what-when-how
In Depth Tutorials and Information
Research has shown that genotype-phenotype correla-
tions are often complex and unpredictable and thus not
all type I patients are the same.
10,12
Most adults with OI
understand the movements that lead to fracture, and
their knowledge of this will help in designing a rehab
protocol. The location of rods or fixators can indicate
which bones are more stable and less susceptible to frac-
ture. Orthopedic special testing will be performed based
on the impairment level of the OI. Due the increased
ligamentous laxity some of the tests may reveal a false
positive so the injured side should always be compared
to that of the non-impaired side.
For patients with more severe OI impairment liga-
mentous testing is not appropriate due to the increased
risk of injury or fracture. Knowing the clinical signs of
an injured ligament, and performing functional testing
or imaging are ways of determining the impairment.
Muscle strength testing should be modified to accom-
modate the level of impairment of the adult with OI.
The longer the lever arm the more force generated on
the bone, thus making the adult more susceptible to frac-
ture. When manual muscle testing these patients, break
testing is recommended so that the force applied to the
limb could be better controlled during testing. For the
patient with a more severe level of impairment strength
testing will have to be modified. Evaluating the effects
of strength in a functional context can give an idea of the
areas of weakness. Muscle length and range of motion
is another critical component to consider in the evalu-
ation. Bone deformity is a frequent occurrence in OI
which significantly impacts muscle length and range of
motion. A muscle that is shortened or lengthened due to
bone deformity is not working at its maximum capacity.
Knowing which muscles are impaired is critical to creat-
ing an appropriate treatment plan.
Cardiac history and precautions are critical to the
evaluation process in adults with OI. These adults have
an increased risk of cardiac issues due to abnormal col-
lagen in their heart (this is discussed further in “Cardiac
Rehabilitation,” below). For this reason it is essential to
communicate with the physician regarding the patient's
cardiac issues and precautions. Basic cardiac testing
including O
2
saturation, heart rate, blood pressure and 6
minute walk/wheelchair propulsion testing adults with
this mobility are needed for the creation of the treatment
plan. Cardiac rehabilitation will be addressed later in the
chapter.
weight bearing status. This is especially true for postoper-
ative repair patients. These patients can have an extended
rehabilitation process postoperatively due to impaired
skeletal structure and the additional hardware needed to
adhere repaired ligaments to compromised bones.
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The goals for rehabilitative care either for non-
operative care or postoperative care are to increase
strength, range of motion and mobility to the affected
limb and restore the patient to their optimal level of
function.
COMPONENTS OF CARE
The progressions listed below are from least to most
severe and the progression should stop along the con-
tinuum based on the highest functioning level that can
be achieved by the patient:
1.
Modalities are useful in the reduction of swelling,
effusion and pain as well as the promotion of
tissue healing after injury. Cryotherapy can be
used to reduce swelling and effusion. Electrical
stimulation in the form of transcutaneous electrical
nerve stimulation (TENS), neuromuscular electrical
stimulation (NMES) or iontophoresis can be a
beneficial modality promoting healing and reducing
pain associated with injury. TENS is a low intensity
electrical stimulation that is applied over the painful
area and designed to work through the pain channels
in the nervous system. When the stimulation is
turned on it will theoretically block the pain stimulus
from traveling along the nerve and thus reduce or
eliminate the pain.
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2.
Neuromuscular electrical stimulation is a higher
level of electrical current applied to the muscle to
cause a contraction. This is designed to strengthen
the muscle and re-educate it on how to move
properly thereby making the joint or limb more
stable and better able to function. NMES can be
put into any functional context to assist with range
of motion, strengthening and even motor learning
activities.
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Iontophoresis delivers medication to a
painful area in a more localized way. The medication
has an electric charge that is matched to the charge
on the electrode pad. When the machine is turned
on the electric charges repel each other and thus the
medication is push through the skin to the area of
injury to reduce the pain.
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3.
Passive, active assistive and active ranges of motion
are the open chain activities that are a critical
component of care. Progression from open chain
activities to strengthening with resistance can be
done for those more mildly affected. Progression
should be monitored closely to ensure proper form
TREATMENT
Below are the general guidelines of rehabilitation.
Close communication between the physician and thera-
pist is critical to guide the rehabilitation process and is
essential for monitoring changes and progression in the
