mycophenolate in advanced fibrosis

[Ajackson10]

Hi

Has anyone with quite advanced fibrosis started on mycophenolate?

My mums scleroderma has seemed to be advancing recently. Her breathing has become very bad she is now struggling to walk at all. Her diffusion rates had dropped to 56% and this was a few months ago so are probably lower than this now. She's also recently had a ct scan to rule out lung cancer due to fast developing mass on lungs which could be fibrosis but still waiting on results.

Is mycophenolate effective at this stage or would it have been needed to have been started sooner? She is under rheumatology (who it is extremely difficult to even see nowadays) but we think she also should be under a lung/breathing specialist. Not sure which way to turn really as it seems impossible to get support from healthcare nowadays!

Thanks

Alex

[Lupiknits]

I’m so sorry about the problems your mother has. I’m not a doctor, and my condition is not as bad as your mother’s, but I am on mycophenolate. It’s my understanding that this dampens down the autoimmune process. All I can say is my rheumatologist would likely have started it earlier for your mother.

Has she had pulmonary function tests? Or echocardiograms? Again, the standard advice is for these to be given annually to check on any possible damage.

Could your GP arrange an urgent appointment with a rheumatologist? Failing that, has the clinic at the hospital have a number you can contact where you can get to speak to a rheumatology nurse? The nurses can speed things up.

I’d say a lung specialist is in order and your GP can be asked to sort this out.

As to rheumatologists in general, it’s unfortunate that many do not have much training in scleroderma as it’s a much less common disorder. I don’t know where you live, and whether it’s affordable, but some on the forum have seen a rheumatologist privately, which has helped them better access the NHS.

Again, I’m so sorry you are both in this position x

[Ajackson10 in reply to Lupiknits]

Thank you for the message ❤️. She does see a rheumatologist currently but finds it extremely difficult to speak with them, and despite requesting it several times she still hasn't been referred to a respiratory specialist as the rheumatologist says they will only treat her the same way. I really find it massively frustrating she is finding it so difficult to get a referral. Shes also asked to be given oxygen so she's more comfortable but they won't do that either! X

[Rups123456 in reply to Ajackson10]

They said the same to me. So I just took it upon myself and I went directly to Brompton, time is of the essence with this condition, Brompton then did the letter to both my mums GP and Rheumatologist , your GP then writes a letter of referrel back to Brompton and refers her. The good thing about Brompton is they have a good relationship with royal free ( I presume your mums with them) so they will most definitely know your consultant and speak to them directly.

[OldTed60]

Hi Alex. Lupiknits has said it all really but just to ask who is looking into your mother’s mass on lungs? if not a lung consultant or rheumatologist but her GP - then it’s up to them to be proactive on her behalf with coordinating and referring to various specialists. But most GPs wouldn’t take on this role with systemic sclerosis but would chase via rheumatology.

The problem seems to be that when things are left too long with systemic sclerosis, it eventually goes for the organs. So regular reviews are needed with rheumatology to oversee the monitoring and this should involve annual pulmonary function tests and echocardiograms - results of each being sent to rheumatology directly and necessary action being taken. So I don’t understand why this hasn’t happened already in your mother’s case? Mycophenolate takes a while to work and should be started earlier on in the disease if possible - so perhaps steroids would be helpful until the Mycophenolate takes over? Alternatively Rituximab or other biologic infusions might be better for your mother now as big gun treatments which are increasingly used when scleroderma is already quite advanced - particularly in the lungs.

I think you posted on behalf of your mum about Mycophenolate a while ago? Did she start it? It’s hard to know but if a person refuses to start the medications they have been offered then the rheumatologist may shrug and say there’s nothing much more they can do. I think a lot depends on a person’s age and temperament and how willing they are to try medications and to adapt their lives to their changing health needs. So it’s great that you’re being proactive on your mother’s behalf at least. If she will let you then I think it would be good if you followed Lupiknit’s suggestions and phone the rheumatology desk yourself and explain the urgency here. You may need to be quite forceful if they aren’t being much use, as it sounds. They should be referring her urgently to a specialist in respiratory medicine. Best of luck xx

[Ajackson10 in reply to OldTed60]

Thanks for the message ❤️. Yeah I posted a month or two ago. She hasn't started mycophenolate yet but she has been waiting on a ct scan to rule out lung cancer. The referral got really delayed and we are waiting on the results now - will prob get them next week. So she didn't want to start the medication until that has been ruled out as she was worried it could affect that. But generally she doesn't tolerate medication well at all so it is sometimes hard to get her to take things. Yeah I'll try pushing a referral for her. Is it a standard for a scleroderma patient with fibrosis to be under respiratory? She also has COPD so I just can't understand why it is so difficult to get her referred! X

[OldTed60 in reply to Ajackson10]

It is standard for anyone with lung fibrosis to be under the care of a respiratory consultant - especially with scleroderma. I’m really shocked that she’s not already under respiratory clinic and also that rheumatology aren’t working with a lung consultant on her case already. Sorry to say but I think you might have to make an almighty stink about this once the results are back - depending which condition you mum’s breathing problems relate to. If it’s scleroderma related then the buck stops with her rheumatologist. If it’s COPD related ie cancer then the buck stops with her GP. Either way she should definitely be seeing a lung consultant. Who will give her the results of the CT? Her GP? I’m so sorry you’re both having to put up with so much prolonged suffering xx

[Rups123456]

Hi Amy your mums situation is almost identical to what my mum has. Myclophenate is for the Sceleredoma part and controlling that so it’s important to stay on that. However for the fibrosis I don’t believe the myclophenate will help the lungs standalone as it didn’t help my mum.

You need to push to get her to get on an anti fibrotic drug.

My mum is currently on tocilizumab which has completely suppressed her immune system. In conjunction with this I pushed to get her the anti fibrotic drug Nintedanib. These two drugs combined plus the Myclophenate is supposed to be effective. We are waiting the CT scan results to see if the progression has slowed down ( and in some cases can miraculously reverse some of the fibrosis)

I hope this helps.

[Rups123456 in reply to Rups123456]

And yes absolutely she should be with a lung specialist seems like ILD which covers Pulmonary fibrosis. If you are based in the UK. Get in touch with Royal Brompton. Your rheumatologist and the lung specialist should absolutely be working together. I hear your pain. It took me multiple consults, meetings and some what aggressive emails for everyone to hear me and take action in a timely manner and work together. Be persistent!

[Ajackson10 in reply to Rups123456]

Can she just contact royal Brompton directly or would she need to be referred there?

[Rups123456 in reply to Ajackson10]

it was too slow for a referral from the rheumatology department at Royal free. So I did my research found the lead consultant at Brompton went privately for a consultation. He reviewed her results he then wrote to my mums GP for a referrel copying in the rheumatologist so they all now speak to each other and work together.

[Ajackson10 in reply to Rups123456]

Thank you ❤️ - I hope it's good news with your mums scan. It's good to know that there are other possible medications. Do they have a lot of side effects do you know? My mum does struggle to tolerate medications. X

[Rups123456 in reply to Ajackson10]

Thank you we are hoping for some positive news.

My my is exactly the same. Gets all the side effects. With Tocilizumab she got mucus and nausea but after 2 months it eased. Wit the anti fibrotic drug she’s on only one tablet a day and has tolerated it. She is supposed to have two but the doctors believe the side effects maybe too harsh. I presume if the fibrosis doesn’t control or get better she will the have to up it.

Let me know how you get on if you need anything my email is [e-mail]

[Whistler81]

Remember we are often told we are all different with some cross over obviously. I’m being treated with mycophenolate for Scleroderma. Early on in my diagnosis I was told I had early signs of fibrosis. Months later and further scans I was informed that the fibrosis appearance has now disappeared.

Best wishes.

[Ajackson10 in reply to Whistler81]

Thanks so much for message this is really good to hear ❤️

[OldTed60 in reply to Whistler81]

Same here. 😊