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There Are New Treatments For Paget's Disease

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By DR. LOREN WISSNER GREENE
New York University School of Medicine
For AP Special Features

Newly available drug treatments are improving the outlook for patients with Paget's disease, a bone-weakening disorder that can cause fractures and bone deformities.

Paget's disease results from overactivity of the cells that break down old bone and replace it with new bone. This excessive activity produces enlarged but weaker bones, which are vulnerable to fracture and deformity. The bones that are most often affected are those of the pelvis, spine, legs and skull.

A newly approved drug for the disease that appears to be effective is alendronate (brand name Fosomax), which inhibits bone breakdown. In addition, calcitonin, a Paget's disease drug that has long been given only by injection, now is available as a nasal inhalant which is much more convenient for patients. Calcitonin is a hormone obtained from salmon. With long-term use, some patients develop resistance to this drug. Drug treatment for Paget's disease may also include etidronate (Didronel) which is used for 3 to 6 months at a time. Gallium nitrate is a new drug that has been used for treatment.

Although the specific cause of Paget's disease is unknown, it tends to run in families, indicating that a genetic factor may be involved. Viral particles have been identified in bone specimens, indicating a possible infectious cause.

The disease is most common in Caucasians of European descent. It also affects black Americans but is rare in persons of Asian descent. It occurs primarily in people over the age of 40 and is most common over age 80.

Paget's disease often causes no symptoms for years. Pain is the most common symptom that brings patients to the physician. The pain can be in the form of a persistent headache (caused by bone pressure on the brain), leg pain or sciatica.

The disease can also cause deformity of the bone with overgrowth of the forehead bones, increased curvature of the spine and frequent fractures. Untreated, Paget's disease can cause hearing loss, as the bones of the inner ear can be affected.

Medical historians have postulated that Beethoven's deafness might have been due to Paget's disease.

An essential step in making the diagnosis is to measure blood levels of alkaline phosphatase, an enzyme that is produced by bone cells. The overactivity of Paget's disease results in excessively high levels of alkaline phosphatase. Sometimes a high alkaline phosphatase on routine testing is the first clue to the diagnosis of Paget's disease.

The physician may also do a bone scan to verify the diagnosis and determine which bones are affected.

Even with drug treatment, surgery may be needed to repair fractures or to repair or replace severely damaged hip and knee bones. Neurosurgery may be needed if deformed skull bones injure the brain or spinal cord.

Because of the possible genetic factor, tests of alkaline phosphatase levels every two or three years are recommended for close relatives with Paget's disease.

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Dr. Loren Wissner Greene is Clinical Associate Professor of Medicine at New York University School of Medicine.

Copyright 1996 Associated Press. All rights reserved. This material may not be published, broadcast, rewritten or redistributed.