Emergency Preparedness in hEDS
Emergency preparedness is a vital but often overlooked aspect of managing hypermobile Ehlers-Danlos syndrome (hEDS), a multisystemic connective tissue disorder affecting people of all ages. Because of the wide range of possible acute complications and comorbidities, individuals with hEDS are more likely to visit the emergency department (ED), require hospitalization, and experience longer hospital stays than the general population. Emergency preparedness involves not only having a plan but understanding the increased prevalence of emergencies, the common causes, and the systemic challenges that affect how hEDS patients are treated. Both patients and clinicians must be equipped to anticipate, communicate, and manage acute medical situations related to hEDS.
Prevalence and Risk of Emergencies in hEDS
People with hEDS have significantly higher rates of ED visits and hospitalizations than those without hEDS.
For example, people with hEDS are more than four times as likely to experience certain types of cardiovascular or nervous system emergencies as people without hEDS.
Common emergency causes include joint dislocations, dysautonomia (especially Postural Orthostatic Tachycardia Syndrome or POTS), gastrointestinal crises, mast cell activation reactions, and acute pain episodes.
Studies also show that people with hEDS are 76% more likely to stay longer in the hospital once admitted.
Healthcare costs are 2–3 times higher in hEDS populations due to emergency visits and hospitalizations.
Problems and Challenges in Emergency Care
Lack of provider awareness of hEDS can lead to misdiagnosis, undertreatment, or dismissal of symptoms.
Patients frequently report stigma, disbelief, or frustration in the ED.
Standard emergency protocols often fail to consider the multisystem nature of hEDS.
Symptoms may mimic psychiatric or functional conditions, leading to inappropriate referrals.
Anesthesia risks, autonomic instability, and medication sensitivities are often overlooked.
Most Common Emergency Scenarios in hEDS
Musculoskeletal injuries: Joint dislocations and subluxations, often with soft tissue damage.
Dysautonomia: Sudden dizziness, syncope, palpitations, and presyncope, especially with heat, standing, or dehydration.
Gastrointestinal emergencies: Severe constipation, vomiting, abdominal pain, and pseudo-obstruction.
Mast cell activation (MCAS): Urticaria, flushing, hypotension, or even anaphylactoid reactions.
Pain flares: Sudden and severe pain episodes that may mimic acute injury or systemic illness.
Cardiovascular complications: Less common but include mitral valve prolapse and aortic root dilatation.
Age-Specific Considerations
Age Group | Common Acute Issues | Special Considerations |
Pediatric | Dislocations, MCAS, dysautonomia, bruising | May be misinterpreted as behavioral or anxiety-related; caregivers must advocate for child safety and documentation. |
Adults | Dysautonomia, GI crises, pain, surgical complications | Require multi-specialty care coordination and awareness of anesthesia and medication risks. |
Older Adults | Falls, fractures, chronic pain, polypharmacy, cardiac risks | Higher risk of adverse outcomes and hospital-acquired complications; monitoring for deconditioning and medication interactions is key. |
Emergency Documentation: What Should Be Included
Diagnoses (hEDS, POTS, MCAS, etc.)
Medications with dosages and allergies
Baseline functional status
History of anesthesia or medication complications
Emergency action plans for common events (e.g., joint dislocations, autonomic crises, allergic reactions)
Contact information for relevant specialists
Past imaging or test results (especially cardiovascular and GI)
These should be kept in
A portable emergency folder
Emergency kits
Shared digital access (e.g., patient portal, USB drive, QR code ID card)
The electronic health record (EHR) wherever possible
Personal Emergency Kits
A well-prepared emergency kit may include
Emergency documentation (above)
Assistive devices (braces, compression garments)
Ice/heat packs
Electrolyte packets
Antihistamines or epinephrine auto-injector if allergic risk is present
Pain relief methods (topicals, oral meds, distraction tools)
Identification with diagnosis and emergency contact info
What Doctors Should Know
Patients with hEDS may present with atypical symptoms or multisystem complaints.
Common acute issues include dysautonomia, GI dysfunction, joint instability, mast cell reactivity, and heightened pain sensitivity.
Anesthesia may be less effective or cause unexpected reactions.
Small fiber neuropathy and central sensitization can complicate pain perception.
Longer-than-usual appointments and coordination across specialties are often necessary.
What People with hEDS Should Know
Carry up-to-date documentation.
Inform care teams (including EMS) of hEDS-specific risks.
Tell Emergency Medical Technician's (EMTs) about
Any history of allergic reactions or MCAS
Risk of fainting, dizziness, or blood pressure drops due to dysautonomia
Any joint instability or prior dislocations that may affect lifting, positioning, or spinal precautions
Difficulty with anesthesia, pain management, or medication reactions
Use a medical ID card or bracelet listing (both can be purchased online)
Diagnoses (e.g., "Hypermobile EDS, POTS, MCAS")
Emergency contacts
Preferred hospital or physician
Carry a wallet card from the Ehlers-Danlos Society
Set up the "Medical ID" function on smartphones with key information accessible from the lock screen
Emergency and Hospital Care Tips
Joint dislocations may need gentle, informed reduction techniques.
Avoid unnecessary imaging unless neurovascular compromise is suspected.
Monitor carefully for autonomic instability (especially with IV fluids or positioning).
Be aware of allergy-like reactions from MCAS, even without known allergens.
Use compression garments and ensure adequate hydration before procedures.
Anesthesia and Surgery Notes
Consider history of anesthesia resistance or prolonged recovery.
Use cautious positioning to avoid subluxations.
Avoid overstretching limbs or neck.
Use non-triggering medications for known MCAS patients.
Monitor closely for delayed recovery or pain flares.
Summary
Emergency preparedness for people with hypermobile Ehlers-Danlos syndrome must be proactive, individualized, and multidisciplinary. Given the heightened risk for acute complications and poor outcomes in emergency settings, advance planning—through emergency kits, documentation, communication, and care coordination—is essential. Both clinicians and patients benefit from increased awareness, standardized strategies, and validated tools to manage common scenarios such as joint injuries, dysautonomia, gastrointestinal crises, allergic reactions, and anesthesia complications. Addressing these challenges head-on can improve safety, outcomes, and confidence across the lifespan. Emergency preparedness is not only about readiness but about overcoming systemic barriers, improving recognition, and ensuring that everyone with hEDS has the support they need in a crisis.
