You can’t know me without understanding Ehlers-Danlos Syndrome (EDS). Because EDS is a genetic disorder, the genetic mutation exists in the DNA of virtually every nucleated cell of my body.
To understand how this affects me (and others with EDS) systemically, one must understand that EDS is a disorder of the collagen assembly line. The job of manufacturing collagen falls primarily to specific cells like fibroblasts (cells that churn out defective collagen in people with EDS), osteoblasts (bone), and chondroblasts (cartilage).
Cells don’t float in a void; they sense their environment through mechanical forces and are anchored to an Extracellular Matrix (ECM). The ECM acts both as mortar that holds the cells together and as a signaling platform. Unfortunately, the ECM is floppy in EDS (much like everything else in the EDS body is stretchy). When a cell tries to grip this loose matrix, the mechanical feedback is wrong and the cell’s internal signaling pathways get confused. Chaos ensues.
Recent research (Zoppi et al.) identified an “Integrin Switch” in EDS fibroblasts. Because the environment feels unstable, cells actually change their receptors to survive. Even if a cell isn’t making the defective collagen, it is forced to live in a structurally unsound house. This leads to a state of chronic cellular anxiety and inflammation, explaining why EDS is felt in every bodily system.
Picture a structurally unsound house (the EDS body). How might this look to you? I imagine my body as a brick house constructed not with durable mortar, but with masking tape that is trying to hold not only the individual bricks together, but the entire house as a functional unit. Because the EDS body is defective in collagen production and processing, this faulty collagen adhesive acts more like masking tape than traditional mortar.
Now picture that tape slowly losing its stickiness, fraying, and failing in unseen places. This is what living with EDS feels like to me: existing in a body that’s held together with tape that loses its stickiness and strength over time.
As I age (and after having six children, including twins at age 39), multi-systemic EDS issues have become more and more prevalent and problematic. In my mid 30s, after having my fourth child, it seemed like the masking tape that I didn’t even know existed as my body’s glue unraveled all at once. Around that time, I received my EDS diagnosis; my health has steadily declined ever since.
I’m now raising six children and caring for an elderly parent, all while navigating the significant health challenges and frequent procedures required to keep my “body-house” as stable as possible. To know me well, you must have some knowledge of the pervasive and wide-ranging effects EDS. To say that my lifestyle has changed in the almost-decade since my diagnosis is a sweeping understatement.
A Body-Wide Challenge: The Multi-Systemic Effects of EDS
An image to leave you with: a graphic that illustrates some of the ways how in which, when a body’s foundational “tape” is weak, the implications are far-reaching. The infographic below clearly shows that a faulty collagen assembly line impacts most every part of the EDS body.
Source: https://webspace.clarkson.edu/~lrussek/docs/hypermobility/RussekHSDSymptoms.pdf
Living Life “Taped Together”
Living with Ehlers-Danlos Syndrome is an intense, full-time job. Living with it alongside my son B, who also struggles with the severe, permeating effects of EDS, is an entirely different level of complexity. Our bodies are bound by the same genetic code, meaning we both have that faulty “glue” affecting every system. While I navigate my own body’s chronic pain, instability, and fatigue, I must also parent and protect a young warrior fighting the very same battles.
By sharing my story and some of B’s, I hope to shine a light on EDS, fostering greater awareness, earlier diagnosis, and better support for those of us living life “taped together.”
