Ehlers-Danlos Syndromes (EDS) encompass a group of inherited disorders that primarily affect connective tissues throughout the body. Individuals often wonder if this condition, present from birth, will worsen over time. This article explores how the symptoms of EDS can evolve and present differently across various life stages, addressing the common question of whether the condition “gets worse with age.”
Understanding Ehlers-Danlos Syndromes
Ehlers-Danlos Syndromes are 13 distinct genetic conditions impacting connective tissue, which provides support, structure, and elasticity throughout the body. These disorders arise from genetic changes that affect the production or function of collagen and related proteins. Collagen supplies strength and structural integrity to tissues like skin, bones, blood vessels, and organs.
When collagen is weaker or deficient, as in EDS, connective tissues lose their intended strength and support. This can manifest in various ways, including joints that are more flexible than usual, skin that is stretchy and fragile, and tissues that are easily damaged. Each specific EDS subtype has unique characteristics and diagnostic criteria. The most prevalent type, hypermobile EDS (hEDS), accounts for about 90% of cases and is characterized by generalized joint hypermobility, joint instability, and chronic pain.
Age-Related Changes in Symptoms
While Ehlers-Danlos Syndromes are lifelong conditions, their symptoms do not necessarily worsen in a simple linear progression as individuals age. Instead, symptoms can change, intensify, or present differently at various life stages, influenced by factors like accumulated wear and tear on the body.
In childhood and adolescence, joint hypermobility is often a prominent feature, allowing for an unusual range of motion. However, this flexibility can lead to frequent joint dislocations or subluxations, where joints partially come out of place. Children may experience pain in their knees and ankles, sometimes mistaken for growing pains, and may fall more often due to joint instability.
As individuals with EDS transition into adulthood, particularly after their mid-30s, joint hypermobility may paradoxically decrease as connective tissues naturally stiffen. This reduction in visible flexibility might seem beneficial, but it can lead to new challenges such as increased muscle stiffness and the development of chronic pain. The cumulative effect of years of joint instability and microtrauma can contribute to early-onset osteoarthritis.
Chronic pain often becomes more widespread and persistent with age for many individuals with hEDS. This pain can stem from joint instability, muscle tension as the body attempts to stabilize loose joints, and nerve entrapment. Fatigue, a common companion to chronic pain, also tends to intensify over time.
Autonomic dysfunction, which affects involuntary bodily functions, can become more pronounced or manifest differently. Symptoms such as postural orthostatic tachycardia syndrome (POTS), characterized by dizziness, lightheadedness, and a rapid heart rate upon standing, may emerge or worsen. Gastrointestinal issues, including irritable bowel syndrome, bloating, and acid reflux, can also persist or develop. For those with classical EDS, skin fragility and problems with wound healing tend to worsen, with many experiencing chronic wounds and atrophic scarring by middle age.
Factors Affecting Symptom Evolution
The way Ehlers-Danlos Syndrome symptoms evolve in an individual is influenced by a complex interplay of factors beyond the genetic predisposition. While the underlying genetic changes are present from birth, lifestyle choices, the presence of other health conditions, and how actively symptoms are managed can significantly modulate the condition’s trajectory.
Physical activity levels play a role in symptom progression. Regular, low-impact exercise, such as swimming or cycling, helps strengthen the muscles surrounding joints, providing better support and potentially reducing the frequency of dislocations. Conversely, a sedentary lifestyle can lead to muscle deconditioning, which may exacerbate joint instability and pain. Avoiding high-impact activities and contact sports is recommended to prevent injuries that could further damage fragile connective tissues.
Co-occurring conditions frequently impact the overall symptom burden. For instance, the presence of mast cell activation syndrome (MCAS) or gastrointestinal disorders can contribute to increased fatigue and pain, influencing how an individual experiences their EDS. Hormonal fluctuations, such as those occurring during puberty, pregnancy, or menopause, are also reported to affect symptom severity for some individuals.
Effective medical interventions and self-management strategies can significantly alter the long-term course of EDS symptoms. Proactive measures like physical therapy, pain management, and addressing related health issues can help mitigate the impact of the condition. Consistent engagement in these strategies can help maintain function, reduce pain, and prevent some of the wear-and-tear complications.
Adapting to Lifelong EDS
Living with Ehlers-Danlos Syndromes involves ongoing adaptation and management as symptoms change over time. A multidisciplinary approach to care is beneficial, bringing together various medical specialists to address the wide range of symptoms that can affect multiple body systems. This collaborative care team may include physical therapists, occupational therapists, pain management specialists, and other targeted specialists.
Physical therapy strengthens muscles to support hypermobile joints and improve stability. Occupational therapy helps individuals adapt daily tasks and environments to reduce physical strain and conserve energy. Pain management strategies, which can range from over-the-counter medications to specialized interventions, are also tailored to individual needs to improve comfort and function.
Self-management is a powerful tool for individuals with EDS, empowering them to take an active role in their health. This includes practices like pacing activities to avoid overexertion and manage fatigue, maintaining a balanced diet, and ensuring adequate hydration. Developing good sleep hygiene and stress reduction techniques, such as mindfulness, can also contribute positively to overall well-being. Proactive measures, such as using supportive footwear or adaptive tools, can further help protect fragile joints and tissues.