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.
Patients with joint hypermobility syndrome show various symptoms and signs: joint hyper-mobility; less skin strength; reduced healing of wounds; easy bruising; skin flexibility and likelihood to dislocate easily. Sufferers from this syndrome may develop a chronic pain syndrome with constant and persistent joint pain, with incorrect muscle balances leading to joint stability problems and poor muscle balance. Functionally hypermobile patients can be very limited in normal activities or suffer pain when undertaking them and are unable to join in with vigorous activities or contact sports.
Patient education for sufferers from benign joint hypermobility syndrome is vital if they are to learn to manage the condition through their lives. The joints will not tolerate significant strains and stresses so end range posture should be avoided as this strains the ligaments and can give pain. Repeated lifting of heavy objects is also likely to be unhelpful. Arthritic patients practice joint protection to manage their condition and this is also important for hypermobile patients, so dislocating a shoulder at a party for fun or getting into extreme positions should be avoided. Strong joint stretching may be unwise as in yoga as may activities with a high risk of joint, tissue or skin damage.
As it takes far less trauma to damage a hypermobile joint than a normal one the incidence of acute injuries is higher in these patients as they go about their daily activities. The joint injuries and general painful problems which occur are managed by physiotherapy intervention. The shoulder is a highly mobile but unstable joint in the best circumstances and in hypermobile patients it presents particular problems of stability. The socket is small and the shoulder girdle muscle control must keep the humeral head aligned against it, difficult if the pattern of muscle action is abnormal. Repeated subluxation or dislocation with consequent pain is common and difficult to treat.
Spinal pain, in the neck, low back or thoracic regions, is a common symptom which hypermobile patients complain of, and physiotherapists interpret this as a lack of stabilising muscle control and muscle balance. Physios do not manipulate these patients but mobilizations, core stability work, strengthening weak muscle groups and general exercise are typical approaches. Increasing the usually low muscle tone by gentle weight training or using resistive bands can help joint control in the mid positions and avoid stresses at end ranges. Hyperextension of the knee is a typical problem, leading to joint pain on weight bearing and later to osteoarthritis. Hamstring work to strengthen the muscle opposing the abnormal movement is useful, with patients typically working on the muscle balance of several body areas.
All postures and activities are a challenge to a patient with hypermobility as unsuitable stresses are very easy to apply, causing pain. The patterns of muscle activity are abnormal when the joints are under load, pushing them into end range positions where the ligaments and capsules suffer from strains. Physiotherapy retraining of poor muscle balance can be helpful but patients need to be constantly vigilant and work at their weaknesses persistently. The most important factor overall is patient education as the condition is a long term one and all physical activities challenge the joints.
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.
Patients with joint hypermobility syndrome show various symptoms and signs: joint hyper-mobility; less skin strength; reduced healing of wounds; easy bruising; skin flexibility and likelihood to dislocate easily. Sufferers from this syndrome may develop a chronic pain syndrome with constant and persistent joint pain, with incorrect muscle balances leading to joint stability problems and poor muscle balance. Functionally hypermobile patients can be very limited in normal activities or suffer pain when undertaking them and are unable to join in with vigorous activities or contact sports.
Patient education for sufferers from benign joint hypermobility syndrome is vital if they are to learn to manage the condition through their lives. The joints will not tolerate significant strains and stresses so end range posture should be avoided as this strains the ligaments and can give pain. Repeated lifting of heavy objects is also likely to be unhelpful. Arthritic patients practice joint protection to manage their condition and this is also important for hypermobile patients, so dislocating a shoulder at a party for fun or getting into extreme positions should be avoided. Strong joint stretching may be unwise as in yoga as may activities with a high risk of joint, tissue or skin damage.
As it takes far less trauma to damage a hypermobile joint than a normal one the incidence of acute injuries is higher in these patients as they go about their daily activities. The joint injuries and general painful problems which occur are managed by physiotherapy intervention. The shoulder is a highly mobile but unstable joint in the best circumstances and in hypermobile patients it presents particular problems of stability. The socket is small and the shoulder girdle muscle control must keep the humeral head aligned against it, difficult if the pattern of muscle action is abnormal. Repeated subluxation or dislocation with consequent pain is common and difficult to treat.
Spinal pain, in the neck, low back or thoracic regions, is a common symptom which hypermobile patients complain of, and physiotherapists interpret this as a lack of stabilising muscle control and muscle balance. Physios do not manipulate these patients but mobilizations, core stability work, strengthening weak muscle groups and general exercise are typical approaches. Increasing the usually low muscle tone by gentle weight training or using resistive bands can help joint control in the mid positions and avoid stresses at end ranges. Hyperextension of the knee is a typical problem, leading to joint pain on weight bearing and later to osteoarthritis. Hamstring work to strengthen the muscle opposing the abnormal movement is useful, with patients typically working on the muscle balance of several body areas.
All postures and activities are a challenge to a patient with hypermobility as unsuitable stresses are very easy to apply, causing pain. The patterns of muscle activity are abnormal when the joints are under load, pushing them into end range positions where the ligaments and capsules suffer from strains. Physiotherapy retraining of poor muscle balance can be helpful but patients need to be constantly vigilant and work at their weaknesses persistently. The most important factor overall is patient education as the condition is a long term one and all physical activities challenge the joints.
About the Author:
Jonathan Blood Smyth is Superintendent of a large team of Physiotherapists at an NHS hospital in Devon. 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 Edinburgh or elsewhere in the UK.
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