My physiotherapist assessed me with the Beighton Scoring System and I got the maximum score.
Joint hyper-mobility syndrome: increased joint flexibility beyond the normal range of motion
POTS: postural orthostatic tachycardia syndrome
HSD: hyper-mobility spectrum disorder
OI: orthostatic intolerance
Annotated Bibliography
Atuesta-Rodriguez, Alexis Javier, Yimy F. Medina-Velasquez, Orfa Motta, Maria Isabel Narvaez-Reyes, and Federico Rondon-Herrera. “Association between Postural Orthostatic Tachycardia Syndrome and Joint Hypermobility.” Edited by Christopher S. Snyder. Case Reports in Cardiology 2021 (April 28, 2021): 1–4. https://doi.org/10.1155/2021/8875003
This is a fascinating case study that honestly reminds me of my own medical history.
From the introduction: “The following is the report of a rare case of JHS asso- ciated with postural orthostatic tachycardia syndrome (POTS). The relevance of this case report lies in the impact that this disease had on the patient’s quality of life and the limitation in the performance of activities of daily living.”
“Patients with POTS sometimes have a history of viral infection or a family member with similar symptoms; however, the actual association is unclear. The pathophys- iology of this disease is mainly due to autonomic nervous system dysregulation, sympathetic hyperactivity, and atten- uated vagal tone, resulting in hypoperfusion [8].” I did not realize blurred vision, which is a bit of a problem for me when I am tired or dehydrated or dizzy, was a POTS symptom. (See table on page two, top of page.)
Boris, Jeffrey R., and Thomas Bernadzikowski. “Prevalence of Joint Hypermobility Syndromes in Pediatric Postural Orthostatic Tachycardia Syndrome.” Autonomic Neuroscience 231 (March 2021): 102770. https://doi.org/10.1016/j.autneu.2020.102770
Our evaluation of a large series of pediatric patients with POTS revealed that over one-fifth of patients had EDS and over one-third of patients had HSD.
Celletti, Claudia, Beatrice Borsellino, Marco Castori, Federica Censi, Giovanni Calcagnini, Filippo Camerota, and Stefano Strano. “A New Insight on Postural Tachycardia Syndrome in 102 Adults with Hypermobile Ehlers-Danlos Syndrome/Hypermobility Spectrum Disorder.” Monaldi Archives for Chest Disease 90, no. 2 (May 20, 2020). https://doi.org/10.4081/monaldi.2020.1286.
Peebles, Karen C., Isabella Tan, Mark Butlin, Felicity Collins, Louise Tofts, Alberto P. Avolio, and Verity Pacey. “The Prevalence and Impact of Orthostatic Intolerance in Young Women across the Hypermobility Spectrum.” American Journal of Medical Genetics Part A 188, no. 6 (June 2022): 1761–76. https://doi.org/10.1002/ajmg.a.62705
This small study did not find a statistically conclusive correlation between orthostatic intolerance and generalized hypermobility disorder or hEDS.
The links between hypermobility, Ehlers-Danlos and endometriosis seem elusive, but could have an underlying genetic deficiency in vitamin B9 (folate) that affects connective tissue and eventually causes myriad seemingly unrelated symptoms including joint pain, chronic fatigue, thin tooth enamel, digestive trouble and migraines, endometriosis and uterine fibroids. While this article only briefly mentions endometriosis, folate does indirectly and directly produces antioxidant effects, which fits the big picture that oxidative stress is an underlying mechanism in endometriosis etiology.