Treatment
There is not yet a cure for O.I. Treatment is directed toward preventing or
controlling the symptoms, maximizing independent mobility, and developing
optimal bone mass and muscle strength. Care of fractures, extensive surgical
and dental procedures, and physical therapy are often recommended for people
with O.I. Use of wheelchairs, braces, and other mobility aids is common,
particularly (although not exclusively) among people with more severe types of
O.I.
People with O.I. are encouraged to
exercise as much as possible to promote muscle and bone strength, which can
help prevent fractures. Swimming and water therapy are common exercise choices
for people with O.I., as water allows independent movement with little risk of
fracture. For those who are able, walking (with or without mobility aids) is
excellent exercise. People with O.I. should consult their physician and/or
physical therapist to discuss appropriate and safe exercise.
Children and adults with O.I. will
also benefit from maintaining a healthy weight, eating a nutritious diet, and
avoiding activities such as smoking, excessive alcohol and caffeine
consumption, and taking steroid medications — all of which may deplete bone and
make bones more fragile. For more information on nutrition, see the O.I. Foundation
fact sheet titled “Nutrition.”
A surgical procedure called
“rodding” is frequently considered for people with O.I. This treatment involves
inserting metal rods through the length of the long bones to strengthen them
and prevent and/or correct deformities.
This treatment is most commonly done in the legs and the arms as
needed. Rodding not only corrects bowing (curving) of the bone, but also
adds an internal support that helps to prevent further fractures. The
age of the child on which this operation is performed depends greatly on
the size of the bones, but it is frequently done on children as young
as two or three years old.
When considering this option for your child, be sure to discuss the
pros and cons of telescoping and nontelescoping rods with your
orthopedic surgeon. Also, keep in mind that this is a treatment that
must be repeated as the child outgrows the rods.
Several medications and other
treatments are being explored for their potential use to treat O.I. These
include growth hormone treatment, treatment with intravenous and oral drugs
called bisphosphonates, an injected drug called teriparatide (for adults only)
and gene therapies. It is not clear if people with recessive O.I. will respond in
the same manner as people with dominant O.I. to these treatments. The O.I.
Foundation provides current information on research studies, as well as
information about participating in clinical trials.
Prognosis
The prognosis for a person with O.I. varies greatly depending on the number and
severity of symptoms. Respiratory failure is the most frequent cause of death
for people with O.I., followed by accidental trauma. Despite numerous fractures,
restricted physical activity, and short stature, most adults and children with
O.I. lead productive and successful lives. They attend school, develop friendships
and other relationships, have careers, raise families, participate in sports
and other recreational activities and are active members of their communities.
