Jean’s Story

We are very excited to share another Still’s disease patient story with you. Because the Still’s disease population is incredibly varied, our goal in sharing these stories is to make sure we give a voice to as many different facets of the Still’s experience as possible. Not all patients have the privilege of sharing their stories without fear of having it impact their personal or professional lives, but this (entirely understandable) need for privacy shouldn’t exclude them from having a voice. Thus, we present to you an anonymous Still’s disease story under the name Jean.

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Personal Information

Name: Jean

Age: Early 30s

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Still’s disease Onset

Age at onset	The onset of my illness was insidious, but in retrospect, I probably had mild symptoms as a toddler. I was 12 when I realized something was wrong and started looking for answers.
Name of disease at onset of diagnosis:	My doctors use SJIA and AOSD interchangeably. My first diagnosis was inflammatory arthritis in my 20s which eventually changed to JIA then SJIA.

Disease onset story:

I had fleeting symptoms of pain and fatigue, as early as age 3. Progression was subtle until age 12, when the I developed join pains, migraines, and flare ups of fatigue and low grade fevers. This was also the first time I remember going to the doctors for symptoms. At 15 the “invisible” illness was “visible” to those who were casually observant. I missed a lot of school and needed a lot more rest than what was normal. Unfortunately, no doctor was able to put the pieces of the puzzle together. I moved a lot which led to fragmented medical care.

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Diagnosis

How long after onset did it take you to get a diagnosis?

It took almost 20 years for me to be diagnosed from the time I first went to the doctors. And almost 10 years after my first diagnosis.

Diagnosis story, if different from disease onset:

At age 20 I suddenly developed shoulder pain that radiated down my spine, up my neck, and into my face. The first rheumatologist I saw diagnosed this pain as inflammatory arthritis, but didn’t do a thorough exam, completely missing significant liver involvement which was eventually diagnosed as an autoimmune liver disease that was unusually refractory to treatment. My symptoms continued to get worse but would be blamed on the liver disease, including the chronic fevers, because I had no additional antibodies. Eventually I did progress to the point where there were other recognizable organ-specific autoimmune “syndromes” but no primary disease that explained it all.

Three primarys, 8 rheumatologists, 4 neurologists, and 3 liver doctors later, I received a diagnosis of JIA in my mid-20s. I asked if it could be SJIA and was told no because my arthritis was mild. I trialed anti-TNF medications to help with the arthritis and liver disease which normalized my liver tests. But I also developed excruciating back pain, lower body numbness/weakness, and difficulty walking. The rheumatologist at the time became frustrated and decided it was best to take me off all medications to see how long my “remission” would last (answer: 2 weeks). I’m pretty sure they decided to ignore me and my other doctors, as efforts to contact them afterwards proved futile. My liver enzymes quickly rose into the quadruple digits, and I asked to be referred to one of the renowned institutions.

The year of the year-long workup was the worst for me health-wise. My liver enzymes remained in the quadruple digits and I developed an unrelenting fever that fluctuated from low to high grade. Eventually after ruling out many other possibilities it was determined I had SJIA/Still’s Disease. My shoulder pain was determined to be a rare neuro-immune process affecting the cranial and spinal nerve roots (cranial neuritis & polyradiculitis) and suspected spinal cord (myelitis). This explained why I reacted so strongly to anti-TNFs as they can trigger Guillain-barre syndrome (a polyradiculitis) and myelitis.

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Your Life Today

How has your disease changed since onset and/or diagnosis?	It’s been 2 years since I was diagnosed, and I do feel a lot better than the hellish year leading up to diagnosis. However overall, my disease has been more aggressive and harder to manage with time. I’ve had a continuous fever for 3 years, which contributes to significant fatigue. I have chronic pain from inflamed spinal and cranial nerves. I am also dealing with the possibility of additional organ involvement.
What is a day like in your shoes as a Still’s disease patient?	I work from home full time and don’t do a whole lot other than that due to the severity of my symptoms. After work I spend the rest of my day wishing I had the energy to do more than waste time on the internet. When I can, I volunteer my time for patient advocacy and support efforts. Pre-pandemic a “busy” day would include grabbing dinner/lunch with a friend; working in the office for a few of hours; or going to a doctor’s appointment. Never all three in one day, of course.
If you could go back in time to when you were diagnosed and give yourself some advice, what would you say?	Essentially, I was diagnosed in the reverse order of when each conditions presented. By the time I was diagnosed with SJIA/AOSD, I had learned how to handle the emotions of being diagnosed with a serious condition But during the 10 years from my first diagnosis of “inflammatory arthritis”, I wish I had known to request medical records regularly, seek second opinions sooner, and that treatment from doctor’s won’t necessarily be any better once you’ve been diagnosed.
What is one thing you wish doctors knew about Still’s disease?	A patient doesn’t need to have severe arthritis, or any arthritis, to have Still’s Autoinflammatory diseases are generally not characterized by autoantibodies and may be an appropriate consideration when a patient with suspected systemic autoimmune disease has no autoantibodies. In regard to the liver, autoinflammatory patients should be monitored for development of an autoimmune liver disease. And autoinflammatory hepatobiliary disease should be considered if the diagnosis of immune-mediated hepatobiliary is elusive.

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Treatment + Medications

Past Medications	Off the top of my head, azathioprine, humira, enbrel, tacrolimus, plaquenil, anakinra, cellcept. I’m sure I’ve forgotten something
Current Medications	Actemra, Prednisone, IVIG