What is Hypermobility?

Hypermobility refers to the ability of some or all of a person’s joints to move beyond the normal range of motion. This condition is often described as having loose joints or being “double-jointed”. Some patients describe it to me as “cracking or popping joints” or “feeling not well held together.” While many people with hypermobility do not experience any problems, others may face significant health issues and challenges.

When you break down the word “Hyper-mobility” it refers to an excess (hyper) of motion or movement (mobility), that can be seen in the joints of a person’s body. This is the opposite of hypomobility, the decrease (lesser/hypo) of movement in the joints. Hypermobility itself is a symptom, indicating that a person’s joints move beyond the normal range of motion. It is not a diagnosis on its own.

How common is Hypermobility?

Hypermobility is quite common and can vary widely among different populations. General Joint Hypermobility (GJH) is found in about 2% to 57% of the population [1]. This wide range is due to differences in age, gender, and ethnicity. For example, children and adolescents tend to have higher rates of hypermobility compared to adults [2]. However, when hypermobility leads to symptoms such as pain, joint instability, or other complications, it can be part of a larger diagnosis, such as Hypermobility Spectrum Disorders (HSD), Ehlers-Danlos Syndrome (EDS), Marfan’s Syndrome or Loeys Dietz Syndrome.

Hypermobility is often a genetic condition, meaning other family members may also have similarly bendy joints. It involves the connective tissues, such as ligaments, which are supposed to stabilise joints. In people who are hypermobile, these tissues are more elastic, allowing for greater or excess movement or flexibility. This condition can be caused by various factors, including the shape of bones, training effects (continuous stretching), or inherited connective tissue disorders.

Signs that hypermobility might be part of a larger syndrome:

• Chronic muscle or joint Pain
• Joint instability
• Fatigue
• Skin or bruising issues
• Gut and bladder problems
• Balance & coordination issues
• Racing heart, dizziness or fainting
• Anxiety or sleep issues
• Difficulty reaching milestones

Why understanding your connective tissues matters

Understanding the role of your bodies’ connective tissues in hypermobility is crucial for managing Hypermobile Spectrum Disorders. Connective tissues include, collagen, and other proteins. Collagen is the most common structural protein in the human body, often described as the “glue that holds us together.”

Connective tissues and especially collagen, provide structural support to various parts of the body, including the:

• Joints
• Skin
• Bones
• Nerves and neural systems
• Gut
• Bladder
• And many other organs.

When connective tissues are more elastic or less effective than usual, they can’t offer the same level of support. This can result in joint instability and a range of other health challenges. In people with Ehlers-Danlos Syndromes (EDS), the collagen is often structurally abnormal (defective), which leads to connective tissues that are weaker or more elastic than normal. In Hypermobility Spectrum Disorders (HSD), the exact cause is less well understood, but it is believed to involve connective tissue differences that similarly result in joint instability and other related symptoms.

What is collagen?

Collagen is a key structural protein in the human body and a building block for skin, bones, tendons, ligaments, cartilage, and other connective tissues. It provides strength, elasticity, and support to these tissues, playing a vital role in maintaining their health. Collagen fibres are incredibly strong and flexible, with strength comparable to steel [4]. However, their elasticity makes them perfect for body tissues that need both strength and flexibility.

In the average human, connective tissues like collagen provide structural support to joints, tendons, ligaments, and other body structures, helping to brace and support blood vessels and organs.

In hypermobile individuals, genetic changes affecting collagen creation and structure means it is often defective or less effective, which can reduce its ability to properly support joints and other connective tissues. This can cause overstretching, difficulty sensing their position in space, joint problems like partial dislocations or full dislocations, and various other health issues throughout the body [5][6][7]. As a result, my patients often notice “issues in their connective tissues” which cause them to not “feel well held together.” Additionally, these issues with collagen creation, structure and metabolism can lead to chronic pain and fatigue [8].

Since collagen is a key component of connective tissues, hypermobility is often referred to as a “connective tissue disorder.” [3]

When it’s more than just being bendy – Understanding Symptomatic Hypermobility

While many people with hypermobility do not experience any problems, others may face significant challenges. When hypermobility is accompanied by symptoms such as pain, frequent injuries, fatigue, joint instability, and other symptoms, it is referred to as symptomatic hypermobility. When these symptoms significantly impact daily life, hypermobility is then referred to as “syndromic” or “symptomatic” [8]. The two primary conditions associated with symptomatic hypermobility are Hypermobile Spectrum Disorder (HSD) and Hypermobile Ehlers-Danlos Syndrome (hEDS) [10].

A medical disorder is an illness or condition that interferes with your normal everyday physical or mental functions. When joint hypermobility leads to problems that disrupt normal function, it is classified as a disorder. However, if joint hypermobility does not cause any issues or pain, it is not considered symptomatic or a disorder.

Types of Hypermobility, Hypermobile Spectrum Disorders and the Ehlers-Danlos Syndromes

Starting in March 2017, a new classification system was established by the International Consortium on the Ehlers-Danlos Syndromes [11]. This new classification includes a review of hypermobility disorders, with hypermobility syndrome now renamed and categorised under the umbrella of Hypermobility Spectrum Disorders. The spectrum of joint hypermobility now includes:

• Asymptomatic Generalised Joint Hypermobility: Hypermobile joints across the body with no symptoms
• Asymptomatic Peripheral Joint Hypermobility: Hypermobile joints in the hands and feet with no symptoms
• Asymptomatic Localised Joint Hypermobility: Hypermobility in single joints or groups of joints with no symptoms
• Generalised Hypermobility Spectrum Disorder
• Peripheral Hypermobility Spectrum Disorder
• Localised Hypermobility Spectrum Disorder
• Historical Hypermobility Spectrum Disorder

This classification is based on the framework outlined in “A framework for the classification of joint hypermobility and related conditions” [7].

Hypermobile Spectrum Disorder (HSD)

Hypermobile Spectrum Disorder (HSD) is a group of conditions characterised by symptomatic joint hypermobility that other connective tissue disorders cannot explain. HSD is diagnosed after excluding other conditions, such as Ehlers-Danlos Syndrome (EDS). The condition can vary greatly in its presentation and severity, meaning it affects individuals in unique ways. For example, patients may experience varying degrees of joint involvement, along with combinations of pain, anxiety, fatigue, or digestive issues, making it a “body-wide” or spectrum disorder. [12]

Symptoms of HSD

Individuals with HSD may experience a range of symptoms, and any two people with Hypermobile Spectrum Disorder may experience very different symptoms. These symptoms can include:

• Pain, chronic pain in muscles and joints
• Joint instability, leading to subluxations and dislocations.
• Fatigue and brain fog
• Mast Cell Activation Syndrome (MCAS)
• Coordination and proprioception issues
• Gastrointestinal issues, such as Irritable Bowel Syndrome (IBS)
• Anxiety and or depression
• Autonomic dysfunction (Dysautonomia), including dizziness, racing heart, temperature dysregulation and fainting.
• Headaches or migraines

While the symptoms of HSD can be challenging, research and patient experiences suggest that they may manifest differently across genders. Biological, hormonal, and structural differences can influence symptom severity, presentation, and frequency. The diagrams below illustrates some of the ways symptoms can present differently across male, female, and non-binary individuals, showing variations in severity and common patterns unique to each group.

Ehlers-Danlos Syndrome (EDS’s) and Hypermobile Ehlers-Danlos Syndrome (hEDS)

EDS is a group of genetic connective tissue disorders characterised by joint hypermobility, skin hyperextensibility, and tissue fragility. There are 13 recognised subtypes of EDS, each with its own set of clinical criteria. The most common type is Hypermobile EDS (hEDS), which shares many features with HSD but includes additional systemic issues.

There is a stricter criterion for diagnosing hEDS compared to HSD. Other notable subtypes of EDS include Classical EDS (cEDS), which features severe skin involvement, and Vascular EDS (vEDS), which involves a high risk of arterial, intestinal, and uterine rupture. Please visit our article on Ehlers-Danlos Syndromes (EDS) Types for more detailed information.

Hypermobile Ehlers-Danlos Syndrome (hEDS)

Hypermobile Ehlers-Danlos Syndrome (hEDS) is a heritable connective tissue disorder that causes generalised joint hypermobility, joint instability, and chronic pain. hEDS is the most common type of EDS, accounting for about 90% of EDS cases. It’s estimated that around 10% of people with General Joint Hypermobility (GJH) experience symptoms that qualify as HSD [1]. This means that a significant portion of the population could be affected, but the exact numbers can differ based on factors like age, gender, and ethnicity.

hEDS is more common than previously thought. Recent research suggests that hEDS and Hypermobility Spectrum Disorders (HSD) are actually the same disorder and may affect approximately 1 in 500 people [14]. This is a significant increase from earlier estimates, which suggested a prevalence of about 1 in 5,000 individuals.

It is important to note that hEDS is often underdiagnosed, and many individuals may go years and even decades without a proper diagnosis due to a lack of awareness and understanding of the condition. Increased recognition and improved diagnostic criteria have contributed to more accurate prevalence estimates [15].

Here at Hypermobility Health Connect we are attempting to further assist with more accurate and earlier diagnosis – and it all starts by Taking the test!

Symptoms of hEDS

Individuals with hEDS may experience a variety of symptoms, which can vary widely among individuals. Here are some common symptoms associated with hEDS:

• Joint hypermobility: Joints that move beyond the normal range of motion.
• Joint pain and instability: Frequent joint dislocations, sprains, and chronic pain.
• Muscle pain: Generalised muscle pain and weakness.
• Stretchy, fragile skin: Skin that bruises easily and may not heal properly.
• Fatigue: Extreme tiredness and low endurance.
• Digestive problems/Gut: Issues such as heartburn, constipation, gastroparesis, and gastroesophageal reflux disease (GERD)and bloating.
• Anxiety and or depression
• Dizziness and increased heart rate: Especially after standing up.
• Problems with internal organs: Such as mitral valve prolapse, or organ prolapse.
• Bladder/Urinary incontinence: Difficulty controlling bladder functions.
• Autonomic dysfunction: Issues with blood pressure regulation and other autonomic functions, including Postural Orthostatic Tachycardia Syndrome (POTS)
• Skin differences: Skin that is soft, velvety, and may be slightly stretchy
• Female health concerns
• Sleep difficulties

It is important to note that not everyone with hEDS will experience all of these symptoms, and the severity can vary widely.

Hypermobility itself is a symptom, indicating that a person’s joints move beyond the normal range of motion. It is not a diagnosis on its own. However, when hypermobility leads to symptoms such as pain, joint instability, or other body wide life impacting complications, it can be part of a larger diagnosis, such as Hypermobility Spectrum Disorder (HSD) or Hypermobile Ehlers-Danlos Syndrome (hEDS).

Next steps…

Symptomatic hypermobility, including Hypermobile Spectrum Disorder (HSD) and Hypermobile Ehlers-Danlos Syndrome (hEDS), can significantly impact an individual’s quality of life. Understanding and managing the symptoms is crucial for improving the well-being of those affected. Diagnosing hypermobility requires a thorough clinical evaluation, patient history, and the use of specific diagnostic tools like the Beighton Score and Five-Point Questionnaire.

If you suspect you have symptomatic Hypermobility, HSD, hEDS or another type of EDS, consult with a healthcare professional for a proper diagnosis and treatment plan. We also welcome you to Take the test! – it’s a very useful tool for understanding your Hypermobility journey.

For more detailed information, you can visit the following resources:

• The Ehlers-Danlos Society
• Hypermobility Syndromes Association (HMSA – UK)
• The Marfan Foundation (Marfan Syndrome & Related Disorders)
• Loeys-Dietz Syndrome Foundation
• The International Consortium on Ehlers-Danlos Syndromes & HSD
• National Institutes of Health (NIH)
• Genetics Home Reference (NIH/NLM)
• Mayo Clinic
• Cleveland Clinic
• NHS UK

References

[1] Blajwajs, L., Williams, J., Timmons, W. et al. Hypermobility prevalence, measurements, and outcomes in childhood, adolescence, and emerging adulthood: a systematic review. Rheumatol Int 43, 1423–1444 (2023). https://doi.org/10.1007/s00296-023-05338-x

[2] Nicholson, L. L., Chan, C., Tofts, L., & Pacey, V. (2022). Hypermobility syndromes in children and adolescents: Assessment, diagnosis and multidisciplinary management. Australian Journal of General Practice, 51(6)

[3] Grahame, R. (1999). Joint hypermobility and genetic collagen disorders: Are they related? Archives of Disease in Childhood, 80(2), 188. https://doi.org/10.1136/adc.80.2.188

[4] Yamamoto, N., & Nakamura, S. (2017). Relationships between the tensile strength and diameter of collagen fibrils isolated from mouse tail tendons. Journal of Biomechanical Science and Engineering, 12(3), 16-00511.

[5] Gensemer, C., Burks, R., Kautz, S., Judge, D. P., Lavallee, M., & Norris, R. A. (2021). Hypermobile Ehlers‐Danlos syndromes: Complex phenotypes, challenging diagnoses, and poorly understood causes. Developmental dynamics, 250(3), 318-344. https://doi.org/10.1002/dvdy.220

[6] Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., … & Tinkle, B. (2020). The 2017 international classification of the Ehlers-Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 5-7.

[7] Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F., & Hakim, A. (2017). A framework for the classification of joint hypermobility and related conditions. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 148-157.

[8] Tinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017). Hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD): Clinical description and natural history. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 48-69.

[10] Hypermobility Syndromes Association. (n.d.). What is hypermobility? Retrieved February 18, 2025, from https://www.hypermobility.org/what-is-hypermobility

[11] Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F., & Hakim, A. (2017). A framework for the classification of joint hypermobility and related conditions. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 148-157.

[12] The Ehlers-Danlos Society. (n.d.). What is HSD? Retrieved February 18, 2025, from https://www.ehlers-danlos.com/what-is-hsd/

[14] Demmler, J. C., Atkinson, M. D., Reinhold, E. J., Choy, E., Lyons, R. A., & Brophy, S. T. (2019). Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum disorder in Wales, UK: a national electronic cohort study and case–control comparison. BMJ open, 9(11), e031365-e031365. https://doi.org/10.1136/bmjopen-2019-031365

[15] Mayo Clinic. (n.d.). Ehlers-Danlos syndrome. Retrieved February 18, 2025, from https://www.mayoclinic.org/