What is hypermobility? (Introduction)
 

 

Many people consider themselves to be flexible or double jointed. But what is the difference between flexibility and joint hypermobility? Well, joint hypermobility is determined by the Beighton Score, a simple joint flexibility scorecard that determines the degree of joint hypermobility.

To find your Beighton Score, refer to the figure and

add up the points from each of the following movements:

  1. Bending your small finger back further than 90 degrees (1 point each side)

  2. Bending your elbow beyond a straight line (1 point each side)

  3. Bending your knee beyond a straight line (1 point each side)

  4. Putting your palms flat on the floor without bending your knees (1 point in total)

  5. Bending your thumb back to touch the front of your forearm (1 point each side)

From that score the general criteria that is used to determine whether you are hypermobile:

  • If you are a child or teenager, a score of 6 or more out of 9 is positive.

  • If you are an adult, a score of 5 out of 9 is positive.

  • If you are over 50, a score of 4 out of 9 is positive.

Being hypermobile is not necessarily a problem, and approximately 5% of the population test positive from the Beighton score. In fact, in many sports a degree of hypermobility provides some advantage, for example; dance, gymnastics, cricket and swimming. If you test positive for hypermobility, you will fall somewhere on the spectrum from being fully functional to being mildly / severely debilitated. Along with the issues associated with the joints, hypermobility is often accompanied by a number of other health issues. These are summarised below:

  • Dizziness and fainting

  • Poor wound healing, abnormal scarring, easy bruising and excessive bleeding

  • Immune system issues such as asthma, hay fever and eczema

  • Gastrointestinal problems such as painful bloating, constipation or diarrhoea

  • Food, medicine and anaesthetic reactions and/or allergies

  • Anxiety and mood disorder problems

  • Hormonal issues

  • Fatigue and sleep issues

  • Headaches

 

The good news is recognising you’re hypermobile at the outset is a start! The Hypermobility Clinic can provide you with the correct diagnosis and ensure you receive a comprehensive and appropriate treatment for all of your symptoms.

Hypermobility comes in many different shapes and forms. The most common of which are the Ehlers-Danlos Syndromes (of which there are 13 sub-types) and Hypermobility Spectrum disorders (which have 4 sub-types ). In addition, hypermobility is seen in other disorders such as Marfan's Syndromes, Loeys-Dietz Syndrome and Stickler Syndrome. Hypermobility is equally present in both males and females, however females tend to be more severely affected.

Ehlers-Danlos Syndrome is commonly abbreviated as EDS, and Hypermobility Spectrum Disorder is commonly abbreviated as HSD.

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The Beighton Score

 
More detailed information

Hypermobility is caused by defective collagen. Collagen is the most abundant protein in our bodies and therefore many organ systems can be affected.

 

Hypermobility Spectrum Disorder

Hypermobility spectrum disorders (HSD) are a group of conditions related to Joint Hypermobility (JH). HSD are diagnosed only after other possible conditions have been excluded, such as Ehlers Danlos Syndrome(s) (EDS) including Hypermobile EDS (hEDS) and the rarer EDS forms.

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Ehlers-Danlos Syndrome

Hypermobile EDS is the most common out of the 13 sub types. However, only for hypermobile EDS, the suspected causal gene has not been elucidated, suggesting it has a complex genotype. The 3 most common sub-types, Hypermobile, Classical and Vascular have an autosomal dominant pattern of inheritance, meaning there is a 50% chance of passing it on to your children. Vascular EDS is associated with fragile tissues (including arteries, muscles and internal organs) that are prone to rupture. In addition, Classical and Kyphoscoliotic EDS can also show cardiac abnormalities, although less common. In high risk sub types, such as Vascular EDS,  a referral for genetic testing is imperative.

For Hypermobile EDS (hEDS), joint hypermobility is the main feature. However, It should be noted that all of the sub-types of EDS have joint hypermobility as a feature in varying degrees.

Subtypes of HSD

  • Generalized (joint) HSD (G-HSD)

  • Peripheral (joint) HSD (P-HSD)

  • Localized (joint) HSD (L-HSD)

  • Historical (joint) HSD (H-HSD)

Subtypes of Ehlers-Danlos Syndrome

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  • Classical EDS (cEDS)

  • Classical-like EDS (clEDS)

  • Cardiac-valvular EDS (cvEDS)

  • Vascular EDS (vEDS)

  • Hypermobile EDS

  • Arthrochalasia EDS (aEDS)

  • Dermatosparaxis EDS (dEDS)

  • Kyphoscoliotic EDS (kEDS)

  • Brittle Cornea Syndrome (BCS)

  • Spondylodysplastic EDS (spEDS)

  • Musculocontractural EDS (mcEDS)

  • Myopathic EDS (mEDS)

  • Periodontal EDS (pEDS)

Co-morbidities of EDS

 

The two most common co-morbidities (associations) linked with EDS are Postural Orthostatic Tachycardia Syndrome (POTS) and Mast Cell Activation Syndrome (MCAS).

POTS is a condition that affects blood flow and blood pressure regulation. POTS is a rapid increase in heartbeat that can come on when standing up from a reclining or sitting position, it is relieved by sitting or lying back down. Heart rate and blood pressure work together to keep the blood flowing at a healthy rate, no matter what position the body is in. The autonomic system in people with POTS can't coordinate the balancing act of blood vessel squeeze and heart rate response. This means the blood pressure can't be kept steady and stable. See a list of the symptoms below.

MCAS occurs when the mast cells in your body become overactive. Mast cells are a type of while blood cell and are part of your immune system. When the body is exposed to stress or danger, the mast calls respond by releasing substances called mediators. Mediators cause inflammation, which in normal amounts help your body heal from an injury or infection. Mast cells are also triggered similarly during an allergic reaction. The mast cells release mediators to remove the allergens. For example, with an allergy to pollen, the mast cells release a mediator called histamine, which induces sneezing to remove the pollen from the body. If you have MCAS, your mast cells are over-reactive and release mediators much too frequently and too often.

Symptoms of POTS

  • heart palpitations (feeling your heart beating or racing)

  • dizziness or lightheadedness

  • fainting (passing out)

  • problems with thinking, memory and concentration (this combination of symptoms) is often called 'brain fog'

  • shaking and sweating

  • weakness and fatigue (tiredness)

  • headaches

  • poor sleep

  • chest pain

  • feeling nauseated (sick)

  • shortness of breath

  • a sense of panic.

Symptoms of MCAS

  • skin: itching, flushing, hives, sweating

  • eyes: itching, watering

  • nose: itching, running, sneezing

  • mouth and throat: itching, swelling in your tongue or lips, swelling in your throat that blocks air from getting to your lungs

  • lungs: trouble breathing, wheezing

  • heart and blood vessels: low blood pressure, rapid heart rate

  • stomach and intestines: cramping, diarrhea, nausea, abdominal pain

  • nervous system: headache, dizziness, confusion, extreme tiredness

 
Diagnosis

At The Hypermobility Clinic a thorough one hour examination is conducted assessing all relevant organ systems as well as family history, medical history, and past history of injuries.

 

To make a clear diagnosis the 2017 International Diagnostic Criteria is used. This criteria incorporates the Beighton Score, skin hyper-extensibility measurements as well as other systemic questions.

In addition, The Hypermobility Clinic conducts a holistic assessment; physical, mental and emotional aspects are evaluated with an empathetic and supportive approach.

 
F.A.Q. (Frequently Asked Questions)

  1. My doctor said I was double jointed, does that mean I have EDS ?
    It's quite possible. but a proper diagnosis cannot be made without a thorough assessment. It could be EDS, HSD or another condition entirely.

     

  2. I suspect I have EDS, what can I do to confirm this ?
    Well as a very rough guide you can follow this questionnaire for a basic screening. However if you suspect a hypermobility disorder, it's well worth getting professionally assessed by a suitably trained health professional. Many of the symptoms can seem somewhat confusing and unconnected.

     

  3. What's the difference between hypermobility and flexibility ?
    The main difference is that hypermobility is due to defective collagen which makes the tendons and ligaments that support the joints stretchier than normal. In some cases leading to multiple joint subluxations or dislocations.  If you have hypermobility, you are born with this condition. Flexibility is trained, whereby the muscle fibres and tendons are lengthened through repeated stretching techniques to extend their range of motion. However flexibility training does not lead to unstable joints.

     

  4. From what age can  you accurately diagnose hypermobility (EDS or HDS) ?
    Some degree of joint hypermobility is common in children, especially in children under the age of 3. As we age we gradually stiffen up. The real noticeable change occurs around puberty when hormones also affect our development. It can be strongly suspected and treated as such in childhood but not accurately diagnosed until adolescence. A provisional diagnosis is highly recommended early in childhood - read my story.

     

  5. Can you be hypermobile without any health problems ?
    Well this is possible, but it's not very common. The human body is a complex machine and all of us have slightly different genetics that make us unique. Some people have very pronounced or wide ranging issues while others may be able to lead a relatively normal life unimpeded by their conditions. In any case it is possible to improve a person's quality of life through proper management and treatment.

     

  6. What role does physiotherapy have in managing hypermobility ?
    Physiotherapy plays an essential role in obtaining successful outcomes for hypermobile clients. Generally speaking, physiotherapy will include gentle stabilisation and strengthening programmes that are vital to protecting the joints and preventing future injuries and pain. This leads to a more functional and active lifestyle.