New member diagnosed in January 2020

[Susan B]

Hi all! I’m Susan—50 years old and from Iowa, USA. I’ve enjoyed looking through the forum for the past several days and have found that many of you all have had similar experiences. Here is my (sorry, long-winded) story with GPA so far.

I began having allergy-like symptoms in April 2019, with severe nasal congestion and sneezing. After maxing out on over-the counter meds and nasal rinses, I got a referral to an ENT specializing in allergies. My nasal walls were so swollen I could feel the insides touching across to the septum at times, and I had a tiny amount of bleeding. Eventually my ears felt full and I could not move any air through my Eustachian tubes to equalize pressure.

My ENT used a scope to view my nasal passages and felt the inflammation was consistent with allergies, and reacted to 10 substances with testing. I was prescribed 2 prednisone tapers which were the only things that provided relief. In October, my ear fullness worsened and eventually had a spontaneous eardrum rupture. Over the course of months, I had multiple visits regarding my “allergies” and expressed surprise that someone my age would develop such severe allergic symptoms and ear problems when I had never had a single ear infection or a reaction to any substance in my entire life. No one seemed impressed. I finally had an ear tube placed in the ear opposite the rupture before I was to embark on my 50th birthday cruise to Antarctica. ENT was not concerned about the ear causing problems during a 3 week trip to a place without ant medical care other than a small ship’s doctor.

Go figure, my GPA decided to ramp up at the most inconvenient time. On the flight, all of my knuckles suddenly became stiff and painful. I mentioned it to my sister, who was traveling with me, and said, “it’s almost like I have some kind of weird autoimmune condition.” (It was a wild yet accurate guess—I had never heard of GPA.) I became seasick as soon as the cruise was underway and started taking Dramamine. Soon, I noticed that the older guests were passing me on our shore outings while I stopped to catch my breath. I was much colder than I would expect for the climate and broke out in sweats at the same time—I attributed this to seasickness, meclizine, and jet lag.

Midway through the cruise I developed mild hemoptysis and consulted the ship’s doctor. He (quite reasonably) diagnosed me with pneumonia and started me on antibiotics. Three days later my symptoms had become so severe that I knew I had to leave the ship. It took the entire day for evacuation to be arranged, and by the time a medical plane landed at a nearby air strip I was receiving oxygen at 15 lpm and barely achieving O2 sats of 90%. The luckiest thing for me, was that the airstrip we were near belonged to Chile, so I was taken to a small private hospital in Punta Arenas — Chilean health care is excellent.

I was admitted to the ICU in Punta Arenas, and was sedated and intubated within hours. I had diffuse alveolar hemorrhage and dumped loads of bloody and secretions via the ET tube and mg hemoglobin dropped to 5.6. I would ultimately receive 8 units of blood. My diagnosis was finally made when the MD performing a bronchoscopy recognized the appearance of vasculitis. I was given massive doses of steroids and became the first patient to ever have plasmapheresis at that hospital. They borrowed the equipment from the Punta Arenas’ public facility.

Being on a ventilator with a knocked out immune system made conditions perfect for a very nasty pneumonia, which led to sepsis. My blood pressure was maintained on vasopressors and they managed to find a multiple antibiotic regimen that could treat the infection. A tracheostomy tube was placed and I was transferred home to the teaching hospital where I work as a nurse. By the time I arrived I was off pressors but still not oxygenating well. A chest tube was put in and yielded 2000 mL of fluid from a massive pleural empyema. Finally, my oxygen levels stabilized.

I woke up to my sister’s voice telling where I was—I had no idea nearly 3 weeks had passed since I arrived in the ICU in Punta Arenas. Fortunately, the rheumatologists at our facility have treated dozens of patients with GPA. I got IGG, proper antibiotics and steroids, and at last a solid plan to whip this GPA into submission.

I spent a total of 7 weeks in the hospital and inpatient rehab. Upon wakening, I had severe neuropathy and could only move my right arm and my head. After a battery of tests including EMG (fun, fun, fun!), the neurology team decided the most likely diagnosis of my weakness and paraesthesia was critical illness neuropathy. On follow-up they are still not convinced my residual neuropathy is due to GPA. Reading you comments here makes me a bit skeptical of that. I was on 80 mg prednisone per day for months and developed steroid-induced osteoporosis which, combined with my non-ballerina-like grace, has resulted in several spine fractures, a broken ankle, and a broken rib.

I’m doing very, very well now. I’m down to 5 mg prednisone daily and Rituximab every 6 months. I returned to work full time in July. I even saw my staff ICU doctor today and was so happy I could tell him in person how grateful I am that they preserved not only my life, but my heart, kidney, and BRAIN function. My lungs are so much better and kidneys are good—thank goodness! No one here wants this diagnosis, but the experience of all of this hasn’t been all bad. I believe I have become a better nurse in subtle yet important ways. I value all of life’s important moments—and simple pleasures all the more.

I’m here to take advantage of the wisdom of all of you who have more experience with GPA, and hope to keep it at bay the best I can. I’ll happily offer my limited insights and support to you all here. Looking forward to getting to know you better!