by: Shalla Newton MSN, RN, NE-BC and David Maher
Imagine for a moment, you are in a casino and before you are two giant dice. The first has on each of its sides the various organ systems while the second is covered in the endless inflammation pathways of the immune system. In this casino, these are the only dice you may use to gamble. However, the house always wins. Oh, and you cannot opt out of playing, no matter how much you lose.
Once you reach a certain level of inflammation, your dice get rolled. And you have to live with the consequences and they are never in your favor. Still’s disease operates like a casino run by a silent film era villain, with every role of the dice causing some kind of misfortune.
Treating Still’s disease mostly reduces how often you must roll the dice, but there is no guarantee. Even with the best of modern treatments, patients and providers are often left feeling like they are waging a war on chaos.
To put this another way, Still’s disease complications appear to be highly stochastic, giving each patient a truly unique set. As one patient recently stated on social media, “it’s like the body is ordering a la carte.” We recommend you hold the cheese!
Turtles all the way down
It is important to remember the complications of Still’s disease are driven by the core autoinflammatory process generated by the innate immune system. This autoinflammation is widespread and aimless. However, it is capable of becoming localized and deliberate when it receives the correct signal(s), leading to the enormous variety of complications. Where and how the inflammation lands, much like a set of dice, is often left to chance.
Still’s disease inflammation is like the old mythological story about the world resting on the back of a turtle. In the story the world turtle supports the earth on its back, and stands on a slightly larger turtle, who stands on yet another larger turtle, and so on. No matter how far you go, it is turtles all the way down.
With Still’s disease, the overarching chronic systemic inflammation drives the disease and each complication thereafter. Every organ system is often affected in some manner. No matter where you look, it is inflammation all the way down.
Why post a series for complications?
Chronic systemic inflammation and innate immune dysregulations drive complications in Still’s disease. Both are also diffuse and undirected. Chronic inflammation and immune deficiencies are like the roll of a gambler’s dice, risky and unpredictable.
Thus, we will break down the complications per system, set or disorder in a series. This allows us a bit more room to dig into each subject, giving a much clearer picture of the insidious manifestations of Still’s disease.
See you next time!