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Tuesday, August 12, 2008

What is Paget’s disease?

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What is Paget’s disease?

* Paget’s (pronounced paj-ets) disease affects bones.
* Throughout a person’s life bone is constantly breaking down and growing back. With Paget’s disease the normal process of bone growth is changed. The bone breaks down more quickly, and when it grows again it is softer than normal bone.
* Soft bones can bend or break more easily. The area affected by Paget’s disease can become shorter because the bone bends.
* With Paget’s disease the bone can also grow larger than before.
* Paget’s disease can affect any bone, but usually affects the skull, the hip and pelvis bones and bones in the legs and back.

Paget’s disease causes a malfunction in the normal process of bone remodelling. Normally, bone is continually breaking down and rebuilding. This usually slow process of bone destruction and growth is somehow altered in Paget's disease.

When an area of bone is destroyed in a person with Paget’s disease, the bone that replaces it is soft and porous. Soft bone can be weak and easily bend, leading to shortening of the affected part of the body. The bone replacement also takes place very quickly and excess bone may be formed. This can cause the bone to get larger, be painful and break easily.

The bone affected by Paget’s disease also tends to have more blood vessels than normal. This causes an increase in the blood supply to the area, and as a result the area may feel warmer than usual.

The disease can affect any bone but more commonly affects the spine, pelvis, skull, thighbone (femur – pronounced fee-mer) and shinbone (tibia – pronounced ti-bee-ah).

Paget’s disease can lead to other medical conditions including osteoarthritis, kidney stones and heart disease.

Paget’s disease is also called osteitis (pronounced ah-stee-eye-tis) deformans. It is named after Sir James Paget, an English doctor who first described the disease in 1876.

How common is Paget’s disease?

* The exact number of people with Paget’s disease is not known.
* Men are more likely to be affected by Paget’s disease than women.
* It usually affects people over age 40.

Paget’s disease is estimated to affect 3% of people over 40. However the exact number is not known because many people who have it do not know it. It occurs all over the world but is more prevalent in some areas, such as in Europe and Australia.

It tends to affect men more than women, and usually those over the age of 40.

What are the warning signs of Paget’s disease?

* Because Paget’s disease comes on slowly many people do not know they have it.
* If you have Paget’s disease the first warning sign may be pain in or over a bone.
* The area may feel extra warm.
* You may feel also feel tired.
* If Paget’s disease affects the bones in a leg, the shape of the bone may change and your legs may bend or bow out.
* If it affects the skull, your head may get bigger. It can also cause you to have trouble hearing.
* Paget’s disease can cause your bones to break more easily.
* Often Paget’s affects only one or two bones.

In many cases, Paget's disease takes a very mild course and a person with it may not have any symptoms. Those who do have symptoms may be affected in various ways.

If you have Paget’s disease your bones may break easily because they are weakened. Your bones may also bend, and if your leg bones are affected you may notice that your legs bow, or a leg may appear to shrink. If your spine is involved, you may feel pain in your back. If the bones in your spine bend or grow larger than usual this can put pressure on your nerves, and you may feel pain or numbness in other areas of your body also.

If Paget’s disease affects your skull, your head may increase in size from front to back. Hearing loss may result if there is involvement of some of the small bones in the middle ear or pressure is placed on the nerves related to hearing.

In late stages of the disease, your hip joint may become damaged if the bones of your pelvis have been involved.

Usually only one or a few bones are affected. However, the disease can be widespread and affect all bones. Because of the increased number of blood vessels in bones affected by Paget’s disease your heart has to work harder to pump blood to them. If many bones are affected this can cause strain on your heart and lead to other problems.

What causes Paget’s disease?

* The exact cause of Paget’s disease is not known.
* Some people with Paget’s disease have other family members with it.

Some studies have shown that up to 30% of people with Paget’s disease have other family members with it. The disease is also more prevalent in areas where much of the population is of Anglo-Saxon descent This has led some researchers to believe there may be a genetic factor in the development of Paget’s disease. A slow acting virus may also be involved, though the virus has not yet been identified.

What can you do about Paget’s disease?

* If your doctor thinks you have Paget’s disease, he or she may perform a physical examination and order tests such as x-rays or blood tests.
* Your doctor may also refer you to a rheumatologist (pronounced room-a-tol-o-jist). A rheumatologist is a doctor who has received special training in the diagnosis and treatment of problems involving the joints, muscles and other parts of the body.
* Treatment is done to reduce the rate of bone loss, build up new bone and lessen pain.
* Learn as much as you can about this disease. Speaking with your doctor or other people who are specialists in arthritis care can provide you with the information you need.

At this time there is no cure for Paget’s disease. Therefore treatment is designed to control the symptoms and change the rate of bone growth. Establishing the correct diagnosis is important because something can be done to manage most forms of arthritis and most therapies work best when started early in the disease.

Your doctor may be able to diagnose Paget's disease based on your medical history and a physical examination. Your doctor may order certain tests to help confirm the diagnosis and to determine what areas of bone are affected. These tests can include x-rays, bone scans and blood and urine tests.

A diagnosis of Paget's disease may be made when you see your doctor for another problem. You may have no symptoms at this time, but routine tests may show that you have the disease.

Once diagnosed with Paget’s disease you may be referred to a rheumatologist or other doctor who specializes in bone disorders, or an endocrinologist, who specializes in metabolic and hormonal disorders (which affect the rates of growth in your body).

There is no cure for Paget’s disease but there are treatments that can help you manage the pain and slow the disease. Your active involvement in developing your treatment plan is essential.

For more information about Paget's Disease, click here.

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