Paget's disease more often affects men and those over the age of 40 years.

The disease occurs in two stages:

Stage 1: - Vascular Stage: bone tissue is broken down but the spaces left are filled with blood vessels and fibrous tissues instead of new, strong bone.
Stage 2: - Sclerotic Stage: the blood-filled fibrous tissue becomes hardened and bone-like but it is weaker and more fragile than healthy bone.

An extremely variable disease, in that it can occur in part or all of one or many bones. The sites most commonly affected, are the hip bone (pelvis) and shin bone (tibia), although; the thigh bone (femur), skull, spinal bones (vertebrae) and collar bone (clavicle) are also frequently affected.

Causes
The cause of the disease is unknown, although early viral infection and genetic causes have been thought to play a major role. Although the disease occurs worldwide, there does seem to be a clearly defined geographical distribution. Being more common in Australia, New Zealand and the UK (especially in the north of England); and extremely rare in countries such as South Africa, Norway and Japan. The reasons for this, are not yet fully understood.

Symptoms
Paget's disease does not always produce symptoms, as it does tend to progress slowly, with many of the initial symptoms going unnoticed. When symptoms are noticed, bone pain, stiffness and repeated fractures are the most common of problems. This discomfort is virtually continuous and is often worse in the evenings, or following a bout of exercise. In the case of the skull being affected, one is likely to suffer from repeated headaches and gradual loss of hearing.

At a later stage the affected bones become enlarged, tender and warm to the touch. Deformities in the form of bowing, vertebral collapse and an increase in skull size are all common, depending on which bones are affected.

Treatment
Although there is no cure for the disease, non-steroidal anti-inflammatory drugs may be prescribed to relieve the pain. Severe pain can often be alleviated through regular calcitonin injections - a hormone made naturally in the parathyroid glands, - which controls the calcium and phosphorous levels in the blood, and commences the production of new bones. Chemotherapy is another method commonly used to prevent further complications, or as a means of suppressing bone cell prior to surgery, as in the case of hip replacement or spinal decompression. Orthopedic surgery would be advised if the need arose to correct specific deformities.

 

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