Paget's Disease of Bone

Risk: Because the disease tends to run in families, there may be a genetic component. Paget's disease is a common disorder of middle-aged and elderly individuals; it rarely occurs in individuals under age 40. Men are twice as likely as women to develop Paget's disease (Altman).

Incidence and Prevalence: About 1% of persons older than 40 in the US have the disease, and the prevalence increases with age (Altman). Paget's disease is more common in Europe (but not in Scandinavia) and in Australia and New Zealand than in the Americas, Asia, and Africa. It is especially common in England.

History: Although the disorder may not produce any symptoms, individuals may report bone pain in any bone that is affected by Paget's disease. Depending on which bones are affected, individuals may report headaches, radicular pain (sciatica), hip pain, muscular pain, or arthritic pain if the cartilage of joints adjacent to the affected bone becomes damaged. Patients may also report hearing loss or dizziness if Paget's disease affects the skull. Nerve pain may occur from pressure on the skull or spine. Patients may comment that they need a larger hat size.

Physical exam: Physical examination may reveal hearing loss (if Paget's disease affects the skull), pressure on the nerves (if the disease affects the skull or spine), increased head size, bowing of the long bones in the legs, curvature of the spine (scoliosis), and damage to cartilage in the joints. The forehead and brow may look more prominent, and the person may take shorter or unsteady steps if the legs or hips are affected. Veins on the scalp may appear more prominent, possibly due to increased blood flow through skull bones.

Tests: If Paget's disease is suspected based on physical examination, the diagnosis can be confirmed by x-ray, blood tests to measure levels of an enzyme called alkaline phosphatase, urine tests to measure enzyme levels (such as Urinary-telopeptide and hydroxyproline assay), and bone scans (radionuclide test using technetium). In individuals with the disorder, the bones appear thickened and porous on x-ray, and the alkaline phosphatase level is elevated. Bone scans are useful in determining the extent and activity of the condition. If a bone scan suggests Paget's disease, the affected bone(s) should undergo x-ray or CT scanning to confirm the diagnosis.

Individuals with Paget's disease may benefit from physical and occupational therapy to reduce pain and maximize functional abilities. However, the condition is frequently asymptomatic and does not require any special rehabilitation measures (Hadjipavlou).

When indicated, therapists teach individuals pain control techniques and establish a gentle stretching and strengthening program that the individual should perform daily. These exercises can be performed independently to help reduce impairment due to pain and bone deformity, as well as secondary arthritis. Individuals can also perform low-impact aerobic exercise to increase strength and endurance as well as to improve stamina. Individuals may use heat to decrease joint stiffness and pain. Individuals unable to perform exercises because of pain may benefit from an aquatic therapy program in a heated pool. Aquatic exercise provides resistant exercise while reducing the stress through an individual's bones and joints because of the buoyant properties of water. Individuals may also be taught to use assistive devices such as a cane or walker.

Occupational therapists address activities of daily living such as dressing and bathing and may provide individuals with assistive devices to lessen the strain of common daily tasks. Individuals may require consultation with a speech therapist if hearing is affected.

For further information about management of this condition and rehabilitation outcome please refer to "Paget's Disease" (Bender).