Paget's disease of bone

Description

Paget's (PAJ-its), disease of bone interferes with your body's normal recycling process, in which new bone tissue gradually replaces old bone tissue. Over time, the bones become fragile and misshapen. The pelvis, skull, spine and legs are most commonly affected.

The risk of Paget's disease of bone increases with age and if family members have the disorder. However, for reasons unknown to the doctors, the disease has become less common in recent years and is less severe when they are not developed. Complications may include bone fractures, hearing loss, and the compression of the nerves of the spine.

Bisphosphonates — drugs that are used to strengthen the bones weakened by osteoporosis are the mainstay of treatment. Surgery may be needed if complications occur.

Symptoms

Most of the people who have Paget's disease of bone have no symptoms. When symptoms do occur, the most common complaint is bone pain.

Because this is a condition that causes your body to generate new bone faster than normal, the rapid bone remodeling occurs less organized and weaker than normal bone, which can lead to bone pain, deformities and fractures.

The disease may affect only one or two areas of your body, or it may be widespread. Its signs and symptoms, if any, will depend on the affected part of your body.

  • The Pelvis. Paget's disease of the bones of the pelvis can cause pain in the hip.
  • The skull. An excessive growth of the bones in the skull can cause hearing loss, or headaches.
  • Of the spine. If your spine is affected, nerve roots can be compressed. This can cause pain, numbness and tingling in an arm or a leg.
  • Of the leg. As the bones weaken, they can bend — causing you to be bowlegged. Enlarged and misshapen bones of the legs can put additional strain on the nearby joints, which can lead to osteoarthritis in the knee or the hip.

When to see a doctor

Talk with your doctor if you have:

  • Pain in the bones and joints
  • Tingling and weakness in a limb
  • Deformities of the bones
  • Unexplained hearing loss, especially if it is only on one side

Causes

The cause of Paget's disease of bone is unknown. Scientists suspect a combination of genetic and environmental factors contribute to the disease. Several genes that appear to be linked to the disease.

Some scientists believe that Paget's disease of bone is associated with a viral infection in the bone cells, but this theory is controversial.

Risk factors

Factors that may increase your risk of Paget's disease of bone include:

  • Age. People over the age of 50 are more likely to develop the disease.
  • Sex. Men are more often affected than females.
  • National origin. Paget's disease of bone is most common in England, Scotland, central Europe and Greece, as well as the countries colonized by European immigrants. It is rare in Scandinavia and Asia.
  • The history of the family. If you have a family member who has the disease of bone Paget's disease, you are more likely to develop the condition.

Complications

In most cases, Paget's disease of bone is progressing slowly. The disease can be managed effectively in almost all of the people. Possible complications include:

  • Fractures and deformities. Affected bones break more easily, and extra blood vessels in these deformation of the bones to make them bleed more during surgery repair. Bones of the leg may arc, which can affect your ability to walk.
  • Osteoarthritis. Deform the bones can increase the amount of stress in the nearby joints, which can lead to osteoarthritis.
  • Neurological problems. When Paget's disease of bone occurs in an area where nerves pass through the bone, such as the spinal column and the cranium, the excessive growth of the bones can compress and damage the nerve, causing pain, weakness or tingling in an arm or a leg, or loss of hearing.
  • Heart failure. In severe cases, your heart has to work harder to pump blood to the affected areas of your body. Sometimes, this increased workload can lead to heart failure.
  • The bone cancer. Bone cancer occurs in up to 1% of people with Paget's disease of the bone.

Paget's disease of bone

Diagnosis

During the physical exam, your doctor will examine areas of your body that are causing the pain. He or she may also order x-rays and blood tests that can help confirm the diagnosis of Paget's disease of bone.

Imaging tests

The changes in the bones can be revealed by:

  • The x-ray. The first sign of Paget's disease of bone is often found anomalies in the X-ray performed for other reasons. The X-ray images of bones may show areas of breakdown of bones, enlargement of the bones and deformities that are characteristic of the disease, such as the inclination of their long bones.
  • Bone scan. In a bone scan, the radioactive material is injected into the body. This material travels to the places where the bones most affected, and until the light in the scan images.

Laboratory tests

People who have Paget's disease of the bone usually have elevated levels of alkaline phosphatase in the blood, which can be revealed by a blood test.

Treatment

If you don't have symptoms, you may not need treatment. However, if the disease is active, indicated by an elevation of the alkaline phosphatase level — and is affecting the high-risk sites in your body such as the skull or spine, your doctor can recommend a treatment to prevent complications, even if you have no symptoms.

Drugs

Drugs for Osteoporosis (bisphosphonates) are the most common treatment for Paget's disease of bone. Bisphosphonates are typically given by injection into a vein, but also may be taken by mouth. When taken orally, the bisphosphonates are generally well tolerated, but may cause irritation to the stomach.

Bisphosphonates administered intravenously are:

  • Zoledronic acid (Zometa, Reclast)
  • Pamidronate (Aredia)
  • Ibandronate (Boniva)

Oral bisphosphonates include:

  • Alendronate (Fosamax, Binosto)
  • Risedronate (Actonel, Atelvia)

Rarely, bisphosphonate therapy has been linked to severe muscle, joint or bone pain, which may not resolve when the medication is discontinued. Bisphosphonates may also increase the risk of a rare condition in which a section of jaw dies and decays, usually associated with active dental disease or oral surgery.

If you cannot tolerate bisphosphonates, the doctor may prescribe calcitonin (Miacalcin), a natural hormone involved in calcium regulation and bone metabolism. Calcitonin is a drug that is administered by injection or nasal spray. Side effects may include nausea, flushing of the face and the irritation at the site of the injection.

Surgery

In rare cases, surgery may be needed to:

  • Help to heal fractures
  • Replace joints damaged by severe arthritis
  • Realign the deformation of the bones
  • Reduce the pressure on the nerves

Paget's disease of bone often causes the body to produce too many blood vessels in the affected bones, increasing the risk of a severe loss of blood during an operation.

If you are scheduled for a surgery that involves bones affected by Paget's disease of the bone, your doctor may prescribe medications to reduce the activity of the disease, which may help to reduce the blood loss during the surgery.

Self-care

To reduce the risk of complications associated with Paget's disease of bone, try these tips:

  • To prevent falls. Paget's disease, which puts you at high risk of bone fractures. Ask your doctor for advice about the prevention of falls. He or she may recommend the use of a cane or a walker.
  • Fall-proof your home. Remove slippery floor coverings, the use of non-slip mats in the bathtub or shower, hide cables, and install handrails on the stairs and grab bars in the bathroom.
  • Eat well. Make sure that your diet includes adequate levels of calcium and vitamin D, which helps absorb the calcium in the bones. This is especially important if you are taking a bisphosphonate. Check your diet with your doctor and ask whether you should take vitamins and calcium supplements.
  • Exercise regularly. Regular exercise is essential to maintain joint mobility and bone strength. Talk with your doctor before you begin an exercise program to determine the type, duration and intensity of exercise for you. Some of the activities you can put too much stress on your bones affected.

Preparing for your appointment

Most people with Paget's disease of the bones do not have symptoms and is diagnosed when a chest x-ray or a blood test for another reason reveals signs of Paget's disease of bone. In some cases, you may be referred to a doctor who specializes in metabolic and hormonal disorders (endocrinologist), or joint and muscle disorders (rheumatologist).

Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test. Make a list of:

  • Your symptoms , including any that seem unrelated to the reason for your appointment
  • Key personal information , including major stresses, recent life changes, and the medical history of the family
  • All medications, vitamins or supplements that you are taking, including dose
  • Questions to ask your doctor

Have a friend or family member, if possible, to help you remember the information they give you.

For Paget's disease of bone, some basic questions to ask your doctor include:

  • What is likely causing my symptoms?
  • What are other possible causes of the symptoms?
  • What tests do I need?
  • Is my condition likely temporary or chronic?
  • What is the best course of action?
  • What are the alternatives to the primary approach you're suggesting?
  • How can I best manage this condition with my other health conditions?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Your doctor might ask you some of the following questions:

  • Has had numbness or tingling?
  • How about the muscle weakness?
  • Any new headaches?
  • Has your hearing worsened recently?
Symptoms and treatment of Paget's disease of bone