Hi All
Well I have now now tried myco and aza in an attempt to reduce steroids. Have just had to stop with azathioprine due to another bad reaction. I am holding out for Mepo..does anyone have experience with with other regime which will not put Mepo on hold? Or should I just suck up steroids for longer? For the past 10 months pred dosages have varied between 40/day to 15/day. Currently on 22.5/day.
HiOnly wish i could be prescribed some effective medication. Was lucky enough to be prescribed Steroids once for 5 days in the last 2 or more years, which allowed me to actually breath normally for a short while
Anything to escape daily and nightly coughing fits and the inevitable constant production of phlegm, only wish i had shares in Kleenex.
It has been over 54 months since diagnosis of EGPA ( Churg Strauss) and I am just about surviving on a Symbicort Inhaler, Carbocisteine capsules, and Montelukast tablets.
Next Consultant appointment Thu 2 Dec 21.
No breath, no energy, no life.
Hi Ruskin, sorry to hear that too. Are you being treated by a Vasculitus specialist?
Unfortunately, there’s so much trial and error in finding the right treatment for each individual.
Wishing you all the best 🙏🏻🥰
Sorry to hear this. I am puffing on my symbicort as well. Its all trial and error and pushing and pushing whilst we have energy
If you have asthma, you may benefit from treatment with a biologic such as Nucala or Fasenra both very effective. Read up on controversy with Montelukast promoting worse symptoms, I have discontinued use approved by my specialist.
Hi EGPAGuy,Sort to hear you’re still having not found your stability.
Are you under a Vasculitus/EGPA clinic/consultant?
I think there’s no set medicine course or treatment unfortunately everyone is so individual.
My sister has EGPA and she’s reacted to Azathioprine, Mycophenolate and retuximab. And had copious amounts of steroids. For now her body has reacted so much to all the drugs that her immune has been battered in to immune suppression, so it appears to be keeping her EGPA at bay.
Because her consultants are hesitant to give her anymore immune suppressants because of her reactions, should she have a flare up, that’s the possible next plan to have mepolizumab.
I will say she managed to stay on retuximab for 4 and a half years before having a reaction, so definitely seemed like the most effective, the other drugs took a year until she reacted.
For us, the main thing is having faith/trust in her consultants, we are lucky as she’s under Vasculitus Clinic at Addenbrooks.
I’d also say, in our experience if you was diagnosed 10 months ago, I’d say you may still be early days of finding the right treatment. Did you start off with cyclophosphamide?
Wishing you all the best of health.
Look after yourself 🙏🏻🥰
Hi Cloe..I feel lucky to have been diagnosed relatively quickly after reading about experiences of others here, EGPA with lung involvement and am being treated at Addenbrookes. Started on pred and then pred and myco and latterly pred and aza and am on the long path to Mepo. Issue is if I go down the rituximab route I have been told that will likely put off Mepo for another year but may have no choice as I want to continue to work. Mepo is not guaranteed but I continue to push and hopeful about getting it this year sometime albeit because of NICE standards only at a third of the recommended dosage which is all we can get in the UK (we should all band together and petition the nhs/NICE to recognise the science for Mepo and EGPA..NICE I believe cut its trial and we all have to suck it up) and thats to treat a different Eosinophillic disorders in my case suspected Eosinophillic Asthma. Still jumping through the hoops. Just reaching out to see whilst I wait if anyone has found a steroid sparing solution that does not involve Myco or Aza as they are both non starters for me now because of really bad side effects. Chemo/MTX or Biologics/ Rituximab are both on the table but want to hold out for Mepo whilst hope exists without destroying my kidneys with pred in the meantime. Suffering from breathlessness, extreme fatigue, cough, loss of feeling in hands/feet and generally feeling weak and unwell...usual stuff although no sinus or ear issues to date and finding it harder to focus at work. My wife (and work colleagues) will tell you that the steroids make me manic and grumpy so kudos to them all for putting up with me.I feel for your sister. As I understand it mepo is a maintenance drug..if we were in USA we would all be on low pred dosage and mepo (3 times the dosage we are allowed in the UK) for maintenance. Cyclo is used to treat flare ups. Could be wrong. We (actually my wife) have done loads of research and continue to push a few boundaries and learn to control our frustrations. Ultimately I guess its all about cost as Mepo is really expensive - even in the USA EGPA patients have to petition Glaxo for assisted programs as the insurance doesn't cover it all.
Best Lev
You are obviously under the same consultant then, I totally agree, madness a drug to treat eosinophillic asthma but seen differently as a condition of EGPA. Ridiculous!! Fingers crossed 🤞🏻 for you that you get the ok for mepolizumab. They are trying to trial it for EGPA so it becomes available for patients, but I guess these things all take time and being a rare condition probably slows the process down more too.
That was definitely my way of managing, research, research and more research!! 🤪 however a lot has changed since my sister was diagnosed 10 years ago. It was a year to 18 months until she was diagnosed, she was so ill but thankfully doesn’t have massive organ damage, did damage her nerves in her hands and feet though.
Good luck, and hopefully you stabilise soon and wish you all the best. 🙏🏻🥰
Thanks Cloe and best to you and your sister..we have probably passed each other in clinic 12 over the past months..may stand up and announce myself next time. Will be up next month.
😀
Hi I’ve never had any major problems with aziathioprine. I’ve been taking it for about 9 years and reduced a bit so I’m on 75mg a day - would like to reduce further. I started on about 200 mg along with high doses of steroids which have extremely gradually reduced. When I came off prednisolone unfortunately my adrenal glands had been affected so I still have to take hydrocortisone- but only a low dose 10mg a day. Sorry I can’t give you any advice about other regimes. Good luck - it’s difficult to find what’s right for you and you often need to be patient and try different things.
Thanks Katie. My experience with aza was shortlived and told to stop immediately yesterday. Was on 150 mg/day. 2 hours after taking cycle was 1. Sweats. 2. Intense dizziness. 3. Nausea 4. Heaving 5. Completely wiped out. My pee turned an odd colour as well and experienced total loss of appetite. Work was very hard as the dizziness was constant. Glad to get off it. I can best describe it as that feeling ...erhem..perhaps some of us may have experienced back in the day before diagnosis after a few too many drinks when the room starts spinning and you begin to feel sick, only it lasts all day. Reaction to myco when I was on 3g/day was 24/7 pain. Lived with that for a few months until told to stop. I haven't had any obvious physical problems with pred except difficulty sleeping and mania although I am sure my kidneys are screaming. Been pretty good with diet cutting out sugar and simple carbs and only having small amounts of fat mainly as olive oil.Hope you are feeling good.
Lev
Struck with severe nerve attack in Nov 2020. Original diagnosis Guillain-Barre. New dx w EGPA Vasculitis in May 2021. First time finding so many other egpa patients. Thnx for your feedback. Weaned down to 10 mg Pred. Taken off Aza after 4 weeks due to high liver markers. Maybe rituxan next,not anxious to start that, too many side effects. I have found that treating the Enosinaphilic asthma is key. Currently receive Nucala monthly injections. Have absolutely NO breathing issues. Fazenra works also. I use Trelegy inhaler every other day.Complete neuropathy in both feet and right hand. Nerve and muscle damage. Take Lyrica & Gabapentin for nerve pain.
Certainly would like to reduce dosage to avoid long term damage. I'm walking and functional so consider myself lucky!
Hang in there everyone!!
Vascular surgeon appointment 
Coming off prednisilone 
joints and muscle inflammation 
Oral steroids 
How do you know when to increase or decrease your steroids?
