Starting Thalidomide 10 years ago

[vivdunstan]

Facebook reminded me that 10 years ago today I started on Thalidomide. Because of the horrific birth defect scandal it is only used now very rarely, for rare cancers and very occasionally for auto immune, including very rarely vasculitis. It is a bureaucratic nightmare to get, especially for a woman who’s pre menopausal. On plus it did help with my life threatening brain form of vasculitis, which had resisted many other drugs over many years since treatment finally started after my very delayed diagnosis in 1997. On downside I suddenly showed signs of peripheral neuropathy damage from it, which is a red flag situation, and meant I had to stop it immediately and permanently. There are similar drugs chemically to Thalidomide that patients who have to stop it can be switched to instead, but I then demanded the Cyclophosphamide chemotherapy infusions which the consultant had ruled out multiple times for various reasons (his concerns about very young me’s fertility + my already appalling bladder incontinence). They really helped. But yes, Thalidomide for most of a year for me, starting 10 years ago. Quite rightly only used with great reluctance and care, but can be life saving.

[piglette]

Thalidimide is supposed to be an excellent drug and recently has been found to be a powerful anti-angiogenic drug.

[vivdunstan Volunteer]

Many thanks. I’m pleased it’s a drug that can help people, but wanted to raise a bit of awareness. Very few people in the public know it’s still used. Even among vasculitis patients it’s little known. I’d have been happy to stay on it if I hadn’t developed the red flag issue. Even with the incredible bureaucracy every month to get it (including a monthly pregnancy test and lots of extra monthly form filling). But being able to finally demand Cyclophosphamide really switched me around. It’s a bit ironic that being on Thalidomide before led me to demand another treatment the consultant had previously ruled out for fertility concerns!

[Dancerina in reply to vivdunstan]

What was your experience with cyclophosphamide like? To what extent did it stop you your brain inflammation? What side effects did you have and how did you handle them?

Over the past 3 years, my son has been on varying doses of prednisone along with Rituximab and later tocilizumab. With both of those infusions, he had breakthrough inflammation once his prednisone got below 20mg/day. He is recovering from a brain hemorrhage & crainectomy. and they are starting cyclophosphamide in the next day or two. They did have him bank sperm. He’s back on higher prednisone dose.

Were you ever able to cut back on the prednisone?

[vivdunstan Volunteer in reply to Dancerina]

I found Cyclophosphamide a very tough treatment, with severe nausea, vomiting, shivering, hair loss etc. On one day I vomited 20 times over a 12 hour period. Initially my body responded with increased record inflammation markers. But my bladder incontinence was improving a lot, so we hung on in there. I had 6 infusions. It took a year afterwards for me to see the full benefits of it. Then I was able to start successfully lowering my steroids for the first time in years. I had battled to lower them ever since my huge relapse in 2004. I was also able to drop my Azathioprine and Methotrexate from my combo cocktail. I am now managing on just 2g Mycophenolate Mofetil and 7mg steroids, and expect to be on those for life, barring another relapse. My disease is still slowly progressive, but as stable as can be. In my circumstances we are taking it as a positive thing.

I will try to attach a picture showing my steroid dose graph. It only dates from 2006 or so. I’ve been on steroids permanently since 1998, and ill since 1994.

Graph of steroid doses over time, showing a rollercoaster, but more recent lowering

[Dancerina]

I should make a steroid graph. Where’s your area of vasculitis inflammation?

He is starting with an 8 day regimen with treatments days 1, 2, 4, 6 & 8. He is getting a gram of iv prednisone along with anti nausea and other protective meds & antibiotics. Then eventually they will repeat some sort of treatment over time, maybe twice? He still has no B cells from two doses of Rituximab. Has tapered 4 or 5 times over past three years but taper ends w 5 days of IV prednisone and then back up at 60 for another taper. One taper was to take him off prednisone to prepare for brain biopsy which confirmed Vasculitis after the cerebral angiogram was negative.

How slowly did you get to 7mg?

[vivdunstan Volunteer in reply to Dancerina]

I can't remember where specifically it was in my brain. I was diagnosed back in 1997, a very long time ago now, and I was extremely ill already by then. But I do remember it's in the small blood vessels in my brain.

I tried dropping steroids for many years after my relapse in May 2004.

I only managed a sustained drop starting from January 2014. From then I had a mainly lowering path, as the graph above shows, with a few small increases along the way. I finally reached 7mg in May 2019, and have stayed there ever since.

But this is only possible because I am also on a steroid sparing medicine Mycophenolate Mofetil, which reduces the amount of steroids I need. I have been on other similar steroid sparing drugs in the past, including Azathioprine and Methotrexate. In my experience it is quite common for cerebral/CNS vasculitis patients to need such drugs over a long term in addition to steroids.