An Introduction to Ehlers-Danlos Syndromes (EDS)
Ehlers-Danlos Syndromes (EDS) are a group of genetic connective tissue disorders caused by mutations that affect the structure, production, or processing of collagen and related proteins. Connective tissue plays a vital role in supporting, binding, and protecting tissues and organs throughout the body. As a result, individuals with EDS often experience a wide range of symptoms affecting multiple systems.
What is Connective Tissue?
Connective tissue acts like the body’s “mortar,” holding organs and tissues in place while allowing flexibility and strength. Collagen—a key component of connective tissue—is found throughout the entire body. When defective, it can lead to issues such as fragile skin, joint hypermobility, and a wide spectrum of internal symptoms.
Understanding EDS
EDS is characterized by abnormally fragile and hyperextensible tissues, leading to a variety of symptoms that can include:
- Joint hypermobility and dislocations
- Chronic pain and fatigue
- Skin that is unusually stretchy, soft, or bruises easily
- Digestive problems and gastrointestinal dysfunction
- Cardiovascular and autonomic dysfunction (e.g., PoTS, low blood pressure)
- Mast cell activation and allergic-like responses
- Musculoskeletal complications
- Neurological, bladder, and gynecological issues
The severity and range of symptoms vary greatly—even among individuals within the same family. Some forms of EDS can cause significant physical disability, and certain rare types may be life-limiting.
The 13 Types of EDS
There are currently 13 recognized subtypes of EDS. Twelve are considered rare and can be identified through genetic testing. The most common form, Hypermobile EDS (hEDS), does not yet have a known genetic marker.
The 13 EDS subtypes:
- Arthrochalasia EDS (aEDS)
- Brittle Cornea Syndrome (BCS)
- Cardiac-Valvular EDS (cvEDS)
- Classical EDS (cEDS)
- Classical-like EDS (clEDS)
- Dermatosparaxis EDS (dEDS)
- Hypermobile EDS (hEDS)
- Kyphoscoliotic EDS (kEDS)
- Musculocontractural EDS (mcEDS)
- Myopathic EDS (mEDS)
- Periodontal EDS (pEDS)
- Spondylodysplastic EDS (spEDS)
- Vascular EDS (vEDS)
Hypermobile EDS (hEDS) and Hypermobility Spectrum Disorders (HSD)
hEDS is the most common subtype of EDS and often involves widespread joint hypermobility, pain, and a host of associated conditions. While hEDS can be inherited, it may also result from a spontaneous genetic mutation. Diagnosis is clinical and based on a combination of:
- Generalized joint hypermobility (assessed via the Beighton scale)
- Musculoskeletal complications
- Systemic features of connective tissue disorders
- Family history
Many individuals experience hEDS-related symptoms but do not fully meet the diagnostic criteria. These individuals may be diagnosed with Hypermobility Spectrum Disorders (HSD), which present similarly and are managed with the same approach.
Hypermobility Spectrum
Joint hypermobility exists on a continuum:
- Simple hypermobility – A benign trait with no symptoms
- HSD – Symptomatic hypermobility not meeting hEDS criteria
- hEDS – Severe, systemic hypermobility disorder
Management strategies for both hEDS and HSD focus on symptom control, physical therapy, lifestyle modifications, and multidisciplinary care.
Support and Resources
EDS UK GP Toolkit
Developed to guide General Practitioners (GPs) in diagnosing and managing EDS and HSD in primary care.
🔗 GP Toolkit
EDS UK School Toolkit
An online resource to help schools understand and support pupils with symptomatic hypermobility and EDS.
🔗 School Toolkit
EDS/HSD Northern Ireland Support
Providing local support through:
- Face-to-face and online meetings
- Private Facebook group
- Awareness events and educational resources
- Guidance on accessing diagnoses, treatments, and benefits
📧 General Enquiries: nisupport@volunteer.ehlers-danlos.org
📧 Engagement: northernireland@volunteer.ehlers-danlos.org
📞 Advice Line: 0800 907 8518
🔗 Facebook Group
Research & Expert Insights
Below are selected research articles, reports, and expert contributions that shed light on the complexities of Ehlers-Danlos Syndromes (EDS), Hypermobility Spectrum Disorders (HSD), and their related conditions. These resources are vital for deepening understanding, improving clinical care, and shaping future research priorities.
Healthwatch Calderdale – Hypermobility Syndromes Report
This comprehensive report explores the lived experiences of individuals in West Yorkshire with symptomatic hypermobility, including those with EDS and HSD. Key findings include:
- Prolonged diagnostic journeys, with many patients waiting years for recognition and support
- Poor understanding of EDS/HSD among GPs and other healthcare providers
- Lack of access to appropriate referrals and services (e.g., rheumatology, physiotherapy, pain management)
- The emotional and psychological toll of feeling dismissed or disbelieved by medical professionals
Why it matters: This report provides compelling evidence of systemic gaps in care and supports calls for better education and care pathways within the NHS and beyond.
British Journal of Midwifery (BJM) – Clinical Update on EDS and Pregnancy
This peer-reviewed article provides clinical guidance for midwives and healthcare providers on managing pregnancies affected by EDS, particularly hypermobile EDS (hEDS). It covers:
- Risks associated with pregnancy, including joint instability, premature rupture of membranes, and autonomic dysfunction
- Recommendations for labor and delivery, including anesthesia considerations and delivery mode
- Postpartum care strategies and implications for future pregnancies
Why it matters: Many women with EDS report negative experiences during pregnancy and childbirth due to a lack of clinical understanding. This guide helps bridge that gap with evidence-based recommendations.
Dr. Jessica Eccles – Research on Neurodiversity and Hypermobility
🔗 Follow Dr. Eccles on X (Twitter)
Dr. Jessica Eccles is a leading UK researcher exploring the neurological and psychological dimensions of hypermobility, including its link to:
- Neurodivergent conditions such as Autism Spectrum Disorder (ASD) and Attention Deficit Hyperactivity Disorder (ADHD)
- Anxiety and mood disorders, which are more prevalent in hypermobile individuals
- Interoception, or the brain’s ability to sense internal bodily states, which may be altered in hypermobility syndromes
Her work highlights the mind-body connection in EDS and HSD and promotes a holistic approach to diagnosis and care that encompasses physical, neurological, and psychological aspects.
Why it matters: This research offers vital insights into why people with hypermobility often report sensory sensitivity, anxiety, or difficulty with emotional regulation—and why treatment needs to reflect that complexity.
hEDS-START Report – University of Edinburgh
The hEDS-START (Supporting Access to Relevant Treatment) report presents findings from a national qualitative study of individuals living with hEDS and HSD in the UK. Key findings include:
- Average time to diagnosis: 20 years
- Many participants experienced medical gaslighting or dismissal
- 77% use mobility aids, often without clinical guidance or NHS provision
- Significant impact on education, employment, and mental health
- Higher A&E attendance rates and associated costs for the NHS
Why it matters: This landmark study provides a strong evidence base for systemic changes in how hypermobility disorders are diagnosed, supported, and funded in the UK healthcare system.
