what-when-how
In Depth Tutorials and Information
sports and physical activities with sudden rotation
movements of the joints are strongly discouraged.
activities or training (when indicated) to counteract
subjective fatigue.
Pain
Pain described in children with OI is, in the major-
ity, diagnosed as bone pain due to (micro) fractures.
Considering the effects of medication on experienced
pain there remain strong contradictions. Phillipi et al.
described in a Cochrane review the general effects of
bisphosphonate therapy in OI (children and adults) and
presented the effects on bone pain as well.
27
The results
of this study indicated a non-significant reduction of
pain after oral bisphosphonate therapy. Similar results
were found in the 2-year follow-up randomized con-
trolled trial (RCT) of Kok et al. regarding the influence
of oral bisphosphonates (olpadronate 10 mg/m
2
/day)
on quality of life and pain in children with OI.
28
The
results of this RCT indicated also a small but not sig-
nificant decrease of pain in the bisphosphonate group.
28
Simultaneously, the study of Letocha et al. also reported
no changes in pain after bisphosphonate treatment.
29
On the other hand, the study of Seikaly et al. reported a
significant decrease of pain in the alendronate group of
their study at 12 months after therapy.
30
Furthermore,
Chevrel et al. reported comparable pain values in their
alendronate and control group at the end of the treat-
ment, up to 30 months and an increase of pain in the
alendronate group at 36 months.
31
In these studies, ten-
dencies regarding the influence of medication on pain
are mentioned; on the other hand, discussion remains
regarding the type, dose and use of medication.
PAR
TICIPATION AND ACTIVI
TIES
Motor Development
Engelbert et al. described the motor development
of children with different types of OI (age of achieving
motor milestones achieved by a postal questionnaire)
and established a delay in achieving motor milestones
in OI type I, comparable to the 95th percentile of the
normal population.
33
In OI type III, the development
of all motor milestones was significantly delayed with
a discrepancy between static and dynamic milestones.
In OI type IV, retardation in motor development began
after the milestone “sitting without support” was
achieved. Motor development in OI types I and IV was
not influenced by intramedullary rodding of the lower
extremities, since rodding was rarely performed before
the milestone “unsupported standing” was achieved.
In OI type III, the influence of intramedullary rodding
on the age of achieving motor milestones remains ques-
tionable. The authors concluded that the severity of OI
has a large influence on the age and sequence in the
development of motor milestones.
33
Recently, the study of Alcausie et al. described the
skeletal and gross-motor developmental effects of intra-
venous pamidronate therapy in a cohort of infants with
OI who started the therapy at age under 36 months.
34
Earlier attainment was noted in rolling over, crawling,
pulling to stand and walking independently but not in
sitting. The authors concluded that intravenous pami-
dronate treatment, commenced before 36 months of age
in children with moderate to severe OI was well toler-
ated and motor milestones were attained at an earlier
age compared with untreated historical controls.
34
Fatigue
The earlier mentioned RCT regarding a supervised
training program of Van Brussel et al. also studied the
subjective fatigue in the same population.
19
Fatigue
was measured with the subscale subjective fatigue
of the self-report questionnaire Checklist Individual
Strength-20 (CIS-20).
32
The CIS-20 asks about fatigue in
the 2 weeks before the assessment. The questionnaire
includes four respective subscales, fatigue, concentra-
tion, motivation and physical activity, consisting of
items scored on a 7-point Likert scale. Van Brussel et al.
reported that subjective fatigue levels were decreased
with borderline statistical significance in the interven-
tion group (adjusted for baseline measures, 4.2 points;
95% CI, 0.3-8.8,
p
-value: 0.068).
19
These results indicate
that fatigue can be influenced by an individualized
intervention program. Unfortunately, the children were
not able to maintain the gained training effects 3 and
6 months after the completion of the intervention pro-
gram. This latter highlights the continuation of physical
Functional Ability
In the prospective study of Engelbert et al.,
10
regard-
ing impairment and disability in 49 children (mean age
(SD): 11.3(3.8); no standard medication) with a follow-
up of 4 years, the level of ambulation, the amount of
functional ability and caregiver assistance were mea-
sured using the Pediatric Evaluation of Disability
Inventory (PEDI).
10
The results of this study indicated
a significant increase in self-care and social function
in children with OI type I. Children with OI type III
showed a significant increase in self-care, mobility and
social function, with a significant decrease in paren-
tal assistance in self-care and mobility. In children
with OI type IV, a significant increase was observed in
