Joint hypermobility, also known as being double-jointed, means that some or all of a person’s joints have a larger-than-normal range of movement.1 It is very common, thought to affect one in every five people worldwide.2 The majority of people with hypermobile joints do not feel any pain as a result.1 In sports such as ballet, gymnastics, acrobatics and yoga as well as when playing musical instruments, having hypermobile joints may allow a person to complete more technically advanced movements and positions.
However, if a person’s joint hypermobility causes them pain, this is known as joint hypermobility syndrome (JHS).3 JHS needs to be managed with careful medical care to minimise its impact on everyday life.
There are various types of JHS, with the most well-known form being hypermobile Ehlers-Danlos Syndrome (hEDS), affecting between 1 in 3100 and 1 in 5000 people worldwide.4 This number is likely to be much greater, as JHS is often misdiagnosed as similar conditions like Fibromyalgia or Chronic Fatigue Syndrome (Myalgic Encephalomyelitis).5 hEDS can affect joints throughout the body, or in some cases only the hands and feet. Other forms include Marfan syndrome, where in addition to hypermobile joints, individuals may be unusually tall, have longer limbs, fingers and toes, and may have eye and heart problems.1 A condition known as Osteogenesis imperfecta, or brittle bone disease, can also cause joint hypermobility.
Read on to find out what causes JHS and how it is diagnosed and managed.
Causes of joint hypermobility syndrome
JHS runs in families, so it is not preventable.6 This is because it is caused by malfunctions in genes that code for key proteins that make up connective tissues, which help support and bind different parts of the body together. Examples of connective tissues include bones, ligaments, tendons, cartilage and fat tissue. For example, EDS is caused by a problem with the gene coding for collagen proteins, which helps connective tissues to stay strong and flexible. Marfan syndrome occurs as a result of a problem with the gene coding for the fibrillin protein, which forms elastic fibres within connective tissues. These protein defects weaken the connective tissues, which in turn causes those with JHS to have loose and hypermobile joints.
Signs and symptoms of joint hypermobility syndrome
Alongside feelings of pain in the areas of your joints and muscles, there are some key signs and symptoms you and your healthcare provider can look out for to assess whether you may have JHS. Some of these are as follows:6
- Regular joint and ligament injuries, such as dislocations or sprains
- Stiffness of muscles and joints
- Recurrent tiredness
- Problems with balance and coordination
- Difficulties with bowel and bladder activity
- Dizziness and fainting
- Skin that appears thin and stretches easily
If you think you may be experiencing any or several of these symptoms alongside joint hypermobility, it is important to speak with your healthcare provider.
Management and treatment for joint hypermobility syndrome
Whilst there is no cure for JHS, there are many ways in which JHS can be managed.3 The main goal is to build muscle strength and physical fitness, which protects your joints. To support this, your healthcare provider may refer you to a physiotherapist, occupational therapist or foot specialist known as a podiatrist to give you the right help.
The following are the aims of these types of support:
- Decreasing pain
- Reducing the risk of dislocations
- Increasing muscle strength and fitness
- Maintaining good posture and balance
You can help reduce your risk of pain flare-ups through low-impact exercises such as swimming or cycling. It helps to pace yourself, so you don’t do too many repetitive movements one after the other. Wearing supportive shoes and using special insoles to line the inside of your shoes can help protect your joints.
As the pain that coincides with JHS can range between different people, it is important to monitor it and seek the best possible treatments as early as possible. For example, over-the-counter medicines such as Paracetamol (also known as Tylenol) and Ibuprofen (also known as Advil) may help with mild pain, whilst more severe pain will need to be treated with stronger painkillers such as opioids or local anaesthetics such as Lidocaine. It can be difficult to know what the best form of treatment is, depending on whether you are experiencing shorter acute episodes of pain or longer bouts of pain, so it is vital to keep track of your experience of pain over time to share with your healthcare provider when you see them.
The infographic below shows some of the recommended ways to manage JHS symptoms:7
Transcutaneous Electrical Nerve Stimulation (TENS) devices, as pictured in the bottom right of the infographic above can temporarily alleviate pain via the skin,8 which may be helpful in JHS. These are battery-operated and are connected to electrode pads that stimulate nerves in the painful area, causing them to send fewer pain signals to the brain and spinal cord. This stimulation also triggers muscle relaxation and the production of the brain chemical serotonin, which is a natural painkiller. Speak to your healthcare provider if you think this may help you or someone you know, as they can loan one and show you how they work.
Elinor Jones, who is 24 and living with hypermobile Ehlers-Danlos syndrome, reflects on how she manages and is coming to terms with the condition:
“In 2023, I'm finally accepting my new normal as someone diagnosed with hEDS. I'm learning how I can enjoy life and my career whilst managing my symptoms.
For me this means pacing myself, stretching, getting enough sleep, taking my meds and finding a balance between activity and rest.”
Before making a diagnosis of JHS, your healthcare provider will most likely run blood tests and an X-ray to rule out other conditions such as arthritis.3 There are some key tests they can run to identify whether you may have JHS. These are as follows:9
- The Beighton Score/ Test – this assesses your joint flexibility on a nine-point scale, where a score of four and above alongside joint pain may lead to a diagnosis of JHS. One point is given for each of the following:
- The ability to bend forward and put your hands flat on the floor without needing to bend your knees
- The ability to bend your elbows backwards (one point for each elbow)
- The ability to bend your knees backwards (one point for each knee)
- The ability to bend your thumbs backwards so they reach your forearm (one point for each thumb)
- The ability to bend your little fingers backwards until they are at a 90-degree angle (one point for each little finger)
- The Five-point Hypermobility Questionnaire – you may be asked these questions in addition to the above questions. Answering yes to two or more of these questions may lead to a diagnosis of JHS. This consists of the following questions:
- Can you currently (or could you previously) put your hands flat on the floor without bending your knees
- Can you currently (or could you previously) bend your thumb to touch your forearm
- When you were a child, did you entertain your friends by placing your body in strange shapes or doing the splits
- When you were a child, did your shoulder or kneecap dislocate more than once
- Do you believe you are double-jointed
The Ehlers-Danlos Society UK has a great infographic showing the different physical examinations that are used to diagnose JHS.
As JHS can vary considerably from person to person, it is not always easy to predict one day from another. This can lead to stigma about the validity of the condition. Not only is JHS valid, but just because the pain caused by JHS isn’t necessarily visible, this doesn’t mean it isn’t there. Having compassion for those living with the complications of JHS is key to ensuring they get the support they need. Anyone can live with JHS, including children and adults of any age, race, gender or body shape.10
Below are some great examples of campaigns and organisations fighting to raise awareness of JHS, such as Ehlers-Danlos Syndrome, which you can support and take part in:
- Ehlers-Danlos UK’s #EnoughIsEnough campaign
- Ehlers-Danlos UK’s support helplines
- MindBodyEDS, supporting people through the impacts of JHS on education and finances
- The Chronic Pain Partners, who provide local support groups for those experiencing JHS in the US
How can I prevent joint hypermobility syndrome
JHS cannot be prevented, as it is caused by faulty genes and runs in families.6 However, spotting signs and symptoms early in family members and children ensures that the most appropriate and helpful treatments are given.
How common is joint hypermobility syndrome
Joint hypermobility syndrome is uncommon, affecting between 1 in 3100 to 1 in 5000 people across the world.4 This number is likely higher, as JHS can often be misdiagnosed as other conditions such as Fibromyalgia and Chronic Fatigue Syndrome, due to an overlap in symptoms.
Who is at risk of joint hypermobility syndrome
You are at risk of JHS if you have JHS in your family.6 It is seen more often in females than males, and in people of colour than those who are white.1
What can I expect if I have joint hypermobility syndrome
If you or someone you know is diagnosed with JHS, it is likely that the symptoms such as pain and stiffness will interfere with everyday activities. For example, if a child with JHS is attending school, they may find it difficult to take part in some sports or other important activities.11 Therefore, it can be helpful to have a supportive adult who can encourage anyone with JHS and who they can talk to if they are struggling with symptoms. This also applies in the workplace, whether that be an occupational therapist or a mental health first aider. It is important that those with JHS feel listened to, as they may find it hard to talk about their condition.
It can be extremely important to implement time for rest, for example after lots of physical activity to prevent pain flare-ups. This can be something that is implemented into a diary or planner around any events and exercise. Additionally, anticipating the need for the management of symptoms through physiotherapists and other healthcare providers means that these can be planned ahead of time.
When should I see a doctor
It is best to go and see your healthcare provider if you experience any of the following:3
- Repeated pain and stiffness in your joints or muscles
- Recurrent strains and sprains
- Multiple dislocations of your joints
- Difficulties with balance and coordination
- Thin skin that stretches easily
- Bowel and bladder issues
Joint Hypermobility Syndrome (JHS) is a rare genetic condition which causes an individual to experience extreme flexibility of joints and associated pain. The condition affects anyone of any age, but is more common in females and people of colour. Most commonly, symptoms include joint pain and stiffness, recurrent ligament and joint injuries such as sprains and dislocations, as well as frequent tiredness. JHS is diagnosed by ruling out other similar conditions, such as Fibromyalgia and Chronic Fatigue Syndrome, as well as conducting a range of physical examinations of joint mobility and symptoms. JHS is treatable and can be managed through physiotherapy, low-impact exercise and appropriate pain relief.
- National Health Service (NHS). [Internet] Joint Hypermobility. Available from: https://www.nhsinform.scot/illnesses-and-conditions/muscle-bone-and-joints/conditions/joint-hypermobility. Date accessed: 2nd July 2023.
- Hakim AJ, Cherkas LF, Grahame R, Spector TD and MacGregor AJ. The genetic epidemiology of joint hypermobility: a population study of female twins. Arthritis Rheum. 2004; 50(8): 2640–2644. https://doi.org/10.1002/art.20376
- NHS. [Internet] Joint hypermobility syndrome. Available from: https://www.nhs.uk/conditions/joint-hypermobility-syndrome/. Date accessed: 2nd July 2023.
- The Ehlers-Danlos Society. [Internet] What is EDS? Available from: https://www.ehlers-danlos.com/what-is-eds/. Date accessed: 2nd July 2023.
- Mikovic T and Sieg VC. Ehlers-Danlos Syndrome. Treasure Island, Florida: StatPearls Publishing; 2023. https://www.ncbi.nlm.nih.gov/books/NBK549814/#:~:text=Patients%20with%20EDS%2C%20especially%20those,they%20have%20on%20the%20patient
- Fawcett DW. [Internet] Connective Tissue. Available from: https://www.britannica.com/science/connective-tissue. Date accessed: 2nd July 2023.
- NHS. [Internet] TENS (transcutaneous electrical nerve stimulation. Available from: https://www.nhs.uk/conditions/transcutaneous-electrical-nerve-stimulation-tens/. Date accessed: 3rd July 2023.
- Peck S. [Internet] Pain Management. Available from: https://www.hypermobility.org/pain-management. Date accessed: 2nd July 2023.
- Cleveland Clinic. [Internet] Joint Hypermobility Syndrome. Available from: https://my.clevelandclinic.org/health/diseases/21763-joint-hypermobility-syndrome. Date accessed: 2nd July 2023.
- The Ehlers-Danlos Society. [Internet] Myths and Facts about EDS and HSD. Available from: https://www.ehlers-danlos.com/myths-and-facts-about-eds-and-hsd/. Date accessed: 2nd July 2023.
- Lowth M. [Internet] Hypermobility syndrome. Available from: https://patient.info/bones-joints-muscles/hypermobility-syndrome-leaflet. Date accessed: 2nd July 2023.