Chronic health conditions can significantly impact daily life, often presenting with a wide array of symptoms that make diagnosis and management challenging. Understanding these conditions is important for those affected and their support networks.
Understanding POTS
Postural Orthostatic Tachycardia Syndrome, known as POTS, is a disorder of the autonomic nervous system, which regulates involuntary bodily functions like heart rate, blood pressure, and digestion. Individuals with POTS experience an abnormal increase in heart rate upon standing or sitting upright. This often leads to symptoms such as lightheadedness, dizziness, fatigue, and cognitive difficulties. Other common symptoms include chest pain, shortness of breath, palpitations, nausea, headaches, and sleep disturbances.
POTS is characterized by a sustained increase in heart rate of at least 30 beats per minute (bpm) within 10 minutes of standing for adults, or 40 bpm for those aged 12-19 years. This heart rate increase occurs without a significant drop in blood pressure, distinguishing it from orthostatic hypotension. Diagnosis often involves a tilt-table test, where heart rate and blood pressure are monitored as a person is tilted from a lying to an upright position. Subtypes of POTS, such as neuropathic, hyperadrenergic, and hypovolemic, are recognized based on underlying mechanisms.
Understanding EDS
Ehlers-Danlos Syndromes (EDS) are a group of inherited connective tissue disorders that affect the body’s collagen production. Collagen provides structure and strength to tissues like skin, tendons, ligaments, and blood vessels. When collagen is compromised, it can lead to symptoms such as joint hypermobility, skin extensibility, and tissue fragility.
Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common type and is frequently observed alongside POTS. Individuals with hEDS often experience generalized joint hypermobility, leading to easily dislocated joints and chronic pain. Other manifestations include soft, velvety, and easily bruised skin, as well as digestive issues and fatigue. Diagnosis of hEDS is primarily clinical, relying on criteria such as the Beighton score to assess joint hypermobility and other systemic signs.
Why POTS and EDS Often Co-Occur
The frequent co-occurrence of POTS and EDS suggests shared underlying biological factors. One hypothesis points to the dysfunctional connective tissue in individuals with EDS, which can affect the integrity of blood vessels. This can lead to weakened blood vessels that struggle to constrict effectively, resulting in blood pooling in the lower extremities when upright, a contributing factor to POTS symptoms.
Other factors contributing to this comorbidity include autonomic nervous system dysfunction, where structural abnormalities from EDS may disrupt nerve and blood vessel function. Mast cell activation syndrome (MCAS) is also frequently observed alongside both conditions, potentially contributing to their complex symptom profiles. The clinical observation of their frequent co-occurrence highlights a clear connection between these conditions.
Approaches to Managing Both Conditions
Managing both POTS and EDS requires a comprehensive, multidisciplinary approach tailored to the individual. Lifestyle adjustments are an important part of management for both conditions. For POTS, increasing fluid intake to 2-3 liters per day and consuming 3-10 grams of salt daily can help expand blood volume and support blood pressure. Wearing compression garments can also help prevent blood pooling in the lower body.
For EDS, physical therapy focused on strengthening muscles around joints can provide support and reduce the risk of dislocations. Gentle, low-impact exercises, such as swimming or using a recumbent bike, are recommended for both conditions, as they help improve cardiovascular health without exacerbating symptoms. A team of healthcare professionals works together to address the diverse symptoms. Symptom-specific pharmacological treatments may also be used to manage heart rate, blood pressure, pain, or gastrointestinal issues.