Ehlers-Danlos Syndromes (EDS) are a group of inherited connective tissue disorders resulting from faulty collagen production. Because collagen is present throughout the body, the effects of EDS are systemic and evolve over time, presenting new and worsening challenges with age for many individuals. While some people report stable or even slightly improved symptoms as they get older, the majority experience a progressive increase in symptoms, especially chronic pain and fatigue. Proper diagnosis and early intervention can be key to slowing this progression and improving quality of life.
Aging with Hypermobile Ehlers-Danlos Syndrome (hEDS)
hEDS is the most common subtype, and its effect on aging is distinct. A common misconception is that joint symptoms improve with age as connective tissues naturally stiffen. While some joint laxity may decrease after the third decade of life, this often leads to increased joint stiffness and a new set of problems.
Musculoskeletal Changes
- Joint Instability and Osteoarthritis: The cumulative wear and tear from years of joint instability, subluxations, and dislocations often leads to early-onset osteoarthritis.
- Chronic Pain and Fatigue: For up to 90% of adults with hEDS, musculoskeletal pain worsens with age, often becoming more chronic and widespread. Fatigue is a near-universal experience, affecting up to 95% of patients over 40 and often proving more debilitating than the pain.
- Muscle Weakness: The body's attempt to stabilize hypermobile joints with muscle tension can lead to widespread weakness and pain. Physical therapy focused on low-impact strengthening is crucial to prevent this decline.
Autonomic and Other Systemic Issues
- Dysautonomia (POTS): Autonomic nervous system dysfunction, like Postural Orthostatic Tachycardia Syndrome (POTS), frequently worsens with age. This can lead to increased fatigue, blood pressure instability, and temperature regulation problems in patients in their 40s and 50s.
- Gastrointestinal Problems: Slowed gastric motility, chronic constipation, and gastroparesis can become more prominent during midlife.
- Pelvic Organ Prolapse: Postmenopausal women with hEDS face a higher risk of pelvic organ prolapse due to weakened connective tissue and hormonal changes.
Aging with Vascular and Classical EDS
Other subtypes, while rarer, present specific age-related challenges. Vascular EDS (vEDS) is particularly concerning for its progressive risks, while Classical EDS (cEDS) involves distinct skin and joint issues that change over time.
Vascular Ehlers-Danlos Syndrome (vEDS)
- Vascular Fragility: The risk of life-threatening arterial dissections, aneurysms, and organ rupture increases significantly with age. Studies show a major vascular event occurs in nearly 80% of individuals with vEDS by age 40, and the median life expectancy is around 48 years.
- Distinct Facial Features: Many with vEDS have a distinct facial appearance that may seem prematurely aged due to thin, translucent skin, prominent veins, sunken cheeks, and thin lips.
Classical Ehlers-Danlos Syndrome (cEDS)
- Skin Fragility: For cEDS patients, skin fragility and poor wound healing tend to worsen over time, leading to more chronic wounds and atrophic scarring by age 50.
- Persistent Joint Instability: While ligaments may stiffen, joint instability often persists. Secondary complications such as cervical spine instability and degenerative disc disease are common with age.
How EDS Subtypes Affect Aging: A Comparison
Feature | Hypermobile EDS (hEDS) | Vascular EDS (vEDS) | Classical EDS (cEDS) |
---|---|---|---|
Genetic Basis | Unknown genetic cause | Caused by COL3A1 mutations | Caused by COL5A1/COL5A2 mutations |
Life Expectancy | Normal life expectancy | Reduced life expectancy, with median age of death around 48 | Normal life expectancy |
Primary Age-Related Concern | Chronic pain, early osteoarthritis, and systemic complications | Increased risk of arterial and organ rupture | Worsening skin fragility, chronic wounds, and atrophic scarring |
Joint Mobility | Joint laxity may decrease, but this leads to new problems like stiffness and arthritis | Joint hypermobility is typically less prominent than in hEDS | Joint instability persists, though some report reduced dislocations as joints stiffen |
Skin Appearance | Soft, stretchy skin that may appear youthful, but can also be thin and fragile | Thin, translucent skin with visible veins; may have a prematurely aged look | Velvety skin with poor wound healing and characteristic 'cigarette-paper' scarring |
Cardiovascular Risk | Increased risk of autonomic issues like POTS; some risk of mitral valve prolapse | Very high risk of aortic and arterial dissections or aneurysms | Some risk of cardiovascular issues, such as mitral valve prolapse |
Conclusion: The Individual Journey of Aging with EDS
EDS fundamentally alters the aging process, but the journey is highly individual and depends on the specific subtype, genetics, and proactive management. While conditions like hEDS may lead to years of escalating chronic pain and early osteoarthritis, vEDS carries a higher risk of life-threatening vascular events with age. Effective long-term management involves a multidisciplinary approach, including physical therapy, pain management, and specialized monitoring for systemic complications. By understanding how EDS affects the body over time, individuals can work with healthcare providers to develop strategies that manage symptoms, reduce complications, and ultimately improve their quality of life.
One of the biggest challenges for aging EDS patients is navigating the medical system and finding specialists who are knowledgeable about the condition and its progression. Resources like The Ehlers-Danlos Society and The Hypermobility Syndromes Association (HMSA) can provide support and educational materials for both patients and clinicians. With a proactive and informed approach, individuals with EDS can better navigate the unique challenges that come with aging.