UCTD with NSIP been offered MMF OR High Pulse Cyclophosphimide and v confused ! Any advice appreciated

[NemoP1980]

Hi All

Am new to Health Unlocked - Im a 36 women, otherwise healthy my whole life until early 2015 - Ive had AA disease (vasculitis) for around 18 months now and more recently been given the title of Undifferentiated connective tissue disease with NSIP (lung disease). I was taken off MTX 22.5mg injections once the CT showed lung involvement about 6 weeks ago and am just on Pred for now. Having met the respiratory consultant my options seem to be MMF or High Pulse Cycophosphimide treatment via IV.

Any advice or observations would be most appreciated as i feel completely in the dark about my options and Dr Google just has horror stories!

Thanks so much

Nemo

[JyotiSmith]

Hey Nemo,

They wouldn't give me methodxrate, although it's apprantely one of the best treatments for UCTD or MCTD it causes scaring of the lungs.

Cyclosphamide for me had to be one the best treatments I have had, I had this with mythpredesilone both Iv.

It's a drug commonly used for cancer but for us not as strong, and the side effects of throwing up and everything all there 😣.

I'm on azathioprine now supposed to have that combined with ciclosporin but it made me sick and I have been taking steroids for 2 years now obviously not happy about the weight gain but it's pros and cons.

The good news is I'm stabilised!! Thanks to the chemo. But my dieseas is at its advanced stage at 27 I'm on ambulatory oxygen and am being assessed for a double lung transplant.

Make sure you drink a lot of water, they will give you sickie pills and mensa which is to stop bladder inflammation after treatment. My Iv lasted 5 hours so be comfortable. And I always got a sandwich. You will have bloods every two weeks and wee sample before each treatment to make sure there's no protein in your wee. Be sure you get plenty of rest!! Because it does take it out of you. You have to be a germaphob Cyclosphamide is a strong immune suppressant so it's best to keep away from anyone who has cold or flu or infections as it could stop your treatment. Which then delays the process. I would search cytotoxic precautions just so you are sure, if you have a partner you have to use contraception as it can cause infertility. Depending on which forms of NSIP you have the chemo will kill that cell (cellular) and if it's not damaged it might have some reverse ability.

It's alot to take in but your respiratory specialist and rhumethologisthe should explain everything to you.

This is all the information I can remember.

Feel free to message if you have any questions I'm happy to help.

Good luck ♡

Jo x

[misty14]

Hi memo

So sorry to read of your serious health problems. I'm sure your medical team will suggest which treatment is best for your lung problems but just want to say maybe try MMF first and keep the Cyclophosphamide as a back up ?. MMF is a well tolerated immunosuppressive that works well. Have to be aware that it can cause skin cancer so extra precautions needed. Can also cause diorrhea when first taken but that should settle and it needs blood monitoring as can cause infections but so can all the immunosuppressives!. It's a very tricky decision you have to make but whatever you decide I hope it works well for you. X

[Fennella02]

Hi Nemo. My feeling after reading your post is that I would think there is a big difference in the impact on your body between Cyclophosphamide and Mycophenolate. It surprises me that they have given you such different options to consider. Unless time is of the essence, I would agree with Misty that Mycophenolate is a drug that is easy to administer and tolerate and is perhaps a good place to start?

Whatever you choose, I wish you all the very best. Clare x

[NemoP1980]

Hi Jo, Clare and Misty - thank you all so much for taking the time to reply -

Jo, im pleased to hear your disease has stabilised with Cyclo treatment but sorry to hear you now need a lung transplant. I was on mtx for 9 months and i suspect this has made my lungs worse as I was constantly complaining of not being able to breathe but the dose kept going up. Tried Azathiprine too but didn't tolerate it well at all.

SO ..now i'm left with MMF and the High Pulse Cyclo treatment. Just keen to push this nasty stupid disease into remission if possible to its positive to hear it did that for you.

I think they feel time is of the essence with preserving lung function and preventing any further scarring.

Nemo x