Postural Orthostatic Tachycardia Syndrome (POTS) and Ehlers Danlos Syndrome (EDS) in Pediatric Patients

Postural orthostatic tachycardia syndrome (POTS) is a disorder of the autonomic nervous system (ANS) that is predominantly found in females and disproportionately affects adolescents. POTS symptoms, including dizziness, fatigue, palpitations, headaches, and difficulty concentrating, are exacerbated by upright posture and tend to improve or resolve with recumbency. Management typically starts with lifestyle recommendations, such as increasing fluid and salt intake in addition to a graduated exercise program, and sometimes includes pharmacologic agents. POTS can significantly impact one’s quality of life. POTS is frequently co-morbid with other medical conditions, including Ehlers Danlos syndrome (EDS). EDS, a group of heritable connective tissue disorders, is characterized by hypermobility, skin hyperextensibility, and tissue fragility. EDS is thought to be present in at least 25% of patients with POTS. Establishing the diagnosis of hypermobile EDS and/or hypermobility spectrum disorder (HSD) typically occurs several years after symptom onset. The symptoms of EDS, including pain, fatigue, and functional disability, have a considerable effect on mood and quality of life in patients. A comprehensive treatment approach is usually recommended and includes both non-pharmacologic and pharmacologic interventions. Adequate psychosocial support is crucial to addressing the emotional aspects of living with these invisible, chronic illnesses, particularly for adolescent patients.