When I was 8 years old, I began to complain to my mother about joint pain. Understandably our pediatrician attributed it to growth pains and sent us on our way with children’s Tylenol. Within a year, however, my joint pain began to present with an array of other symptoms including high fevers, blotchy red rashes, massive hematomas, nausea, and vomiting. But it wasn’t until I was 11 that we got answers.
I was in sixth grade and I was at an evening band practice for the school’s upcoming holiday concert. At the end of practice when I stood up, my legs went out from under me and I passed out. My mother called our pediatrician who met us at Boston Medical Center that night. Five days, and dozens of tests later (including lymph and bone marrow biopsies to rule out Leukemia), we had our answer: Systemic Juvenile Rheumatoid Arthritis (JRA). Just in time for Christmas, I was discharged with a prescription for Naproxen and a referral to see Dr. John Yee at Boston Children’s Hospital.
Immediately after the new year, we met with Dr. Yee, and within minutes he told my mother “You should put her in a wheelchair now. She needs to get used to being disabled.” The appointment ended there, and on the car ride home, my mom promised me that we would find a new doctor who could provide us with a better outcome. Our quest to find a new doctor was interrupted, however, when, a little over a week later, I was airlifted from our local ER to Children’s in acute congestive heart failure.
Systemic JRA can affect internal organs such as the heart, liver, and spleen. When the heart is involved JRA (and RA as an adult) causes swelling and irritation of the pericardium (the thin sac-like membrane surrounding your heart) which impairs heart function. This is known as Pericarditis. The Naproxen I was prescribed addressed the symptomatic joint pain that I experienced but did nothing to treat the inflammation around my heart or to stop the progression of my disease. After several weeks at Children’s, I was discharged with Prednisone (steroids) in addition to the Naproxen to alleviate the inflammation around my heart.
My disease remained relatively stable for a while, but less than two years later we would learn that my disease had attacked my hips so aggressively and damaged my bones so extensively that I would need both hips replaced. I was 13 and in eight grade by then. The tricky thing about juvenile joint replacements is that one’s growth plates need to be closed completely before surgery can be performed. I went home on crutches to alleviate the pain that even just walking was causing.
The first half of my freshmen year of high school passed in a blur. I was on massive doses of pain meds for my hips, and I moved from class to class in a fog. Finally in February of 1998, I had my right hip replaced. Three months later in May my left hip was replaced. After my hips were stabilized it was time to address my knees: My Sophomore and Junior years featured a Synovectomy on either knee. Senior year was surgery free and I set a personal record for myself by missing only 10 days of school that year!
A Fresh Start
Leaving for college presented me with a rare opportunity: a chance to reinvent myself as “Anna” and not “the girl with Arthritis” or “Anna. She has Arthritis or something”. This freedom would prove short lived. During my first semester finals, I was admitted to the hospital with a ruptured Ovarian cyst made more complex by my suppressed immune system. Second semester featured bacterial pneumonia and another hospital admission. Little did I know that Sophomore year would prove even more challenging: over Christmas break that year, I would undergo hip surgery #3 to revise my right hip replacement.
Balancing my Arthritis with college proved far more challenging than I could have ever imagined, and I eventually found myself withdrawing from school. Eventually, I retreated home to care for my mother who was battling her own terminal illness. During this time my mother’s illness offered me an escape from my own and caring for her a distraction from my symptoms. When she passed away in 2006 the emotional pain I felt eclipsed any joint pain until complete and total numbness set in. I got a job bartending and moved through the next year in a fog not dissimilar to the one I felt while waiting for my first hip replacement.
One Step Forward, Two Steps Back…
In 2007 I got what would be a life changing job at Marriott and by 2008 I was enrolled in classes part-time. I was finally moving forward with my life again, but ignoring my Arthritis while doing so. Finally in 2009, my joint pain, especially my hip pain, far exceeded the 2,000 mg of Ibuprofen I was self-medicating with daily, and I reluctantly made an appointment with a new Rheumatologist at Tuft’s Medical Center.
On the day of my new patient intake, I had every single joint in my body x-rayed. When I returned a few weeks later for my first real appointment with Dr. A my hip x-rays were conspicuously missing which Dr. A had referred to Dr. S, my new orthopedic surgeon (my previous surgeon had retired by this time). At my first appointment with Dr. S he seemed edgy, almost nervous. Finally, he put my x-rays on the screen and told me, “I hate that I have to tell you this the first time that we are meeting, but you have suffered a catastrophic failure of your hip replacements. We need to schedule you for surgery immediately.” Eleven years after my first set of hip replacements, and with one revision under my belt already, my hip replacements had failed.
In July of 2009, one week after my 26th birthday, I had both of my hip replacements revised in a single surgery (which I count as hip surgeries/replacements 4 AND 5). While I was in post-op recovery, Dr. S would tell my family that there was no medical explanation for the fact that I had walked into the hospital that day. When they opened my right hip up there was no bone, just metal on metal. Extensive bone grafting was required to repair the damage to the joint. Recovering from that surgery proved more complicated than I was prepared for. Having both hips done at the same time meant being non-weight bearing on both sides = using a wheelchair instead of crutches. As a result, I couldn’t be discharged straight home, and instead spent several weeks in a rehabilitative hospital before going home, wheelchair and all.
My sixth month follow up appointment held more bad news: while the bone grafting in my left hip proved successful, once again my right hip had failed. It would take another surgery to (hopefully) stabilize the joint. I was crushed. In between surgeries I had moved into a third-floor walk up on Hanover Street in Boston’s historic North End. My dream apartment in my dream neighborhood. First things first, I could not recover from surgery while living there. A few days later I called Dr. S and asked, “If I put off surgery will I do additional damage to the joint?” Dr. S told me no, but that the pain would only increase over time. I scheduled my surgery for January 2011 and spent 2010 focused on work and school and life.
Sixth Time’s a Charm
Dr. S wasn’t taking any chances this time. After hip surgery #6 I was 100% non-weight bearing on my right hip, and I spent January through May cooped up in our new Somerville apartment recovering one excruciating day at a time.
That was four years ago, and, for the first time ever, I have graduated to seeing Dr. S every other year in January (instead of annually). I would be lying if I said that I didn’t miss him a little. Despite that initial meeting, we have formed a strong doctor-patient bond and he empowers me to live the life that I want every single day.
This isn’t to say that the last four years have been easy. While my hips have been on the mend, my heart has become increasingly inflamed and there’s evidence of permanent damage from years of swelling. My wrists show extensive damage and joint narrowing, I have cysts on my shoulders and early symptoms of Osteoporosis in many of my joints. Many times I wonder: Just how many good days do I left? How many surgeries can I have before we run out of options? How long before I am in a wheelchair? How long can I reasonably expect to live?
These questions are not me being overly dramatic, overly worried. They are simply reality. A painful truth of this insidious disease.
There is a poem by Dawn Markova called I Will Not Die an Unlived Life which goes like this –
I will not die an unlived life.
I will not live in fear
of falling or catching fire.
I choose to inhabit my days,
to allow my living to open me,
to make me less afraid,
to loosen my heart
until it becomes a wing,
a torch, a promise.
I choose to risk my significance,
to live so that which came to me as seed
goes to the next as blossom,
and that which came to me as blossom,
goes on as fruit.
And, despite my RA, neither will I.