Pagets Disease

Pagets Disease is a chronic condition that results in enlarged and deformed bones. Although any bone in the body may be affected, the most common sites are the spine, skull, pelvis, thighs, and lower legs. The disease does not usually spread to other normal bones.

Causes

Pagets Disease is caused by a malfunction in bone formation. Normally, bones are constantly being broken down by cells called osteoclasts, and rebuilt by cells called osteoblasts. With Pagets, bones are broken down abnormally fast, and new bone replacement is loose and bulky, instead of strong and compact. These poorly formed bones may become weak, and may bend over time. The exact cause of this bone malformation is unknown, but it is associated with heredity. Some experts believe that Paget's may be activated early in life by a viral infection.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.
  • Family history of Paget's disease, especially in a parent, sibling, or child
  • Race: Anglo-Saxon, European
  • Age: 40 or older
  • In US, residence in northern states (rather than southern states)

Symptoms

In the early stages, most people have no noticeable symptoms. In later stages, symptoms may include:
  • Swelling or deformity of a limb
  • Increased head size
  • Bowing of a limb
  • Curvature of the spine
  • Chronic bone pain, especially legs, hips, or spine
  • Frequent fractures
  • Problems with walking or gait
  • Headaches, facial pain, or hearing loss when the skull bones are affected
  • Swollen or painful joints due to damaged cartilage
  • Numbness, tingling, pain, or weakness from pressure on nerves
Depending on the bone site, Pagets may result in:
  • Arthritis, usually osteoarthritis or gout
  • Carpal tunnel syndrome
  • High calcium in blood
  • Kidney stones
  • Hearing problems
  • Dental problems
  • Rarely:
    • Eye problems
    • Congestive heart failure
    • Bone cancer called sarcoma

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include:
  • Blood test for alkaline phosphatase or calcium
  • X-ray - a test that uses radiation to take a picture of the bones
  • Bone scan - a series of pictures of bones taken after injection of a small amount of radioactive material

Treatment

Treatment may include:
Medication
Medications may include:
  • Pain medicine – such as acetaminophen or nonsteroidal anti-inflammatory drugs
  • Bisphosphonates – usually by tablet or by injection
  • Calcitonin – administered by injection or nasal spray
Surgery
Surgery may be required if you have one of the following conditions:
  • Bone fracture
  • Severe degenerative arthritis
  • Bone deformity
Self Care
Recommendations include:
  • Intake of 1000-1500 mg calcium per day
  • Adequate exposure to sunshine to promote vitamin D production
  • Intake of 400 mg of vitamin D per day
  • Regular exercise to maintain skeletal health, joint mobility, and normal body weight
  • Avoidance of excess mechanical stress on involved bones
  • A splint for an area at high risk for fracture

Prevention

There is no known way to prevent the onset of Pagets Disease. People with primary family members who have Pagets Disease are encouraged to have a routine alkaline phosphatase blood test every two to three years after age 40.