My story ... journey to a diagnosis

[356bubbles]

My problems start in January 2008 following the birth of my first child. Over the next 10 years I suffered from pain in joints, pins and needles in my feet and hands and a skin infection every 6 - 9 months. Each time the GP would give me various diagnoses and prescribe me something to get me through the flare up. During this time, I saw many consultants including rheumatologist, immunologist and a cardiologist. No one could give me a diagnosis and things appeared to be getting worse. In July 2017 I had a small flare up which only lasted a week (quite small timescale for me) however, once the spots had gone, I continue to get worse and was eventually admitted into hospital in August 2017 with kidney failure. Having been transferred to a specialist kidney unit, I was diagnosed with ANCA related Vasculitis. Over the next three weeks, I had blood transfusions, kidney biopsy (which went wrong but that’s another story!) , 10 sessions of plasma exchange and began cyclophosphamide and large dose of steroids. After 12 weeks on cyclophosphamide, I moved to azathioprine. Over the last 18 months, I have twice almost got into remission but each time we withdrawal steroids my MPO measure quickly goes through the roof. Now we are trying Rituximab and I have just had my second transfusion.

[Christophene47]

Oh my! I can only relate to your story in so far as my daughter, after giving birth to her first and only child, suffered terrible musculoskeletal pain. She had every test in the book, and we learned that the practice of rheumatology is not a very precise speciality. Also the baby was premature and in the NICU for 5 weeks; of course, we were all thrilled when baby came home.

She is 13 now and an honor student in her school. My daughter continued to suffer as you did being in such pain and needing to care for a preemie at the same time. The farthest she got with rheumatology was a diagnosis of rheumatoid arthritis. She tried an IV infusion of Remicade, and became unable to breathe so the treatment was stopped.

She decided not to continue with Remicaid, and, unfortunately fell into the hands of a pain management practice which got her addicted to every narcotic available.

She realized that was no way to live and with the help of a detox doctor. got off all the narcotics. She recently was accepted into a medical marijuana program, which is legal where we live, and she not only works, but in hard physical labor, and is doing well and happy.

I too was diagnosed myself of C-ANCA vasculitis in 9/16 and immediately put on Prednisone and in less than 3 days ,felt great.I had a PR3 titre of. 1.6 which is very low; I sought a 2nd opinion at the Vasculitis Center at Johns Hopkins University in Baltimore. MD.in the US in Baltimore MD, I was required to send in all medical records, and 5 mo later had an and was met by a lovely doctor who took 1.5 hours with me, and bluntly said. "I don't think you have vasculitis." Upshot is I don't, and, thereafter my pain was attributred to polymyositis rheumatica still hanging on to 4 mg.

Unfortunately, I recently fell down stains at home; lucky no broken bones, but bad soft tissue damage including a hematoma lump on top of pain from PMR. I was ready before the fall to decrease my prednisone to 3 mg, but figured , not a good time to reduce dose..

Your story struck me because your problems started with the birth of a baby, and so did my daughter's .I'm sorry you have gone through so much; and I am waiting for remission as you are.

I hope you feel in good care; I don't blame rheumatologists so much because they are dealing with a tricky set of diseases, for which there are no easy answers.

I was offered Rutiximab, but I was waiting for my appt. with the specialists. I was also given Azanthopine which I could not tolerate, but did better with Methotrexate until a 2nd rheumatologist and a hematologist advised against it for me. Now I am wondering if I was right.

I hope you will get your remission soon. Seems odd that only during your hospitalization did doctors think to look for C-ANCA vasculitis and that it wasn't discovered until a hospitalization.

Best of luck to you and your little one . Do let is know you progress.

[Webbyj]

Thank you for sharing your sharing your story so far. Good luck with the Rituximab. Keep us posted.

[356bubbles in reply to Webbyj]

Thank you, will do. Second infusion went well - no immediate side effects. Am now feeling completely wiped out! Hoping that today will be the last day of rest and back to work tomorrow. Would be interested in hearing from others who have had Rutiximab infusion particularly how long it was until they felt better and were able to resume ‘normal life’.

[Boffy08 in reply to 356bubbles]

Hi there,

I’m just waiting for my 4th infusion(two infusions each time) because of a flare up. I also have had GPA since 2007 and have had numerous treatments over the years. Rituximab works well for me, but does take at least 2 months, after second infusion, before my my symptoms disappear and I start to feel well again. The last infusion lasted 12 months before I started to flare again. I’ve had to increase my steroids as the last couple of days the symptoms are getting much worse and there was a 2.5 week wait for the treatment.

All in all Rituximab has been fantastic for me and I would highly recommend it.

[356bubbles in reply to Boffy08]

That’s really great to hear. In between infusions when things are going well, do you manage to come off steroids completely?

[Boffy08 in reply to 356bubbles]

No 😞 I’ve been on steroids since 2007! The lowest dose I have been on is 5mg but in the last few years it’s been more like 10mg. It take seconds for your steroids to be increased but months to get them down again. Saying that, I have been very healthy and able to most things in the last year.

[Maria_B]

Hi 365bubbles, I am sorry to hear about your lengthy journey! Having read the other replies I am sure that gives you the hope that the treatment will be worth it - although it is not immediately.

I think all of us suffering with a vasculitis of one type or another is very pleased to have any treatment they suggest in the hope that it will help improve or reduce the symptoms.

I have had 6 months of cyclophosphamide which I completed in February only to be told that it hasn’t worked. The plan now is to be started on Rituximab which I wasn’t looking forward to but hearing from those who have had their treatment and improved after is very uplifting. At the moment I have yet another chest infection which unfortunately led to a few days in hospital- now home again managing it myself which I am happier with!. I need to be well with no infection in order to start the Rituximab on the 9th of April.

I wish you luck on your treatment and hopefully you will get the same effect as the others by eventually feeling a benefit from it. I certainly feel now that I am not so worried about the treatment but so happy to hear that it has improved the quality of life for others.