Dysautonomia in hEDS

(Excluding POTS — see separate information page)

While Postural Orthostatic Tachycardia Syndrome (POTS) is the most well-known form of dysautonomia associated with hypermobile Ehlers-Danlos syndrome (hEDS), it is far from the only one. People with hEDS may also experience other types of autonomic nervous system dysfunction, which can affect multiple body systems and significantly impact quality of life.

This page outlines types of non-POTS dysautonomia seen in hEDS, including symptoms, prevalence, diagnosis, treatment, and special considerations for care. For information on POTS, click here:

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What Is Non-POTS Dysautonomia?

Dysautonomia refers to a dysfunction of the autonomic nervous system (ANS)—the part of the nervous system that controls automatic bodily functions such as heart rate, blood pressure, digestion, sweating, and bladder control.

In addition to POTS, the most common forms of non-POTS dysautonomia seen in hEDS include:

• Orthostatic Intolerance (OI) – symptoms such as dizziness or fatigue upon standing, but without the significant heart rate increase seen in POTS.

• Orthostatic Hypotension (OH) – a drop in blood pressure when standing, leading to lightheadedness, weakness, or fainting.

• Neurally Mediated Syncope (Vasovagal Syncope) – fainting due to sudden drops in blood pressure and/or heart rate, often triggered by pain, stress, or standing still for long periods.

• Small Fiber Neuropathy (SFN) – damage to small nerve fibers that affects both sensation (e.g., burning, tingling) and autonomic function (e.g., sweating, temperature control, heart rate regulation).

Prevalence in hEDS

Dysautonomia is very common in people with hEDS.

• 44–65% of hEDS patients are estimated to have some form of dysautonomia.

• Around 20% of hospitalized EDS patients have measurable autonomic dysfunction, including forms like OH, OI, or vasovagal syncope.

• Orthostatic intolerance without POTS is also widespread, affecting up to 31% of hEDS patients in some studies.

This means even if someone doesn’t meet the formal criteria for POTS, they may still experience significant symptoms related to autonomic dysfunction.

Symptoms of Non-POTS Dysautonomia

Symptoms vary depending on the type of dysautonomia, but often overlap and fluctuate. Common symptoms include:

• Dizziness or lightheadedness when standing

• Fainting or near-fainting (presyncope)

• Palpitations (feeling of heart racing or skipping)

• Fatigue and exercise intolerance

• Nausea, bloating, constipation, or diarrhea (GI dysmotility)

• Difficulty urinating or urinary urgency

• Excessive or reduced sweating

• Headaches or brain fog

• Pain, burning, or temperature sensitivity (if small fiber neuropathy is present)

These symptoms often come and go and may worsen with heat, prolonged standing, dehydration, or illness.

Diagnosis

A medical diagnosis may involve

• History and symptom review: Identifying typical patterns (e.g., symptoms that worsen when upright).

• Standing tests or tilt-table testing: To check for changes in blood pressure and heart rate when upright.

• Sweat testing or skin biopsy: To diagnose small fiber neuropathy by measuring sweat response or checking for nerve fiber density.

• Blood and urine tests: To rule out other causes of fatigue, dizziness, or nerve pain.

There are no universal tests for all forms of dysautonomia, so diagnosis often requires a combination of clinical evaluation and exclusion of other conditions.

Treatment and Management

Treatment is usually supportive and symptom-focused, aiming to reduce severity and improve daily functioning.

Non-pharmacologic strategies

• Fluids: Aim for 2–3 liters per day unless restricted for medical reasons.

• Salt: Up to 10–12 grams per day if tolerated (under medical guidance).

• Compression garments: To reduce blood pooling in the legs.

• Graded exercise: Start slowly with low-impact, horizontal or seated activity (e.g., swimming, recumbent bike).

• Environmental adjustments: Avoid overheating, dehydration, and long periods of standing.

Medications (if needed)

• Fludrocortisone: To boost blood volume for orthostatic hypotension.

• Midodrine: To constrict blood vessels and raise blood pressure.

• Beta-blockers: For symptoms like palpitations or mild tachycardia.

• Neuropathic pain medications: For small fiber neuropathy (e.g., gabapentin, duloxetine).

Most medications are used off-label and based on experience rather than large clinical trials. A trial-and-error approach may be necessary, always under medical supervision.

Small Fiber Neuropathy in hEDS

Small fiber neuropathy (SFN) is increasingly recognized in hEDS. It affects small sensory and autonomic nerve fibers and may explain many puzzling symptoms, including:

• Burning pain, numbness, or tingling (especially in hands and feet)

• Cold or heat sensitivity

• Sudomotor dysfunction (sweating too much or too little)

• Fluctuating heart rate or blood pressure

SFN can be diagnosed via skin biopsy, nerve conduction studies, or quantitative sensory testing. Management may include medications for nerve pain, lifestyle changes, and supportive therapies for associated autonomic dysfunction.

Special Considerations for People with hEDS

• Joint instability and pain may limit exercise options. Careful physical therapy and pacing are essential.

• GI symptoms, such as gastroparesis or IBS, may interfere with recommended high fluid and salt intake.

• Medication sensitivities are common, requiring individualized dosing and careful monitoring.

• Mast cell activation syndrome (MCAS), another common comorbidity in hEDS, may worsen autonomic symptoms and affect medication tolerance.

A multidisciplinary care team—including a neurologist, cardiologist, pain specialist, physical therapist, and dietitian—can be invaluable.

Summary

• Non-POTS forms of dysautonomia are common in people with hEDS and can significantly affect quality of life.

• These include orthostatic hypotension, orthostatic intolerance, vasovagal syncope, and small fiber neuropathy.

• Symptoms can involve dizziness, fatigue, GI upset, abnormal sweating, and nerve pain.

• Treatment focuses on fluids, salt, exercise, compression, and medications as needed.

• Special attention must be paid to joint stability, GI health, and comorbid conditions when designing treatment plans.