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cardiac rehabilitation principles can be extrapolated
and applied to the adult OI population when cardiac
changes occur. The goal of cardiac rehabilitation, per the
American Association of Cardiovascular and Pulmonary
Rehabilitation, is to improve or maintain cardiovascu-
lar fitness.27 27 This can be accomplished through risk fac-
tor modification in addition to a consistently performed
home exercise program. Risk factors that potentially can
be modified include hypertension, smoking, obesity, sed-
entary lifestyle, diabetes mellitus and elevated cholesterol
and lipids. 28 Risk factors that cannot be modified include
age, male gender and family history. Goals of therapeutic
exercise include decreasing bone loss, increasing strength
and improving balance to prevent falls.
There are three phases in cardiac rehabilitation: (1)
the acute inpatient phase; (2) the supervised outpatient
phase which lasts 3-6 months and (3) the maintenance
phase. The goals are to improve: exercise tolerance,
symptoms and blood lipid levels. 29
After cardiac surgery, isometrics should be avoided as
these can increase heart rate. Valsalva maneuvers should
also be avoided as this can promote arrhythmias. Raising
legs above the heart can increase preload, as can arm
ergometry exercises.
After discharge and during the subacute phase, cal-
isthenics and low grade ambulation using treadmill
training can be incorporated into the rehabilitation pro-
gram. A graded exercise test may also be indicated, as
this functional tool assesses tolerance of increased physi-
cal stress and demands. 27,29 As the supervised outpa-
tient phase of cardiac rehabilitation progresses, a low
impact exercise program can be incorporated including
swimming, stationary cycling or the use of an elliptical
machine. Aquatic therapy has been shown to improve
strength and mobility. 30 A trial of each type of low impact
exercise is important to determine which is best tolerated
by the patient, as the ultimate goal for the patient is to
consistently perform the program independently during
the maintenance phase.
lung disease. In restrictive lung disease, almost all lung
volumes are decreased. The majority of acute respiratory
failures as a result of restrictive lung disease are due to
impaired clearance of secretions. Rehabilitation for indi-
viduals with restrictive lung disease includes educa-
tion of patients on signs and symptoms of pneumonia
due to impaired secretion clearance; reinforcement of
importance of vaccinations such as influenza and pneu-
mococcal as indicated; and education on potential aspi-
ration risks. Instruction in glossopharyngeal breathing,
deep breathing, insufflation, manually assisted cough
and suctioning can be provided to the patient and their
caregiver. The use of noninvasive ventilation (including
positive pressure body ventilators, negative and positive
pressure body ventilators, and negative pressure venti-
lators) can be initiated as well. A pulmonologist as well
as a specialist in pulmonary rehabilitation can collabora-
tively decide on the best method to improve exercise tol-
erance, dyspnea and overall quality of life. 32,33
CONCLUSION
OI, a connective tissue disorder that involves multiple
organ systems, can affect an individual's function as they
age. While research specifically focusing on the rehabili-
tation of adults with OI is meager, rehabilitation princi-
ples, strategies and treatments can be extrapolated from
other musculoskeletal and rehabilitation data to guide
appropriate plans of care in this patient population that
are safe and effective in improving mobility, endurance,
transfers, range of motion and pain.
References
[1] Shapiro JR, Germaine-Lee EL. Osteogenesis imperfecta: effect-
ing the transition from adolescent to adult medical care.
J Musculoskelet Neuronal Interact 2012;12(1):24-7.
[2] Montpetit K, Dahan-Oliel N, Ruck-Gibis J, Fassier F, Rauch F,
Glorieux F. Activities and participation in young adults with
osteogenesis imperfecta. J Pediatr Rehab Med 2011;4(1):13-22.
[3] Amako M, Fassier F, Hamdy RC, Aarabi M, Montpetit K,
Glorieux FH. Functional analysis of upper limb deformities in
osteogenesis imperfecta. J Pediatr Orthop 2004;24(6):689-94.
[4] Wekre LL, Froslie KF, Haugen L, Falch JA. A population based
study of demographical variables and ability to perform activi-
ties of daily living in adults with osteogenesis imperfecta.
Disabil Rehabil 2010;32(7):579-87.
[5] Gentry BA, Ferreira J, McCambridge AJ, Brown M, Phillips
CL. Skeletal muscle weakness in osteogensis imperfecta mice.
Matrix Biol 2010;29:638-44.
[6] Kaux J, Foidart-Dessalle M, Croisier J, Toussaint G, Forthomme B,
Crielaard J. Physiotherapy intervention for joint hypermobil-
ity in three cases with heritable connective tissue disorders.
J Musculoskelet Pain 2010;18(3):254-60.
[7] Van Brussel M, et  al. Physical training in children with osteo-
genesis imperfecta. J Pediatr 2008;152(1):111-6.
[8] Yochum TR, Kulbaba S, Seibert RE. Osteogenesis imperfecta in a
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PU LMONARY REHABILITATI ON
The incidence of scoliosis in OI is between 39% and
80%. Up to 60% of individuals with OI have significant
chest wall deformities. Increased stiffness of the chest
wall, such as that due to scoliosis, can result in impaired
lung ventilation which in turn can lead to respiratory
insufficiency. Pulmonary compromise is the leading
cause of death in adults with OI, 31 and restrictive lung
disease is the most common type of pulmonary pathol-
ogy due to the above. Although there is a paucity of
research on the effects of pulmonary rehabilitation in
adults with OI, there is literature on the benefits of pul-
monary rehabilitation for individuals with restrictive
 
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