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Paget disease is a condition of unknown cause that involves alternating cycles of bone destruction and bone reconstruction. This disease tends to slowly get worse over time.

What is going on in the body?

Paget disease alternately destroys and then reconstructs bone. Normal bone is replaced with excessive amounts of abnormal bone. The abnormal bone has less calcium and is weaker.

What are the signs and symptoms of the disease?

Paget disease usually comes on gradually. In the early stages, 90% of people have no symptoms. Later, the most common symptom is a dull, mild pain in the affected bones. Often the pain is constant, present at all times of the day, and made worse by exertion. The most common locations are the skull, neck, hips, or pelvis. Because blood flow is increased in the affected bones, the affected bones may feel warm. Bones often get larger in Paget disease. When the skull is affected, there may be an increase in hat size.

• bone fractures
• dizziness
• headaches
• hearing impairment
• ringing in the ears

Over time, deformities such as bowlegs, a barrel chest, and other bone problems can occur.

What are the causes and risks of the disease?

The cause of Paget disease remains unclear, though genetics definitely play some part, as evidenced by the tendency of the disease to occur in more than one family member.

Some theories about the cause of Paget disease include:

• an autoimmune disorder, in which a person's immune system attacks his or her own body
• a hormone disorder, in which the body produces abnormal amounts of hormones that regulate bone maintenance
• an inflammatory condition or an immune response to injury
• a metabolic disorder, or abnormalities in physical and chemical processes within the body
• a slow viral infection

Paget disease is thought to affect about 3% of the population older than 40. The disease is twice as common in males. Paget disease is also more common in the United Kingdom, Australia, and New Zealand, and less often seen in Asia, Africa, and Scandinavia. In the U.S., it is more common in the northern states than in the southern states.

What can be done to prevent the disease?

There is no known way to prevent Paget disease.

How is the disease diagnosed?

Diagnosis of Paget disease begins with a history and physical exam. In many cases, the disease is diagnosed from routine X-rays that were taken for other reasons. Paget disease can also be seen on other special X-ray tests, such as a bone scan. Blood tests may be ordered to help in the diagnosis.

What are the long-term effects of the disease?

• anemia, or low red blood cell counts
• arthritis
• congestive heart failure, in which a weakened heart is unable to pump blood effectively to body organs
• hearing impairments, including deafness
• kidney stones
• paralysis
• visual impairments, including blindness

The abnormal bone structure causes weak bones which fracture easily. Bone cancer can develop in affected areas and is usually fatal.

What are the risks to others?

Paget disease is not contagious. Some cases of Paget disease are thought to be inherited.

What are the treatments for the disease?

Often, a person with no symptoms is not treated. If symptoms occur, they can be treated with nonsteroidal anti-inflammatory medicines (NSAIDs) such as ibuprofen (i.e., Advil, Motrin).

Other kinds of medication may be used to inhibit bone destruction and reduce pain in people with more severe disease. These include injectable calcitonin (i.e., Miacalcin), nasal spray calcitonin (i.e., ) and biphosphonates, such as etidronate (i.e., Didronel), risedronate (i.e., Actonel), alendronate (i.e., Fosamax), and pamidronate (i.e., Aredia.

Other treatments that are used for Paget disease include:

• eating a diet rich in calcium and vitamin D
• following an exercise program prescribed by the healthcare professional
• taking calcium, magnesium, and boron supplements to support bone growth
• taking glucosamine, a supplement that is also used for arthritis

Long-term treatment may be needed if the disease is severe, especially if the person has involvement of the nerves or spinal cord. Adaptive equipment, such as canes and crutches, may be needed. Physical therapy may be ordered to maintain mobility and function. Surgery may be required for severely affected bones.

What are the side effects of the treatments?

All medicines can have side effects, including allergic reactions. For example frequent side effects of calcitonin include nausea and skin flushing. Herbal remedies may interfere with prescription medicines and cause bleeding problems. Surgery can be complicated by bleeding, infection, or a reaction to the anesthetic.

What happens after treatment for the disease?

Paget disease often requires lifelong treatment. If the symptoms go away, treatment may be stopped for a while.

How is the disease monitored?

A person with Paget disease needs to make regular visits to a healthcare professional. Blood and urine tests may be performed. Any new or worsening symptoms should be reported to the healthcare professional.