Ehlers-Danlos Syndrome (EDS) is a group of inherited disorders affecting connective tissue throughout the body. Connective tissue provides the structure and support for skin, bones, blood vessels, and organs, and defects in this tissue often lead to chronic pain. Back pain is a very frequent complaint among people with EDS, often beginning at a young age and persisting throughout life.
Understanding Ehlers-Danlos Syndrome
Ehlers-Danlos Syndromes are genetic conditions that affect the body’s connective tissue. The integrity of this tissue depends on the protein collagen, which provides both strength and elasticity. In EDS, a genetic mutation impacts the production or processing of collagen, resulting in tissue that is weaker, more flexible, and more fragile than usual.
The current classification includes 13 different types of EDS, each resulting from variants in specific genes. Hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common form, characterized primarily by joint hypermobility and chronic pain. The condition is associated with systemic issues related to overly flexible and unstable joints. The musculoskeletal system, particularly the spine, is a primary source of chronic discomfort.
The Mechanics of EDS-Related Back Pain
The most direct cause of back pain in EDS stems from the widespread laxity of ligaments, which hold bones and joints together. Spinal ligaments are meant to be taut to keep the vertebrae aligned, but in EDS, they are often too loose, allowing for excessive movement. This ligament laxity leads to spinal instability, where the vertebrae shift beyond their normal range during routine activities.
This excess movement, often called segmental instability, causes small, repetitive traumas and strains to the surrounding spinal structures. Over time, this micro-trauma leads to secondary conditions that cause significant pain. For instance, the constant microscopic shearing from unstable vertebrae can accelerate the deterioration of the spinal discs, resulting in early-onset degenerative disc disease.
Muscles surrounding the spine attempt to compensate for the slack ligaments, working overtime to stabilize joints. This continuous overcompensation leads to chronic muscle fatigue, tension, and pain. Studies have shown that paraspinal muscles in people with hEDS must fire for longer periods during simple movements, resulting in fatigue-related soreness that contributes to the persistent ache.
The instability can also directly affect other bony structures of the spine, such as the facet joints, which link the vertebrae and guide spinal movement. Hypermobility causes premature wear and tear on these joints, leading to painful facet joint arthropathy. Furthermore, the inherent tissue fragility can predispose individuals to spondylolisthesis, the slippage of one vertebra over another. Spinal instability can also lead to nerve root irritation or compression as the vertebrae shift, causing neuropathic pain that radiates into the limbs.
Managing Spinal Symptoms in EDS
Managing back pain in EDS requires a specialized approach that focuses on promoting stability rather than flexibility. Traditional physical therapy emphasizing stretching is often detrimental, as it further loosens already lax ligaments. Instead, the focus must be on low-impact strengthening of the core and postural muscles to create a “muscular corset” around the spine.
Targeted strengthening exercises, such as those that improve deep core strength and muscle endurance, help the muscles take over the stabilization role lost by the compromised ligaments. A knowledgeable physical therapist can guide patients through specific exercises to build this support system without causing joint injury. Non-steroidal anti-inflammatory drugs (NSAIDs) or muscle relaxers may be used to manage acute flare-ups of pain and muscle spasms.
Due to the chronic nature of the pain, long-term management often requires a multidisciplinary approach involving pain physicians and psychologists. Lifestyle adjustments are also important, including the use of ergonomic supports or temporary bracing during flare-ups to reduce stress on unstable joints. Patients are advised to avoid high-impact sports and activities that involve extreme joint motion to minimize the risk of injury and further instability.