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[SophiasMom]

Dx'd in August 2016 with GPA - Prior to this diagnosis, I was a healthy, active, health conscious 54 year old. I had been dx'd with seronegative RA 10 years ago that was managed easily with minimal medication and in remission. (We now think that I never had it...but Wegener's all along). We had been in Colorado on vacation, came home and got sick. Tested positive for the flu. The cough got out of hand, so we ended up at the ER. Chest XRay revealed pneumonia (It wasn't). Admitted to the hospital on the weekend, so had to deal with the interns. They tested me for everything from A-Z. One night early on, I lifted the sheet and noticed that my leg was swollen to three times its normal size...blood clot. By the time the surgeon got involved, he told my family that he was unsure if it could be saved. Yes. But, thankfully due to a skilled surgeon and the prayers of family and friends, it was saved and I now only deal with some nerve damage. It was determined that my lungs were full of blood, so I was intubated. Due to the DVT, I had to be placed on blood thinners which made the bleeding in my lungs continue. I eventually came off the vent, went to rehab only to have to return to be intubated again. The doctors decided to place a IVC filter to prevent a clot from reaching my heart. I had two rounds of plasmapharesis with 4 units of plasma and 6 units of blood. High doses of Prednisone and Rituxin were the drug therapies chosen. After 31 days, I got to go home 25 lbs lighter. My husband was able to take another month off of work to care for me. I left the hospital in a wheelchair due to the muscle atrophy that occurred from being in the bed so long. I experienced the typical prednisone side effects that I hated...moon face, mood swings, emotions on a rollercoaster, heart rate acceleration, shortness of breath, and my food didn't taste normal. I have now tapered down, but may be going up soon....I have a feeling. My hair started falling out in November due to telogen effluvium. I have lost 2/3 of it and have to wear it up for it to look close to normal. But, it is already growing back..so it's ok! That is one of the many things I've learned since August of last year...gratitude. I've always been a positive person and thankful for my family and health, but this has made me even more so. This was my second attempt at posting this...I wasn't ready the first time. Too emotional. Anyway, I'm here now and hope I can be of help to someone new that was as scared as I was at diagnosis. I'm still scared, but have learned to live with my new normal. :rolleyes1:

[renidrag]

Thank you for sharing. I was diagnosed in 2009 and in remission since June 2010 with one small bump in the road. Others will be along to welcome you also, best to you.
Dale

[Alysia]

Wow, such a tough onset and journey. Thanks for sharing your story and welcome to the forum. I hope that the worst is over. It seems that you got the best treatment possible and you sound like a strong lady. Sending prayers ❤ Please update us.

[Debbie C]

What a ride you were on. Glad they were not only able to save your leg but save you also. What are the meds are you on now,what dose of pred. Be very careful when weaning down on that, you can easily get a flare if you go to fast. The body will tell you better than the dr. Welcome to the forum,sorry you had to find us but there are so many good people here and since it is world wide ( and most of us on pred ) there is usually someone to talk to 24-7. Glad you are home and your hubby is able to help.Keep us updated or just come on here to chat..Take care

[SophiasMom]

Thank you and the others that have replied. It's nice to talk to otherz that have an idea of what I've been through. I came home from the hospital on Sept 1 on 80mg... tapering down to 2.5 this month, but went back up to 10 when I developed a cough, now going back down to 5mg. I just had a Rituxin infusion last Thursday. My ANCA scores taken that day showed normal on C-ANCA and P-ANCA, but the Proteinanase PR3 test was positive for the first time. I had asked on another thread what that meant, and have been told that it could mean nothing or could mean that I'm either having a flare or about to. Hoping that the Rituxin infusion I just had would stop anything on the horizon. What is usually the course of action for a flare?

[Debbie C]

That seems like an awful quick drop on the pred. I was dxed in 2011 with involvement in my lungs. Not as bad as you by far. I too was perfectly healthy but was rear ended while driving back from a health food store..of all places !! But I went to get cked at the hosp. and they did a ct scan that showed I had several nodules in my lungs. At first they thought I had cancer but they had to do a partial lobectomy to get the dxed of wg. I was put on high does of pred, cytoxin,Bactrim and folic acid for 6 months. I then went into remission and have been ( thank God ) since then. I was given 1 round of rtx that didn't go well so never had another.So now the only med I am on is 5 mg of pred and my dr at Cleveland Clinic said I will be on it for life since my adreanal glands have completely shut down and I am not on any other maintence drug.
Is pred the only drug you are on ? The rtx infusion will take awhile for the full effects to kick in so if it is a flare you may want to ask your dr. about upping your pred again. Several other people here also take methotexate Sorry I can't answer the PR3 question.Hopefully someone will be able to answer that for you. Good luck to you

[SophiasMom]

This is my third series of Rituxin. I have 1g, in two infusions two weeks apart every 4 months. I suspect I may have to stay on the Prednisone due to adrenal fatigue also. My cortisol was a little off before all of this took place. Aside from all of the crappy side effects of the Prednisone, I felt really great while I was on the higher doses. I guess we need to find my happy place. :) Above 10 is not gonna be it.

[lovetoswim]

Hi Debbie. The irony of your onset happening after a trip to the health food store is almost too much to bear! Can I ask what happened with the Rtx that you said "didn't go well"? Glad to hear you are doing well.

[lovetoswim]

I am sorry to hear that your onset was so serious. What a wake up call for us "health fiends." I am in my mid 60's, have exercised every day for 30 years, but in the last year, my life has been a continuum of sinus infections, parotid gland infection and other weird URIs. And exercise has often taken a back seat. Some days I think my healthy lifestyle has prevented a serious onset, but after reading some of these posts from other healthy individuals, a serious onset can come suddenly out of the blue. I've always cherished my ability to exercise, play the piano, etc., and lived life to the fullest. One of the scariest things about Wegs is the loss of control of a lifestyle I appreciated and loved so much. I hope you continue to improve.

[Debbie C]

Hi Debbie. The irony of your onset happening after a trip to the health food store is almost too much to bear! Can I ask what happened with the Rtx that you said "didn't go well"? Glad to hear you are doing well.

Hi lovetoswim..Yea that was bad timing for me but I would have never have know I was sick unless the accident happened.
Since I only had 1 infusion I didn't know what the "norm" was supposed to feel like afterwards, But I just wanted to sleep for about a week.The problem was the from the facility I had it done at. It was their first time giving a rtx infusion and it was getting close to their quitting time for the day so they pushed the remainder of the iv in too fast. I was fine throughout the treatment besides that.

[lovetoswim]

Hi lovetoswim..Yea that was bad timing for me but I would have never have know I was sick unless the accident happened.
Since I only had 1 infusion I didn't know what the "norm" was supposed to feel like afterwards, But I just wanted to sleep for about a week.The problem was the from the facility I had it done at. It was their first time giving a rtx infusion and it was getting close to their quitting time for the day so they pushed the remainder of the iv in too fast. I was fine throughout the treatment besides that.

Debbie, same goes for me. If my primary doc didn't recommend I see an immune doc, I never would have known I had a high pANCA level. Next week, I see a rheumy and will learn more. If you don't mind my asking, had you had the flu in the past and this last flu set off the serious onset?

[Jaha]

Welcome to the forum. I am glad that you made it though that horrible onset of this very unpredictable disease. I wish you all the best with your journey. Please take care of yourself!

[Debbie C]

Debbie, same goes for me. If my primary doc didn't recommend I see an immune doc, I never would have known I had a high pANCA level. Next week, I see a rheumy and will learn more. If you don't mind my asking, had you had the flu in the past and this last flu set off the serious onset?

Lovetoswim, I really don't want to hijack sophiasmom thread but to answer your question. I get a flu shot every year at the Cleveland Clinic with my rheumy. I have not gotten the flu or had any serious onset from it. There is a lot of different opinions on the flu shot that I really don't want to get going on that. If you like you can pm me. Good luck seeing a rheumy,make sure you write down a questions for him/her and try to take any med, records you have with you.

[lovetoswim]

Thanks, Debby.

[marta]

This is my third series of Rituxin. I have 1g, in two infusions two weeks apart every 4 months. I suspect I may have to stay on the Prednisone due to adrenal fatigue also. My cortisol was a little off before all of this took place. Aside from all of the crappy side effects of the Prednisone, I felt really great while I was on the higher doses. I guess we need to find my happy place. :) Above 10 is not gonna be it.

Yuck, what a horrible start of the journey, but sadly, too many people with this disease have to go through hell before starting treatment.

As I was reading down this thread, a few things popped up that I want to share comments on.

First regarding the hair loss. Fret not. The hair that comes back after losing your hair due to the drugs is amazing. Mine came back at least twice as thick as it was when I was healthy (before diagnosis) and also with a beautiful wave to it (it use to be as straight as straight comes.)

Second regarding the pred. My first introduction into the world of WG, took me about 5 years to get off the pred. Everyone that uses it more than a week, gets adrenal atrophy. That's because after about 10-14 days of using high doses of pred, your adrenals go "wait what, we don't have to do anything, vacation time". The only way to get back to having your adrenals activate again is to wean off the pred in a way to wake them up slowly enough to not put yourself into 'adrenal crisis' (which can kill you, and it totally sucks) or worse, trigger a flare if you're a Weggie. I got a pretty fantastic flare last year (March) complete with granulomas, actual holes (perfectly round lesions, the biggest being 30mm) in my lungs along with clots as well, so I too am on the thinners since. I just got off the pred from that flare and I was being quite aggressive about it since I didn't want to be on pred for another 5 years.

So knowing that getting off the pred too quickly can either trigger a flare or set off adrenal crisis, my advice to you is to not go from 10mg to 5mg. The wean below 10mg should be done a little slower, and 1mg at a time. Your body normally makes between 9-11mg / day of corticosteroids, and the worst part of the wean (in my experience) was between 8mg and just below 5mg. That's when the adrenals are forced back into action, and your body has to go through a deficit in corticosteroids for the adrenals to wake up, but it sucks. You're going to have to know that this process is not even remotely fun. But, and this is a big but, it's a lot less painful if you do it 1mg at a time and follow your own body's clues as to the timing than 5mg at a drop. If you want, I can tell you my tecnhique for knowing timing but I wont put it here because already this is too long a post (in typical Marta fashion ;) )

We all feel great while on the high doses. As sick as I was earlier, when I was on the high doses, you wouldn't believe the home projects I did. I have one wall that I tiled with slate, and in my mind that's the pred wall. I also tiled my bathroom in less than 3 hours, ha ha, pred makes you superhuman. Dropping pred, makes you supermush. Having dropped pred completely and being on the other side, also makes you feel super human because you did it.

Lastly, a comment on the RTX. I understand that they really want to beat the beast down, but a treatment every 4 months seems kind of aggressive in my limited knowledge base. My personal choice is to keep RTX as the big gun when I really need it to knock down a flare. I know many docs and patients use it as a maintenance drug and use it every 6 months, but my doc and I wont even go there. I only have RTX to knock a flare down, and it has been proven that the efficacy diminishes with each use, and another thing they know is that WG gets harder to treat with each flare. So in my humble, uneducated opinion, it's good to have a big gun handy for those moments when you need a big gun. For me it's all I have left for a big gun since I've taken my life supply (and then some) of CTX, so I treat it like a golden gun not to be touched unless a Zombie attack (a.k.a. Wegener's flare) is imminent.

There. I'm stopping my rambling. Take it with a grain of salt as it's all based on my experience and might be totally different from your experience, but I think experiences are worth a consideration so you become a long lived patient. I know other experiences on this forum saved my life in the first year of being a Weggie, so hopefully mine can help someone else.

[SophiasMom]

Thank you Marta! Based on your profile pic...pretty sure we could be bff's :) Skiing is my favorite outdoor activity. I haven't put up my skis yet, so to speak...but I may be forced to give it up. But, that thought is for another day, not today! The hair...mine is coming back in great and is also curly right now...looks like a Cocker Spaniel on the top. Mine fell out mostly on the top and back. So, I have enough to pull up and wear a fake hair thing around my piddly pony tail. So, if what's left will hang on, no complaints! Also, you are so right about the prednisone. I felt better in the fall and winter than I had in years. I didn't tile a bathroom, but I had great energy! The month before I got sick, I was on a roof putting on sheet metal. So, I was very active and a wanna be Joanna Gaines (Fixer Upper). 10mg seems to be a good place for me right now. I had developed a strange rash a month or so ago, that I now think is Wegener's related, based on what I have read. Dermatologist thought so too, so prescribed a topical steroid. He thought it came about because of the fairly rapid drop of Prednisone, and using the topical form would calm it down. It did, but the consistent 10mg of Prednisone seems to have stopped it. About the RTX, I am having my last infusion of this series Thursday and I plan on talking to my rheumatologist about it. Originally, she said every four months the first year, then every 6 for 3 years. So, I will be asking. I see my pulmonologist tomorrow. I am going to ask if I had granulomas in my lungs...I never thought about what caused the bleeding. I had these gross sores on my legs and feet..one on my hand. They took forever to heal. They looked like blood blisters. Anyway, thanks to you and everyone that has responded. It helps so much to talk to people that actually know what I've been through.:love: