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In Depth Tutorials and Information
3.
Family involvement in goal setting for their children is
essential.
In general, knowledge of OI is essential and tailored
care should be based on the individual patient's
complaints and needs. Tailored care in terms of
evidence-based diagnostic procedures and clinical
expertise is essential in order to construct classification
models as well as optimized treatment strategies. A
multidisciplinary approach is frequently indicated;
however, this needs further scientific and clinical
exploration. Therefore, diagnostics and treatment
should be based on evidence-based practice, individual
outcome measures and clinical reasoning which
eventually leads towards individual classification
and tailored treatment strategies. Furthermore, a
multidisciplinary approach, in which the parents
should be involved, is frequently indicated.
The family and all the associated care givers should
also be involved when children enter the transition
into adulthood. Recently, Shapiro et al. reported in an
observational report the importance of the transition
effect in children from adolescent to adult medical
care.
44
They presented methods for dealing with
transitional issues. Coordinated team support should
provide the best level of care for the child with OI in
which topics related to effecting the transition from
pediatric to adult care can be addressed: transitioning
and maintaining health-preserving or improving the
level of function-assuring continuity of medical/
surgical care-restructuring psychosocial and work-
related systems. The authors also mentioned that the
process of transition requires active communication
between the pediatric and adult team members along
with a proactive approach by the patient and family.
In addition, as the transition is established, the
patient with OI should be encouraged to be his/her
own advocate and care coordinator.
4.
The role of physical fitness becomes more essential.
The literature concerning the relation between
physical activity levels and its influence on systemic
health is piling up. The adagium “active now, healthy
later” currently guides patient care and therapeutic
strategies. Studies regarding muscle strength
in this specific population are relatively scarce;
however, they indicate that muscular strength can
be affected in children. Nonetheless, the studies are
too scarce and heterogenic in order to make general
statements. Studies concerning the effects of (non)
pharmacological interventions seem promising
for the mild to moderate types of OI, especially
pamidronate therapy and exercise training. However,
determination and standardization of a core set
for the assessment of physical fitness and function
are essential for future studies. As pediatric health
professionals, we must understand the effect of each
chronic condition on specific components of physical
fitness and, in turn, the effects of these impairments
on functional capacity. Many children with chronic
conditions have reduced levels of aerobic fitness
(expressed as VO
2peak/kg
) as do children with
OI. Frequently, these reductions are caused by a
combination of condition-related pathophysiology
itself, treatment of the condition (such as certain
medications), hypoactivity and deconditioning.
Determining physical fitness levels in children
with chronic medical conditions is of great clinical
relevance, as this variable is a powerful predictor
of mortality in people with or without disease.
40,45
The randomized and controlled exercise training
study of van Brussel et al.
19
showed significant
improvements in the physical fitness domain;
however, little information is yet available on the
clinical characteristics of children that may be used
to predict who is likely to benefit with improvements
in exercise capacity.
37
The clinician would benefit
from prediction rules for identifying which children
might benefit from an exercise program and exercise
guidelines on how to design a disease and child-
tailored training program. The first step in this
process is to determine whether the particular
individual is actually physically deconditioned. When
deconditioning is objectively determined, possibly
through exercise testing, the next step (and possibly
the most important one) is to determine whether
deconditioning is either due to inactivity, medication,
nutritional status or disease-related pathophysiology
or due to a combination of these factors. In other
words, the therapist should determine all the real
and/or perceived limitations imposed by the child's
chronic condition. Type of activity should always be
suited to the specific chronic condition; in the context
of this chapter, contact sports and physical activities
with sudden rotation moments of the joints are
strongly discouraged for children with OI.
37
Future
pediatric and transitional studies should also focus
upon determination of lung function in children with
OI, because in the adult phase pulmonary complaints
are more and more evident and limit their physical
activities.
5.
From case-reports to cross-sectional to longitudinal
studies and randomized controlled trials
.
Scientific research in OI is essential to understand
the pathophysiology and the effects of intervention.
Little information regarding functional outcome of
conservative, surgical and medication intervention
in randomized controlled trials is available and
should be expanded in future. Development of
disease-specific instruments, instead of using generic
instruments, should be discussed as well as using
disease-related reference values.
