Hypermobility Syndrome - The Facts

     

    The Hypermobility Syndrome (HMS) is one of a group of inherited
    diseases which affect the connective tissues of the body. It is a multi-system disorder which may result in a wide variety of clinical features and disabilities.

    Fibrous proteins (collagens, elastins, fibrillins) give the body
    its strength. A defect in genetic information which determines the biochemical structure and strength of those proteins may cause structural weakness in muscle, tendons, ligaments cartilage, bone, the blood vessels, eyes and; skin.
     
    The clinical effects depend on the functional particular tissue
    affected. Joints may become lax, and stable and hypermobile with increased tendency to dislocation and fun mobility to the effects of injury. Bones may become osteoporotic, predisposing to fractures. The body-shape may take on characteristic body proportions (called "Marfanoid") with long slender limbs, twisting of the spine and chest deformity.

    Skin shows increased stretchiness and the blood vessels (vasculature) may also be affected in certain of the diseases associated with HMS, such as the Ehlers-Danlos syndrome, with involvement of the heart and major vessels. Eye may occur as dislocation of the lens in the Marfans syndrome where the lens ligament is lax and unable to hold the lens in a stable manner.


    The hypermobile joint is vulnerable this means that if the
    hypermobile back is used excessively, this can be subject to prolapsed discs, stress fractures, spinal and narrowing and other mechanical problems. People with severe forms of hypermobility syndrome (particularly those with greater degrees of tissue laxity and fragility) may need a restricted life because they
    tissues are so fragile.


    Symptoms tend to be similar in hypermobility syndrome their
    respective of the cause. Due to the weakness in muscle, ligaments, tendons and; cartilage etc, there may be joint pain, dislocation of joints, and fractures. In any hypermobile joint, "over-use" i can cause pain and loss of function.

    The same is true of the back. Stress fractures of bone are not
    uncommon. Joints and/or muscle pain can be a prominent symptom. Hypermobility may be a serious or potential source of problems in children. Many will develop osteoarthritis in time. With age, joined hypermobility declines, but other complications resulting from HMS may arise, such as secondary osteoarthritis;
    osteoporosis with reluctant fractures; and loss of balance particularly in the older person, which may result in falls, especially if there is also impaired vision.

    CARE NEEDS


    (i) People with severe forms of hypermobility syndrome may be
    infrequent or constant pain that is worsened by movements, especially those involved in physical effort such as lifting, moving around etc. Joints may dislocate doing quite simple movements e.g. with the handle shoulders.

    When the tissues are damaged, physically demanding activities
    are also painful and give rise to care needs from another person. Depression may ensue -based partly because of the pain, partly because of the inability to perform some normal daily tasks and to enjoy a normal life. Periods of rest throughout the day may be required especially after what a normal person might
    consider as a modest about of physical activity. Falls may occur so that certain activities such as bathing, using stairs, may also need to be supervised, particularly in elderly people who syndrome.


    (ii) Main meal preparation, especially cutting up vegetables,
    opening to yours, lifting hands and using taps may prove to be difficult in those with more advanced disease.

    MOBILITY CONSIDERATIONS

    (i) Because the connective tissues are lax and fragile they may
    be easily injured. The combination of joint pain (especially in the knees) and instability (back-bending, knees etc) may make walking difficult and there may also be problems with balance.

    (ii) The ability to walk may be limited in people with severe
    forms of the syndrome, requiring the use of walking aids (cane, crutches) or wheelchair. The person may have a tendency to lose balance and fall with difficulties getting up.

    DURATION OF NEEDS


    (i) Many of the clinical manifestations of Hypermobility syndrome result from sudden injuries to the soft tissues which heal within weeks or months, either spontaneously or after medical, surgical or physiotherapy treatment. Once severe irreversible damage
    has taken place joins the outlook is largely determined by whether that joint is amenable to joint replacement. Disability associated with chronic painful conditions (e.g. back pain or widespread osteoarthritis) is less likely to respond to treatment and may persist indefinitely.