What Is Fibromyalgia?
20:38 Sep 07 2010
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Fibromyalgia is a chronic condition of widespread pain and profound fatigue. The pain tends to be felt as diffuse aching or burning, often described as head to toe. It may be worse at some times than at others. It may also change location, usually becoming more severe in parts of the body that are used most.
The fatigue ranges from feeling tired, to the exhaustion of a flu-like illness. It may come and go and people can suddenly feel drained of all energy – as if someone just “pulled the plug”.
Fibromyalgia is a common illness. In fact, it is more common than rheumatoid arthritis and can even be more painful. Prevalence of Fibromyalgia: A Survey in Five European Countries (see www.fmauk.org/prevalence for details) put the prevalence of FM at between 2.9 and 4.7%. People with mild to moderate cases of fibromyalgia are usually able to live a normal life, given the appropriate treatment.
If symptoms are severe, however, people may not be able to hold down a paying job or enjoy much of a social life. The name fibromyalgia is made up from “fibro” for fibrous tissues such as tendons and ligaments; “my” indicating muscles; and “algia” meaning pain.
My form is severe, complicated with the EDS and I can not take any of the drugs used to control or ease it.
What is EDS?
20:08 Sep 07 2010
Times Read: 616
Introduction
Ehlers-Danlos Syndrome (EDS) is a heterogeneous group of heritable disorders of connective tissue, characterised by skin extensibility, joint hypermobility and tissue fragility. There are different types of EDS and these were reclassified into six major types in 1997. They are classified according to signs and symptoms with each type running true in a family thus an individual with one type will not have a child with a different type.
EDS is caused by a defect in a protein called collagen, of which there are more than 30 different types. It is a protein and is the main building block of the body providing strength and support. Some examples are ligaments, tendons and cartilage. Consequently, if the collagen itself is defective, it can produce many problems throughout the body.
Prevalence
EDS is known to affect both males and females of all races and ethnic backgrounds. The exact incidence is not known, but is estimated at 1 in 5000, however, it may be more common.
Diagnosis
Diagnosis is based on the presenting symptoms and family history of a patient. Many EDS sufferers, however, do not fit conveniently into the definition of a specific type, and are frequently misdiagnosed.
A skin biopsy may be taken to confirm the diagnosis and determine the type. Of the six types of EDS, there is only one type, the Hypermobility type, that does not have a specific test. In each of the other five cases, the specific gene mutation has been identified and can be tested for. Where the Hypermobility type is suspected, it is possible to examine the collagen in the skin sample for abnormalities.
Prognosis
The prognosis depends largely on the type of EDS the patient has. For instance, life expectancy in the Vascular Type (formerly type lV) is generally shortened to around 40 years due to the rupture of large blood vessels and the major organs. Pregnancy can be life-threatening in the Classical (formerly types I and II) and Vascular Types.
SYMPTOMS
Skin
Hyperextensibility
Cutaneous hyperextensibility (stretchy skin) characterises all types of EDS, except for the Vascular Type (type lV), which has noticeably translucent skin with visible veins. When skin is over-stretched it still retains normal elastic recoil and snaps back once released. This is best tested at the neck, elbows, or knees.
Cutaneous fragility
Easy splitting of the skin is particularly common in Classical Type. Gaping, 'fish-mouth' or 'cigarette paper' scars follow minimal trauma over sites of bony prominence and areas prone to trauma such as the forehead, chin, elbows, knees, and shins.
Epicanthic folds
These are additional symmetrical folds of skin at the inner aspects of the eyes producing apparent broadening of the nose.
Molluscoid pseudotumours
These are firm, fibrous lumps measuring up to 2 - 3 cm which develop over pressure points such as the elbows and knees.
Spheroids
Approximately one third of affected individuals describe small, firm nodules like 'ball-bearings' just beneath the skin (sub cutis). These consist of fibrotic and calcified fat which overlay bony areas such as the shins.
Piezogenic papules
These small, soft, skin-coloured lumps appear on the side of the heel when standing and disappear when the foot is elevated. Although usually symptom-less they can occasionally be painful.
Varicose veins
These are more common in many types of EDS, than in the general population.
Joints
Joints are hypermobile (lax) due to the extra elasticity of the ligaments resulting from the collagen abnormality. The degree of hypermobility is assessed using the Beighton Scale 2. A score of 5/9 or higher defines hypermobility.
The laxity of the joints makes them susceptible to subluxation and dislocation, of which the patient often has a history. This often occurs without significant trauma as would be necessary to cause dislocation in somebody who does not have EDS. Those with the Vascular Type do not generally exhibit joint laxity.
Pain is a common feature with hypermobile joints, even when skeletal X-Rays are normal.
Bruising and Haematomas
Easy bruising, accompanies most forms of EDS, often as a result of minimal trauma. This implies increased fragility of dermal blood capillaries and poor structural integrity of the skin. When bruising presents in a child it may be incorrectly attributed to non-accidental injury.
Mitral Valve Prolapse
This is quite common and should be diagnosed by echocardiography, CT or MRI.
Less Common Features
Arterial, uterine and intestinal ruptures may occur due to the fragility of tissues. These ruptures are more commonly found in the Vascular Type, but also occur in other types. Inguinal and hiatus hernias are also relatively common.
Scoliosis (bend in the spine) may be present at birth or can develop in later life.
Gum disease.
Gastrointestinal diverticula.
TREATMENT AND MANAGEMENT
This depends on the presenting symptoms but simple precautionary measures will greatly lessen the chances of accidental trauma, scarring and bruising. It is important to carefully balance the advantages of less frequent injuries and the disadvantages of over-protection in a child. Simple measures like padding of the lower legs and elbows in children may reduce the number of injuries.
Surgery and skin suture should be undertaken with great care as fragile tissues may tear. Sutures need to be left in longer than normal.
Bracing may be used to support unstable joints. Orthopaedic surgery may be necessary but is not always successful.
Physiotherapy and Occupational Therapy advice may be sort to strengthen muscles and teach aids to daily living.
Psychological
The main problem with having Ehlers-Danlos Syndrome is that the person can look very fit and may often not be believed that they have joint pain etc. Diagnosis is often delayed and misdiagnosis is relatively common. Some forms of EDS may be misdiagnosed as child abuse/self-inflicted injury.
Where there is severe skin involvement scarring can be severe and the person needs to learn to cope with disfigurement.
Information taken from the Ehlers-Danlos Support Group.
COMMENTS
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Angelus
00:51 Sep 09 2010
appreciated.