Pagets Disease of Bone

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Pagets Disease of Bone is a condition that affects the normal biological processes of your bones.

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. Pagets 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.

Pagets disease of bone becomes more common with age. It 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.

Pagets 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. Affected areas may include:

  • 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 Pagets disease of bone 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 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 of bone 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

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

    Some scientists believe Pagets 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.

    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 Pagets disease of bone, but you can't take them if you have serious kidney disease.
      Oral bisphosphonates are generally well tolerated, but may irritate your gastrointestinal tract. Some bisphosphonates aren't available as oral medications, and you must receive them through a vein (intravenously). Intravenous administration offers a more rapid response than oral medications do and provides an option when you can't tolerate or are not a candidate for 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
    Pagets disease of bone 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 of time — 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 Pagets disease of bone 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 in 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 Pagets disease of bone, your doctor may prescribe medications to reduce the activity of the disease, a step that tends to reduce blood loss during surgery.


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