Not another patient diary on a disease…nope, we are not here for that!
Hello and welcome to Still’s disease, the 411! Shalla and David, our two authors, are excited to share with you a scientific perspective on Still’s disease, while still giving some laymen vernacular helpful for patients at all levels of understanding of their disease. As Still’s disease patients ourselves, we have been frustrated by the lack of information focused towards patients or even appropriately summarized for medical providers on our disease. We both feel strongly to foster further advocacy, education, research and treatment options for the larger umbrella nomenclature of Still’s disease, we needed a grander voice for the adult population diagnosed with the disease. We both have worked with and communicated with the amazing patient organizations and foundations out there, and we’re so humbled by the response of so many wanting this further information as well. Currently, the existing organizations and FDA approved treatments give a voice to Still’s disease, but with the focus on the pediatric version, or Systemic Juvenile Idiopathic Arthritis (SJIA), leaving out a large adult onset population, known as Adult Onset Still’s Disease (AOSD). We are hoping to be that voice and to bridge the two spectrums of the disease to create a larger collaborative definition for further research and treatment options for the larger class.
Still’s is the umbrella vernacular for two diagnoses: Systemic Juvenile Idiopathic Arthritis (SJIA) for patients under 16 (or 18 in some countries) and Adult-Onset Still’s disease (AOSD) for patients over 16. Still’s disease is considered a rare systemic autoinflammatory disease that can strike at any age, with an annual incidence rate (the number of new cases a year) estimated between 0.16 and 0.4 per 100,000 to 200,000 persons worldwide. The reported prevalence rates (the number of diagnosed cases) of Still’s range from 1 to 34 cases per 1 million people — if those numbers don’t mean much to you, that’s okay. Just know that it means Still’s disease is very rare. In terms of the U.S. population of 350 million, that means that there are 280–1,400 people per year diagnosed and about 350–11,900 living with it. In reality, the incidence numbers for Still’s disease vary a lot between countries. We computed using the full data set, because it gives the best picture, even though it’s about as precise as taking a selfie with an old-school flip phone.
We have become accustomed to the reality that AOSD has been lost in the overarching Still’s disease vernacular. There is quite a bit of discussion regarding Still’s that freely uses SJIA, but with no mention of AOSD as related or even relevant. Furthermore, the few treatments approved for Still’s disease are approved under SJIA. We see two paths for correcting this issue, inclusion of AOSD in FDA indications or a move to approve treatments under the more inclusive and grounded, “Still’s disease.” A third option exists to rename the disease using the modern descriptive naming schemes, but this is unlikely to happen until the cause of the disease is discovered.
For those SJIA patients and their amazing parents who fight for them daily, we also recognize a need for further AOSD information and advocacy for this population who will age out of the pediatric system and become quite aware of the challenges those of us with AOSD face in accessing care and treatment. As stated above, in our future blogs, we hope to foster a bridging of the two diseases on the Still’s spectrum to collectively amplify our voices, push for research, and new treatments very much needed in our disease.
We are also here to fight the misinformation and misrepresentations often found on internet sites, social media (including many well-meaning patients within support groups) and your favorite TV medical drama — in a recent episode of one show, the protagonist in the episode was cured by 5 ml of IV steroids. If that were the truth, sign us all up, STAT!
Never heard of Still’s disease? You are not alone.
If you have Still’s disease, you know this common scenario: during your first meeting with a new doctor you talk about your diagnosis, he or she swivels the chair to face a computer and frantically types in “Still’s disease” — 30 seconds of silence later, there is a slow swiveled return to you with either a puzzled or elated look after the triumphant Google search. At this point, you either silently mutter “did you just google my diagnosis?” or utterly blurt this out (both Shalla and David opt for the latter most times).
If you are quite accustomed to this (and most of us with AOSD are), we then politely (or not so politely) correct the provider and offer more comprehensive scientific information on our disease. We walk the doctor through the symptoms that might not appear in the recent Google search or antiquated textbook criteria. We often run into misunderstandings and gross over-generalities of the disease by our providers, such as “Oh, so you have an autoimmune disease with rashes and joint pain?” We might then respond with, “May I have my copay back?”
This response sounds comical and fictional, but happens quite often with the lack of medical knowledge on any rare disease, especially autoinflammatory diseases in the rheumatology space. The concept of autoinflammation was only introduced in the medical research community in the late 1990s. Many providers are still unaware of autoinflammatory versus autoimmune. Like many rare diseases, the patient is often the expert looking to partner with the provider in patient centered-outcomes and care.
More and more it has become clear with Still’s disease that the atypical is indeed the normal. In the future, we plan on sharing patient experiences that will highlight the different types of Still’s, and how unique individual presentations can be. While the old textbook definition of Still’s can be helpful, our goal is to show that Still’s disease is subtle, but with a clear signature. Furthermore, it is clear that the antiquated diagnostic criteria for Still’s disease leave out the very important individual nuances and symptom presentations.
- Amplify the collective patient voice in the Still’s disease community by bridging the SJIA and AOSD continuum.
- Arm patients with the science behind Still’s disease to further engage patients and providers in shared medicine.
- Provide an online resource hub for both Still’s disease community.
- Engage the adult rheumatology practice community with their pediatric counterparts.
It is our hope that by painting a more complete picture of Still’s disease, we can change the patient experience by putting the needed information in the hands of patients and providers. We hope you will join us on this ambitious journey! We would love to hear from you and your stories. We encourage you to email us your questions and any suggested related topics to Still’s disease at SDthe411@gmail.com or find us on twitter @StillsDis_411.
Who are we? What makes us experts on Still’s disease? Let’s make like Barbara Walters and get personal.
You can read Shalla’s Story here.