Treatments |
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Commonality is rare |
Further Tests |
Paget’s disease is a chronic disorder that can result in enlarged and misshapen bones. The excessive breakdown and formation of bone tissue causes affected bone to weaken – resulting in bone pain, misshapen bones, fractures, and arthritis in the joints near the affected bones. Paget’s disease typically is localized, affecting just one or a few bones, as opposed to osteoporosis, for example, which affects all the bones in the body.
Scientists do not know for sure what causes Paget’s disease. In some cases, the disease runs in families, and so far two genes have been identified that predispose affected people to develop Paget’s disease. In most cases, however, scientists suspect that environmental factors play a role. For example, scientists are studying the possibility that a slow-acting virus may cause Paget’s disease.
An estimated one million people in the U.S. have Paget’s disease, or about 1.3 people per 100 men and women age 45 to 74. The disease is more common in older people and those of Northern European heritage. Men are about twice as likely as women to have the disease. Research suggests that a close relative of someone with Paget’s disease is seven times more likely to develop the disease than someone without an affected relative.
Many patients do not know they have Paget’s disease because they have no symptoms. Sometimes the symptoms may be confused with those of arthritis or other disorders. In other cases, the diagnosis is made only after complications have developed.
Symptoms can include:
Any bone or bones can be affected, but Paget’s disease occurs most frequently in the spine, pelvis, legs, or skull. Generally, symptoms progress slowly, and the disease does not spread to normal bones.
Paget’s disease is almost always diagnosed using x rays but may be discovered initially by either of the following tests:
If a blood test or bone scan suggests Paget’s disease, the affected bone(s) should be x rayed to confirm the diagnosis.
Early diagnosis and treatment are important to minimize complications. Siblings and children of people with Paget’s disease may wish to have an alkaline phosphatase blood test every 2 or 3 years starting around the age of 40. If the alkaline phosphatase level is higher than normal, a bone scan may be used to identify which bone or bones are affected and an x ray of these bones is used to verify the diagnosis of Paget’s disease.
The outlook for people diagnosed with Paget’s disease is generally good, particularly if treatment is given before major changes in the affected bones have occurred. Treatment can reduce symptoms but is not a cure. Osteogenic sarcoma, a form of bone cancer, is an extremely rare complication that occurs in less than one percent of all patients with Paget’s disease.
Paget’s disease may lead to other medical conditions, including:
Paget’s disease is not associated with the following disorder:
The following types of medical specialists are generally knowledgeable about treating Paget’s disease:
Drug Therapy: The Food and Drug Administration (FDA) has approved several medications to treat Paget’s disease. The medications work by controlling the excessive breakdown and formation of bone that occurs in the disease. The goal of treatment is to relieve bone pain and prevent progression of the disease. People with Paget’s disease should talk to their doctors about which medication is right for them. It is also important to get adequate calcium and vitamin D through diet and supplements as prescribed by your doctor, except for patients who have had kidney stones.
Bisphosphonates are a class of drugs used to treat a variety of bone diseases. Of the six bisphosphonates currently available to treat Paget’s disease, the most commonly prescribed are the four most potent: Actonel1, Fosamax, Aredia, and Reclast. Didronel and Skelid may be appropriate therapies for selected patients, but are less commonly used. None of these drugs should be used by people with severe kidney disease.
Calcitonin is a naturally occurring hormone made by the thyroid gland. The medication may be appropriate for certain patients but is less effective than bisphosphonates and seldom used. The nasal spray form of this medication is not approved for the treatment of Paget’s disease.
Surgery: Medical therapy before surgery helps decrease bleeding and other complications. Patients who are having surgery should discuss pretreatment with their physician. Surgery may be advised for three major complications of Paget’s disease:
Complications resulting from enlargement of the skull or spine may injure the nervous system. However, most neurological symptoms, even those that are moderately severe, can be treated with medication and do not require neurosurgery.
Diet and Exercise: There is no special diet to prevent or help treat Paget’s disease. However, according to the National Academy of Sciences, everyone over age 50 should get 1,200 mg of calcium and at least 400 International Units (IU) of vitamin D every day to maintain a healthy skeleton. People over the age of 70 need to increase their vitamin D intake to 600 IU. People with a history of kidney stones should discuss calcium and vitamin D intake with their physician.
Exercise is important because it helps preserve skeletal health, prevent weight gain, and maintain joint mobility. Patients should discuss any new exercise program with their doctor before beginning, to ensure that undue stress on affected bones is avoided.