Paget's disease of bone is a condition affecting how your bone breaks down and rebuilds. Healthy bone metabolism allows for old bone to be recycled into new bone throughout your life.

In Paget's disease of bone, the rate at which old bone is broken down and new bone is formed is distorted. Over time, Paget's disease of bone may result in your bones becoming fragile and misshapen.

Paget's disease of bone is more common with age. Many older people with discomfort in their bones and joints assume the discomfort is a natural part of aging, and don't seek treatment. But to prevent the most serious complications of Paget's disease of bone, it's important to get treatment as soon as possible after symptoms appear.

Paget's disease of bone affects each person differently. Most people with Paget's disease have no symptoms. When symptoms do occur, they typically present in specific areas affected by the disease, although they may be widespread.

The condition usually affects the skull, the spine, and the bones in your arms, legs and pelvis. The disease may affect only one or two areas of your body, or may be widespread. Your symptoms, if any, will depend on the part of your body that's affected, including:

• Bones. Pain in the affected bones is the most common symptom of Paget's disease of bone. Your pain may be constant, aching and deep, and may be most severe at night.
• Joints. Paget's disease may damage the cartilage lining the joints near your affected bones. This wear and tear often leads to osteoarthritis in your affected joints, a condition that may cause pain, swelling and stiffness.
• Nerves. Enlarged bones can compress your spinal cord or the nerves exiting your brain and spinal cord. Pain resulting from nerve compression is more severe than the bone pain associated with Paget's. The location of the pain caused by nerve compression depends on the nerve that's affected. You may notice pain radiating from your lower back into your legs (sciatica) if the lower region of your spine is affected. Pressure on a nerve can also cause numbness, tingling, weakness, hearing loss and double vision.

Other signs and symptoms of Paget's disease may include:

• Warmth in your skin over the affected area
• Neurological problems, such as hearing loss, headache and rarely, vision loss
• Bone deformities, such as bowlegs and enlarged head size
• Fractures

When to see a doctor
Talk to your doctor if you have:

• Symptoms of Paget's disease of bone, such as pain in your bones and joints
• Symptoms of pinched nerves, such as pain, tingling and weakness
• Bone deformities

If you have Paget's disease of bone, contact your doctor if you experience:

• Signs and symptoms of the disease affecting your skull, such as hearing or vision loss, double vision, facial weakness or numbness
• Loss of bladder or bowel control accompanied by weakness in your legs, which can indicate severe spinal damage
• Sudden or severe pain or a change in the pain that you've been experiencing

Even after you've reached your full height, your bones don't stop growing. Bone is living tissue engaged in a continual process of renewal. During this constant process called remodeling, old bone is removed and replaced by new bone. Paget's disease of bone disrupts this process.

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.

Scientists haven't identified a cause of Paget's disease of bone, though they have discovered several genes that appear to be linked to the disorder.

Some scientists believe Paget's is related to a viral infection in your bone cells that may be present for many years before problems appear. Hereditary factors seem to influence whether you're susceptible to the disease.

Age and heredity are the only known risk factors for Paget's disease of bone. Occasionally, the disease runs in families. People older than 40 are the most likely to develop Paget's disease. Men are more commonly affected than are women.

In most cases, Paget's disease of bone progresses slowly. The disease can be managed effectively in nearly all people and is rarely fatal. Possible complications include:

• Osteoarthritis. This degenerative joint disease is a common long-term complication of Paget's disease.
• Heart failure. Unusually extensive Paget's disease may force your heart to work harder to pump blood to the affected areas of your body. In people with pre-existing heart disease, this increased workload can lead to heart failure.
• Sarcoma. A rare complication is a bone cancer known as sarcoma, also called osteosarcoma or osteogenic sarcoma, which may develop in bones affected by Paget's disease. This complication occurs in less than 1 percent of people with Paget's disease and usually doesn't develop until many years after the onset of Paget's.

If the disease affects bones in your head, you may experience hearing loss, loss of teeth and, rarely, loss of vision.

Most people with Paget's disease of the bone don't have any symptoms, are and diagnosed when an X-ray or blood test taken for another reason reveals signs of Paget's disease.

However, in some cases you may experience pain and other symptoms that lead you to schedule an appointment with your doctor. In some cases, you may be referred immediately to a doctor who specializes in metabolic and hormonal disorders (endocrinologist) or in bone and muscle disorders (rheumatologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment. Even though possible changes such as hearing loss, constipation, or areas of skin warmth may seem unrelated to your pain, your doctor will want to know about them.
• Make a list of your key medical information, including other conditions you're being treated for and the names of the medications that you're taking. Your doctor will also want to know the medical history of your close relatives — especially if one of your family members has had Paget's disease of bone.
• Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Prepare a list of questions so that you can make the most of your limited time with your doctor. List your questions from most important to least important in case time runs out. For Paget's disease of bone, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• Other than the most likely cause, what are possible causes for my symptoms or condition?
• What kinds of tests do I need now?
• Will this condition get worse over time?
• Do I need treatment?
• If you don't think treatment is indicated now, how will you know when I do need to start being treated?
• Will I need follow-up testing? On what schedule?
• I have these other health conditions. How can I best manage them together?
• Are there any restrictions that I need to follow?
• What diet and lifestyle changes would you recommend?
• Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist?
• Are my children and other close relatives at risk of developing this condition? What can they do to catch the disease early, if it develops?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• When did you first begin experiencing symptoms? How severe are they?
• Have your symptoms been continuous, or occasional? Do they seem to be getting worse?
• Where is your pain located? Is your skin warm over the areas of pain?
• Have you been taking pain relievers or using other approaches to relieve your discomfort? Does anything seem to work?
• Do you have any additional signs and symptoms, such as hearing loss, fatigue, changes in your bowel habits or appetite?
• Has anyone in your family been diagnosed with Paget's disease of bone?
• Have you been diagnosed with any other health conditions?

What you can do in the meantime
To ease your discomfort while you wait to see your doctor, try taking a nonsteroidal anti-inflammatory drug (NSAID) such as aspirin or ibuprofen (Advil, Motrin, others). Acetaminophen (Tylenol, others) also may relieve mild to moderate pain.

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Paget's disease of bone

Detecting Paget's disease of bone early may help prevent serious complications. The following procedures may help your doctor detect Paget's disease of bone:

• Blood test. If you have a sibling or parent with Paget's disease, talk to your doctor about requesting an alkaline phosphatase blood test every two to three years after age 40. Alkaline phosphatase is produced by bone cells that are responsible for forming new bone and is elevated in most people with Paget's disease.
• X-rays. The first indication of Paget's disease is often either an elevated alkaline phosphatase level or abnormalities found on X-rays done for other reasons. X-ray images of your bones can show areas of bone reabsorption, enlargement of the bone and deformities that are characteristic of Paget's disease, such as bowing of your long bones. Your doctor may be able to base a diagnosis of Paget's disease on the bone X-ray findings.
• Bone scan. In some cases, your doctor may recommend a bone scan. Bone scans can pick up Paget's disease before it can be seen on an X-ray. Doctors also use bone scans to determine which bones are affected. In a bone scan, radioactive tracers are injected into your body. The tracers are taken up by your bones and give off radiation that's captured by a special camera, which produces a picture of your skeleton. Areas of bone that are affected by Paget's disease are darker than normal on the scan.

If you don't have symptoms, you may not need treatment. However, if the disease is active — indicated by an elevated alkaline phosphatase level — and is affecting high-risk sites in your body, such as your skull or spine, your doctor may recommend treatment to prevent complications, whether or not you have symptoms.

Treatment for Paget's disease can help alleviate pain and may halt the damage done to your bones. In many cases, treatment can cause remission of the disease, which may be prolonged in some people.

Doctors generally recommend treatment when:

• You experience bone pain or neurological signs or symptoms related to Paget's disease.
• You're planning to have surgery to repair damage related to Paget's disease. In this case, your doctor will prescribe medications to minimize blood loss during the operation.
• You're at risk of serious, long-term complications due to the aggressiveness of your disease and the location of your affected bones. Involvement of the spine, skull, bones near major joints, and the long bones of the arms and legs is more likely to result in complications.
• Your heart is overworked because of widespread Paget's disease.

If any of the above criteria apply to you, your doctor may recommend treatment.

Medications
Your doctor may recommend bone-regulating medications if you have Paget's-related pain or if you're at risk of serious complications. Doctors use two kinds of medications to treat Paget's disease of bone:

• Bisphosphonates. Doctors commonly use these medications to treat osteoporosis and increase bone density, but they may also use them to reduce the activity of Paget's disease. Treatment with these agents helps restore more-normal-appearing bone and may produce long-term remission of Paget's disease. Bisphosphonates are currently the treatment of choice for Paget's disease, but you can't take them if you have serious kidney disease.

  Some bisphosphonates are given as oral medications, while others are given through a vein (intravenously). Oral bisphosphonates are generally well tolerated, but may irritate your gastrointestinal tract. Intravenous administration offers a more rapid response than oral medications do and also provides an option when you can't tolerate oral bisphosphonates.

  Doctors usually prescribe bisphosphonates for two to six months, depending on the drug used. You may need to switch to another brand if you take these medications long term because resistance to one bisphosphonate may build over time.

• Calcitonin. If you can't tolerate bisphosphonates, your doctor may prescribe calcitonin (Miacalcin), a naturally occurring hormone involved in calcium regulation and bone metabolism. Calcitonin is a drug that you administer to yourself by injection.

  Side effects may include nausea, facial flushing and irritation at the injection site. Stopping treatment with calcitonin usually results in a rapid reactivation of the disease and recurrence of symptoms.

  Your doctor may use blood tests measuring your alkaline phosphatase level to monitor your response to these medications. If therapy is effective, your alkaline phosphatase level will decrease and may return to normal.

Dealing with arthritis
Paget's disease may also require treatment to reduce pain or treat the inflammation associated with arthritis. Treatment options include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs alleviate pain and reduce the inflammation that's often the source of pain in arthritis related to Paget's disease. NSAIDs come in prescription and nonprescription forms. You may need to try more than one NSAID before finding the one that's most effective for you. Long-term use of NSAIDs or use of more than one NSAID can cause side effects, such as ulcers, heartburn, nausea and stomach bleeding. Large doses of NSAIDs can lead to kidney problems.
• Acetaminophen. Acetaminophen (Tylenol, others) may provide some relief of your pain, but it doesn't improve inflammation. It's generally safe if you take it for a short period of time and adhere to the daily dosage guidelines. If taken at the maximum dose for extended periods — especially when combined with regular alcohol intake — acetaminophen may damage your liver.

Surgery
In rare cases, you may require surgery to help fractures heal, to replace joints damaged by severe arthritis or to realign deformed bones. If Paget's disease affects your spine or your skull, you may need surgery to reduce pressure on nerves and prevent serious complications.

Paget's disease often causes the body to produce an excessive number of blood vessels (hypervascularity) to the affected bones. This change increases the risk of serious blood loss during an operation. If you're scheduled for surgery that involves bones affected by Paget's disease, your doctor may prescribe medications to reduce the activity of the disease, a step that tends to reduce blood loss during surgery.

Take the following steps to reduce your risk of complications from Paget's disease of bone:

• Prevent falls. Paget's disease puts you at high risk of bone fractures. Ask your doctor for advice on preventing falls. He or she may recommend that you use a cane or a walker. Take measures to fall-proof your home. Remove slippery floor coverings, use nonskid mats in your bathtub or shower, tuck away cords, and install handrails on stairways and grab bars in your bathroom.
• Eat well. Be sure your diet includes adequate levels of calcium and of vitamin D, which facilitates the absorption of calcium. This is especially important if you're being treated with bisphosphonates. Generally, you need at least 1,000 milligrams (mg) a day of calcium up to age 50, and 1,200 mg a day after age 50. You need 400 international units (IU), or 10 micrograms (mcg), a day of vitamin D up to age 70, and then need to increase your intake to 600 IU (15 mcg) daily. Review your diet with your doctor and ask him or her whether you should begin taking vitamin and calcium supplements.
• Exercise regularly. Exercising on a regular basis is essential for maintaining joint mobility and bone strength. Focus on weight-bearing exercise — such as walking, jogging or dancing — to maximize the benefit to your bones. Be sure to include strength training in your exercise program — strong muscles help support damaged joints. Talk to your doctor before beginning an exercise program to determine the right type, duration and intensity of exercise for you. Some activities may stress your affected bones.
• Maintain a healthy weight. Excess body weight may cause additional damage to your joints and may worsen pain in your joints. If you require surgery, excess weight also increases the risk of complications.

Living with a chronic condition such as Paget's disease of bone, even if you don't have symptoms, can be stressful. Focus on what you can do to reduce your risk of complications, such as exercising regularly and eating a healthy diet. Discuss any new symptoms or changes in your symptoms with your doctor. Effective treatments are available.

You may also find it helpful to join a support group for people with Paget's disease. To learn about support resources available in your area, contact The Paget Foundation.