Episode 50: Paget’s Disease (Bone) - A Disease of Prehistoric Repercussions - Anatomy Of Illness

Episode Transcript
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(00:00):
Lately, you have been struggling with a lot of hip pain, which you initially shrugged

(00:03):
off as a consequence of getting older, especially with your 60th birthday just around the corner.
However, you've recently noticed that your knees are also beginning to act up, making
it difficult to chase after your grandchildren when they visit.
Just the other day, your daughter made an observation that sent a chill down your spine.
She pointed out that your legs appeared oddly bent, almost bow-legged.

(00:24):
You brushed off her concerns at first, but the nagging feeling that something isn't quite
right has lingered.
After discussing it with your wife, she encouraged you to go to the doctor.

(00:48):
Hello and welcome to Anatomy of Illness.
Today's episode is about Paget's disease of the bone.
Before we get into the condition, we are going to start with the history.
So why do we know Paget's disease of the bone exists?
I may also call it osteitis deformans or simply Paget's disease throughout the episode.
We begin in 1876, as this is when the first description of Paget's disease would occur.

(01:08):
This was by Sir James Paget.
He was at the time a prominent English surgeon.
He described the cases of five men who had at least two deformed areas of the skeleton.
He called the condition osteitis deformans.
What he thought he was describing was a new and rare inflammatory disorder.
However, this was not the case.
Bones with evidence of Paget's disease could be found in France and Italy as far back as

(01:29):
the Neolithic period, which dates between 3500 BC to 2000 BC.
There is also evidence of it being found in the Late Bronze Age period in Greece and through
much of Europe throughout the Roman Empire and medieval periods.
Evidence of this condition has also been found across the Americas in the pre-Columbus period.
This has been found as early as 1300 AD to before the arrival of the Spanish in the Americas.

(01:55):
However, none of these would be the original case of the condition.
For that, we have to travel back even further and even leave our species.
Yes, that is right.
We are moving to the Late Jurassic period 150 million years ago.
So please, eyes and ears out, arms to selves if you want to keep them.
A dinosaur from this period, which was found in current day Tanzania, was found to have

(02:17):
Paget's disease.
This was found rather recently though, in 2011, leaving the Jurassic period and heading
back to the times of humans.
In 1885, Butlin claims to have found evidence of Paget's disease in a Neanderthal, specifically
the skull.
This was dating back to the prehistoric period.
However, this has not been proven to be Paget's disease.

(02:37):
This was due to, at the time, no established differential diagnosis existing for the symptoms.
So whether or not it is Paget's disease is unknown.
In 1888, the condition would first be referred to as Paget's disease.
So prior to this, it was osteitis deformans.
This was done by Sir Jonathan Hutchinson in a publication on the condition.
With the discovery of x-rays not long after the first description of Paget's disease,

(03:01):
we would have the visualisation of what the condition actually caused the bones to look
like in living people.
In 1896, the first x-ray report of Paget's disease occurred.
With the dawn of x-ray came a new ability.
We could detect bones that otherwise on the outside seem unaffected.
So we were able to diagnose people earlier.
The UK in 1929 would recognise the first biochemical marker for Paget's disease.

(03:25):
This would not only help with diagnosis, but also the monitoring of the condition.
So what actually is Paget's disease?
Paget's disease is when there is a rapid growth of new bone.
This new bone is structurally less organised and weaker in comparison to the old bone.
This can lead to an increase in fractures and breaks, but it can also lead to damaging
of the nerves.
What causes Paget's disease?

(03:46):
The specific cause of Paget's disease is currently unknown.
There are several theories surrounding the cause behind Paget's disease, some more
accepted than others.
Speaking of accepted, if you want to be seen as the cool kid, be sure to check out merchanatomy.com
for the coolest clothes and accessories.
Anyway, back to theories behind what causes Paget's disease.
These theories include genetic factors.

(04:06):
Several genes have been found that appear to be linked to developing Paget's disease.
Environmental factors.
This seems to be combined with various genetic factors as an increased risk of developing
Paget's disease.
And of course, viral.
This is the idea that there is a viral infection in the bone cells contributing to the occurrence
of Paget's disease.
However, this is less accepted.

(04:27):
What are the risk factors for developing Paget's disease?
Some of the risk factors for developing Paget's disease include age.
People older than 50 are more likely to develop Paget's disease.
However, it can also occur at a younger age.
So this may be early onset or considered juvenile if it is a young person.
Men are more likely than women to get Paget's disease.

(04:49):
It's about a three to two ratio.
So three men to two women.
Family history.
If you have a relative who has Paget's disease, you are more likely to develop the condition
yourself.
This is again why they think genetics are also in play.
Ethnicity.
Paget's disease of the bone is more common in specific areas.
These being Greece, England, Scotland, and Central Europe.

(05:10):
This also extends to the countries where people who descend from these origins have migrated
to.
The risk factors for developing Paget's disease of the bone is less common in areas like Scandinavia
and Asia.
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it with them.
How do we test for Paget's disease?
Firstly, a doctor will examine areas of the body that are causing pain.
After this, blood tests may be ordered.

(05:30):
Specifically a blood test to determine the levels of alkaline phosphatase in the blood
as elevated levels of these can be a sign of Paget's disease.
Loving my rhymes.
Anyway, imaging tests will also be done.
This can either be an x-ray or a bone scan.
Or it can be both.
On an x-ray there may be signs of bone breakdown, enlargement of the bone, and deformities such

(05:51):
as bowing of the long bones.
In a bone scan, radioactive material is injected into the body.
This will indicate the areas most affected by the condition.
We'll get right into the symptoms and presentation right after this little break.
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(06:13):
What symptoms might you have if you had Paget's disease with a bone?
The symptoms will vary depending on what bone is affected.
But many with the condition will initially not have any symptoms.
For those who have Paget's disease affecting the skull, this can cause symptoms of hearing
loss and headaches.

(06:34):
For those whose spine is affected, they may experience compression of the nerves and nerve
roots.
To pain, tingling, and numbness in either an arm or a leg, or sometimes in multiple
places.
If your pelvis is affected, then you may experience hip pain.
And for those whose legs are affected, with the bones weakening they can bow, leading
to a bow-legged appearance.
These enlarged and misshapen bones can then put more stress on your joints, leading to

(06:58):
osteoarthritis of the hip or knee, or sometimes both.
What are some complications a doctor should be aware of when it comes to Paget's disease
of the bone?
When it comes to managing Paget's disease, a doctor should be aware of fractures and
deformities.
People with Paget's disease are more likely to experience bone fractures in their affected
bones, as these bones do break more easily than normal bones in other areas.

(07:20):
These affected bones also have more blood vessels, which cause more bleeding during
surgeries that fix these fractures.
With Paget's disease, your legs can also bow, causing difficulty with walking.
Osteoarthritis Due to Paget's disease causing misshapen
bones, more stress is often put on the nearby joints.
This can lead to osteoarthritis.
Neurological issues Paget's disease can occur in areas where

(07:41):
the nerves pass through the bone.
This is areas like the skull and the spine.
Bone overgrowth in these areas can compress and damage the nerves.
This causes pain, weakness or tingling in the arm or leg, or even in some cases hearing
loss.
Heart failure In some of the more severe cases of Paget's
disease, the heart will have to work harder to pump blood to the affected areas of the
body.

(08:02):
This increased workload, however, can have a negative effect on the heart, causing heart
failure.
Bone cancer Bone cancer occurs in up to 1% of people with
Paget's disease.
This is due to the increased bone growth caused by Paget's disease.
Some of it can become diseased or cancerous.
How do we treat Paget's disease?
Well this really depends.
For those who are asymptomatic and those who do not have active disease, treatment may

(08:23):
not be needed.
However, for those who do have active disease, or if your disease is in a high risk site,
like your skull or your spine, they may recommend treatment even if it is inactive, so not currently
progressing.
The disease is considered active if you have high levels of alkaline phosphatase in the
blood.
So this is considered a sign that there is a lot of bone growth.

(08:44):
Treatment is often bisphosphonate therapy.
These are medications commonly given for osteoporosis, so they help to prevent loss of bone density.
These are often given intravenously, so into a vein.
An example of this is pamidronate, but in some cases it can be prescribed and given
orally, so by mouth.
An example of this is alendronate.
If you can't tolerate bisphosphonates, there are other options.

(09:04):
This includes calcitonin, which is a hormone that is part of the calcium regulation and
bone metabolism.
This can be done via injection or nasal spray.
Surgery may also be recommended as part of the care.
This may be done in several cases, such as realigning deformed bones, reducing pressure
on nerves, replacing joints that are damaged by severe arthritis, and also to help fractures
heal.

(09:25):
There are also many self-care recommendations that may be helpful in the case of Paget's
disease that your doctor may suggest.
These include exercising regularly.
This helps to maintain joint mobility and bone strength.
Eating well.
Making sure you have a diet with plenty of vitamin D and calcium to ensure you have strong
bones.
Foolproofing your home.
So installing handrails and grab bars in areas like stairways, steps, and bathrooms.

(09:48):
Making sure the bathroom has plenty of non-slip mats, because those tiles can become quite
slippery with water.
Removing any slippery floor coverings you may have, and making sure there are no trip
hazards on the floor, like cords.
Preventing falls.
People with Paget's disease are at a higher risk for fractures and breaks, especially
if they do have a fall.
So doctors may recommend using a walker or a cane to help prevent falls.

(10:11):
If they recommend them, be sure to actually use them.
These tools are only effective if they are used.
Are there any famous people who have or have had Paget's disease?
There is Dom DiMaggio.
If you are a follower of American baseball, you are sure to know his name.
Otherwise he was a star American baseball player, who was one of the three players to
average more than 100 runs per season throughout his entire career.

(10:34):
Dom DiMaggio was diagnosed with Paget's disease in 1962.
He lived with the condition for around 47 years when he passed away at the age of 92
in 2009.
There was also potentially Ludwig van Beethoven, the world renowned composer.
Beethoven died in 1827.
On autopsy they found he had a prominent forehead, an enlarged jaw with a protruding chin, and

(10:55):
also a thickened skull vault.
All of these are signs of Paget's disease.
Beethoven also went deaf.
Another sign of Paget's disease, especially affecting the skull.
However, this was before the condition was a diagnosable illness, or even known about.
This leaves his case up to interpretation, as we can't just go back in time and ask
for a blood sample.
Sadly that doesn't happen.

(11:16):
I don't have a time machine on me.
Hate to break it to you.
So Beethoven is a potential case of Paget's disease, depending on how you interpret his
symptoms.
If you'd like to check out a foundation there is, for those in the UK, the Paget's
Association.
This foundation supports those who are affected with Paget's disease, provides funding for
research into the condition, and also raises awareness of its existence.

(11:37):
There are not really any other foundations that specifically focus on Paget's disease
in other countries.
However, foundations focusing on bone health and bone diseases do provide resources and
care for people with Paget's disease.
So if you are looking for a foundation, make sure to check one out if that's in your
country.
For more information on other sources, social media links, or any other links you can head
to anatomyofillness.com.
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(11:59):
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You can also check us out on YouTube for more great content.
Otherwise stick around for the next episode.

(12:23):
Why did the orthopedic surgeon never get lost?
He had a bone-ified sense of direction.
Get it?
Bone?

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Episode 50: Paget’s Disease (Bone) - A Disease of Prehistoric Repercussions

Episode Transcript

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