Paget’s Disease of Bone: Causes, Symptoms and Care

Paget’s disease (PDB) is a relatively common skeletal growth disorder. Based on 2023 reports, PDB has a 1.5% to 8.3% prevalence rate, making it the second most common metabolic bone disorder on the globe. ^[1]

This condition can affect either one or multiple bones in the body. Many of the people affected have no symptoms. But when symptoms do occur, most patients complain that their bones ache.

The disorder tends to manifest in older patients, typically in their late 50s. It is also more prevalent in men than women. PDB is a relatively complex disease as it involves both environmental and genetic factors. Here is a detailed guideline on what to expect with PDB.

About Paget’s Disease of Bone

Paget’s disease is a condition where patients experience unusual bone growth abnormalities. The disorder causes changes in the structure and density of the affected bones. This can lead to widespread pain in the muscles and bones.

In PDB, there’s too much activity from certain bone cells called osteoclasts. This prompts a compensatory response from another type of bone cell called osteoblasts. This leads to the formation of disorganized and less sturdy bone tissue.

This affected bone is less compact, has more blood vessels, and is more likely to break. Luckily, over 3/4 of people with Paget’s disease don’t show any symptoms. Studies show it’s the second most common bone disorder in older patients, right after osteoporosis. ^[2]
This condition can affect one bone or several bones. However, it usually impacts the central part of the skeleton, including the skull, spine, and pelvis.

Note: Paget’s disease doesn’t spread to other normal bones. But it can get worse in the areas where it’s already present.

What Happens in Paget’s Disease?

In our bones, there are two important elements: the “builders” called osteoblasts and the “removers” called osteoclasts. Osteoblasts construct new bones, while osteoclasts help eliminate the old bones. They work together to keep our bones healthy.

As we grow older, it is completely normal for the process of removing old bones and adding new ones to slow down. But, for someone with PDB, that balance is disturbed. As the condition progresses, new bone grows more quickly than the old bone is removed. ^[3]

Paget’s disease happens when there’s too much breakdown of bones. This is a chronic disorder that makes the bones grow bigger, weaker, and more susceptible to damage. This process forces the osteoblasts to become more active.

The bone changes can make the bones ache. The abnormalities can also lead to misshapen bones, fractures, and inflammation. The disease usually begins in small areas but tends to develop gradually, in stages, and it can get worse. ^[4]

PDB can cause redness and warmth in the affected area, which might also put a strain on the heart. The potential strain on the heart comes from the fact that these extra blood vessels require the heart to pump more blood to supply them.

This increased demand on the heart can be particularly noticeable if PDB affects a large portion of bone, as it may require a higher volume of blood flow. But it’s important to point out that it is a relatively rare complication of PDB.

Who Gets Paget’s Disease?

Some people are more prone to Paget’s disease. The factors that can increase your risk may include:

• Family history: Research suggests that there is a genetic predisposition to developing this condition. In about 15% to 30% of these cases, there is a family history of PDB. Specifically, mutations in the SQSTM1 gene. This gene plays a key role in bone remodeling. ^{[5] [6]}

• Age: Bone diseases tend to develop predominantly in older patients. The cumulative effect of genetic predisposition and environmental factors increases the risk of PDB. Age-related changes in bone metabolism and hormonal levels may also play a role. As people grow older, they can experience changes in the balance of bone formation and resorption.

• National origin: Being of Anglo-Saxon descent is one of the risk factors for PDB. This disease is more prevalent in specific regions, such as New Zealand, Australia, North America, and some parts of Europe. But it is not common in Scandinavia, Africa, and Asia.
• Sex: Male patients are more prone to PDB than women. Paget’s disease typically impacts older men. For every two women affected, about three men may experience this condition. ^[7]

Which Bones Are Affected by Paget’s Disease of the Bone

While Paget’s disease can affect nearly any bone, it tends to favor the longer bones, spine, and skull. It can also affect the pelvis, tibia, and femur. But the disease rarely affects hands, feet, clavicles, ribs, and shoulder blades.

It is rare for a normal bone that wasn’t affected when a patient was first diagnosed to develop the condition later on. There are also cases where PDB can be associated with osteoarthritis, especially in big joints like the knee and hips, if it is near the joint or there is a bone deformity. ^[8]

If bones bend, like the femur or tibia, it can lead to pain. When that happens, patients often struggle to walk. Fractures may be painful and are more common on the curved side of a bone. When the skull is involved, veins on the scalp may become more prominent.

Symptoms and Signs of Paget’s Disease

Paget’s disease can often go unnoticed since many patients don’t experience any obvious signs. However, it can lead to fractures or bones becoming misshapen – this can result in discomfort.

This bone discomfort, which is the primary indication of the disease, is wrongly attributed to arthritis or similar conditions. Typically, symptoms emerge gradually. Those who develop PDB can experience discomfort in any bone. But it tends to affect the following:

• Skull
• Leg bones (tibia and femur)
• Pelvis
• Spine

Patients with advanced stages of PDB can develop bone abnormalities and misshapen bones. Such as curving of the spine, enlargement of the skull, and bending of a limb. Other symptoms of Pagets disease can include: ^[9]

• Hearing problems and headaches – these are common symptoms when PDB affects the skull.
• Hip pain – these symptoms develop when the condition affects the thighbone or pelvis.
• Numbness and tingling – these signs can occur if the vertebrae become enlarged and press on the spinal nerves.

Based on which bone or bones Paget’s disease affects, various other health issues may arise, including:

• Enlarged bones in the spine and skull can put pressure on different parts of the body. Such as the spinal cord, brain, and nerves. This pressure could potentially reduce blood flow to these areas.
• An injury to joint cartilage may result in arthritis.
• When the disease affects the bones in the face, it can loosen your teeth. This can make it harder for you to chew.
• Abnormalities in the bones of the head and ear can cause hearing impairment.
• People with pre-existing heart conditions and advanced Paget’s disease may experience heart failure due to the added strain of pumping blood.
• Although uncommon, people with PDB may occasionally develop bone cancer.

What Causes Paget’s Disease of the Bone?

We don’t know the exact cause of Paget’s disease. But research estimates that it occurs due to a mix of genetic and environmental factors. Many genes can increase the susceptibility to the disease.

Some experts also believe that PDB could be associated with viral infections in the bone cells. However, that theory lacks sufficient evidence. ^[10]

A lot of people want to know why PDB causes pain. When it comes to bone pain causes, this disorder can cause pain because of structural changes and increased blood vessel growth.

The affected bones in PDB have more blood vessels than normal bones. This increased vascularity can lead to a sensation of warmth over the affected area. It can also contribute to the perception of pain, as the blood vessels may put pressure on surrounding tissues.

What Are the Possible Complications of Paget’s Disease?

We can’t cure Paget’s disease, but we can use treatment to curb the symptoms and decrease the odds of developing complications. Here is a quick look at the potential complications that can happen from untreated PDB.

1. Bone Expansion

In Paget’s disease, affected bones can enlarge and become misshapen. This can lead to physical deformities, especially in weight-bearing bones. If that happens, the disease can affect your mobility.

2. Fractures

In Paget’s disease, the new bone that forms is disorganized and less compact than healthy bone. It is also more susceptible to microfractures or fissures. These small fractures can cause localized pain.

These fractures can occur even from minor traumas or normal activities. While fractures can be painful, they may also go unnoticed. So, the more activities you do around the house, garden, or at work, the bigger the odds of further damage to your bones.

3. Nerve Compression

Enlarged bones can put pressure on adjacent nerves, especially in the spine. This can lead to symptoms like pain, tingling, numbness, and muscle weakness in the affected areas.

Severe nerve compression can result in more significant neurological symptoms. Such as nerve damage and chronic pain. It is important to relieve the pressure so the nerve can work properly.

3. Deafness

When Paget’s disease affects the bones of the skull, particularly those around the ears, it can affect your ability to hear. You can experience hearing loss, which can be progressive and gradual.

4. Osteoarthritis

PDB can lead to changes in joint structure and alignment. It could potentially cause or exacerbate osteoarthritis. This can result in joint pain, stiffness, and a reduced range of motion. That’s why people with bone disorders should take better care of their bone health.

5. Tumours

Although rare, long-term Paget’s disease is associated with an increased risk of bone tumors, including osteosarcoma. These tumors may develop within the affected bones. ^[11]

How Is Paget’s Disease Diagnosed?

To diagnose PDB, your healthcare provider will start with a physical exam and talk about your overall health. If you experience any of the symptoms of Pagets disease or believe to be at risk of developing the condition, the doctor can suggest:

• X-rays
• Urine and blood tests
• Bone scan

Additional testing may be necessary, such as a CT or an MRI scan.

What Treatments Are There for Paget’s Disease?

Bisphosphonates (osteoporosis medications) are a go-to choice for treating PDB. But other methods may be suggested, depending on the symptoms and causes of the disease. ^[12]

1. Physical Therapies

Patients with PDB should stay physically active. That’s because exercise can improve joint mobility and bone health. But you should avoid aggressive exercises like bending, twisting, running, or jumping because the risk of fracture and bone damage is relatively high.

2. Eat a Good Diet With Enough Calcium and Vitamin D

Your bones need all the nutrients, vitamins, and minerals they can get. A balanced diet can help with that. It can support immune function, enhance the quality of life, and aid in recovery. Calcium and vitamin D are critical for bone health.

3. Supplements

Supplements can work alongside a well-balanced diet. With Flexoplex Bone and Joint Support Supplement, you can receive all the necessary nutrients for optimal bone function. It is a comprehensive solution with a powerful formula.

Flexoplex is formulated with a blend of clinically-backed ingredients, such as natural compounds, minerals, and vitamins. It is designed to reduce discomfort, enhance mobility, and promote strong bones.

4. Surgery

Surgery might be necessary if the enlarged bone is starting to put pressure on the nerves. Particularly when that pressure is affecting the skull or the spine. Through surgery, doctors can alleviate the pressure and treat the pain.

Risk Factors of Paget’s Disease

Paget’s disease tends to occur in patients who already have a genetic risk. It is slightly more prevalent in elderly men than women. Particularly in patients of Anglo-Saxon descent.

Takeaway

Paget’s disease of bone is a complex condition that affects bone structure. Recognizing its signs and seeking specialized care can help you improve your quality of life. Don’t hesitate to talk to a bone health specialist to find the right treatment for you.