Skip to main content
Updated date:

PAGET’S DISEASE OF THE BONE: A Rare Disease

Mona is a veteran writer for Pressenza, columnist for Enrich Magazine, and life coach. She holds webinars on writing and emotional health.

pagets-disease-of-the-bone-a-rare-disease

Paget's disease of the bone (PDB) is a rare, long-life disease that affects 1.5% to 8.3% of people globally. This chronic disease is experienced by people age 50 and over.

A study by Robert A. Wermers et. al. determined that this life-long disease will worsen over time, but it isn’t fatal. However, it is excruciatingly and relentlessly painful, affecting the quality of one’s daily life.

Normally, upon adulthood, the bone remodels itself every 10 years. Remodeling involves the elimination of old bone and its replacement with new bone. The timing of this process is perfectly harmonious.

But PBD interrupts this process by speeding up the elimination of one’s old bone. In response, the replacement of new bone tissue is also hastened. The result is bone abnormalities. The new bone is weak, dense, thick, fragile, and can easily fracture.

Usually, PDB affects the femur (thigh leg), tibia (shin bone), pelvis, skull, and spine. But any bone can be affected with PDB, and oftentimes more than one bone is affected at the same time.

A person with PBD may experience arthritis and debilitating bone pain. Even worse, the deformed bones can have extra blood vessels that will cause excessive bleeding with repair surgery.

There is no cure for PDB. However, with proper treatment, you can manage your symptoms. Over time, as the bones continue to grow and are misshapen, it may stress other joints nearby, which can lead to osteoarthritis. On rare occasions, Paget's can evolve to become bone cancer.

PBD is not widely known by your average bone doctor because of its rare occurrence. They don’t know what causes it, but they believe it may be hereditary. This illness is also difficult to identify, and more often it’s discovered while testing a person for another ailment.

Usually, people with PBD are treated by an endocrinologist and/or rheumatologist. People with this disease may find themselves going through many doctors and hospitals until they find someone who can really be of help to them. This was the case with Keith Simpson, a trustee of the Paget’s Association. His story follows:

Definitely, there is a need for more research as to what causes Paget’s disease, how it can best be treated, and hopefully, a cure will be discovered. What we do know is that there’s no guarantee, even with good treatment, that the pain will go away. We also know that Pagets, if it perseveres, may even lead to compressed or impaired nerves.


pagets-disease-of-the-bone-a-rare-disease

Symptoms

The symptoms you should be wary of, which may indicate Paget’s are:

  • Joint pain, with rigidity and inflammation.
  • Persistent, blunt pain in the bone.
  • Immobility and stinging pain.
  • Darting pain that runs through the body.
  • Loss of mobility in affected parts of the body

You should see your doctor when:

  • Malformation of any bone or some bones is evident.
  • Constant agony of the joint or bone is felt.
  • There are signs of nerve complications such as dullness, stinging, or immobility.
Paget's on the pelvis

Paget's on the pelvis

The tests for PDB include:

  1. Blood tests. This will check if your alkaline phosphatase (ALP) level is above normal. If it is, it may indicate PDB.
  2. X-ray scan. If there is any bone enlargement, PDB is confirmed.
  3. Scintigraphy. This follows the progression of your PDB. It’s done by injecting a small amount of a radioactive substance. The radioactive substance will enable:
  • Bone scan. The radioactive substance will collect in the areas where there is heightened bone renewal in your body.
  • Gamma camera. This will highlight the parts of your body that are affected by PDB.

    4. Additional tests. If you have severe Paget’s disease, bone cancer may be indicated. These tests will rule out cancer:

  • A bone biopsy. A part of the bone is removed for further testing and examination.
  • CT scan. This produces a series of x-rays to create a three-dimensional image of the afflicted bone range. This enables a more detailed view of the area.
  • MRI scan. This is superior to a CT Scan because images are more discernible. You can see the affected bones in greater detail. You also will see some diseases that aren’t visible with a CT scan, such as cancer and incidence of metastasis. An MRI uses potent magnetic fields and radiofrequency instead of radiation.
Normal vs. Paget's bone on leg

Normal vs. Paget's bone on leg

Available treatments

PDB is incurable, but there are treatments to help deal with symptoms. They include:

  • Bisphosphonate medication. These medicines lend some control to bone regeneration.
  • Painkillers. The usual painkillers like ibuprofen and paracetamol are normally prescribed.
  • Supportive therapies. These include walking sticks, shoe inserts, physiotherapy, and occupational therapy.
  • Surgery. This will be required if there are deformities, fractures, or excessive pain in the joints.
  • Vitamins. Supplements can help, including calcium and vitamin D.

Progressive PDB

  • Bones become fragile and break more easily.
  • Bones grow bigger and/or are malformed.
  • Affected skull bones may cause loss of hearing.
  • Excess calcium enters the blood, which can cause kidney stones and affect your brain and heart functioning.
  • In rare cases, Paget’s can lead to bone cancer.

NOTE: Originally published in Enrich Magazine, August 2021 edition

Comments

Mona Sabalones Gonzalez (author) from Philippines on October 24, 2021:

Hi John, thank you for your kind words. I'm glad to know that you liked the article. Cheers!

John Hansen from Gondwana Land on October 20, 2021:

This is very informative. I had heard of the condition but never knew much about it. Thank you for sharing.

Mona Sabalones Gonzalez (author) from Philippines on October 20, 2021:

Thanks MG, for your kind comments and the visit. Hope things are going well with you:):):)

MG Singh emge from Singapore on October 04, 2021:

Very informative.

Mona Sabalones Gonzalez (author) from Philippines on October 04, 2021:

Yes Jo, I also hope you never get it. Thank you for the visit, and thank you for your kind words:):):)

Jo Miller from Tennessee on October 04, 2021:

You have provided some very good useful information for this disease that I hope I never need.

Good job!

Related Articles