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Ehlers-Danlos syndrome is a single or group of rare connective tissue disorders caused by 20 genes that “alter biosynthesis, organization, and/or supramolecular assembly of collagen fibrils in the extracellular matrix” (Syx, Delfien; Malfait, Fransiska, 2024). This disorder causes the following symptoms:
- Skin hyperextensibility and fragility
- Abnormal wound healing
- Joint hypermobility
- Connective tissue frailty
The overall fragility brought about by EDS can lead to many life threatening instances, like the hollowing of organs or a weakened arterial tree.
The diagnosis of EDS is aided by something called a Beighton score. “Diagnostic and symptom checklists, including how to conduct a Beighton assessment for joint-hypermobility, are…readily available online”(Black, William R;Black, Lora L;Goldstein-Leever, Alana;Fox, Lisa, 2024). The Ehlers-Danlos Society describes it as a 9-point scale where “A positive Beighton score is any score greater than or equal to 5/9 points in adults, 6/9 points in children (before puberty), and 4/9 points in adults over age 50.”(The Ehlers-Danlos Society, 2024). Beighton assessments include an evaluation of the:
- Thumbs
- Elbows
- Pinkies
- Knees
- Spine
During these assessments, patients are asked to hyperextended each joint. If the joint extends farther than the normal range, a point is added on the scale. One point is added for each elbow, thumb, pinky, and knee with the 9th point being singular for the hyperextension of the spine.
My Project
For this project, I made a physical snapshot of connective tissue containing elastic fibers and fibroblasts. I wanted to take on a more abstract approach to Ehlers-Danlos Syndrome by using elastic bands (made of rubber) to symbolize collagen/collagen fibers. Many instances of EDS have to do with the irregular spacing or small diameters/irregular spacing of the collagen fibrils in the extracellular matrix. To convey this, I linked some elastic bands on my structure together to provide varying thicknesses of fibers and even pulled some bands around to rearrange them, causing the overall structure of my project to weaken.
References
Black, W. R., Black, L. L., Goldstein-Leever, A., Fox, L. S., Pratt, L. R., & Jones, J. T. (2024). The need for primary care providers in the clinical management of hypermobility spectrum disorders and ehlers-danlos syndrome: a call to action. Rheumatology International, 44(11), 2273–2278. https://doi.org/10.1007/s00296-024-05676-4
Syx, D., & Malfait, F. (2024). Pathogenic mechanisms in genetically defined Ehlers-Danlos syndromes. Trends in Molecular Medicine, 30(9), 824–843. https://doi.org/10.1016/j.molmed.2024.06.001
The Ehlers Danlos Society. (2024, August 9). Assessing joint hypermobility. The Ehlers Danlos Society. https://www.ehlers-danlos.com/assessing-joint-hypermobility/