by the wayside" in less global cases of Weggies because of its high toxicity and unpleasant side effects even though it still seems to be the starter drug for lots of people on here not being treated at major centers?

Another thing on the drug front -- my insurer (Cigna) confirmed that I don't have to fail on any other drugs to get rituxan (not that I need it right now, but perhaps I might in the future). It just has to be precertified through the physician. Maybe this is another positive change coming for us Weggies. Wouldn't it be nice if we could get the best treatment to control the disease with the least amount of toxicity?

Sangye
02-11-2010, 04:45 AM
It's not as straight-forward as that, unfortunately. They try not to use ctx when other options are there, but there aren't any hard and fast rules like "No kidney involvement= No ctx" (No rules for the other meds, either) It would sure make it easier for non-Wegs docs to treat properly if that were the case.

I have never had kidney involvement. Last summer when my lungs started hemorrhaging again, my Wegs doc at JHU put me on ctx. We only switched to rtx because the ctx failed again.

There are many other variables involved that your doc may not explain unless you delve into it with him/her. Impossible to do in the first few visits, usually. I've been seeing my doc for over a year and I'm just getting around to asking him some of this stuff. One of the variables is this: They know ctx is highly toxic and can only be given a limited number of times in one lifetime. They also know rtx is very new to Wegs treatment and don't have years and years of data concerning the long-term efficacy OR safety in Weggies. The age of the patient-- and therefore how many years of Wegs treatment and damage may lay ahead-- is often the deciding factor. My doc told me that because I'm only 46 and have had refractory Wegs since dx, he would feel uncomfortable giving me tons of rtx. I don't know if I'm explaining this well.

onatreetop
02-11-2010, 05:00 AM
Thanks.Just got my explorer out from being stuck again Husband tried to get out to the store and didnt make it out of the driveway. It is a giant slush pit out right now. Just switched back to snow again. I am better than last night sleeping proped up was hard ended up on the couch with lots of pillows and waking with dry mouth bad. Nose is packed. Sleep was bad but if thats the worst of it now thats not so bad. We are hoping the snoring will be helped by this too!!! I asked when my husband would go in? He said never! Nice right!!

I am relieved for Lila and her sister now too. The trip is a big one but so worth it!!! Congrads!

Jack
02-11-2010, 05:07 AM
onatreetop - do you flush your nose with saline to clear it? You may have mentioned this before, but I can't remember. If not, please give it a try, everyone swears by it and it certainly worked for me.
See - http://www.wegeners-granulomatosis.com/forum/general-wg-chat/114-sinus-irrigation-bottle-do-you-know-about.html

JanW
02-11-2010, 06:08 AM
I use the Neilmed nose wash -- the saline stuff (I used the brand name Saltaire) was much, much too strong for me. It was the saline, not the water that was burning like crazy. It seems liked the water flush alone cleared everything well.

What is 'refractory' Wegs, Sangye?

elephant
02-11-2010, 06:18 AM
I use the Neilmed nose bottle wash twice a day. Sometimes I only do once a day. It helps, and it keep things clean. No need for nose picking. :)

Sangye
02-11-2010, 08:13 AM
Jan, refractory means "unresponsive to treatment." I've never been in remission-- not even close-- in 3.5 yrs post-dx. Some of the research on rtx has been specifically aimed at people with refractory Wegs.

Rtx has been shown to work better in refractory Wegs vs non-refractory Wegs in at least one study. (I don't remember the others) This makes me think that perhaps refractory Wegs is toxin-based. Ctx is a major toxin, but rtx isn't. So giving ctx would make an already toxin-overloaded person even sicker. I do believe the Wegs I have was caused by toxins. I lack any of the other common associations (infection, heredity, etc...) but have a strong history of exposure to toxins.

JanW
02-11-2010, 08:28 AM
Very interesting, Sangye. Do you doctors have any stats on what percentage of cases are refractory? Reading on the internet seems to suggest that as many as 50 percent of Weggies go into remission entirely and never flare again after the first flare, but on this board it doesn't seem as though I am seeing any (although admittedly the bias is towards the newly diagnosed and perhaps some of those for whom Wegs has affected multiple systems)...I haven't seen the term refractory before, although my rheumy made very clear that remission is not a "given" outcome in any case.

elephant
02-11-2010, 08:43 AM
My thought is that it depends on what is the definition of" Remission" ....Is it defined by the doctor that is treating you? I think many Weg are on some kind of medicine to keep them from flaring for the rest of thier life.
The chart the Sangye and Lightwarrior made " list of symptoms" helps but ...I don't have any zero's or is this the "New Normal?" I think I'm developing a twitch in the left eye. wink wink..

Sangye
02-11-2010, 09:28 AM
There are 2 types of remission: drug-maintained or drug-free.

Remission doesn't necessarily mean "symptom-free." It just means the symptoms are not active Wegs.

Some people do achieve drug-free and symptom-free remission. I can only think of one in our group--Mike Caven, who was in total remission for about 27 yrs and was completely fine. Maybe there are others, but his is the most extreme case I've ever heard.

I've never asked my doc about stats for refractory Wegs. I'm not sure I want to know.

The stats on the JHU website are:
90% of patients with severe disease respond to treatment
75% are able to achieve disease remissions
50% of those who achieve remission will relapse

Which means
10% die or remain refractory
25% never go into remission or die
50% who achieve remission never relapse

elephant
02-11-2010, 10:30 AM
That helps Sangye. I am always thinking ....but with those stats 10% die or remain refractory- is it because they didn't try other options or to late when they were diagnosed. I know that some have died because of delayed diagnosis. I am wondering the medicines ...they were given to the 10% who died or 25 % never go into remission.
What makes the ones who go into remission stay in remission. I wonder if Mike Caven can let us know his journey( lifestyle, food, medicines, exercise, spiritual) so that we may learn something ...just maybe or anyone who has been in remission for over 20 years.
Just curious, hope I'm not driving people nuts...

Sangye
02-11-2010, 10:44 AM
Some people are diagnosed when it's too late. The drugs just can't work fast enough, or maybe there's not enough viable tissue to work with.

The chronically refractory are a big mystery to the MDs. To holistic docs, not so much. It takes chi to recover, to respond to drugs. The drugs have to have something to work with. You can put gas in a car but without spark plugs it won't run. Maybe too much chi was lost by being ill a long time pre-diagnosis, or maybe the drugs have an especially deleterious effect on the person. Maybe both, as in my case.

We asked Mike some things when he first joined. It sounds like he just responded right away to the meds--textbook response-- and kept going. He flared last year (after about 27 yrs of remission) and again responded quickly to the meds. He went back into remission fairly quickly.

JanW
02-11-2010, 11:17 AM
This is fascinating, Sangye. By the time you started conventional medical treatment (if I am remembering your story correctly you had been trying to work with natural remedies for some time but getting increasingly ill), did your doctors believe that you would respond well to treatment given your history, or was it more a case of "let's try whatever we can and see if it works because she is really ill?" Are there treatments that you received that you believe actually may have retarded your healing? I hope this is not too personal; feel free to tell me if you think it is.

Sangye
02-11-2010, 11:53 AM
LOL-- it's not too personal, no worries.

I'd been trying purely wholistic remedies for 8-10 months pre-dx. The last 6 months of it were especially intense, as I was completely crippled by pain and doing a great deal of holistic treatment.

My first docs were far from Wegs specialists--really had no clue. They also had zero belief in holistic remedies, so they completely discounted that period. They just thought it was a natural progression of untreated Wegs. They did the "standard" protocol for someone with severe alveolar hemorrhage--oral ctx 150 mg and pred beginning at 1,000 mg IV solumedrol (equiv 1,250 mg oral pred). I stayed on ctx for 7-8 months and was transitioned to mtx for a couple months. I quickly developed infiltrates and had to stop mtx and pred.

The ctx weakened me terribly. It is overly toxic to me-- destroys my bone marrow faster than I can make it. It was clear within 3 months. They ignored that and kept me on it far too long. The massive doses of pred did me in. They didn't taper it correctly at all once I got under 20 mg. Way too fast, ignoring my complaints that the horrific Wegs pain was coming back. So the drugs weakened me terribly and still never put the Wegs fire out.

The fact that I developed uncountable leg and lung clots the week I was diagnosed also was a big factor. My lungs had been bleeding for 3 months and were then packed with clots. The tissue was really damaged. I lived at high altitude as well, which made it even harder to recover.

Another factor that weakened me was not getting me into aquatic physical therapy right away. My muscles wasted, I lost a tremendous amount of range of motion and had no possible way to strengthen. I went 3 yrs without PT.

I've always been very sensitive to meds. I never could tolerate regular doses. So when they give me standard doses of these drugs, it's a big problem. My JH docs understand that, and do use lower doses when we can.

JanW
02-11-2010, 12:06 PM
So when you say that they discounted the period when you were trying holistic cures, do you mean that this discounted the fact that this could have been contributing to your symptoms (i.e. some things in your body were caused by WG and some were caused by reactions to the holistic medications)? Did they not know that your bone marrow was being destroyed? Your story just sounds so harrowing -- and so unfortunate that after all that time on those medications, it didn't bring the disease under control. What do the docs think will be the biggest factors in your getting better now -- or is it really too much of a crapshoot to say?

Sangye
02-11-2010, 12:20 PM
The Wegs train was progressing terribly, but I inadvertently supplied another engine by boosting my immune system so thoroughly. They didn't believe holistic treatments can boost the immune system at all. That's what I meant by discounting.

In my case the holistic treatments were so powerfully diametrically opposed to what I needed, that it deepened the imbalance and weakened my foundational reserves.

This is exceedingly rare. I don't know of it happening with anything other than Wegs, actually. However, the converse is exceedingly common: medical treatments of many conditions are so powerfully diametrically opposed to what is needed that they deepen the imbalance. Allergies and recurrent infections (eg strep throat) are typical examples.

elephant
02-11-2010, 12:29 PM
Thanks Sangye you really make sense when you explain medical terminology. Everyone is different. It's amazing to see why some can just take cytoxan for a few months and go into remission for 27 years! Wow! " I will have whatever he is eating." LOL
Sangye you are going to get better.

Sangye
02-11-2010, 12:33 PM
ROTFL-- I want that meal, too. And I'd like a muffin on the side.

Thank you for the reassurance about getting better. It's powerful to see it in print, you know?

onatreetop
02-12-2010, 05:43 AM
Holy Moly I'll take the buffet !!!!!! I have to saline rinse 4 x a day now. The gel packing is starting to come out Yuck!!!! No more bleeding. Still swollen and sore cant touch my nose. Limited to do no lifting, excerise, or putting my head down. finally took the drain catcher off after my shower today. Sofar no major drainage. I have had to shovel and do a lot with all this weather but so far so good. i stop when I know I need too. The kids are getting crazy in the house.We lost power last night for 8 hours too. That was fun I am just glad we have heat and water again.

Lila, how are you faring in this mess?

JanW
02-12-2010, 07:21 AM
Sangye - wonder why this wouldn't happen with any other AI disease? Let's say someone goes to the acupuncturist with undiagnosed RA and gets their immune system boosted. Wouldn't that also be the exact opposite of what that RA patient should have?

Sangye
02-12-2010, 09:27 AM
Sangye - wonder why this wouldn't happen with any other AI disease? Let's say someone goes to the acupuncturist with undiagnosed RA and gets their immune system boosted. Wouldn't that also be the exact opposite of what that RA patient should have?
Actually, no. Holistic physicians and practitioners have a long history of successfully treating RA and other autoimmune diseases by doing the opposite of the medical treatment (ie, regulating the immune system, not suppressing it). With the exception of a handful, other AI diseases are not fatal and don't do extensive silent damage like Wegs. So if the treatment were worsening the condition it's quite noticeable and can be changed or stopped in time.

If some AI diseases are caught in the early stages and treated holistically, they can often be cured because the underlying imbalance can be found and corrected. (Some known causes are food sensitivities and toxins.) Once the disease has become more entrenched a cure is not likely. The goal is then prevention of disease progression and relief of symptoms. Again, holistic methods are highly successful at this.

Some patients use a combination of holistic and medical treatments. It can work well under certain circumstances.

You rarely see a holistic practitioner or long-time patient getting an AI disease. None of my holistic buddies have ever heard of one other than me, and most have practiced for 20-30 yrs. It was a real shock when this happened to "one of us." This is also one reason why I'm not a big fan of the genetic theory of causation.

Lila
02-12-2010, 03:13 PM
Thanks Sangye!
I think there are 2 Dr. Kim's there...the one she was going to see couldn't get her in the same day as her follow-up with Dr. Levine, so she's seeing the male Dr. Kim?! I think...
Yea, I know she started her meds today, and I didn't even bother calling her. She was back to her ol' self and now....UGH....the PRED!!! I did tell Dr. Levine about how nasty she gets...him and her both laughed about it. He says that since she's not 'depressed'-cause she goes out, that he'll hold off putting her on anything.Maybe she'll start taking the Xanax....I dunno!
I'm just relieved~
How are u?

Lila
02-12-2010, 03:16 PM
Wow Sangye, I posted a response and now it's not here.....
I'm to tired to retype the whole thing! I just jumped on here real quick....I'll be back tomorrow.
How are u?

Lila
02-12-2010, 03:17 PM
oops! there it is!! LOL! I told u I was tired!! :)

Lila
02-12-2010, 03:18 PM
Thanks Jola!

Lila
02-12-2010, 03:19 PM
Thanks so much Moyan!!! Im so relieved too!

Lila
02-12-2010, 03:22 PM
She got the trache because she couldn't breathe which, I believe was due to the stenosis. Dr. Levine didn't seem too thrilled that the ENT at Jefferson didn't seek the help of a Wegener's doc while treating her. She didn't have any trauma....Sorry if these responses are short...Im falling asleep... ;0

Lila
02-12-2010, 03:25 PM
Im glad everything worked out well for u Onatreetop! This weather is ridiculous but we made it there! YIPPEE! It was well worth the drive!
Dr. Levine mentioned something about Ritux ? (spelling)...maybe in the future. But, he has alot of hope that the trache can be out within a few months!
Take Care! I'll get back on tomorrow when Im not so sleepy! Goodnight! :)

Sangye
02-13-2010, 12:59 AM
Lila, she definitely has to see Young Kim (male) not Jean Kim (female). He's the Wegs guy, she isn't. The receptionists don't know that. They're really nice receptionists and very truthful about him running late, etc... They'll tell you how long a wait to expect with a particular appt time.

The reason I suggested a psychiatrist eval is because evaluation for meds and their use is tricky business. What looks like anxiety is often depression. It's incredibly complicated stuff. Dr Levine is a great doc and knows Wegs inside-out of course. But brain chemistry isn't his specialty and an appt to discuss Wegs is not a psychological evaluation. It's just as important to go to the specialists for that-- moreso. I suggest a psych eval and ongoing therapy. I sure couldn't have gotten through all this without seeing a therapist.

onatreetop
02-14-2010, 11:52 AM
Glad to hear she is taking the meds. Even if it comes with mood slings!!!!! Not swings! My EMG went well from what the doc said at the end. Have more results on wednesday. See ENT on tuesday about biopsy and fallow up. See Prim about fallow up on pulse. And go back to work everyday. I feel So sluggish after a week off. I feel like a blob! The docs are checking into the diabetes now too. This blows Snow!!! I am boarderline and know it from the labs but poop!

Hope the meds work quickly for your sis! An the tra. is out soon for her. Hope you are all dug out and ready for more snow. I am not.