PAGET'S DISEASE OF THE BONE

Introduction

Paget’s disease is a condition which bone formation is speeded up, changing the strength and shape of the bone. The disease is named after a mid-century English surgeon, Sir James Paget, who also identified Paget’s disease of the breast. Bone is a living tissue engaged in a continual process of renewal. Paget’s disease of the bone disrupts this constant remodeling process of old bone being removed and replaced by new bone. Early in the course of the disease, old bone starts breaking down faster than new bone can be built. Over time, your body responds by generating new bone at a faster than normal rate. This rapid remodeling produces bone that’s softer and weaker than normal bone, which can lead to bone pain, deformities and fractures.
Paget’s disease of the bone becomes more common with age. It often begins between the ages 50 and 70. Also it is slightly more common in men than in women No definite evidence exits indicating that paget’s disease is hereditary. However, in some instances, it tends to run in families. The disease usually affects the skull, the spine and the bones in your arms, legs, and pelvis. It may affect only one or two areas of your body, or may be widespread.

Causes, Signs and Symptoms

The cause of Paget’s disease is unknown. Some scientists believe it is related to a viral infection in your bone cells that may be present for many years before the problems appear. They also have discovered several genes that appear to be linked to the disorder. Hereditary factors seem to influence whether you’re susceptible to the disease.
Most people with Paget’s disease have no symptoms. When symptoms do occur, they typically affect each person differently. Affected areas may include:
1. Bones --- pain may be constant, aching, deep and most severe at night. Bone deformities occur such as bowlegs, enlarged head size and fractures.
2. Joints --- wear and tear in your cartilage lining of the affected bones cause pain, swelling, stiffness in your affected joints, and warmth in your skin over those affected joints.
3. Nerves --- enlarged bones can compress your spinal cord or the nerves exiting your brain and spinal cord causing numbness, tingling, weakness, hearing loss, double vision and other neurological problems
Eventually, the rapid bone breakdown and disorganized bone formation slows down, and Paget’s disease appears to “burn out”. Symptoms may come and go but, any bone enlargement or bowing that has occurred will remain.

Diagnostic tests and Treatments

Although there is no way to prevent Paget’s disease, it can be detected before it becomes a serious problem. The following procedures may help detect Paget’s disease of the bone:
1. X-rays --- images can show bone reabsorption, enlargement, and deformities
2. Blood test --- check every 2-3 years after age 40 for elevated alkaline phosphatase level (responsible for forming new bone)
3. Bone Scan --- radioactive tracers can pick up this disease before it can be seen on an X-ray
4. Urine tests --- 24 hour urine collection can determine if an increased amount of hydroxyproline ( another product of bone breakdown ).
Often, people with Paget’s disease of the bone have no symptoms at all and may not require treatment other than regular monitoring. But if signs or symptoms are painful and troublesome, treatment for Paget’s disease of the bone include the following:
1. Occupational or Physical Therapist
2. Medications
3. Healthy Diet
4. Surgery
For more information: www.orthop.washington.edu or www.mayoclinic.com/health/