Autonomic dysfunction, often referred to as dysautonomia, is a broad term describing malfunctions within the autonomic nervous system. It’s a condition frequently observed in various medical contexts, and notably, it has a significant association with Ehlers-Danlos Syndrome (EDS), especially the hypermobile type (hEDS). For individuals seeking specialized care, Eds Doctors are crucial in managing the complexities of EDS and its related conditions.
One of the primary challenges faced by those with EDS is orthostatic intolerance. This condition manifests as symptoms that arise when standing upright and are typically alleviated by lying down. The most prevalent orthostatic intolerance syndromes seen in hEDS and Hypermobility Spectrum Disorders (HSD) include orthostatic hypotension, Postural Orthostatic Tachycardia Syndrome (POTS), and recurrent vasovagal syncope. These syndromes share overlapping symptoms that can be significantly debilitating, impacting daily life and overall well-being. These symptoms encompass pre-syncope and syncope (fainting or near fainting), heart palpitations, persistent fatigue, excessive sweating, headaches, reduced tolerance to exercise, blurred vision, and nausea. Seeking guidance from eds doctors is vital for accurate diagnosis and effective management strategies.
Orthostatic hypotension is clinically defined by a notable drop in blood pressure – specifically, a decrease of more than 20 mmHg systolic or 10 mmHg diastolic – when transitioning from a lying to a standing position.
POTS diagnostic criteria include:
- A sustained increase in heart rate of 30 beats per minute or greater upon standing from a recumbent position, without a corresponding drop in blood pressure indicative of orthostatic hypotension.
- For adolescents aged 12 to 19, this heart rate increase threshold is even higher, set at 40 beats per minute.
- Crucially, the elevated heart rate must be accompanied by the characteristic and frequent symptoms of POTS to warrant a diagnosis.
It’s important to note that a patient can be diagnosed with POTS, orthostatic hypotension, and vasovagal syncope concurrently, as these conditions are not mutually exclusive. Fortunately, the initial treatment approaches for these three conditions are generally similar. These typically involve lifestyle adjustments such as increasing fluid and salt intake, making dietary modifications, employing postural maneuvers to help prevent fainting episodes, and engaging in carefully graduated exercise programs. In addition to these lifestyle changes, medication can also play a beneficial role in managing these conditions. Eds doctors can provide tailored treatment plans that address the specific needs of each patient.
Another condition gaining increased attention in the EDS community is Mast Cell Activation Syndrome (MCAS). MCAS is an immunological disorder characterized by the inappropriate and excessive release of chemical mediators from mast cells. These cells, part of the body’s innate immune system and found throughout the body, when activated improperly, can trigger a wide spectrum of chronic inflammatory and immune symptoms. In severe cases, this can even lead to anaphylaxis or near-anaphylactic reactions.
The relationship between MCAS and hEDS is a relatively recent but increasingly discussed topic among both patients and healthcare professionals. Particularly in the United States, a significant number of patients are being diagnosed with both conditions. Observational studies suggest a potential overlap between EDS and MCAS. However, it is crucial to acknowledge that research into a definitive link and the underlying mechanisms is still in its early stages. For individuals suspecting MCAS in conjunction with EDS, consulting eds doctors who are knowledgeable about both conditions is advisable.
For further in-depth information, resources are available that explore MCAS and its connection to EDS, providing valuable insights for both patients and medical professionals. These resources can aid in understanding the complexities of these overlapping conditions and inform discussions with eds doctors for comprehensive care.
