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FOP
FAQs
Q
- What does FOP stand for and when was it first documented?
A - FOP or Fibrodysplasia Ossificans Progressiva (Fibro-dis-playsha
Os-sih-fih-cans Pro-gress-eva) means "soft connective tissue
that progressively turns to bone." The earliest documented
cases date back to the 17th and 18th centuries.
Q
- How many people have FOP?
A - It is estimated that FOP affects about 2,500 people
worldwide, or approximately one in two million people. Such
statistics may be better grasped by the following example:
if a large football stadium holds 100,000 fans, one would
need to fill nearly 20 football stadiums to find one person
who has FOP. At the present time, researchers are aware of
approximately 600 people throughout the world who have FOP.
Q
- Can the extra bone be removed?
A - The technical answer is surprisingly "yes," but
that does not tell the whole story. Surgical intervention
often results in a worsening of the condition. New bone will
grow back and further impair mobility.
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Q
- How is the extra bone in FOP different from normal bone?
A - The extra bone in FOP forms by a progressive transformation
of soft tissue into cartilage and bone. This is the same process
by which bone regenerates (heals) after a fracture (break) occurs
and is nearly identical to the process by which bone forms normally
in an embryo. The abnormality in FOP occurs not in the manner of
bone formation but rather in its timing and location.
Q
- What is a flare-up and is it painful?
A - A flare-up occurs when the body starts to generate new
bone. No one knows what initiates this process, but once it begins,
it leads to tissue swelling and much discomfort. Flare-ups are usually
painful. Sometimes the individual will not feel well and may develop
a low-grade fever. While there is no medication or therapy that
can stop the process of bone formation once it has begun, a physician
can prescribe medicine to help relieve the pain. A single flare-up
may continue for as long as 6-8 weeks. However, major overlapping
flare-ups may occur in which pain does not subside as quickly. When
a flare-up is in progress, joint stiffness may occur overnight.
The stiffness comes from swelling and pressure inside the muscle
during the earliest stages of new bone formation.
Most
people find that the pain subsides when a flare-up is over; it appears
that it is the process of extra bone formation, rather than the
extra bone itself, that usually leads to pain. FOP is not always
painful.
Q
- Will FOP get worse? Does it ever stop or go away?
A - Unfortunately, FOP does not improve over time. The "P"
in FOP stands for "Progressiva." That means that FOP will progress,
or get worse, as a person ages. As FOP is part of a person's genetic
make-up, people with FOP are born with the condition, even though
the extra bone may not have appeared at birth. So people with FOP
will not outgrow the condition. Nor can the extra bone that has
been produced by FOP disappear. The body of a person with FOP does
not make extra bone all of the time; a person with FOP may go months
or years without a flare-up. Nevertheless, there is always a chance
that extra bone can form, either without any warning, or as a result
of trauma (injury), such as a bump or a fall. There are also times
when, in spite of obvious trauma, FOP does not manifest itself.
It is unclear why the disease is active some times and quiet or
dormant at other times.
Q
- What parts of the body are involved in FOP? How does FOP affect
mobility?
A - FOP affects the neck, spine, chest, shoulders, elbows,
wrists, hips, knees, ankles, jaw, and many areas in between. The
progression of ossification follows a characteristic pattern. Usually
extra bone forms in the neck, spine, and shoulders before developing
in the elbows, hips and knees. The muscles of the diaphragm, tongue,
eyes, face, and heart are characteristically spared. The well-documented
and characteristic progression of FOP, as well as the regions not
affected, likely hold important clues to the cause and development
of the disease.
FOP
affects mobility because the body's joints, such as the knees or
the elbows, connect the bones and aid in movement. In FOP, extra
bone replaces the ligaments (which cover the joints), as well as
muscles and tendons (which move the joints). Consequently, movement
in areas affected by FOP becomes difficult or impossible.
Q
- What physical variations are seen from person to person?
A - The largest variation seen is the timing and rate of
extra bone formation. Another common variation includes the severity
of movement restriction. For example, an elbow could be fixed in
a closed position, putting the hand permanently across the chest,
or the elbow may be left with some range of motion.
Q
- Is there any treatment for FOP?
A - At this time, there is no treatment for FOP. There is
a drug currently under development which may one day be used to
help control extra bone growth. For now, medication is only helpful
to manage the symptoms of FOP (pain, inflammation, etc.) Read our
medication guide for more information.
Q
- What can I do to help?
A - There are four main things you can
do to help:
- Make sure tissue is collected during
appropriate emergencies
- Arrange for bone marrow to be removed
from the person with FOP within appropriate timeframe
- Publicize yourself to help attract funding and more correct diagnoses,
maybe even multigenerational ones
- Fundraise
Q - How does FOP affect a child's school life?
A - FOP does not affect a person's intelligence or cognitive abilities. It does, however, negatively impact the child's ability to safely access the school premises, use and manipulate the school's materials, and function academically in a typical way. For a detailed discussion of the various issues that may arise in school and the ways others have dealt with them, please click here or go to “Members” and “Parents' Place” or Chapter 6 of the Overcoming Obstacles catalogue where you will also find other relevant ideas and information.
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Symptoms