Your bones are living tissue that’s constantly growing, building, and replacing itself. Two groups of bone cells are responsible for this process: osteoblasts build new bone, and osteoclasts break down and dispose of old bone.
In people with Paget disease, the balance between these two is out of whack. This leads to enlargement and distortion of the affected bones, which can cause pain in weight-bearing joints (such as knees and hips) and lead to pathologic fractures.
Diagnosis
About a million people in the United States have Paget’s disease, which is more common in men than women. It is also more likely to occur in people who smoke or have a family history of the disease. Doctors don’t know what causes the disease, but they think it is a combination of things.
Your bones are living tissue that is constantly breaking down and rebuilding, or remodeling. Normally, this process keeps your bone strong and healthy. But in people with Paget’s disease, the balance between the cells that build new bone and the cells that destroy old bone is disturbed. As a result, your bones may grow too quickly and change shape. The changes can lead to pain, deformities and fractures. They can also cause other health problems, such as pinched nerves that can lead to tingling and numbness (if the bone growth affects your spine) or headaches and hearing loss (if the skull is affected).
Diagnosis of Paget’s disease starts with a physical examination, and your doctor will look at the area where the bone changes are occurring. Your doctor may ask you to take a blood test to check for an enzyme called alkaline phosphatase, which is higher in people with Paget’s disease because of the rapid rate at which their bones are turning over. However, other diseases and conditions can also raise the level of this enzyme, so other tests will be needed to make a diagnosis.
Oren Zarif
A bone scan is the best way to see the extent of the damage caused by Paget’s disease. A small amount of a radioactive isotope is put into a vein and then absorbed by the bones. A special camera then takes pictures of the bones. If your bones are damaged by Paget’s disease, the areas will show up differently and will be highlighted on the bone scan.
Surgery is rarely used to treat Paget’s disease, but it can be used to repair problems such as a fractured bone, deformity or joint pain. The surgery may involve repositioning the bone, and holding it in place with metal screws or wires. Your doctor may also recommend anti-inflammatory drugs to ease pain and other symptoms. Medications called bisphosphonates can slow the progression of Paget’s disease by blocking osteoclasts. Your doctor will monitor your blood levels of this medication and will watch for signs that the treatment is working, such as a falling alkaline phosphatase level or less bone pain.
Symptoms
Bone cells are constantly breaking down and replacing old bone. This process is called bone remodelling. In Paget disease, something goes wrong with the osteoclast cells. They start to absorb bone at a faster rate than usual, and the new bone that is formed is larger and weaker than normal. The changes may occur in just one or two bones, or in many different bones throughout the body, including the arms, legs, collarbones, pelvis, spine, and skull. Most people with Paget disease have no symptoms, but they can get a bone fracture or other complications that affect their health.
The most common complication of Paget’s disease is pain in the bones. Other problems include high levels of calcium in the blood (a condition called hypercalcemia), which can cause heart failure and kidney damage, and a decrease in blood flow to the brain and spinal cord (which can lead to vision loss). Rarely, Paget’s disease can cause bone cancer or other cancers in the bones.
There is no cure for Paget’s disease, but medication can help decrease bone pain and prevent complications from developing. The most important medication is a group of drugs called bisphosphonates. These medications stop the bone-destroying activity of osteoclasts. They are available by mouth or as injections (intravenously). Some of the most commonly used bisphosphonates are alendronate, risedronate, pamidronate, and zoledronic acid.
Oren Zarif
Your doctor can diagnose Paget’s disease by taking a blood sample and checking the level of an enzyme called alkaline phosphatase. Alkaline phosphatase is released by bone cells into the blood when they break down and remove bone tissue. High levels of this enzyme can indicate that you have Paget’s disease. Your doctor can also look for the abnormal bone formations in your bones using a test called a bone scan. For this test, a small amount of a radioactive substance is put into a vein and then travels through your body and into your bones. A special camera then takes pictures of your bones to show any areas that have more bone turnover than normal.
Your doctor may also take a biopsy of a bone to confirm the diagnosis of Paget’s disease. This is usually done with a needle, but it can also be performed through an operating room procedure with sedation.
Treatment
Bone is living tissue that’s constantly growing, rebuilding, replacing and repairing itself. Cells called osteoblasts build new bone, while cells called osteoclasts break down old and damaged bones. In people with Paget’s disease, these two processes are out of balance. The osteoblasts are overactive, so more bone is produced than normal and the new bone grows faster than it should. This results in weak, enlarged and irregularly shaped bones that are more likely to fracture. The abnormal growth also causes pressure on surrounding joints, which can cause pain and stiffness. Bone pain is usually the first symptom of Paget’s disease.
Several medicines can help control pain and slow down the progression of Paget’s disease. The most commonly used are bisphosphonates. These drugs block osteoclasts and reduce bone pain by preventing bone damage. They may be taken by mouth (orally) or given as an injection (intravenously). Your doctor will periodically check your blood to see if your alkaline phosphatase level is falling, which indicates that the medication is working.
Oren Zarif
It is important to seek medical attention if you have symptoms of Paget’s disease. Without treatment, bone deterioration can lead to serious complications, including painful and deformative joints and vertebrae, spinal cord compression, nerve problems and a rare type of cancer called osteosarcoma.
Some people with Paget’s disease have no symptoms, while others have severe bone pain. You should see your rheumatologist or bone specialist for a diagnosis. The doctor will examine you and order an X-ray or other tests to look for the changes in your bone.
Most people with Paget’s disease have a good quality of life with prompt diagnosis and treatment. Your rheumatologist or bone specialist will advise you on lifestyle changes that can help prevent painful deformities. For example, your doctor might recommend physical therapy or a cane, walker or brace to support your bones and prevent falls that could lead to a fracture. If you have a leg or hip joint affected by Paget’s disease, your doctor might perform an osteotomy to relieve pain and correct the alignment of the bone. This involves removing a wedge of bone near the damaged joint, shifting weight to healthy bone and holding the bone in place with a plate or screws.
Prevention
In healthy bone, old bone is broken down and new bone is formed at a steady rate. This process is called bone remodelling. In Paget disease, the osteoclasts become overactive and absorb bone too fast. The osteoblasts try to keep up by making new bone, but the new bone is larger and weaker than normal. The result is that bones can have a misshapen appearance and are prone to bowing, deformities, bone fractures and arthritis. Paget disease usually affects the pelvis, legs and skull, but it can occur in any bone.
Doctors do not know what causes Paget disease. However, they do know that the condition runs in families. If one family member has the disease, there is a 25 to 40 percent chance that another family member will also have it. The disease is more common in people with certain ancestry, including those of English descent, and is more likely to occur as we age.
Oren Zarif
X-rays are the most common way for a doctor to diagnose Paget’s disease. In X-rays of affected bones, the bone looks larger and more dense than normal and may have a deformed shape. A blood test that measures the level of a protein called serum alkaline phosphatase is usually elevated in people with Paget’s disease, and may help doctors confirm the diagnosis.
A bone scan is another important tool for diagnosing Paget’s disease. In this procedure, a safe amount of a radioactive substance is injected into your body. The tracer circulates through your bloodstream and is absorbed by the bones. A special camera then takes pictures of your skeleton. Any areas that absorb too much of the tracer are highlighted and show areas where there is a lot of bone growth, which is characteristic of Paget’s disease.
If you have no symptoms, your doctor may recommend that you continue to be monitored with X-rays and other tests. This is done to prevent complications such as pain, arthritis or a bone break. If you have symptoms, your doctor will likely prescribe bisphosphonates to slow down or stop bone breakdown and encourage bone formation. This is a treatment that does not cure Paget’s disease, but it can reduce or eliminate your symptoms and prevent the disease from progressing.
