• What is Paget's disease?
• Who gets Paget's disease?
• What are the symptoms of Paget's disease?
• How is the diagnosis made of Paget's disease?
• What is the prognosis (outlook) with Paget's disease?
• What other medical problems can be caused by Paget's disease?
• What is the relationship between Paget's disease and osteoporosis?
• What type of medical specialists are generally knowledgeable about treating Paget's disease?
• What is the goal of drug treatment?
• What medications are approved for Paget's disease?
• When may surgery be recommended for Paget's disease?
• How do diet and exercise help in Paget's disease?
• Paget's Disease At A Glance

What is Paget's disease?

Paget's disease is a chronic bone disorder that typically results in enlarged, deformed bones due to irregular breakdown and formation of bone tissue. Paget's disease can cause bones to weaken and may result in bone pain, arthritis , bone deformities and fractures. Paget's disease is also known as osteitis deformans.

Who gets Paget's disease?

Paget's disease is rarely diagnosed in people under 40. Men and women are affected equally. The prevalence of Paget's disease ranges from 1.5 to 8% depending on age and country of residence. The prevalence of familial Paget's disease (where more than one family member has the disease) ranges from 10 to 40% in different parts of the world. Because early diagnosis and treatment is important, after age 40, brothers, sisters and children of someone with Paget's disease may wish to have an alkaline phosphatase blood test every 2 or 3 years. If the alkaline phosphatase level is above normal, other tests such as a bone-specific alkaline phosphatase test, bone scan, or x-ray can be done.

What are the symptoms of Paget's disease?

Many people do not know they have Paget's disease because they have a mild case of the disease with no symptoms. Sometimes, 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:

• Bone pain -- the most common symptom. Bone pain can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints.
• Headaches and hearing loss -- may occur when Paget's disease affects the skull.
• Pressure on nerves -- may occur when Paget's disease affects the skull or spine.
• Increased head size, bowing of limb, or curvature of spine -- may occur in advanced cases.
• Hip pain -- may occur when Paget's disease affects the pelvis or thighbone.
• Damage to cartilage of joints -- may lead to arthritis.

How is the diagnosis made of Paget's disease?

Paget's disease may be diagnosed using one or more of the following tests:

• X-rays -- Pagetic bone has a characteristic appearance on X-rays.
• Alkaline phosphatase test -- An elevated level of alkaline phosphatase in the blood can be suggestive of Paget's disease.
• Bone scan -- Useful in determining the extent and activity of the condition. If a bone scan suggests Paget's disease, the affected bone or bones should be x-rayed to confirm the diagnosis.

What is the prognosis (outlook) with Paget's disease?

The outlook is generally good, particularly if treatment is given before major changes in the affected bones have occurred. Paget's disease occurs most frequently in the spine, skull, pelvis, thighs, and lower legs. In general, symptoms progress slowly, and the disease does not spread to normal bones. Treatment can control Paget's disease and lessen symptoms but is not a cure.

What other medical problems can be caused by Paget's disease?

Paget's disease may lead to other medical conditions, including:

• Arthritis -- Long bones in the leg may bow, distorting alignment and increasing pressure on nearby joints. In addition, Pagetic bone may enlarge, causing joint surfaces to undergo excessive wear and tear. In these cases, pain may be due to a combination of Paget's disease and osteoarthritis .
• Hearing loss -- Loss of hearing in one or both ears may occur when Paget's disease affects the skull and the bone that surrounds the inner ear. Treating the Paget's disease may slow or stop hearing loss. Hearing aids may also help.
• Heart disease -- In severe Paget's disease, the heart works harder to pump blood to affected bones. This usually does not result in heart failure except in some people who also have hardening of the arteries.
• Kidney stones -- Kidney stones are somewhat more common in patients with Paget's disease.
• Nervous system pressure -- Pagetic bone can cause pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord.
• Bone sarcoma -- Paget's disease is associated with the development of osteosarcoma, a malignant tumor of bone. It is, fortunately, rare and occurs in less than 1% of all patients with Paget's disease. However, when there is a sudden onset or worsening of pain, sarcoma should be considered.
• Teeth loosening -- When Paget's disease affects the facial bones, the teeth may become loose. Disturbance in chewing may also occur.
• Decreased vision -- Rarely, when the skull is involved, the nerves to the eye may be affected, causing some loss of vision.

What is the relationship between Paget's disease and osteoporosis?

None. Paget's disease is not associated specifically with osteoporosis . Although Paget's disease and osteoporosis can occur in one and the same person, they are completely different disorders. But, despite their marked differences, many treatments for Paget's disease can also be used to treat osteoporosis.

What type of medical specialists are generally knowledgeable about treating Paget's disease?

The following types of medical specialists are, as a rule, generally knowledgeable about treating Paget's disease:

• Endocrinologists -- Internists who specialize in hormonal and metabolic disorders.
• Rheumatologists -- Internists who specialize in joint and muscle disorders.
• Other specialists -- Orthopedic surgeons, neurologists, and otolaryngologists (physicians who specialize in ear, nose, and throat disorders) may be called upon to evaluate specialized symptoms.

What is the goal of drug treatment?

The goal of treatment is to control Paget's disease activity for as long a period of time as possible. Treatment options include aspirin, other anti-inflammatory medications, pain medications, and medications that slow the rate of bone turnover, such as calcitonin (Calcimar, Miacalcin), etidronate (Didronel), alendronate (Fosamax), and pamidronate (Aredia). A newer drug, risedronate (Actonel), appears to have a powerful effect against severe Paget's disease. It has the added advantage of requiring only 2 months of initial treatment and is given by mouth.

What medications are approved for Paget's disease?

The U.S. Food and Drug Administration (FDA) has approved the following treatments for Paget's disease:

Bisphosphonates

Five bisphosphonates are currently available. As a rule, bisphosphonate tablets should be taken with 6-8 oz of tap water on an empty stomach. None of these drugs should be used by people with severe kidney disease.

• Didronel (etidronate disodium) -- Tablet; approved regimen is 200-400 mg once daily for 6 months; the higher dose (400 mg) is more commonly used; no food, beverages, or medications for 2 hours before and after taking; course should not exceed 6 months, but repeat courses can be given after rest periods, preferably of 3-6 months duration.
• Aredia (pamidronate disodium) -- Intravenous; approved regimen 30 mg infusion over 4 hours on 3 consecutive days; more commonly used regimen 60 mg over 2-4 hours for 2 or more consecutive or non-consecutive days.
• Fosamax (alendronate sodium) -- Tablet; 40 mg once daily for 6 months; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down (patient may sit).
• Skelid (tiludronate disodium) -- Tablet; 400 mg (two 200 mg tablets) once daily for 3 months; may be taken any time of day, as long as there is a period of 2 hours before and after resuming food, beverages, and medications.
• Actonel (risedronate sodium) -- Tablet; 30 mg once daily for 2 months; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down (patient may sit).

Calcitonin

• Miacalcin is administered by injection; 50 to 100 units daily or 3 times per week for 6-18 months.

When may surgery be recommended for Paget's disease?

Medical therapy prior to surgery helps to decrease bleeding and other complications. Patients who are having surgery should discuss pre-treatment with their physician. There are generally three major complications of Paget's disease for which surgery may be recommended.

• Fractures -- Surgery may allow fractures to heal in better position.
• Severe degenerative arthritis -- If disability is severe and medication and physical therapy are no longer helpful, joint replacement of the hips and knees may be considered.
• Bone deformity -- Cutting and realignment of Pagetic bone (osteotomy) may help painful weight-bearing joints, especially the knees.

How do diet and exercise help in Paget's disease?

In general, people with Paget's disease should receive 1000-1500 mg of calcium, adequate sunshine, and at least 400 units of vitamin D daily. This is especially important in patients being treated with bisphosphonates. Patients with a history of kidney stones should discuss calcium and vitamin D intake with their physician.

Exercise is very important in maintaining skeletal health, avoiding weight gain, and maintaining joint mobility. Since undue stress on affected bones should be avoided, patients should discuss any exercise program with their physician before beginning.

Historical Note

Paget's disease is named after the English surgeon, Sir James Paget, who described it in 1877. Paget also discovered the worm that causes trichinosis and described what is called Paget's disease of the breast . Together with Rudolph Virkow in Germany, Paget was one of the founders of pathology.

Paget's Disease At A Glance

• Paget's disease is a chronic bone disorder.
• Paget's disease frequently causes no symptoms.
• Paget's disease can cause pain in the bones or joints, headaches and hearing loss, pressure on nerves, increased head size, bowing of limb, or curvature of spine.
• Tests used to diagnose Paget's disease include x-rays, blood tests, and bone scanning.
• Paget's disease can lead to other medical conditions.
• Treatment options include aspirin, other anti-inflammatory medications, pain medications, and medications that slow the rate of bone turnover.

For further information, you can contact:

The Paget's Disease Foundation
120 Wall Street
Suite 1602
New York, NY 10005-4001
212-509-5335
212-509-8492 Fax
e-mail: pagetfdn@aol.com

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