By Heather Kelly, MSN, RN, BCPA, CPC
Ehlers-Danlos syndromes (EDS) represent a group of hereditary connective tissue disorders characterized by abnormalities in collagen, the primary structural protein that supports joints, skin, vasculature, and internal organs. These disorders present along a spectrum, with hallmark features including joint hypermobility, skin hyperextensibility, and tissue fragility
Hypermobile Ehlers-Danlos syndrome (hEDS) is the most common subtype and is closely related to hypermobility spectrum disorders (HSD), conditions in which patients experience symptomatic joint hypermobility without meeting full diagnostic criteria for hEDS.
Unlike many other EDS subtypes, hEDS does not currently have a confirmed genetic marker, making diagnosis reliant on clinical evaluation and history
Rising Awareness and Increasing Diagnoses
Historically, EDS was considered rare, with prevalence estimates around 1 in 5,000 individuals, though emerging evidence suggests this may be an underestimate.
More recent studies indicate that hEDS and hypermobility-related conditions may be as common as 1 in 500 individuals in some populations, reflecting both improved recognition and evolving diagnostic criteria.
Several factors are contributing to this upward trend:
- Improved clinical awareness and education
- Updated 2017 international diagnostic criteria
- Better differentiation between hEDS and HSD
- Increased patient advocacy and awareness campaigns
Impact on Younger Populations
Symptoms often begin in childhood or adolescence, though recognition may lag for years.
Emerging research highlights that adolescents with hEDS/HSD frequently experience:
- Chronic pain and fatigue
- Reduced physical activity levels
- Functional limitations affecting daily life
For case managers, this underscores a critical shift: earlier identification opportunities exist but so do risks for prolonged diagnostic delays.
Team Approach
Patients often experience years-long delays, as symptoms are misattributed to more common conditions.
Multisystem Involvement
Patients may engage with:
- Primary care
- Rheumatology
- Gastroenterology
- Cardiology
- Behavioral health
Patients with Ehlers-Danlos syndrome are often high utilizers of healthcare services due to the complexity of their condition. Chronic pain, persistent fatigue, and a wide range of multisystem comorbidities contribute to frequent healthcare encounters and require ongoing, coordinated management across multiple specialties. Without coordination, care becomes fragmented.
Mental Health and EDS
Nearly half of patients diagnosed with Ehlers-Danlos syndrome (EDS) or hypermobility spectrum disorders (HSD) experience co-occurring psychiatric conditions, highlighting the significant mental health burden associated with these disorders. A clinical study found that approximately 49% of patients had at least one psychiatric disorder, with depression being most common, followed by other conditions such as anxiety and somatoform disorders; notably, many individuals experienced multiple mental health diagnoses. These findings underscore the strong connection between chronic pain, gastrointestinal issues, and psychological distress in this population. For case managers, this reinforces the importance of a holistic, biopsychosocial approach to care, including routine mental health screening, coordination with behavioral health providers, and ongoing support to address the emotional impact of living with a complex, chronic condition.
Proactive case management can play a critical role in improving quality of life by ensuring mental health needs are recognized, validated, and integrated into the overall care plan.
Actionable Strategies for Case Managers
To improve outcomes, case managers can:
1. Identify Patterns Early
- Recurrent joint injuries without trauma
- Chronic pain in younger populations
- Multi-specialty involvement without clear diagnosis
2. Support Appropriate Referral Pathways
- Rheumatology or genetics evaluation
- Physical/occupational therapy
- Pain management and behavioral health support
3. Facilitate Multidisciplinary Care
Evidence supports coordinated care models including:
- Physical therapy for joint stabilization
- Patient education and self-management
- Psychological support for chronic illness burden
4. Advocate for the Patient
- Validate symptoms, especially in younger populations
- Address stigma and misdiagnosis
- Ensure continuity across care settings
The Case Manager’s Impact in EDS Care
Ehlers-Danlos syndrome and hypermobility spectrum disorders are no longer niche conditions, they are increasingly recognized, frequently underdiagnosed, and clinically complex.
For case managers, the opportunity is clear:
- Recognize early warning signs
- Understand diagnostic pathways
- Coordinate multidisciplinary care
By doing so, case managers can significantly reduce diagnostic delays, improve patient outcomes, and deliver more holistic, person-centered care, especially for younger populations navigating these lifelong conditions.
References:
Cleveland Clinic. (2025). Ehlers-Danlos syndrome (EDS): Symptoms, treatment & tests.
Demmler, J. C., et al. (2019). Prevalence of Ehlers-Danlos syndromes and hypermobility spectrum disorders.
Ehlers-Danlos Society. (2017). Diagnostic criteria for hypermobile Ehlers-Danlos syndrome (hEDS).
Ehlers-Danlos Society. (n.d.). Assessing joint hypermobility: The Beighton scoring system.
Ehlers-Danlos Society. (n.d.). Hypermobile Ehlers-Danlos syndrome.
Inácio, P. (2019, April 10). Almost half of EDS/HSD patients have psychiatric disorders, study finds. Ehlers-Danlos News. https://ehlersdanlosnews.com/news/almost-half-of-eds-hsd-patients-have-psychiatric-disorders/
Mayo Clinic. (2026). Ehlers-Danlos syndrome: Symptoms and causes.
Rheumatology Advisor. (2025). Challenges and progress in diagnosing Ehlers-Danlos syndrome.
Smith, C. (2021). Diagnosis and management of hypermobility disorders.
Yew, K. S., Kamps-Schmitt, K. A., & Borge, R. (2021). Hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders. American Family Physician, 103(8), 481–492
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Bio: Bio: Heather Kelly, MSN, RN, BCPA, CPC, is a registered nurse with more than 30 years of healthcare experience, including oncology, hospice and palliative care, and healthcare administration. She brings a strong background in care coordination, medical policy, and clinical program strategy, with a focus on improving access, quality, and patient‑centered outcomes. Heather currently serves as a Senior Clinical Strategist, collaborating with multidisciplinary teams to support effective care management and program development. She is a frequent community speaker on cancer survivorship and end‑of‑life planning and is deeply committed to patient advocacy and professional mentorship.