Newly Diagnosed

[CapeKay]

On April 6, 2023 a lung biopsy result officially diagnosed me with GPA. It took nearly 2 years. I have a history (beginning 2010) of being treated for Giant Cell Arteritis, a vasculitis of the temporal artery. Not an ANCA associated vasculitis. Back in 2021 a CT scan radiologist review recommended an image guided biopsy of 2 fairly large nodules in my lung. My pulmonologist failed to follow through, although he claimed he tried. Nobody was acting quite right during Covid. I have a history of COPD from years of smoking. However, there was a sudden change that alleviated after a spell of antibiotics. I suspected infection and that inflammation was playing a role. Fast forward a year and nodules had now tripled in size and were called masses. I was treated for pneumonia, which I had no symptoms of, although I had been feeling horrible for a year at least. My Rheumatologist also ignored many symptoms, noting that I had previously told him I was feeling pretty good. In retrospect I believe the earliest sign was in my sinus's. I had a chronic runny nose and swollen lymph nodes in my neck for 3-4 years. Again, after an antibiotic, that temporarily would go away. I made note of this to all my Doc's but none really responded. At some point I noticed the bridge of my nose collapsing but chalked it up to yet another undignified consequence of getting old. I will note that my runny nose was not accompanied by a lot of the other horrible symptoms I have read that others have suffered from. Occasional bleeding and a lot of rawness from all the mopping but not so bad.
After the Treatment in 10/2022 for pneumonia, a 10 day course of 2 antibiotics, I went to a walk in clinic. An Xray showed alarming changes in the past year and Wegener' was listed in the differential diagnosis. I knew of this disease by this name. At the time I was diagnosed with GCA in 2010 a good friends father died from Wegener's (which is what they called it back then). His diagnosis came too late. At this time I had an appointment with a new pulmonologist, but, as a new pt. it was several months out. Doc at walk in called him and arranged for him to see me ASAP. I feel very lucky. He tried to rule out cancer, fungi, and he ran ANCA tests, which were positive. So, now I had to find a new Rheumatologist. I live on Cape Cod MA. Specialty Drs. are just very hard to come by. If you can find one you will wait an eternity to get an appointment. My brother offered to speak with his rheumatologist of 20 years at Brigham Woman's Hospital in Boston. He agreed to see me quickly. He re ran his own tests, scheduled a biopsy quickly. I began Rituximab infusion on April 20.2023 and had my 2nd on May 4, 2023. At this time I was on 60 mgs. prednisone and oxygen 24/7. The last 6 weeks, the disease escalated so fast it was unbelievable. On the eve of the first infusion the Rheumatologist called me and instructed me to go down 10 mgs. prednisone on day 1 of infusion, and another 10 after 2 weeks. I was actually rather shocked, because this protocol seemed to not take into consideration how I would be doing or feeling when that time came. When I told him I had a few questions he said "no questions, I ask the questions". I am a very smart and curious person and am fascinated by disease. I was a manager in a pathology lab for many years. Through my own disease I have sought to learn as much about them as I possibly can. I learn so much here because I believe the people who are going through this can best tell us what they feel is going on.
Anyway, I have been experiencing horrendous assaults on my body that I can only describe as Trachea involved, with intense pain that begins in lower chest and radiates up through back, neck, jaw, ear and eye. The pain can be mind blowing. I had reported these to him on my initial visit. I also told him I had experienced something similar with less intensity and only on 3-4 occasions years ago with GCA. After my 2nd infusion and 20 mgs. decrease in prednisone these began nightly, around 1am, after only 2 hrs. of sleep, and they lasted 3 hours. I also had simultaneously on 2 night intense pain in my legs. I worried I had blood clots. These episodes sort of paralyzed me. I just tried to focus and get through them. I would stand because I was too afraid to sit back down. So, after a week to 10 days of feeling totally drugged, terribly weak (on all that Pred I had lost 13 bs in a month or less) and was getting roughly 2 hrs. of sleep a night, I decided to add back some pred. The Doc was out of the country at this time and I did this for 3 days. I know a lot about what can happen when withdrawing too quickly from prednisone after long term, high dose use. When I told him he was furious with me, as I suppose he had a right to be. But when I told him what I was going through he told me it had nothing to do with the disease and to go get some prilosec.I told him that was not so and that tracheal involvement was well documented. I realize something has been going on there possibly for a long time too and have an appt. with an ENT end of week. I tried to tell him my concerns and that nobody wants more to get off prednisone than I do. I lost 3.5 inches in height 10 years ago and my spine is a mess. I know the price. I just think he needs to consider how I am feeling all along the way. This is not his specialty. His specialty is RA. I have learned here and through all my research, how specialized treatment of this disease is. I am contemplating scheduling an appointment at the Cleveland Clinic. It is 650 miles away, but I can stay with family in New York, make it a 2 day trip and it would possibly be reasonable. I know others here swear by the care they have received there and that some consult I find brevity difficult so sorry to go on and on. I will turn 70 in a few months and I really did not think I was going to survive this a few months ago. I can also be very optimistic and realize that it is also possible that some of what has happened in recent months may reverse. I am losing sight in one eye and hearing in one ear. I do understand this is most likely due to prednisone. Thanks for listening. I welcome feedback.

[Pete]

@CapeKay

You’ve had quite the fight with both vasculitis and your doctor. Here are a couple of thoughts:
> While the trip to Cleveland Clinic is a good idea, there may be a better choice in Boston or New York. The Vasculitis Foundation website has a find a doctor feature.
> I see Dr Alexandra VillaForte at Cleveland Clinic. She’s great. She’s cared for my vasculitis for 11 years. Cleveland is a little over two hours drive from my home in suburban Columbus. There’s not much vasculitis expertise here in Columbus, the Ohio State Medical center not withstanding.
> You may want a Cleveland doc to coordinate/direct your care with a doc closer to you. Your current doc doesn’t sound like a good candidate for that, though.

I hope you soon get the care you need.

[CapeKay]

Thanks so much Pete for your advice. I knew I was likely over reacting, out of fear mostly. I believe I did peek at the Vasculitis Foundation website awhile ago but that was before recent events. I found that site so informative years ago. You and others here were my inspiration. Get the best care you can. Although I was looking at it as more of a good consultation. There is a more specialized group at MGH that I did call. They are part of the same MGH/BWH system. I was a bit afraid of how this Doc I am seeing would react. Anyway I have to get over that. Thanks for sll your support and inspiration.

Kathy

[J@$0N]

Sorry to hear about all you are going through. I don't understand doctors telling you not to ask questions. I was very fortunate with my Rheumatologist.. he and his nurse are very knowledgeable and tell me to call anytime. I lost about 30lbs while on high dose prednisone when originally diagnosed. It is strange how each of us are affected by not only the disease, but also the medication. I hope you get better treatment and start to improve soon.