Our ligaments, tendons, discs and skin are made up of forms of collagen, one of the most important structural proteins in our bodies. This gives our tissues the ability to heal, its elasticity, its integrity and its strength, allowing us to have strong and healthy joints and skin which will put up with the stresses life puts upon them. Collagen is also responsible for the strength and integrity of our arteries and many other bodily structures. Human populations show great variation in collagen function from those who are very stiff jointed to those who are very mobile jointed or "double jointed".
Ehlers-Danloss syndrome is caused by an abnormality in the way collagen is produced and acted upon in the body, causing an inheritable deficiency in the strength of the substance. 10 forms of EDS are known to exist, with much overlap, and EDS Three is considered the same as benign joint hypermobility syndrome, called benign because the serious changes such as in the arteries are not present in this form. Very hypermobile joints are the most obvious sign of this syndrome, with a smooth, flexible skin which tends to heal slowly and scar poorly in terms of wide and thin scars.
Joint hypermobility syndrome exhibits a series of symptoms and signs including some fragility of the tissues, slower and poorer wound healing, a propensity to joint dislocations, flexible skin and hypermobility of the joints. Along with these findings patients often complain of multiple chronic joint pains which can develop into a pain syndrome. Poor muscle balance around the major joints causes instability and incorrect muscle patterning which can cause joint and muscle pain and limit the ability to do functional things. Contact sports or vigorous physical activities are not appropriate for this group of people.
Advice to patients is very important and education should be a priority to allow them to manage their condition over the long term. Avoidance of excessive or repeated heavy lifting and other movements which put stresses and strains on the hypermobile joints is important and patients should avoid end-range postures which strain the lax ligaments. Joint protection, as in arthritic joints, is essential and patients should avoid showing off by performing "party pieces" such as dislocating a joint voluntarily or other extreme tissue manoeuvres. Stretching the joints strongly may worsen the underlying problems, and any activity carrying high risk of skin damage should be avoided.
The stresses and strains of daily life and recreational activities tend to result in more acute injuries and pain complaints in hypermobile patients which are managed by physiotherapy to the joints and muscles. Even in normal circumstances the shoulder is very mobile yet unstable but in hypermobile patients the lax connective tissue makes the joint very unstable and difficult to control. The shoulder and surrounding muscle must keep the large ball of the arm bone aligned with the small socket during large movements and this is difficult with hypermobility, leading to abnormal muscle patterns and pain. It is common to have pain due to this and to repeated dislocations.
Spinal pain is common in hypermobility perhaps secondary to the difficulty in keeping good stability of the joints as forces act upon them. Gentle mobilizations can be performed for local problems but manipulation is likely to be unwise. General exercises to keep the joint moving can be helpful but end range and stretching exercises should be avoided. Gentle weight training and core stability work may help stabilise the spine and other joints, increasing the muscle tone to hold joints in their mid ranges and prevent excessive play. The knees may bend back excessively and suffer arthritic change in time, so work on the hamstrings to counteract this in standing is an option. Often patients need to work on several areas, maintaining muscle strength and control.
Overall, hypermobile patients have a challenging condition to manage, as over-stress of their joints in any activity can lead to pain. When their joints are stresses they respond by recruiting abnormal muscle patterns, allowing joints to be placed in unhelpful positions and causing them to suffer from end range strains. Patient education is the most important factor in self management, although physiotherapists can help with advice and postural training.
Ehlers-Danloss syndrome is caused by an abnormality in the way collagen is produced and acted upon in the body, causing an inheritable deficiency in the strength of the substance. 10 forms of EDS are known to exist, with much overlap, and EDS Three is considered the same as benign joint hypermobility syndrome, called benign because the serious changes such as in the arteries are not present in this form. Very hypermobile joints are the most obvious sign of this syndrome, with a smooth, flexible skin which tends to heal slowly and scar poorly in terms of wide and thin scars.
Joint hypermobility syndrome exhibits a series of symptoms and signs including some fragility of the tissues, slower and poorer wound healing, a propensity to joint dislocations, flexible skin and hypermobility of the joints. Along with these findings patients often complain of multiple chronic joint pains which can develop into a pain syndrome. Poor muscle balance around the major joints causes instability and incorrect muscle patterning which can cause joint and muscle pain and limit the ability to do functional things. Contact sports or vigorous physical activities are not appropriate for this group of people.
Advice to patients is very important and education should be a priority to allow them to manage their condition over the long term. Avoidance of excessive or repeated heavy lifting and other movements which put stresses and strains on the hypermobile joints is important and patients should avoid end-range postures which strain the lax ligaments. Joint protection, as in arthritic joints, is essential and patients should avoid showing off by performing "party pieces" such as dislocating a joint voluntarily or other extreme tissue manoeuvres. Stretching the joints strongly may worsen the underlying problems, and any activity carrying high risk of skin damage should be avoided.
The stresses and strains of daily life and recreational activities tend to result in more acute injuries and pain complaints in hypermobile patients which are managed by physiotherapy to the joints and muscles. Even in normal circumstances the shoulder is very mobile yet unstable but in hypermobile patients the lax connective tissue makes the joint very unstable and difficult to control. The shoulder and surrounding muscle must keep the large ball of the arm bone aligned with the small socket during large movements and this is difficult with hypermobility, leading to abnormal muscle patterns and pain. It is common to have pain due to this and to repeated dislocations.
Spinal pain is common in hypermobility perhaps secondary to the difficulty in keeping good stability of the joints as forces act upon them. Gentle mobilizations can be performed for local problems but manipulation is likely to be unwise. General exercises to keep the joint moving can be helpful but end range and stretching exercises should be avoided. Gentle weight training and core stability work may help stabilise the spine and other joints, increasing the muscle tone to hold joints in their mid ranges and prevent excessive play. The knees may bend back excessively and suffer arthritic change in time, so work on the hamstrings to counteract this in standing is an option. Often patients need to work on several areas, maintaining muscle strength and control.
Overall, hypermobile patients have a challenging condition to manage, as over-stress of their joints in any activity can lead to pain. When their joints are stresses they respond by recruiting abnormal muscle patterns, allowing joints to be placed in unhelpful positions and causing them to suffer from end range strains. Patient education is the most important factor in self management, although physiotherapists can help with advice and postural training.
About the Author:
Jonathan Blood Smyth is a Superintendent Physiotherapist at an NHS hospital in the South-West of the UK. He specialises in orthopaedic conditions and looking after joint replacements as well as managing chronic pain. Visit the website he edits if you are looking for physiotherapists in London.
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