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It is widely accepted that hypermobility is generally inherited, in fact, around three quarters of people affected have a previous family history of it.  Some may remain ‘asymptomatic’ or only experience a few symptoms, for others their hypermobility may cause symptoms that  have a large impact on everyday life, to the point where everyday tasks become difficult to manage.

 

It is not, as yet, fully understood why the presentation of hypermobility varies so greatly from person to person, but we do know that many factors are involved.  These factors include variable gene expression, which infulences the range of signs and symptoms that can occur, muscle tone, our sense of joint proprioception, our age, our gender and our hormones.  We also know that a symptomatic person will have a varying combination of the three types of hypermobility (see below), and that, of these three, one type will often predominate, which may cause symptoms to vary extensively (Bird H. 2007 / Knight I. 2011 / Celletti C. 2013)

 

 

Bony hypermobility – Characteristics


• Shallow joint sockets that may dislocate easily

• Less hypermobility overall, but profound at a smaller range of joints

• Involves the joint articulating surfaces





Collagen-related hypermobility - Characteristics



• More likely to be hormonally dominant – for example more likely to dislocate during progesterone changes in menstruation in females.

• Stretchy skin.

• May be more likely to have problems with other collagen structures, e.g. bladder (weakness),lungs (asthma), bowels (IBS).



Neuropathic hypermobility - Characteristics

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• Late walkers, problems in maintaining core stability, clumsy gait.

• If linked to the bony type of hypermobility, it is possible that some of the bones of the spine may    be abnormal.

• Sometimes linked to proprioceptive defect and new research is highlighting problems with the reflex arc. (Ferrell and Ferrell 2010)

 

 

 

 

Even in the same family, one member of a family may develop a variety of complications, whilst another hypermobile family-member may have no problems at all. The ‘range’ of severity within family members is also still a mystery.

 

As we have discussed previously, symptoms relating to hypermobility may be part of Heritable Disorders of Connective, such as osteogenesis imperfecta, which causes bone fragility, Marfan’s syndrome, which involves the heart, eyes and blood vessels and, of course, Ehlers–Danlos syndrome, or Joint Hypermobility syndrome.  

 

It is, however, also possible to ‘aquire’ hypermobility and, if problems are subsequently experienced, to acquire symptomatic-hypermobility.

 

How is it possible to ‘acquire’ hypermobility?

 

A non-hypermobile person can acquire, what would be considered ‘advantageous’ hypermobility through intensive stretching of the joints, for example, dancers and gymnasts put themselves through gruelling  workouts in order to produce graceful, flowing movements. 

 

How is it possible to acquire ‘symptomatic’ hypermobility?

 

It can be hard to understand how a person can go through life ‘asymptomatic’ and then suddenly become symptomatic, for example experiencing the onset of pain or joint subluxation. 

 

In some cases, a person may have an underlying collegen defect, which, although it has not caused problems to-date, has left them pre-disposed to developing symptoms later in life. In others, a change of job, lifestyle, or injury, may trigger the onset of pain.

 

Some will ‘acquire’ symptomatic hypermobility through conditions such as Lupus or Rheumatoid Arthritis (where, otherwise healthy, collagen fibres are attacked). Some may acquire problematic symptoms of hypermobility through ‘overuse’ of particular joints in the body, for example gymnasts, or ballet dancers, who train their bodies to be hypermobile, or massage therapists who may develop hypermobile joints in their thumbs, fingers and wrists from giving deep tissue massage.  Research has also identified other triggers including, biomechanical problems and muscle deconditioning, caused by a sudden change from an active to less active life-style.

 

In females, hormonal fluctuations at key points in their lives, such as puberty, the onset of hormonal contraception, or the peri-menoupause can trigger symptomatic hypermobility. It is now recognised that, for many women, symptoms significantly increase at certain points during their monthly menstruation cycle. 

 

In men, it is thought that their increased ability to build more muscle mass and the presence of testosterone offers a larger degree of protection.  It should be remembered however, that men are also often less vocal on forums and statistically less likely to visit their GP to pursue treatment or a diagnosis and, therefore, may be under-represented in statistics.

 

'Some drug treatments for acne, which commonly affects young males, contain hormones that may influence the joints.  In an older age group, hormones used in the treatment of prostatic cancer may also have a deleterious effect on hypermobile joints.  Some diuretic or water tablets are also distantly related to progesterone' (Quote: Prof. H. Bird) 

 

Confusion regarding ‘acquired’ versus ‘inherited’ hypermobility is understandable because the term ‘hypermobility syndrome’, is currently used by many to describe Joint Hypermobility syndrome (which we now know is one of the Heritable Disorder of Connective Tissue (HDCT), along with Ehlers-Danlos syndrome, Marfan syndrome etc.) and is also used to describe acquired problematic biomechanical symptoms (not HDCT related) / hypermobility resulting from disorders such as Lupus or Rheumatoid Arthritis:

 

‘Whenever symptoms commence, and irrespective of the cause of the hypermobility, the term ‘hypermobility syndrome’ is used to describe the condition.' (Quote: Prof. R. Grahame CBE, MD, FRCP, FACP)

 

It would, perhaps, be beneficial for medical professionals to begin using more precise definitions such as:

 

1)  Simple or 'general' hypermobility - Those who are hypermobile, but asymptomatic, possibly finding it of benefit

 

2)  Joint Hypermobility syndrome - The most common of the Heritable Disorders of Connective Tissue

 

3)  Acquired Hypermobility - symptomatic hypermobility that has occured due injury or overuse, or due to disorders such as Lupus, Rheumatoid Arthritis or Down's syndrome.

 

 

 

The following are some examples, which demonstrate how hypermobility can seem to become problematic over a period of time.

 

First we will look at a person who has experienced symptoms, which, with hindsight, can often be traced back to early childhood (names have been changed to protect identity):

  

It was always clear to Alice’s family that something was not quite right – she was slow to walk, and preferred to sit in the “W” position (a child’s way of compensating for the poor balance associated with weak core muscle strength).  As she grew older, her parents noticed that she experienced easy bruising and poor wound healing, and would carry out ‘party-tricks’ with her bendy fingers, but they never understood the cause. 

 

In the absense of an obvious family history, and with no more-visable symptoms, such as dislocations, having occured, it is perhaps understandable that no underlying HDCT was checked for.  It is, after all, notoriously difficult to diagnose EDS-H or JHS in young children, as most children are flexible in their early years. From puberty, however, symptoms became far more apparent, she experienced pain, joint instability and autonomic dysfunction, particularly around her time of menstruation. Despite repeated trips to their GP no diagnosis was made and, although pain killers were prescribed, no appropriate management plan was implemented.  As is the case for many, the lack of awareness within the medical community, meant that Alice was not diagnosed until after the birth of her first child, by which time, for her, unexplained injuries, chronic long term pain, fatigue and anxiety had become commonplace.

 

The second example we consider Harriet, who is hypermobile, but went through childhood untouched by any negative hypermobility symptoms (i.e. she was asymptomatic). Harriet’s ballet teacher spotted her potential early on, as hypermobility allowed her to achieve the desired ballet positions easily. Out of the blue, at age 17, Harriet twisted her ankle during a dance routine and the injury refused to heal, she began to walk with a limp, causing a knock on effect to her knee and hip and the physical strain on her joints from relying on crutches soon caused further problems. Harriet began to develop pain in her shoulders and wrists as well. Within months she found herself in a downward spiral as her body became deconditioned and pain and muscle fatigue became a daily occurance.  

 

When an injury does not heal properly, a patient’s gait may change to compensate for the injury, causing a knock-on effect to other joints for example in the knees or hips. Alternatively, a patient may resort to surgery, only to find that this goes on to trigger problems in other joints. 

K. J. Murray and P. Woo, write in the Oxford Journals:

 

‘A promising dancer or gymnast, selected because of their extended range of motions and ability to create beautiful lines, is cut off before they have reached their prime, due to hypermobility related injury. '   'It is not uncommon to gain a history of a child who has been proficient at sport, gymnastics or ballet who has had to give up participation because of musculoskeletal symptoms or problems increasing at the time of increased demands of training and frequency of competition.' 

 

Once a hypermobile dancer, or athlete, is injured, the likelihood of future injury is high.  The crucial aspect, in an ideal world, lies in educating teachers to screen students for hypermobility, in order that suitable strengthening techniques can be taught and muscles can be trained to support the joint’s extra range of movement, thus preventing injury from happening in the first place.

 

Whatever the cause of symptomatic-hypermobility, early diagnosis, advice, education and appropriate investigations where necessary, are essential to tools in helping those affected better manage their symptoms and live life to the full.

 by edhs.info