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About
OI
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Causes
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Fractures
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Treatment
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OI is treated primarily by
managing fractures and promoting as much mobility and
independence as possible. Prolonged immobility can
further weaken bones and lead to muscle loss, weakness,
and more fractures. Many orthopedists prefer to treat
fractures with short-term immobilization in lightweight
casts, splints, or braces to allow some movement as soon
as possible after the fracture.
Various minerals and medications
have been tested throughout the years to determine if
they strengthen bone in OI. Most of these substances have
not been proven effective. Current therapy is focused on
bisphosphonates.
- Healthy Lifestyle
- Cyclic intravenous
Pamidronate
- Intermedullary Rodding to
support long bones
- Somatic Cell
Therapy
Healthy Lifestyle
People with OI benefit from a
generally healthy lifestyle, including safe exercise and
a nutritious diet. Adequate intake of nutrients, such as
calcium (to maintain bone density) and Vitamin C (to
promote healing) is important. However, megadoses of
these nutrients are not recommended.
Evaluation by a physician or
registered dietitian will help people with OI determine
adequate nutrient intake for their body size and age. It
is also recommended that people with OI avoid smoking,
excessive alcohol or caffeine consumption, and steroid
medications, which may affect bone density.
Physical therapy is beneficial for
children and adults with OI to maintain independence and
bone and muscle mass. The long-term goal for children
with OI is independence in all life functions (e.g.,
self-care, locomotion, recreation, social interaction,
and education), with adaptive devices as needed. Swimming
and water therapy are particularly well-suited for people
with OI of all ages, as they allow independent movement
with little fracture risk. Walking is also excellent
exercise for those who are able (with or without mobility
aids).
Cyclic intravenous
Pamidronate
Cyclic intravenous
Pamidronate is a safe short-term therapy for the
treatment of osteoporosis in children with Osteogenesis
Imperfecta.
Cyclic Intravenous Pamidronate
therapy uniformly results in improvement with
- increased mobility,
- decreased pain,
- increased feeling of
well-being,
- decreased fractures,
and
- an increase in bone
density.
For many children there is a return
to normal ranges of bone density within two to three
years of commencing therapy. The largest changes are
visible in infants and during puberty.
Bone densitometry can be
standardised for children and cyclic intravenous
Pamidronate can be shown to increase BMD throughout the
skeleton. There is a positive enhancement of growth, a
trend towards normal remodelling of long bones and
spine.
Two regimens have been
effective:
- A single intravenous dose given
either monthly or second monthly or
- Three doses given on
consecutive days every three-four months.
While monitoring needs to be
undertaken in centres equipped to undertake this, therapy
can be given at local hospitals supervised by
paediatricians or family practitioners.
Short-term safety has been
established however long-term safety remains a concern.
For example, the safety of pregnancy in adults who have
received bisphosphonates for many years in childhood has
yet to be established.
The data from the randomised study
performed at The Children’s Hospital at Westmead
indicates that the maximum treatment effect occurs in the
first six months after commencing treatment. The
treatment regimen for children commencing therapy for
Osteogenesis Imperfecta consist of intravenous
Pamidronate 1 mg/kg/month given as a single infusion over
two hours in the first six months. Depending on the
response, therapy should then be continued with cyclic
intravenous Pamidronate given every second monthly until
BMD is in the normal range. Outcomes should be evaluated
by centres with the experience in the clinical and
rehabilitation assessment of metabolic bone disease, and
with the laboratory and bone densitometry services able
to offer expert monitoring.
> Read
report from Professor David Sillence regarding
Bisphosphonate Treatment Program - The Children's
Hospital at Westmead, 2000
> Read
report on Conference in 2001 on the use of
Bisphosphonates - Royal North Shore Hospital in Sydney in
July 2001
> Read
1998 Report from Shriners Hospital for Children in
Montreal
Intermeduallary Rodding
Surgery
 Many
children with OI undergo a surgical procedure known as
rodding, in which metal rods are inserted into the long
bones to control fractures and improve deformities that
interfere with function. There are two basic types of
rods. Nonexpandable rods are more versatile but often
must be replaced as the child grows. Expandable rods can
grow with the bone, but are only appropriate for larger
bones (such as the femur) due to their thickness and need
to be firmly anchored at both ends.
Somatic Cell Therapy
Somatic cell therapy such as
bone marrow transplantation plays no role at the present
time in the management of OI.
Stem cell therapies are being
investigated in laboratory and animal studies.
Other agents such as parathyroid
hormone, and prostaglandins have been studied but at the
present time have no therapeutic application in the
Brittle Bone Disorders. Other treatments, including
growth hormone, gene therapies, and cell therapies, are
also being researched.
References
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