Paget's disease

Paget's disease
is a relatively common bone condition

that affects the skeleton.

It could arise
in one bone in the skeleton

or in several bones in the skeleton.

It's commonly found in the skull,
the spine, the pelvis,

the hip and the lower legs.

It's a relatively rare condition
in younger people.

But around about the age of 55,

it's found in about one to two per cent
of white adults.

And this increases by the age of 80
when it's thought to occur

in approximately eight per cent of males
and five per cent of females.

I've got it in my left tibia,
the bone I've broken twice,...

...and in the right-hand side
of my pelvis.

I'm very mindful of it. I'm not allowed
to run, but that's no hardship

because I've never enjoyed sport

I'm not allowed to jar it.

I mustn't because
it could break again quite easily.

The one thing that has ceased
to be a pleasure is walking.

It's really a condition
which is concerned with bone turnover.

We think about the bone
as being a living, dynamic tissue

which is constantly being
remodelled and renewed.

There are two types of cells
involved in this process.

One type of cell is the osteoclast,

which is the bone
that destroys the old bone,

then there is a type of cell called the
osteoblast, which builds up new bone.

In Paget's disease, the osteoclasts,
there are a lot more of them,

and they work very, very rapidly.

In response to this, the osteoblast
cells also work very rapidly.

The result of this
at the end of this process

is that the bone
becomes much, much bigger.

You might think that that bigger bone
means healthy bone.

But in this particular case,
the inside is fragmented,

and it's much weaker,
which is why it can easily break.

I don't have a lot of inconvenience.

I have odd twinges of being made aware
that that is the spot that you broke.

In the front of my left tibia,
I have odd twinges.

But I really don't have any pain.

One way of diagnosing it is by having
an x-ray taken of the affected area.

But it can also be diagnosed
on an isotope bone scan

which is when an individual
has radio isotope tracer

injected into the blood
which goes round the whole body.

If there are areas of Paget's
in bones around the body,

these are highlighted
as what could be defined as hot spots.

By looking at the result through
a radio isotope bone scan

you can actually see
areas of Paget's disease.

Another critical way of diagnosing it:

there is a substance released by
the bone cells when they are overactive.

This substance is elevated.

That is checked very easily
by performing a simple blood test.

One of the most interesting things
about Paget's disease

is a lot of people have it
and have no symptoms whatsoever.

It's sometimes discovered because
they have an x-ray for another reason

or they have a blood test taken.

But for those people who do have
symptoms of Paget's disease,

the most important symptom is pain.

The pain is often associated
with a feeling of heat from the bone.

For example, if someone
has Paget's disease in the shinbone,

if you hold the hand next to the shin,

you can sometimes feel heat
coming off the bone.

In addition to pain,
it is possible to have deformity

at the site of the Paget's disease.

And so, for example, if somebody
has Paget's disease in the skull,

the skull will be larger than normal
and the forehead can look flattened.

if somebody has Paget's disease

in the lower shinbone or the hipbone,

the bone itself
can actually bow outwards and distort.

Until about 30 years ago, there was
limited treatment for Paget's disease.

People had to suffer the pain
and deformity,

but then there was a group of drugs
discovered called bisphosphonates.

These drugs are used it the treatment
of a lot of bone disorders

and Paget's is one of them.

They are particularly specific
to the activity of the bone cells.

They calm down the activity
of the bone cells

and subsequently will reduce
the bone turnover

which causes the pain and the problems.

My treatment's gone extremely well.

I've had various courses
of bisphosphonates.

Three or four I must have had,

with this latest infusion, and it has,
I think the term is, put it to sleep,

that it isn't progressing.

Gradually, each time
after a course of treatment

your alkaline phosphatase will rise,

and when it gets to the level
that they feel it needs treatment,

then you have
another course of treatment,

be it tablets or infusions.

And so it goes on.
You go up and it's kept control of.

In fact, there are slightly divided
opinions about when you do treat.

In certain situations,

certain doctors would consider treatment
even without symptoms,

because they feel symptoms will arise
and therefore they should treat.

On the other hand,
there is another school of thought

which favours the approach of
let's wait and see,

let's see if and when
symptoms do develop.

(Elizabeth) The most important thing
is to see a consultant

who knows about Paget's,
knows a lot about Paget's.

I know that the National Association
for the Relief of Paget's Disease

will advise people
on who in their locality

is, or knows about who is,
an expert in Paget's.

I really think
that is the most important thing.

I think very much the outlook these days
is very optimistic.

Obviously, in the olden days,
people weren't treated adequately.

They did develop deformity, they did
develop fractures in the affected bones.

But I think importantly,
drugs are available,

and provided somebody is adequately
diagnosed and adequately treated,

they should be able to contain the
disease and stop it getting any worse.