Ehlers-Danlos Syndromes (EDS) are a group of genetic disorders that primarily affect connective tissue throughout the body. These conditions arise from mutations in genes responsible for producing collagen and related proteins, which form the body’s fundamental structural framework. While the underlying genetic defect does not typically intensify over time, the effect of living with flawed connective tissue leads to a functional progression of symptoms for many individuals, especially those with the most common type, hypermobile EDS (hEDS). The cumulative physical impact often results in increasing disability and symptom severity, even though EDS is not a traditional degenerative disorder.
The Mechanism of Cumulative Connective Tissue Damage
The core issue in EDS is the structural abnormality of collagen, the protein that provides strength and elasticity to skin, joints, blood vessels, and organs. This faulty construction means that connective tissues possess an inherent weakness from birth, making them less resilient to the mechanical stresses of daily life. The body is constantly subjected to microtrauma—small, repetitive injuries that occur simply through movement and load-bearing activities. Over decades, this continuous wear and tear on already compromised ligaments and tendons accumulates, leading to progressive tissue degradation.
Normal aging processes, such as the natural decline in muscle mass and reduced tissue elasticity, further amplify the effects of the underlying genetic disorder. In a person with EDS, the supportive structures are already lax. When compensatory muscle strength naturally diminishes with age, the instability worsens dramatically. This process creates a cycle where weak tissue leads to injury, which is then poorly repaired due to the collagen defect, resulting in progressively weaker structures over a lifetime.
Progression of Joint Instability and Chronic Pain
The musculoskeletal system is where the age-related impact of EDS is often most noticeable, with the profile of joint problems changing over time. In childhood and early adulthood, the main characteristic is often generalized hypermobility, where joints easily move beyond the normal range of motion. By middle age and beyond, many individuals report that their joints feel less “loose” but suffer from greater pain and stiffness. This transition occurs because years of repeated partial or full joint dislocations, known as subluxations and luxations, cause permanent structural damage.
The constant abnormal movement within the joint capsule erodes the cartilage, leading to early-onset degenerative joint disease or secondary osteoarthritis. This joint deterioration begins much earlier than in the general population, becoming a major source of chronic, widespread pain. Furthermore, as hypermobility decreases, patients frequently develop chronic muscle tension. This continuous muscular effort attempts to stabilize joints that the ligaments cannot properly support, contributing significantly to a more persistent pain profile.
Increased Severity of Systemic Manifestations
Beyond the joints, the systemic nature of EDS means that non-musculoskeletal issues can also become more pronounced with age due to poor connective tissue support. Autonomic dysfunction, particularly Postural Orthostatic Tachycardia Syndrome (POTS), often manifests as worsening symptoms of light-headedness, palpitations, and chronic fatigue. The fragile vascular tissue may lead to less effective blood pressure regulation, as the vessels struggle to constrict properly, exacerbating symptoms like near-syncope.
Gastrointestinal issues also tend to increase in severity as the smooth muscle and connective tissue within the digestive tract lose tone. Many patients experience slower gastric motility, leading to complications like chronic constipation, delayed gastric emptying, and gastroparesis. Structural complications, such as the increased risk of pelvic organ prolapse, are also a consequence of lifetime stress on lax supportive tissues. For individuals with Vascular EDS (vEDS), age increases the cumulative lifetime risk of life-threatening arterial dissections or organ ruptures due to the inherent fragility of blood vessel walls.
Long-Term Management and Lifestyle Adaptations
Proactive management is instrumental in mitigating the age-related decline and functional progression of EDS symptoms. The primary focus of physical therapy shifts from general flexibility to rigorous joint stabilization and strengthening the surrounding musculature. Tailored exercise programs concentrate on low-impact activities, such as swimming or using a recumbent bicycle, to build muscle tone without risking further joint trauma. Strengthening the muscles acts as an external brace, compensating for the laxity and weakness of ligaments and tendons.
Effective long-term care requires a multidisciplinary approach, involving specialists in pain management, physical medicine, and cardiology, as symptoms become more complex. Pain management protocols often avoid long-term use of traditional anti-inflammatory medications due to potential gastrointestinal side effects, favoring nerve-modulating drugs or specialized therapies. Lifestyle adjustments, including maintaining excellent hydration, optimizing sleep hygiene, and using supportive devices like braces or compression garments, are adopted to manage the compounding effects of chronic pain and fatigue. By adopting these strategic adaptations, individuals can significantly influence their quality of life as they age with the condition.