Paget's disease: symptoms, causes and how to treat it

Definition of Paget's disease

What is Paget's disease?

Paget's disease or Paget's disease is a disease that causes interference with the bone regeneration process. Bone regeneration is the process of gradually forming new bone to replace old damaged bone tissue.

In normal bones, the regeneration process occurs every day. Bone cells called osteoclasts will absorb bone and osteoblasts will make new bone.

However, in people who have Paget's disease, osteoclasts are more active than osteoblasts. That means, bone absorption occurs more than usual. This causes osteoblast cells to compensate for osteoclasts.

However, this actually overreacts and causes the bones to enlarge abnormally, change shape, and do not fit together. These abnormal bones are also weak and brittle, so they are prone to fractures.

The most common fractures are hip, skull, spine, and lower leg fractures.

How common is this disease?

Paget's disease of the bones is a fairly common disease. Most people affected by this disease are people over 50 years of age. This disease is very rare in someone under 50 years of age.

Signs & symptoms of Paget's disease

In most cases, Paget's disease causes no symptoms at all. The disease is often discovered only after imaging tests are performed because of other health problems or when blood tests show high levels of serum alkaline phosphatase.

Even so, there are some who feel symptoms. Symptoms of Paget's disease that commonly occur are:

Pain in the bones

Pain in the bones is the most common symptom of Paget's disease. The pain can appear in one or more areas of the bone because this disease can attack several bones at once.

Sometimes, the appearance of pain is associated with complications, such as:

• Fractures (broken bones) because bones are very brittle.
• Arthritis of the joints near the affected bone

Symptoms in the bones and nerves

Apart from pain, physical changes are also a symptom of Paget's disease, including swelling and bending of the thighs, lower legs, and forehead area.

In addition, this bone deformity can also cause the body to bend over like a person with kyphosis. When this condition occurs, it will be difficult for you to walk well and it will be difficult to maintain your balance.

Paget bone, which attacks the skull, generally experiences headaches and hearing loss. Additional symptoms that accompany are blindness and hydrocephalus (buildup of fluid in the brain).

These musculoskeletal disorders can also cause nerve problems, because they put excessive pressure on the nerves. As a result, you may feel numbness, tingling, or weakness in the muscles.

Meanwhile, if the disease attacks the spine, complications of spinal stenosis can occur. This condition indicates an abnormal narrowing of the space within the spinal canal, spinal nerve root canal or vertebrates.

Other symptoms

Overactive osteoclasts can release calcium from the bones, causing an increase in calcium levels in the blood. High levels of calcium in the blood may occur if the disease affects more than one bone.

If this condition occurs, you will experience additional symptoms, such as:

• The body is weak and tired easily.
• Decreased appetite.
• Stomach pain accompanied by constipation.

When to see a doctor?

If you experience the signs and symptoms listed above, see a doctor immediately. Especially if you experience the following symptoms:

• Pain in the bones or joints that is persistent.
• There is a deformity of one of the bones in the body.
• Experiencing nerve problems, such as numbness, tingling, or muscle weakness for no apparent reason.

Causes of Paget's disease

The exact cause of bone paget disease is not known with certainty. However, researchers argue that the cause may be from a variety of factors, such as genetics and certain environments.

According to the National Organization for Rare Disease website, research shows that Paget's disease is linked to bone infections that occur over the years.

About 15-30% of cases are likely due to a family history of the same condition. The study found several genes that can increase the risk of paget's disease, namely the sequestosome 1 gene, the TNFRSFIIA gene that encodes the RANK protein, and the VCP gene.

However, how the mechanism can cause this bone disorder is not certain.

Risk factors for Paget's disease

Although the exact cause of bone paget disease is not known, experts have found a variety of factors that can increase the risk, such as:

• Increasing age

As we get older, the risk of this disease also increases. Especially, after you enter the age of 40 years.

• Male gender

In many cases, men get this disease more often than women.

• Family medical history

If you have relatives suffering from this bone disease, it is very likely that you are also at risk for the same disease.

Complications of Paget's disease

Paget's disease is a progressive disease, which can worsen over time. Without proper treatment, Paget disease can cause complications, such as:

• Broken bones and abnormal bone shape, for example, bent legs. This condition can cause extra blood vessels that can cause excessive bleeding when the operation is performed.
• Osteoarthritis due to deformed bones can increase the pressure on the surrounding joints.
• When these bone problems occur near the nerves, there is excessive pressure that can damage the nerves.
• In the long term, this disease can force the heart organ to work harder to pump blood to the affected area of ​​the body. This increase in load can lead to heart failure.
• Although rare, this bone disease can trigger the cells around the bone to become abnormal, causing cancer.

Medicine & treatment of Paget's disease

The information provided is not a substitute for medical advice. ALWAYS consult your doctor.

To make a diagnosis of Paget's disease, your doctor will ask you to do a series of medical tests, such as:

• Physical test. This medical test is done by examining the area of ​​the body that causes pain. Your doctor may also check your and your family's medical history.
• Imaging test. The changes that occur in your bones can be seen through an X-ray imaging test. Starting from the enlargement of the bone, its deformity, to the reabsorption area can be observed from the results of the images.
• Bone scan test.In this medical test, radioactive material will be injected into your body. Then, the material will spread and collect in the affected bone area. The affected area will be visible from the scanned image.
• Laboratory test.People suffering from this bone disease will usually have elevated levels of alkaline phosphatase in their blood, which can be determined by a blood test.

What are the treatment options for Paget's disease?

If the level of alkaline phosphatase in the body is high, the new doctor will prompt the appropriate treatment. The following are treatments for Paget's disease that doctors usually recommend are:

Take medicine

Bisphosphonate drugs, which are drugs for osteoporosis, are also often prescribed by doctors to treat Paget's disease.

This medicine is available in pill form by mouth, and as a liquid that is injected. Side effects can be tolerated, but can irritate the digestive tract if used long-term.

Examples of bisphosphonate drugs that are also used to treat Paget's disease are:

• Alendronic (Fosamax)
• Ibandronic (Boniva)
• Pamidronate (Aredia)
• Risedronate (Actonel)
• Zoledronic acid (Zometa, Reclast)

If the risk of side effects is high, the doctor will prescribe calcitonin (Miacalcin). Calcitonin is a natural hormone drug that is involved in the regulation of calcium and metabolism in the body.

This medicine is usually given by injection or sprayed into the nose. Side effects that may occur are stomach nausea, facial flushing, and irritation of the skin after the injection.

Calcitonin is a medicine that you give yourself by injection or nasal spray. Side effects may include nausea, facial flushing and irritation at the injection site.

Operation

If the above treatments are not effective, the doctor will recommend a surgical procedure as a treatment. The goal is to replace joints that may be damaged by inflammation, realign bent bones, and reduce pressure on the nerves.

Some types of surgery to treat Paget's disease that are generally performed are:

• Internal fixation

In this operation, the bone fragments are first corrected to their normal position. Then, it is secured with screws or metal plates that are fixed on the outside of the bone.

• Osteotomy

This surgery can help relieve pain and restore alignment of the weight-bearing joints affected by the disease, especially the knee and hip areas. During the procedure, the doctor will remove bone incisions near the damaged joint to transfer weight to other parts of the joint that are healthier.

• Total joint replacement surgery

In this medical procedure, the affected or damaged part of the joint is removed and replaced with a metal or plastic device called a prosthesis. The prosthesis is designed to mimic normal, healthy joint movements.

Home remedies for Paget's disease

Apart from doctor's treatment, lifestyle changes are also needed by patients with Paget's disease which include:

• Be careful in your activities so you don't fall or slip. The goal is to prevent injury or fracture. If you have trouble walking, you can use a cane or a walker.
• Ask family members or caregivers to place a mat on the slippery floor to avoid slipping. Also, organize your house wiring so you don't trip over them.
• Eat bone-strengthening foods, which are rich in vitamin D, calcium and phosphorus. The food choices are quite diverse, ranging from dairy products, nuts, green vegetables, and various types of fish.
• Bask in the morning regularly to get sufficient vitamin D. If you are interested in taking supplements, consult your doctor first.
• Do regular exercise to maintain body mobility and increase bone strength. Consult with your doctor first, about an exercise plan and the type of exercise that you are safe to do.

Prevention

How can you prevent Paget's disease?

Paget's disease is one of the bone diseases that cannot be prevented, considering that the cause is not known with certainty. Until now, health experts are still observing more deeply about the prevention of this disease in the future.