Hypermobility Spectrum Disorder: All You Need to Know

Unravel what is Hypermobility Spectrum Disorder, its causes, symptoms, and management strategies.

steven zauderer
Steven Zauderer
April 26, 2024
9 min read
min read

Understanding Hypermobility Spectrum Disorders

Hypermobility Spectrum Disorders (HSD) are a group of conditions related to joint hypermobility. To better understand these disorders, it's crucial to delve into their definition, prevalence, symptoms, and impact on individuals.

Definition and Prevalence

Hypermobility Spectrum Disorders refer to a group of conditions that cause the joints to be more flexible than average. This excessive flexibility, or 'laxity', can lead to a wide range of symptoms and health problems. According to Versus Arthritis, hypermobility affects 1 in 4 people, making it a relatively common condition.

Symptoms and Impact

The symptoms of Hypermobility Spectrum Disorders can vary greatly from person to person and can affect many parts of the body. They can range from mild to severe and can include pain, fatigue, and injuries to joints and ligaments. For some people, the symptoms may be so mild that they don't even realize they have the condition, while for others, the symptoms can be severe and debilitating.

In addition to joint problems, people with hypermobile joints may have weak collagen in other parts of the body, which can lead to additional conditions or symptoms. For instance, hypermobile individuals may also experience conditions such as postural orthostatic tachycardia syndrome (POTS), digestive issues, and other rarer conditions.

The impact of HSD on an individual's life can be significant, affecting their mobility, daily activities, and quality of life. It's important to note that while there is currently no cure for HSD, many people with the condition lead full and active lives with the right treatment and management strategies.

In the following sections, we will delve deeper into the types of Hypermobility Syndromes, their causes and risk factors, how they are diagnosed, and the available treatment options. This comprehensive overview aims to provide a clear understanding of what Hypermobility Spectrum Disorder is and how it can be managed effectively.

Types of Hypermobility Syndromes

There are distinct types of hypermobility syndromes that have been identified by the medical community. It's important to understand the differences between these conditions to create an effective treatment plan. The two primary types we will discuss are Hypermobility Spectrum Disorder (HSD) and Ehlers-Danlos Syndrome (EDS).

Hypermobility Spectrum Disorder (HSD)

Hypermobility Spectrum Disorders (HSDs) is the updated term used to describe Joint Hypermobility Syndrome (JHS), which is characterized by joint hypermobility (JH) paired with secondary musculoskeletal manifestations, excluding hypermobile EDS (hEDS). These disorders are considered common, with a prevalence of 1 in 600 to 1 in 900.

Joint hypermobility is a clinically identifiable finding that refers to the ability of a joint or group of joints to move beyond normal limits along physiological axes. It can be diagnosed using a screening questionnaire or physical examination using a goniometer or the Beighton scoring system.

HSDs have four distinct subtypes:

  1. HSD (historical JH)
  2. H-SD (JH without musculoskeletal manifestations)
  3. H-HSD (JH with historical manifestations)
  4. HSDm (JH with musculoskeletal manifestations)

These subtypes are distinguished based on the presence of secondary musculoskeletal manifestations and the historical presence of JH.

Ehlers-Danlos Syndrome (EDS)

The Ehlers-Danlos syndromes were reclassified in 2017, and the hypermobility spectrum disorders were introduced. As a result, the diagnosis of Joint Hypermobility Syndrome became outdated. Most individuals previously diagnosed with this syndrome are now classified as having either hypermobile Ehlers-Danlos syndrome (hEDS) or a type of HSD.

The combined prevalence of HSDs and hEDS is estimated to be 1 in 600 to 1 in 900. The prevalence of generalized joint hypermobility (GJH) in children and young adults is around 34%, with higher prevalence rates among professional dancers.

The awareness of these different types of hypermobility syndromes can help in understanding the intricacies of these disorders and their impact on the lives of those affected by them. The next section will discuss the causes and risk factors associated with these conditions.

Causes and Risk Factors

To gain a better understanding of Hypermobility Spectrum Disorder (HSD), it's important to examine the potential causes and risk factors associated with this condition. HSD, including its most common form, hypermobile Ehlers-Danlos Syndrome (hEDS), is primarily influenced by genetic factors and weaknesses in collagen structure.

Genetic Factors

The main cause of HSD and hEDS is genetics. Hypermobility often runs in families and cannot be prevented. For hypermobile Ehlers-Danlos syndrome, the most common form, there is currently no genetic testing available to confirm a diagnosis.

Individuals with the hypermobile type of Ehlers-Danlos syndrome are characterized by an unusually large range of joint movement (hypermobility). This often results in weak muscle tone (hypotonia) in infants and children, leading to delays in motor skill development such as sitting, standing, and walking. The loose joints are unstable, prone to dislocation, and may cause chronic pain.

Collagen Weakness

HSD and hEDS are often characterized by faulty or weak collagen, which affects the strength and stability of joints. Collagen, a protein that provides structure to much of your body, including skin, hair, nails, and bones, plays a crucial role in joint health. When collagen is weak or faulty, it can lead to the hypermobility and instability seen in these disorders [1].

Different types of Ehlers-Danlos syndrome can also present a variety of symptoms related to collagen weakness. For instance, the classical form of the syndrome presents individuals with soft, velvety, highly stretchy (elastic), and fragile skin. Affected individuals bruise easily, and wounds can split open with little bleeding, leaving characteristic "cigarette paper" scars that widen over time. The dermatosparaxis type of the disorder is characterized by loose skin that sags and wrinkles, and extra folds of skin may be present.

Bleeding problems are common in the vascular type of Ehlers-Danlos syndrome, a condition caused by unpredictable tearing (rupture) of blood vessels and organs. This can lead to complications such as easy bruising, internal bleeding, intestinal perforation, and stroke. Pregnant women with vascular Ehlers-Danlos syndrome may experience uterine rupture [5].

The understanding of the genetic factors and collagen weakness associated with HSD provides a basis for further exploration of this complex disorder. While there are no preventative measures against the genetic factors, understanding and managing the symptoms associated with collagen weakness can significantly improve the lives of those affected by HSD and hEDS.

Diagnosis and Assessment

Diagnosing Hypermobility Spectrum Disorder (HSD) involves careful assessment by a healthcare professional. There are various tools and criteria used to evaluate the presence of joint hypermobility and related symptoms.

Beighton Score

The Beighton Score is one such tool that is commonly used to measure joint hypermobility. It uses a 9-point scale to assess the range of motion in various joints. A positive score, indicating hypermobility, is any score greater than or equal to 5 out of 9 points in adults. However, hypermobility can also be present in joints not measured by this score [3].

The Beighton Score assesses the following joints:

  1. Left and Right Little Finger: Can it be bent backwards to 90 degrees? (1 point each)
  2. Left and Right Thumb: Can it touch the forearm when bent forwards? (1 point each)
  3. Left and Right Elbow: Can it bend backwards? (1 point each)
  4. Left and Right Knee: Can it bend backwards? (1 point each)
  5. Trunk: Can the individual bend forwards and place their hands flat on the ground without bending their knees? (1 point)

It's important to note that joint hypermobility, as determined by the Beighton Score, does not automatically mean a diagnosis of HSD. Joint hypermobility is actually quite common, with up to about 20% of people having hypermobility into adulthood.

Diagnostic Process

The diagnostic process for HSD goes beyond the assessment of joint hypermobility. Healthcare professionals also consider other factors, such as the presence of joint instability. Joint instability occurs when the bones of a joint are not held in place securely. This can lead to joint subluxations, dislocations, sprains, and other injuries. Joint instability can cause both acute and chronic pain, impacting daily life [3].

Furthermore, the diagnostic process takes into account recent changes in the classification of these disorders. In 2017, the Ehlers-Danlos syndromes were reclassified, and the hypermobility spectrum disorders were introduced. As a result, most people previously diagnosed with Joint Hypermobility Syndrome are now classified as having either hypermobile Ehlers-Danlos syndrome (hEDS) or a type of HSD.

A comprehensive diagnosis of HSD requires a thorough medical history, physical examination, and possibly additional tests to rule out other conditions. It's essential to consult with a healthcare provider who is familiar with these disorders to ensure an accurate diagnosis and appropriate treatment plan.

Management and Treatment

Managing and treating Hypermobility Spectrum Disorders (HSD) involves a combination of lifestyle adjustments and medical interventions. These approaches aim to alleviate symptoms, prevent injuries, and improve the overall quality of life for individuals with HSD.

Lifestyle Adjustments

Lifestyle adjustments are often the first line of defense in managing HSD. These adjustments might include the following:

  • Physical Therapy: Regular physical therapy can help strengthen muscles, improve coordination, and increase joint stability. This can help reduce the risk of dislocations, sprains, and other injuries associated with joint instability.
  • Pacing Activities: Pacing day-to-day activities can help manage fatigue, a common symptom in people with HSD. This might involve taking regular rest breaks and avoiding activities that cause pain or discomfort.
  • Adequate Hydration and Nutrition: Maintaining a balanced diet and staying well-hydrated can help support overall health and wellbeing. Some individuals with HSD may also benefit from a specialized diet or nutritional supplements, based on their specific health needs.
  • Mindfulness and Relaxation Techniques: Techniques such as mindfulness, meditation, and deep breathing exercises can help manage stress, improve sleep, and alleviate pain.

Medical Interventions

Medical interventions are typically recommended when lifestyle adjustments are insufficient to manage HSD symptoms. These might include the following:

  • Pain Management: Over-the-counter or prescribed pain relievers can be used to manage acute or chronic pain. It's important to use these medications under the guidance of a healthcare provider to avoid potential side effects and interactions.
  • Joint Supports or Braces: Supports or braces can be used to stabilize hypermobile joints and prevent injuries. These devices should be used as recommended by a healthcare provider or physical therapist.
  • Surgery: In some cases, surgery might be recommended to repair or stabilize a joint. However, surgery is typically considered a last resort due to the potential risks and recovery time involved.
  • Specialist Referrals: Given the wide range of symptoms and associated conditions that can occur with HSD, individuals may benefit from seeing various specialists. These might include cardiologists, gastroenterologists, and neurologists, among others.

Remember, the management and treatment of HSD should be individualized, based on each person's specific symptoms, health needs, and lifestyle. Ongoing communication with healthcare providers and a proactive approach to self-care are key to successfully managing HSD.

Dietary Considerations

While managing Hypermobility Spectrum Disorder (HSD) and Ehlers-Danlos Syndrome (EDS), diet plays a crucial role. It is important to understand potential nutritional deficiencies and consider specialized diets that might help alleviate symptoms.

Nutritional Deficiencies

Individuals with EDS and hypermobility often experience fatigue, which can be exacerbated by nutritional deficiencies. These can arise due to difficulty in absorbing essential vitamins and minerals such as vitamin D, vitamin B12, and iron. Therefore, it is important to monitor these nutrient levels and work with healthcare professionals to ensure optimal intake [6].

Nutrient Role
Vitamin D Bone Health
Vitamin B12 Energy Production and Nerve Function
Iron Oxygen Transport

Hydration and sodium intake are also crucial, especially for individuals with Postural Orthostatic Tachycardia Syndrome (POTS), often linked with EDS. Sodium intake helps the body retain water and increase blood volume, which can alleviate POTS symptoms. Individuals with POTS should aim for a sodium intake of 3,000-10,000 milligrams per day and consume 2-2.5 liters of fluids daily [6].

Specialized Diets

There is no universally prescribed diet for EDS or HSD. The decision to exclude specific foods from the diet should be personal, based on individual experiences, and guided by professional advice. Certain health conditions, such as irritable bowel syndrome (IBS), gastroesophageal reflux disease (GERD), or celiac disease, may necessitate specific dietary adjustments [6].

One specialized diet that has been well-received by individuals with hypermobility and EDS is the FODMAP diet. This diet eliminates certain foods known to cause gastrointestinal symptoms. The low FODMAP diet has been shown to improve symptoms such as bloating, abdominal pain, and IBS symptoms in those with hypermobility. However, it is important to work with a professional and follow a proper protocol for maximum effectiveness.

While there is a lack of comprehensive research on hypermobility diets, the FODMAP diet has shown promising results in improving symptoms in hypermobility patients. However, more rigorous, large-scale studies focusing on hypermobility diets are needed to provide evidence-based guidelines.








steven zauderer

CEO of CrossRiverTherapy - a national ABA therapy company based in the USA.

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