What is Dysautonomia?
Dysautonomia refers to a spectrum of neurological disorders that involve the autonomic nervous system (ANS). A hallmark symptom of dysautonomia due to sympathetic failure is a fall in blood pressure during standing or neurogenic orthostatic hypotension (nOH). When the ANS misfires and produces excessive sympathetic activity the symptoms can present as hypertension or a tachycardia (rapid pulse rate) as seen in patients with Postural Orthostatic Tachycardia Syndrome (POTS) and Hyperadrenergic POTS (hPOTS). Other forms of Dysautonomia include Neurocardiogenic Syncope (NCS), Orthostatic Intolerance (OI), Autoimmune Ganglionopathy (AAG), Pure Autonomic Failure (PAF), Multiple System Atrophy (MSA) and others.
Symptoms may affect every system in the body, are unpredictable, can be debilitating at times or permanently disabling to some. These disorders affect children, teens, and adults. There is currently no cure but ongoing research is offering hope for better understanding and new treatment options. Over 70 million people in the world are living with one or more forms of dysautonomia.
PLANING FOR SUCCESS
A comprehensive resource guide for families with children going to school with the challenges that Dysautonomia and related disorders can bring. This is intended to help families navigate the educational system.
Ehlers-Danlos Syndrome & Hypermobility Spectrum Disorders
Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD) are a group of inherited connective tissue disorders, caused by various defects in the synthesis of collagen. EDS and HSD are known to affect men and women of all socioeconomic, racial and ethnic backgrounds. They are in a class of genetic disorders that are present at birth but can often go unrecognized for years, even decades. Patients often present with a "constellation of symptoms" as they age that make this elusive syndrome difficult for the untrained eye to recognize.
Nearly all types of EDS and HSD share the symptoms of hypermobility and joint laxity. Many types of types of EDS also share the following symptoms: soft skin; easy bruising; and some systemic (widespread) manifestations in the tissue, vascular system, and organs - including autonomic dysfunction. Each type can cause minor to serious deficits in joint, tissue, organ and skin integrity and may impact patients' overall quality of life. Diagnosis includes a Beighton test (depicted below) to asses hypermobility, a thorough patient and family history, and genetic testing. Classification is based-on the 2017 International Classifications from the Ehlers-Danlos Society.
Expanded classifications include 14 distinct types of Ehlers-Danlos syndromes as well as the newly defined hypermobility spectrum disorders. Each type is thought to involve a unique defect in connective tissue, although not all of the genes responsible for causing EDS have been found. Life expectancy is shortened with a majority of individuals living only into their forties with vascular type (vEDS), the most serious form of EDS. The gene for the hypermobile type (hEDS) has yet to be identified. Current research is focused on identifying these still unidentified genetic defects. For questions about the new classifications, see the EDS Society's 2017 Classification FAQ.
Strategic, customized exercise programs, an informed and skilled medical team, adequate pain management and proper protection of fragile joints and organs are essential for good outcomes!
Studies have found that EDS is associated with dysautonomia including OI and POTS. One such study - from the Oxford University Press - explains OI was prevalent in hEDS (EDS: 74.4%, control: 34.3%, p = 0.001), and frequently expressed as POTS (41.0% of the EDS group). Patients in this study responded to tilt with a higher heart rate and lower total peripheral resistance (p < 0.001; p = 0.032). This altered response correlated with fatigue in daily life (CIS). In the hEDS group, tilt provoked significantly more fatigue (numeric rating scale increase: EDS: +3.1 (1.90), controls: +0.5 (1.24), p < 0.001); finding that OI is an important determinant of fatigue in hEDS. [Source: http://rheumatology.oxfordjournals.org/content/early/2016/04/18/rheumatology.kew032.abstract] More studies can be found in the EDS section of the Journal Articles page of the DSN Library.
The Resonance Project
Jordan Plotner is a British/American award-winning composer and writer based in Los Angeles living with EDS/dysautonomia. He is working on a film/music project that deals with the effects of chronic illness on daily life.
Jordans music has appeared in over forty films, television shows, and commercials worldwide, and his concert music has been premiered in New York, Los Angeles, London, Beijing, Shanghai, Kuala Lumpur, and Malta. He has worked with esteemed film composers Marco Beltrami and Joe Trapanese, and has worked on projects for artists ranging from Hans Zimmer and Angelina Jolie to Daft Punk.
After graduating from The American School in London and the Royal Academy of Music pre-college program (concentrating in double bass and jazz studies), and after a brief gap-year stint as an avocado farmer, Jordan enrolled in the Yale Class of 2017. Thanks to a Creative and Performing Arts award, his original filmed opera, Harold, was premiered at Yale, and won “Best Narrative Film” at the Yale Student Film Festival.
More recently, Jordan began the "Resonance Project". He wrote a piece of music for the project with the goal of bringing together the world's largest "virtual" ensemble of chronically ill musicians. To participate, musicians from anywhere in the world can simply record a video of themselves singing/playing their part of the song, then upload it to Jordan's website. After each part has been recorded, Jordan will then patch all of the pieces together to make the full song.
You can visit Jordan's website to learn more about or get involved with the world's largest ensemble of chronically ill musicians! There you can find the song's sheet music, information about the available parts, and in information guidelines for participants. To visit Jordan's website please click the link below: