Racing Against Still's Disease

Twenty-two years ago when I was diagnosed with Systemic Juvenile Idiopathic Arthritis, or Still’s Disease, at age 11, there were no biologics, a new and innovative treatment now widely prescribed for Still’s Disease patients.

All we had available in trying to stem the tide of my disease were NSAIDS and DMARDS, neither of which proved effective. Just three years after my diagnosis, I underwent bilateral total hip replacements at age 14. I had two more knee surgeries before even graduating high school, and in February I had joint surgery number ten.

It all came to a head when I was in sixth grade and collapsed at practice for the school’s holiday concert.

I received my diagnosis in 1994 just days before Christmas. Three years prior, I started articulating symptoms to my parents. It started with achy joints, but progressed to include rashes spanning from my chest down to my thighs. Fevers that would spike to 103 at night, but be gone by morning. Incidental sports contact that left me bruised and swelling.

It all came to a head when I was in sixth grade and collapsed at practice for the school’s holiday concert.

I was rushed to the ER where I was admitted for a battery of tests including lymph node and bone marrow biopsies to rule out leukemia and cancer. Even being diagnosed with the rarest form of childhood arthritis paled in comparison, and I was relieved to be discharged with a prescription for NSAIDS and a referral to a pediatric rheumatologist.

Anna Chair

This relief was short lived when the first doctor we saw told my mother, “You should put her in a wheelchair now so that she can get used to being disabled.”

We went on to find a new doctor, but for the first seven years after my diagnosis, true disease management and treatment remained elusive. I was sixteen when the first biologic drug was approved for use in juvenile arthritis, and I finally felt a glimmer of hope. But, despite my rheumatologist’s best efforts, my access to the drug was denied due to insufficient pediatric clinical trials and another two years went by without relief. Shortly after my eighteenth birthday, I finally began treatment and my health almost immediately stabilized.

As a result, I was able to attend college, join a sorority, go on spring break – and do all of the “normal stuff” my peers did that for years felt so far out of reach.

Fifteen years after beginning that first medication, I’ve tried and failed most of the drugs that are currently available, but this new class of biologic drugs continues to be as life changing as they were when I was 18. Almost two years ago, I started my current treatment protocol during one of the worst disease flares of my adult life. My commute to work sapped all my energy and I struggled to hide heating pads and support pillows in an open floor plan office. On bad days I had to decide which impeded my work less: soul crushing pain or taking narcotic medications. On my worst days, I couldn’t move from bed.

This relief was short lived when the first doctor we saw told my mother, “You should put her in a wheelchair now so that she can get used to being disabled.”

Worst of all, my Pericarditis was in an acute flare as well. A comorbidity of SJIA and other forms of autoimmune arthritis, Pericarditis is swelling of the pericardium which is the thin sac-like membrane surrounding your heart, and the inflammation of this small, delicate space constricts the heart's function causing excruciating pain. During that time it hurt to breathe 24 hours a day.

By the time the loading dose of my current drug was complete, my health stabilized in familiar fashion. And, just three months after starting treatment, I ran my first Spartan race.

Anna Spartan

Today my disease remains in a relatively well controlled state, but I still experience spikes in disease activity and breakthrough pain due to advanced joint destruction. Perhaps even harder than the physical pain, is figuring out how to cope with my prognosis both mentally and emotionally.

Due to the way my disease has advanced over time, remission (especially medication free remission) is no longer medically considered something I will be able to achieve. Instead, my care team and I focus on enhancing my quality of life, however that is defined, to the best of our ability. Today this includes life as a “RheumAthlete” – or athlete with Rheumatoid Arthritis, but in the future simply preserving my ability to walk is a probable goal.

Perhaps even harder than the physical pain, is figuring out how to cope with my prognosis both mentally and emotionally.

Just recently I was dealt yet another unexpected blow in the progression of my disease when a routine cardiology checkup revealed "RA-related heart disease," in the form of extensive functional and structural changes to my heart that have emerged seemingly overnight. As my cardiologist put it: I have the heart damage of someone twice my age. Even worse is the uncertainty of not knowing how this will continue to progress – it could worsen in two years or in 25...or, if I’m lucky, the current available treatments may be equipped to stop it.

Anna athlete

As much as I’m defiant, I also remain hopeful. I’ve grown up and witnessed firsthand advances in treatment that previous generations never could’ve dreamed of. My own mother passed away when I was 22, and while my health had improved dramatically by then, I’m certain she never envisioned a future for me that would include running alongside other competitive endurance athletes. So I hold out hope that new treatments will come to exist before I’ve exhausted my remaining options, and that maybe, just maybe, remission is possible after all.

I hold out hope that new treatments will come to exist before I’ve exhausted my remaining options, and that maybe, just maybe, remission is possible after all.

anna strong smile
anna_legassie
Anna is an athlete (and enthusiastic cook and management consultant and bionic woman) who lives with Systemic Juvenile Rheumatoid Arthritis. She blogs about her adventures and trials at Six Hips and Counting. You can follow her on Instagram and Twitter.