Silent Diseases: What is Paget’s Disease?

The body is constantly recycling bone matter. Over time, new bone tissue grows in to replace the old as we age. Medical experts refer to this process as bone remodeling. There are several conditions that affect (or are related to) this process, with the most common being osteoporosis.

Paget’s disease has a very interesting relationship with osteoporosis because it produces the same results despite being the complete inverse of the latter. With osteoporosis, the body loses too much bone, makes too little, or some combination of the two. On the other hand, Paget’s disease causes new bone tissue to grow in places that it shouldn’t. Additionally, Paget’s disease may cause old bone to be removed from areas where it should remain.

Despite the inverse nature of these two conditions, they both produce similar outcomes. Either condition may lead to weak bones, pain, skeletal deformities, spinal arthritis, and spinal fractures. Unfortunately, most patients do not realize that they have Paget’s disease until it gets to that point. Symptoms may appear mild or they may not be detectable at all. Additionally, these problems do not heal easily because of the abnormalities that the disease causes in bone remodeling.

Symptoms of Paget’s Disease

As previously mentioned, most people with this condition have mild symptoms or no symptoms at all. Normally, when the patient experiences symptoms, they manifest in bone pain.

Paget’s disease causes the body to produce new bone faster than it normally does. Because these bones are produced so quickly, they are usually softer and weaker than normal bone. In healthy bones, the structure is overlapping and well supported—much in the same way that a brick wall is sturdy. When Paget’s disease forms bones, it disrupts this figurative foundation. Instead, the pattern becomes irregular and it does not support bodily weight nearly as well. This leads to excessively large, haphazardly placed, and unusually brittle bones.

Paget’s disease may be isolated, or it may affect multiple regions of the body. If the patient experiences any symptoms at all, it will depend on the affected area(s) in the body.

• Paget’s disease in the skull causes hearing difficulties as well as headaches.
• In cases affecting the legs, the patient may become bow-legged and develop osteoarthritis.
• Paget’s disease in the spine may lead to lumbar radiculopathy (aka a pinched nerve). Nerve compression such as this causes a variety of symptoms. Normally, these symptoms manifest as pain, numbness, and/or tingling.
• A patient with abnormal bone growth in the pelvis may experience hip pain.

In a very small number of cases, Paget’s disease may lead to Paget’s sarcoma—a form of bone cancer. Although very rare, Paget’s sarcoma causes extreme pain in the affected region of the body. The tumors associated with this cancer are often very aggressive and the prognosis is generally poor.

You should consider seeing a doctor if you experience chronic pain, especially in the bones and joints. Additionally, other neurological symptoms—such as tingling and weakness—serve as red flags to consult your physician.

Causes of Paget’s Disease

Unfortunately, the cause of Paget’s disease is unknown in the world of medicine. That being said, there are a handful of identified risk factors that may lead to this condition. The following factors increase your chance of developing Paget’s disease:

• Age: Like osteoporosis, Paget’s disease is more likely to affect a patient as he or she ages. In fact, it is very rare for a person under 40 to have the condition.
• Heredity: Patients with Paget’s disease often have family members who have a history of the condition. In fact, roughly 30 percent of patients with Paget’s disease know a close relative who also has the condition.
• Lineage: Interestingly, people who live in certain geographic areas are statistically more likely to develop the condition. The most concentrated number of cases occur in European countries such as Scotland, Central Europe, and Greece. Additionally, countries with European immigrants, such as the United States, are more statistically susceptible. Likewise, it is quite rare for people in the far east (Japan, China, etc.) to develop Paget’s disease.
• Environmental Factors: Evidence suggests that certain exposures may increase a person’s chance of developing this condition. Although unproven, some believe that there is a connection between measles and Paget’s disease. Additionally, the number of people with Paget’s disease is decreasing over time. This suggests that the condition is not solely tied to genetics; otherwise, the numbers would remain more constant.

Unfortunately, there is no known way or method to prevent this condition. The best advice that medical experts can give is to simply maintain skeletal health and joint mobility. To do this, doctors recommend eating a healthy diet and getting regular exercise as often as possible. For a diet that supports the skeletal system, look for foods that are high in calcium and vitamin D.

Paget’s Disease Diagnosis

Most of the time, Paget’s disease is discovered by happenstance when lab tests are performed for an unrelated reason. If abnormalities suggest Paget’s disease, doctors will perform additional tests for confirmation. These tests include:

• Blood Tests: Patients with this condition have higher than normal alkaline phosphatase levels, which a blood test will detect. This indicates a high turnover rate for bone formation.
• Urine Tests: A doctor may request this in lieu of a blood test as a urinalysis can also reveal elevated bone loss.
• X-rays: When Paget’s disease affects the bones, they appear enlarged and are much denser than normal. Additionally, there may be abnormalities with the bone’s shape—another common indicator of Paget’s disease. X-rays provide images of the skeletal structure that makes these signs observable to the naked eye.
• Bone Scan: Doctors use this type of test to isolate where the Paget’s disease begins and ends. Paget’s disease makes affected bones appear as “hot spots” through using a radioactive dye and a specialized camera.
• Biopsy: A doctor may also perform a biopsy to confirm or deny the existence of Paget’s disease in the patient. Usually, a biopsy involves removing a small sample of the affected bone and viewing it under a microscope. A trained medical specialist will be able to detect abnormalities at the microscopic level.

Paget’s Disease Treatment

There is a whole slew of both conservative and surgical treatments for relieving the symptoms of Paget’s disease. That being said, there is no way to reverse the damage that has already been done. Instead, treatment for Paget’s disease focuses more on alleviating pain and preventing future problems from developing.

Non-surgical Treatment Options

If the patient has Paget’s disease with no negative symptoms, then treatment is not necessary. Instead, the doctor will simply monitor the patient through regular visits until symptoms occur. If symptoms do occur, there are a variety of nonsurgical methods that alleviate the condition.

• Anti-Inflammatory Medications: A doctor may prescribe NSAIDs to the patient to help alleviate mild bone pain associated with Paget’s disease.
• Braces: Some patients need braces to help improve bone stability or to prevent misalignment. If Paget’s disease affects the legs, a cane may allow the patient to walk more easily and prevent falls.
• Bisphosphonate Medications: Doctors will recommend this treatment to patients with more severe cases where the pain is extreme. These drugs block osteoclasts (cells responsible for normal bone breakdown) from performing abnormal functions, decreasing pain dramatically.

Surgical Treatment Options

If conservative treatments do not provide the patient with ample relief, then a doctor may recommend surgery. There are both traditional and minimally invasive surgical options for patients who suffer from Paget’s disease. Normally, a doctor will steer more toward less invasive procedures; however, this is not always viable. Some cases require total joint replacement, whereas others are easier to treat. The following examples are minimally invasive surgeries that your doctor may recommend:

• Vertebroplasty: Doctors use this procedure to treat fractured vertebrae. For this surgery, a special cement is injected into the affected area, relieving pain and restoring spinal flexibility.
• Osteotomy: This procedure treats areas that have been damaged by arthritis. If the patient has Paget’s disease, arthritis may develop and this becomes a viable treatment route. For this surgery, a small wedge of the affected bone is added or removed to more properly distribute body weight.
• Nerve Decompression Surgery: Decompression surgery is a rather broad subject as there are a variety of different methods involved. The specific type of your decompression surgery will depend highly on the nature of your case. As the name suggests, decompression surgery takes pressure off of affected nerves. Because Paget’s disease sometimes leads to nerve compression, this procedure may be viable for some cases.

If you are experiencing chronic pain or have a family history of Paget’s disease, please contact us at (855) 210-0899. New York City Spine hosts a team of specialized experts who will work tirelessly to get you the best treatment for your case. Our back doctors are well-versed in both conservative and minimally invasive treatments for a large variety of medical problems. We look forward to meeting you and answering any questions that you may have!