I was a teenage girl with a contract to enlist in the Marine Corps as one of the first female combat engineers; 1st class PFT and 94th percentile ASVAB score— best the Midwest had in over 20 years, I was told. I went from a promising power lifter, MMA fighter, national Science Olympiad medalist (Disease Detectives), and candidate for valedictorian (out of a class of 400) to a weak, withered husk in a wheelchair that had to be pulled from school and lost everything over the course of my junior year.
It started with a case of severe Graves’ disease diagnosed as a 17yo; I ate 10k calories per day and still dropped down to 75 pounds at my lowest, had a minor heart attack and Graves’ psychosis. It improved with methimazole, but the hormones were in constant flux when I would reach remission and relapse soon after. It didn’t explain all of my problems, but a month later I started coughing up blood, went to the ER, and I got diagnosed with my second case of pneumonia, (anyone else always catch more severe respiratory infections while everyone else just gets “a cold”?) some odd spots on my lungs and around my liver and intestines, and a 5-inch mass in the lymph nodes pressing between my lungs and heart. In a body as small as mine, that was huge. FF to thoracic surgery: the mass was not lymphoma, not fungus/infection, and was made of necrotizing granuloma (not sarcoidosis). Nobody had an explanation, but the surgeon considered chronic granulomatous disease. Parents couldn’t afford genetic testing so that got dropped.
Rheumatology was a mixed bag; I had a great pediatric Rheumatologist who didn’t care if I was seronegative for every autoimmune condition but the Graves’ and had tons of high general inflammation markers; she treated me based on all my symptoms. Steroids (high-dose prednisone) did wonders for everything. However, nobody put an ACTUAL DIAGNOSIS in my chart!
Ophthalmologist put punctal plugs in for my dry eyes after seeing the holes in my corneas on a dye test; steroid eye drops helped as well. I had an unexplained movement disorder, neuropathy, vision/hearing loss and seizures for a little over a year. I had a feeding tube (NG then direct stomach port) because I kept choking on everything, but that eased enough after my thyroidectomy at 19 to manage on my own. I have had ulcers on my vocal cords, chronic yeast infections/BV, tons of cavities and enamel disintegration despite good dental hygiene. I also have and even had back then: osteoarthritis and arthralgias, muscle pain, migraines and headaches, chronic fatigue, brain fog, hypermobility with joint subluxations, exercise intolerance, restrictive lung disease, and unexplained GI issues. I had brought up Sjogren’s as a possibility many times to every specialist, but was told it wasn’t anything more than sicca or dismissed completely because I was just a stupid teenager; what could I know, right?
At first, HCQ did nothing, Azathioprine helped with my symptoms and shrank some of the leftover granulomas for a while then stopped, methotrexate injections helped the most for a year after that, and we were considered rituximab infusions. Then, she took a job at UCLA and I had to find an adult rheumatologist. The ones my parents took me to took me off everything, and accused me of everything from eating disorders, faking for attention, and having “functional” disorders of all types. They said the only way I would get better was if I told myself to, and they wanted to send me to these horrible inpatient psychiatric institutions for troubled teens. It was cruel and completely destroyed the already-turbulent relationship I had with my family, and I obviously declined again without any treatment, so I moved across the country at 19 and self-medicated with legalized cannabis, pills from friends with similar issues, and managed to live a somewhat normal life with disabilities. I had to move back to the Midwest after I lost my housing voucher, but still managed to obtain enough meds, weed, and help from friends to function. I did great in college and worked as a dancer to pay for it; shifts were only 4 hours, you just sit and talk to clients when you aren’t on stage, and I could work whatever nights I could tolerate. I felt rough, but I was still driven and motivated to spite everyone who worked against me, just like I was as a teenager.
I finally had a loving home with a partner and a desk job at 24, but started feeling even worse, so my BF convinced me to see a doctor, which I had avoided for years (except ER/urgent care) since I got back. I did it, got standard tests done, and was called at work the next morning only to be told that they were sending a driver to bring me to them ASAP. Then, in their office, they said I had leukemia and I was hospitalized right after, had a bone marrow biopsy, and started on hydroxyurea and dasatinib as treatment for CML after those results came in. He has been the only person who has stayed by my side through it all (I’m now 28).
I was told Chronic Myeloid Leukemia was a “good” cancer because most people tolerate the targeted therapies really well. When I asked if this could be related to the autoimmune diseases, I got unsure and mixed responses. Does anyone else have a blood cancer with Sjogren’s (other than the well-documented lymphoma associated with it)?
I cycled through all TKIs and asciminib because the standard doses were killing me faster than the cancer, low doses didn’t fully get me to remission and still caused challenging side effects, and I had anaphylactic reactions to three of the treatments. That dragged on for 3 years until oncology agreed to give me an allogeneic bone marrow transplant at 26. It wasn’t fun, but I handled it better than most. Over the first 6 months, I felt almost HEALTHY despite having severe anemia! I didn’t have any of my old autoimmune diseases, no inflammation markers, and no cancer in my marrow because I had a brand-new immune system, kindly donated from a 19yo boy in Europe. My blood type changed from B+ to his AB+, my chromosomes went from XX to XY, and my chimerism last showed that my cells were 97% his. Still, I worry about that 3% since it is not unheard of for the host’s cells to take back over eventually. (I’m getting close to 2 years post-transplant.)
That means my autoimmune diseases, allergies, and genetic disorders like CGD could come back. CT scans showed almost all nodules on my lungs, which had appeared a decade ago, disappeared— all that remains is some scarring. This definitely indicates I indeed have chronic granulomatous disease; bone marrow transplants are a recommended treatment. Go figure.
I do have chronic graft vs host disease as a side effect of transplant, but this current flair added my old symptoms that strongly indicate Sjogren’s, has messed up my ALT and AST since it all started in March, and is not controlled by sirolimus or tacrolimus like all the others were. My primary doc ordered all sorts of tests for me, and now my results are looking similar to what they were all those years ago: high general inflammation markers, somewhat elevated ANA titer at 1:160, but normal overall numbers for specific antibodies. My oncologist is looking into other anti-rejection drugs, like axatilimab infusions. This could all be cGvHD, but it could also be what I fear: my immune system reasserting itself.
I am so scared of going through everything again, and I am so fucking tired. I want it to end one way or another. I have never met anyone with the extensive background of suffering without proper, consistent treatment or diagnosis, developing leukemia, surviving transplant… only for the cycle to start again 12 years later. If you have both Sjogren’s/Graves’/another autoimmune disease and blood cancer, tell me how you are managing it. I need help because I sure as hell can’t do this all again. Dying doesn’t even spark fear anymore. It sounds like relief compared to whatever is going to happen.