What are the three stages of EDS? A guide to the hypermobility progression

According to the Ehlers-Danlos Society, there are several types of Ehlers-Danlos Syndromes, but hypermobile Ehlers-Danlos Syndrome (hEDS) is the most common form. For individuals with hEDS, the condition often progresses through three general stages: the hypermobility phase, the pain phase, and the stiffness phase, which we will explore in detail.

Quick Summary

The progression of hypermobile Ehlers-Danlos Syndrome often involves three key phases: an initial hypermobility phase in childhood, followed by a pain phase in adulthood, and a final stiffness phase later in life.

Key Points

• Hypermobility Phase: Often occurs in childhood, marked by unusual joint flexibility and mild symptoms that are sometimes mistaken for 'party tricks' or normal flexibility.

• Pain Phase: Typically starts in young adulthood, bringing chronic, widespread pain, frequent joint subluxations and dislocations, and fatigue, making an accurate diagnosis more critical.

• Stiffness Phase: Develops later in life, characterized by reduced joint hypermobility due to degenerative changes and osteoarthritis, which can alter the nature of chronic pain.

• Not a Cure: EDS is incurable, but understanding the phase progression allows for proactive and tailored management strategies at each stage.

• Multidisciplinary Care: Effective management across all stages requires a comprehensive approach involving various healthcare professionals to address the broad range of symptoms.

• Diagnosis is Key: Getting an accurate diagnosis, especially during the pain phase, is essential for implementing appropriate treatment and support.

Understanding the Ehlers-Danlos Syndromes

Ehlers-Danlos Syndromes (EDS) are a group of inherited connective tissue disorders that are caused by defects in collagen production or structure. Connective tissue is what gives structure and support to the body's skin, bones, blood vessels, and other organs. The faulty connective tissue in people with EDS can lead to a wide range of symptoms, with joint hypermobility and skin fragility being among the most common. While there are numerous types of EDS, the hypermobile type (hEDS) is the most prevalent and is often characterized by a distinct progression through different life stages.

The Hypermobility Phase: The 'Party Trick' Years

The first stage of hypermobile EDS is often referred to as the hypermobility phase. This phase typically occurs during childhood and adolescence. During this time, the symptoms might be seen as unique talents or 'party tricks' rather than signs of a medical condition. Children may be able to perform impressive feats of flexibility, such as bending their fingers and toes into unusual positions, putting their feet behind their head, or easily walking on their elbows. The joints are often described as being unusually flexible or 'double-jointed.' While this hypermobility might seem harmless, it can be a warning sign for potential future issues. In some cases, children may also experience mild growing pains or an increased number of bruises and minor injuries. This stage can often be overlooked by parents and doctors, and a diagnosis may not be considered yet.

Features of the hypermobility phase

• Extreme Joint Flexibility: Individuals can easily hyperextend joints.
• Apparent 'Talent': Flexibility is often perceived positively, masking the underlying issue.
• Minor Symptoms: Minor joint instability or 'growing pains' may be present but are not typically severe.
• Lack of Diagnosis: EDS is often not suspected during this phase.

The Pain Phase: The Shift to Chronic Symptoms

The second stage is known as the pain phase, which commonly begins in late adolescence or early adulthood. The perceived 'talent' of hypermobility fades as more significant and debilitating symptoms begin to emerge. This phase is characterized by the onset of chronic, widespread pain. Joint instability becomes more pronounced, leading to frequent sprains, subluxations (partial dislocations), and full dislocations. The pain is not limited to the joints but can be widespread, affecting muscles and soft tissues. Fatigue is a common and often debilitating symptom during this stage. The pain is often neuropathic or nociplastic in nature, meaning it is not just caused by tissue damage but also by changes in how the central nervous system processes pain signals. It is during this period that many people begin seeking medical help, though they may face challenges in getting an accurate diagnosis as their symptoms can be mistakenly attributed to other conditions like fibromyalgia or chronic fatigue syndrome.

Manifestations of the pain phase

• Chronic, Widespread Pain: Pain is a dominant and persistent symptom.
• Joint Instability: Sprains and dislocations become more frequent and severe.
• Systemic Involvement: Fatigue, headaches, and gut issues may arise.
• Diagnostic Challenges: Patients often face a long and frustrating diagnostic journey.

The Stiffness Phase: The Later-Life Transformation

The third and final stage is the stiffness phase, which typically occurs later in life, often starting in a person's fifties, sixties, or seventies. As a person with hEDS ages, the joints that were once hypermobile begin to stiffen due to degenerative changes and the development of osteoarthritis. While this can lead to decreased hypermobility and potentially a reduction in dislocations, it introduces a new set of challenges, including joint degeneration and persistent osteoarthritic pain. This does not mean the pain disappears entirely; rather, the nature of the pain may change. For some, the increased joint stiffness is a relief from the constant threat of dislocation, while for others, the new stiffness and arthritis cause a different kind of chronic discomfort. The management of symptoms often shifts towards addressing arthritis and reduced mobility.

Characteristics of the stiffness phase

• Decreased Hypermobility: Joints become less flexible over time.
• Osteoarthritis: Degenerative joint disease is common.
• Reduced Dislocations: The frequency of joint dislocations may decrease.
• New Pain Challenges: Pain management focuses on arthritis and reduced mobility.

Comparing the Three Phases of Hypermobile EDS

Feature	Hypermobility Phase	Pain Phase	Stiffness Phase
Age Range	Childhood/Adolescence	Young Adulthood to Middle Age	Later Adulthood
Primary Feature	Excessive joint flexibility (hypermobility)	Chronic pain and joint instability	Progressive joint stiffness and degeneration
Common Symptoms	Mild joint pain, 'growing pains', bruising, party tricks	Widespread chronic pain, frequent subluxations/dislocations, fatigue, gut issues	Osteoarthritis, decreased mobility, reduced dislocations
Diagnosis Likelihood	Low, often dismissed as normal flexibility	Higher, as debilitating symptoms emerge	Often co-occurs with arthritis diagnosis
Treatment Focus	Gentle strengthening, education	Pain management, physical therapy, symptom control	Arthritis management, joint preservation, mobility aids

The Importance of Early Diagnosis and Management

Early recognition of these phases, particularly the transition from the hypermobility to the pain phase, is crucial for effective management. While EDS is not curable, proactive strategies can significantly improve quality of life. During the hypermobility phase, low-impact exercise and strengthening exercises can help stabilize joints and prevent future injuries. In the pain phase, a multidisciplinary approach involving physical therapy, pain specialists, and other allied health professionals can help manage chronic symptoms. Finally, in the stiffness phase, appropriate arthritis management and maintaining as much mobility as possible are key. Awareness of this typical progression can help patients and clinicians better understand the long-term journey of hypermobile EDS.

For more in-depth information and resources on EDS, consider visiting the official website of The Ehlers-Danlos Society.

Conclusion

While the journey with hypermobile Ehlers-Danlos Syndrome can be complex and challenging, understanding its typical progression through the hypermobility, pain, and stiffness phases can provide clarity and empower individuals to seek the right care at the right time. Each phase presents unique symptoms and requires a tailored approach to management. By staying informed and working with healthcare professionals, those with hEDS can effectively navigate the changes their body undergoes throughout their lives, focusing on symptom management and improving their overall quality of life. This guide serves as a foundational overview, but individuals are encouraged to seek expert medical advice for their specific situation.

Frequently Asked Questions

Does every person with EDS go through these exact three stages?

These three stages—hypermobility, pain, and stiffness—are most commonly associated with hypermobile Ehlers-Danlos Syndrome (hEDS). While this progression is typical for many with hEDS, not everyone will experience it in the same way or with the same severity. Symptoms and progression can vary widely among individuals.

Is EDS curable?

No, there is currently no cure for Ehlers-Danlos Syndromes. It is a genetic, lifelong condition. Management focuses on treating the symptoms, preventing complications, and improving quality of life through a combination of physical therapy, medication, and lifestyle adjustments.

At what age do the pain and stiffness phases typically begin?

The pain phase often begins in young adulthood, typically in the second to fourth decade of life. The stiffness phase usually occurs later, during the fifth, sixth, or seventh decade, as a result of age-related joint degeneration and osteoarthritis.

What kind of exercises are safe during the hypermobility phase?

Safe exercises during the hypermobility phase typically focus on low-impact activities that build muscle strength to support and stabilize the joints, rather than emphasizing flexibility. Physical therapy and exercises like swimming or gentle yoga, under a professional's guidance, can be very beneficial.

Can joint stiffness in the third stage be reversed?

Joint stiffness that develops as part of the third, or stiffness, phase is typically a result of degenerative changes like osteoarthritis and is not reversible. Management at this stage focuses on managing pain and maintaining as much function and mobility as possible.

Are there other types of EDS that don't follow these stages?

Yes. These stages specifically describe the typical progression of hypermobile EDS (hEDS). Other types of EDS, such as Classical or Vascular EDS, have different characteristic symptoms and progressions and do not follow this three-phase model.

Why is it so difficult to get a diagnosis during the early stages of EDS?

Early on, hypermobility is often seen as harmless or a positive trait. In the pain phase, the widespread and systemic symptoms can mimic other conditions like fibromyalgia, and without a thorough medical history and awareness of EDS, it can take years for patients to receive an accurate diagnosis.