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What is Paget’s disease?
Paget‘s bone disease is one of a chronic (long-holding) disease that causes bones to grow bigger and weaken more than normal. The disease typically only affects one or a couple of bones. The bones most frequently affected by Paget‘s illness include:
- Pelvis.
- Skull.
- Spine.
- Femur and tibia (leg bones).
Paget‘s bone disease is a chronic skeletal condition. A process called remodeling removes old bits of bone in healthy bone and replaces them with new, fresh bone. Paget’s disease causes the mechanism to change out of control, resulting in an abnormally formed, frail, and brittle new bone. Paget‘s disease most often affects older people, occurring in around 2 to 3 percent of the population over 55 years of age.
Many people with Paget‘s disease have no symptoms at all and are unaware that they may have the disease before they undergo x-rays for some cause. If bone pain and other signs are present, they can be due to the disease itself or disease complications such as arthritis, bone deformity, and fractures.
In most cases, Paget‘s disease treatment includes taking drugs to help delay or prevent disease progression. Surgery may be required to realign deformed bones or to help heal fractures in patients with complications.
What happens in Paget’s disease:
The body permanently replaces old bone over the lifespan and adds new bone to the skeleton. As we age it is natural that this phase should take place at a slower rate. When the illness begins, the body loses much more bone than it does. As Paget‘s disease progresses, new bone develops at a rate that is faster than the pace of removal of old bone. However, the new bone does not shape properly, leading to wider, smaller, and thinner bones than normal.
Who gets the disease:
- Frequency of occurrence:
Paget ‘s disease follows osteoporosis as the second most common form of bone disease. About 1 million people have Paget ‘s disease in the United States.
Paget ‘s disease appears to occur in both older adults and Northern European people. The condition happens to any two women with Paget ‘s disease in three persons.
- Sex:
Studies indicate that men are probably marginally more likely to have the disease than women.
- Age:
Paget’s disease is rare in people under the age of 40, and as you age the risk of contracting the disease rises.
What are the symptoms of Paget’s disease?
You do not know that you have Paget‘s disease because there are no signs in many people with the illness. But fractures or bones that are misfigured can develop and cause pain. Often this bone pain is mistaken for arthritis or other conditions which is the most common symptom of the disease. Symptoms usually grow slowly, and the disease doesn’t spread to normal bones.
Because this disease causes the body to develop new bone faster than normal, the rapid remodeling produces bone that is thinner and weaker than normal bone, which can lead to bone pain, deformities, and fractures.
The disease can only affect one or two areas of the body, or maybe widespread. If any, the signs and symptoms depend on the part of your body that’s affected.
Pelvis. Paget’s disease of bone in the pelvis can cause hip pain.
Skull. An overgrowth of bone in the skull can cause hearing loss or headaches.
Spine. If your spine is affected, nerve roots can become compressed. This can cause pain, tingling and numbness in an arm or leg.
Leg. As the bones weaken, they may bend — causing you to become bow legged. Enlarged and misshapen bones in your legs can put extra stress on nearby joints, which may cause osteoarthritis in your knee or hip.
What Causes Paget’s disease?
Researchers have yet to determine the conclusive cause of Paget‘s disease.
Paget‘s condition seems to be circulating inside families. More than one family member has the condition in 30 percent of cases, according to the American College of Rheumatology.
Another explanation is that the condition may have arisen during adolescence due to infection with the measles virus. Recent studies have indicated that measles may alter the bone formation process, contributing to Paget‘s illness.
Researchers, however, have not yet discovered a direct connexion between the virus and Paget‘s disease.
Evidence shows that, over the past 25 years, the number of people with Paget‘s disease has declined. Some scientists in several countries have related the rise in vaccination and the subsequent decrease in the number of people with measles to declining rates of Paget‘s disease.
How is Paget’s disease diagnosed?
After undergoing scans for other disorders or to better determine the cause of their pain, many people with Paget‘s disease are diagnosed. Some popular diagnostic tests used for Paget‘s disease include:
X-ray. This is the most prevalent procedure used by physicians to diagnose the disease. An x-ray will determine the condition of the bone for Paget‘s illness.
Blood test. Your doctor can order a blood test to test for the alkaline phosphatase enzyme. This test can indicate either the disease of Paget or other conditions such as liver disease. If the blood contains elevated enzyme levels, this may be a symptom of the illness.
Bone scan. A bone scan is a test that helps doctors recognize which bones Paget‘s disease affects. The test will help the doctor comprehend the magnitude of the disease. You inject a small quantity of radioactive material into a vein in your arm during a bone scan. The substance circulates through the bloodstream and “highlights” locations in the skeleton where Paget‘s disease may be present by accumulating in areas where Paget‘s disease is marked by enhanced blood flow and bone-forming cell activity.
What is the treatment for Paget’s disease?
Paget‘s disease has no cure and no means of mitigating its effects on the body. Care focuses on symptom relief and the avoidance of future complications.
If you do not have pain or other symptoms, no treatment is required. Your doctor may recommend simply monitoring your condition with regular office visits and periodic x-rays to watch for changes in the affected bone and to ensure that complications do not develop.
If symptoms do occur, your doctor may recommend one or more non-surgical treatments.
Nonsteroidal anti-inflammatory drugs (NSAIDs). Medications such as ibuprofen, naproxen, and aspirin can help relieve mild bone pain that arises from Paget’s disease or from arthritis that may be associated with the disease.
Assistive devices. If your pelvis or leg is affected by the disease, using a cane can help relieve pain by decreasing the forces going through the bone. Using a cane can also help prevent falls, so there is less risk of fracture in the bone — a common complication of Paget’s disease.
Wearing a brace can help relieve pain by preventing malalignment of the affected bones.
Bisphosphonate medications. When bone pain is more significant, medications called bisphosphonates are the treatment of choice. These drugs block osteoclasts and can be very effective in treating Paget’s disease.
There are several types of bisphosphonates. Some are given by mouth (orally) and others are given as an injection (intravenously). Your doctor will talk with you about which type is best for you and how long you will need to take it.
During treatment with bisphosphonates, your doctor will perform blood tests periodically to check your alkaline phosphatase level. A falling alkaline phosphatase level and improvement in bone pain are indications that treatment is working.
Surgical Care:
In some cases, surgery may be needed to treat the complications of Paget’s disease, including:
- Bone fractures
- Malalignment or deformity of bone
- Severe arthritis
The surgical procedures used to treat fractures, malalignment, or arthritis in patients with Paget’s disease are similar to those used to treat similar conditions in people with normal bone.
These procedures may include:
- Internal fixation. This procedure can be used to treat fractures in bone affected by the disease. In internal fixation, bone fragments are first repositioned into their normal alignment, then held in place with screws, wires, pins, or metal plates attached to the outside of the bone.
- Osteotomy. An osteotomy can help relieve pain and restore alignment to weight-bearing joints that are affected by Paget’s disease, especially the knee and hip. During the procedure, your doctor will remove a wedge of bone near the damaged joint in order to shift weight onto a healthier part of the joint.
- Total joint replacement. In this procedure, parts of an arthritic or damaged joint are removed and replaced with a metal, plastic or ceramic device called a prosthesis. The prosthesis is designed to replicate the movement of a normal, healthy joint.
Surgery may also be needed if an enlarged bone begins to compress nerves, especially in the spine or skull. In the rare case of Paget’s sarcoma, surgery is almost always used to try to remove the tumor entirely. Chemotherapy and radiation therapy may also be used.
Because Paget’s disease increases the blood supply to bones, your doctor may recommend taking bisphosphonates for a period of time before surgery to help reduce potential blood loss.
Bones affected by Paget’s disease may take longer to heal than normal bones. A longer period of rehabilitation may also be necessary.
Coping with PDB:
Fortunately, existing therapies can help most people lead healthy lives with Paget‘s disease. You will lower the risk of having complications or major changes in your bones if you obey your doctor’s treatment plan. You also should:
Maintain a healthy weight, which is especially necessary if Paget’s disease has progressed to weight-bearing joints such as hip or knee arthritis. Excess weight can result in greater stress — and pain — on affected joints.
Prevent falls by:
- Checking your home for dangers such as loose rugs and poor lighting.
- Installing grab bars and handrails.
- Using non-skid mats in the bathroom and tub.
- Having regular eye exams.
- Increasing your balance and strength by exercising every day.
To preserve good bone health live a healthy lifestyle. Smoke not, and stop if you do smoke. Do this in moderation, if you want to drink alcohol. It is safer for women to have no more than one drink a day, and men to have no more than two drinks a day.
References:
https://www.medicalnewstoday.com/articles/177668
https://www.niams.nih.gov/health-topics/pagets-disease-bone
https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-bone
https://orthoinfo.aaos.org/en/diseases--conditions/pagets-disease-of-bone
By,
Gopala Krishna Varshith,
Content Developer & Editor,
Clipo.
