The risk, burden, and management of non-communicable diseases in cerebral palsy: A scoping review

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DOI: 10.1111/dmcn.13737
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Abstract
Aim To examine the risk, burden, and management of non‐communicable diseases (NCDs) among people with cerebral palsy (CP). Method Databases (Ovid MEDLINE, Embase Ovid, CINAHL Plus) were systematically searched up to August 2017. Data on the prevalence of risk factors for, and the burden and management of, cardiovascular diseases, diabetes, cancers, and respiratory diseases were extracted. Results Thirty‐six studies that examined the prevalence of risk factors among people with CP were identified. There was inconsistent evidence that people with CP had higher prevalence of metabolic risk factors such as hypertension, hyperlipidaemia, and obesity, but strong evidence that they participated in low levels of physical activity, compared with people without CP. Seven studies reported on the burden of NCDs. Adults with CP had a higher risk of NCDs, including stroke, chronic obstructive pulmonary disease, and other heart conditions, and death due to NCDs, including cancers, chronic obstructive pulmonary disease, stroke, and ischaemic heart disease, compared with the general population. Only one study reported on the management of NCD, specifically the uptake of breast cancer screening among females. Interpretation The burden of NCDs is higher among adults with CP compared with the general population. Further research is required to determine the prevalence of metabolic risk factors and management of NCDs among people with CP. What this paper adds • Adults with cerebral palsy (CP) have an increased risk of non‐communicable diseases (NCDs) and increased risk of death because of NCDs. • Evidence is inconsistent about the elevated prevalence of metabolic risk factors for NCDs. • Evidence is consistent that people with CP participate in reduced physical activity. • Only one study reported on management of NCD among people with CP. • Available evidence suggests people with CP are less likely to receive preventive medicine.
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DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY SYSTEMATIC REVIEW
The risk, burden, and management of non-communicable diseases
in cerebral palsy: a scoping review
JENNIFER M RYAN
1,2
|
ELIZABETH ALLEN
3
|
JOHN GORMLEY
4
|
EDWARD A HURVITZ
5
|
MARK D PETERSON
5
1College of Health and Life Sciences, Brunel University, London, UK. 2Department of Epidemiology and Public Health Medicine, Royal College of Surgeons in
Ireland, Dublin, Ireland. 3Department of Medical Statistics, London School of Hygiene and Tropical Medicine, London, UK. 4School of Medicine, Trinity College
Dublin, Dublin, Ireland. 5Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, MI, USA.
Correspondence to Jennifer M Ryan at RCSI Department of Epidemiology and Public Health Medicine, Royal College of Surgeons in Ireland, Lower Mercer Street, Dublin 2, Ireland.
E-mail: Jennifer.Ryan@brunel.ac.uk
PUBLICATION DATA
Accepted for publication 30th January
2018.
Published online
ABBREVIATION
NCD Non-communicable disease
AIM To examine the risk, burden, and management of non-communicable diseases (NCDs)
among people with cerebral palsy (CP).
METHOD Databases (Ovid MEDLINE, Embase Ovid, CINAHL Plus) were systematically
searched up to August 2017. Data on the prevalence of risk factors for, and the burden and
management of, cardiovascular diseases, diabetes, cancers, and respiratory diseases were
extracted.
RESULTS Thirty-six studies that examined the prevalence of risk factors among people with
CP were identified. There was inconsistent evidence that people with CP had higher
prevalence of metabolic risk factors such as hypertension, hyperlipidaemia, and obesity, but
strong evidence that they participated in low levels of physical activity, compared with
people without CP. Seven studies reported on the burden of NCDs. Adults with CP had a
higher risk of NCDs, including stroke, chronic obstructive pulmonary disease, and other heart
conditions, and death due to NCDs, including cancers, chronic obstructive pulmonary
disease, stroke, and ischaemic heart disease, compared with the general population. Only
one study reported on the management of NCD, specifically the uptake of breast cancer
screening among females.
INTERPRETATION The burden of NCDs is higher among adults with CP compared with the
general population. Further research is required to determine the prevalence of metabolic risk
factors and management of NCDs among people with CP.
Cerebral palsy (CP) is a developmental disorder that
results from an injury to the developing fetal or infant
brain.
1
Diagnosis of CP is typically made between 12 and
24 months using a combination of standardized motor
assessments, neuroimaging, and a medical history.
2
CP is
the most common cause of childhood physical disability
with a prevalence of 1.5 to 3.8 per 1000 live births in Eur-
ope, Australia, and the USA.
35
The causal pathway to CP
is not well understood but risk factors include low birth-
weight, placental abnormalities, birth asphyxia, neonatal
infections, emergency Caesarean delivery,
6
maternal obe-
sity,
79
and low socio-economic status.
10
Recent evidence
suggests that a significant proportion of cases have a
genetic component
11,12
and those with a family history of
CP have increased risk of CP.
13
Regardless of the aetiol-
ogy, the hallmark features of CP are abnormal fine and
gross motor functioning.
1
Substantial reductions in mortality rates among children
with CP have been observed over the past decades, with
most children expected to survive to adulthood.
1416
Increases in life expectancy have also been observed among
adolescents and adults with CP who are unable to self-
feed, and this aligns with improvements observed in the
general population.
15
However, life expectancy among
adults with CP who self-feed has not improved at the same
rate as life expectancy in the general population over the
past three decades.
15
The cause of this widening gap in life
expectancy between adults with CP and the general popu-
lation is not well understood. It is clear, however, that
mobility status and particularly the maintenance of inde-
pendent mobility into middle age are strongly predictive of
survival.
16,17
Over the past three decades there has been a substantial
shift in disease burden worldwide from communicable to
non-communicable diseases (NCDs).
18
NCDs now account
for nearly two-thirds of deaths worldwide.
19
The leading
causes of death due to NCDs are cardiovascular diseases,
cancers, respiratory diseases including asthma and chronic
obstructive pulmonary disease, and diabetes. In total, these
four types of disease account for 82% of NCD deaths.
20
©2018 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac KeithPress. DOI: 10.1111/dmcn.13737 1
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The underlying causes of NCDs are shared and modifi-
able risk factors: tobacco use, unhealthy diet, physical inac-
tivity, and excess intake of alcohol.
19,21
These four
modifiable risk factors contribute to metabolic risk factors
for NCDs including overweight and obesity, high blood
pressure, hyperlipidaemia, and hyperglycaemia.
2226
Lead-
ing risk factors for NCDs in the UK are tobacco smoking,
high blood pressure, high body mass index (BMI), alcohol
consumption, hyperlipidaemia, and hyperglycaemia.
27
However, the combination of physical inactivity, high
BMI, and unhealthy diets represents the largest overall
contributor to disease burden.
28
Physical inactivity
accounts for 3.2 million deaths and overweight and obesity
accounts for 3.4 million deaths worldwide per year.
29
In
20062007, the cost to the UK’s National Health Service
related to poor diet was estimated to be £5.8 billion, physi-
cal inactivity was £0.9 billion, smoking was £3.3 billion,
alcohol was £3.3 billion, and obesity was £5.1 billion.
30
Of
importance, there is a robust interrelationship between
physical inactivity, unhealthy dietary practices, and obesity;
thus it is difficult to unravel the direction of association
between these factors, and the progression of and burden
attributed to preventable NCD.
The management of NCDs worldwide should focus on
reduction of risk factors through primary prevention.
21
Primary prevention includes targeting reductions in
tobacco smoking and alcohol consumption, and promoting
healthy diets and participation in physical activity.
21
A sec-
ond priority for prevention of NCDs is opportunistic
screening of adults attending primary health care facili-
ties.
21
Screening of adults at risk for cardiovascular disease
followed by a multi-factorial lifestyle intervention consist-
ing of individual and group-based counselling can improve
long-term alcohol habits, as well as the adoption of a
healthy diet and physical activity.
3133
The World Health
Organization endorses total disease risk rather than risk
management of single factors, to allow early detection and
more cost-effective management of NCDs.
19,34
Timely
treatment of NCDs through universally available, afford-
able, and high-quality drugs is also a priority, as it can
reduce NCD deaths and may be cost-effective by reducing
the need for more expensive treatment as disease
progresses.
21
Although CP is considered a non-progressive disorder,
in that the injury to the brain does not progress with time,
adults with CP experience increases in pain, fatigue, and
chronic musculoskeletal conditions with age.
3539
At least a
quarter of adults with CP also experience declines in
mobility by the age of 40 years.
4042
It has been proposed
that a model of worsening mobility in young adulthood,
accelerated loss of muscle mass, muscle weakness and fati-
gue, progression of motor dysfunction, and reduced partic-
ipation in physical activity exists among people with CP.
43
The consequence of this negative cycle of events may be
an increased risk of NCDs and multimorbidity.
43,44
Addi-
tionally, people with physical and intellectual disabilities
may be less knowledgeable about modifiable risk factors
for NCDs, and therefore less likely to participate in health
screening services for these diseases.
45,46
The aim of this review was to examine the risk, burden,
and management of NCDs among people with CP. The
review focused on the four leading causes of death due to
NCD: cardiovascular diseases, cancers, chronic respiratory
diseases, and diabetes.
20
The specific objectives were to examine (1) the preva-
lence of risk factors for NCDs among people with CP, (2)
the burden of NCDs among people with CP, and (3) the
management of NCDs among people with CP.
METHOD
Inclusion criteria
Full reports of original research published in English that
aligned with the objectives of the review were included.
No restrictions were placed on the type of study included
in the review. Participants were people with CP of any
age, any type of CP, and any functional ability. Studies
that included people with CP as a subgroup and reported
results for people with CP separately were included.
Search strategy
All available years of the following databases were searched
from inception to August 2017: Ovid MEDLINE (1946 to
August week 1, 2017); Embase Ovid (1974 to August 11th,
2017); CINAHL Plus (Cumulative Index to Nursing and
Allied Health Literature; 1937 to August 13th, 2017). The
reference lists of eligible studies were also searched.
Search terms
The full search strategy for each database is reported in
Appendix S1 (online supporting information). Terms relat-
ing to CP (e.g. cerebral palsy, hemiplegia, spastic, ataxic),
NCDs (e.g. cardiovascular disease, diabetes, respiratory dis-
ease, cancer), modifiable and metabolic risk factors (e.g.
physical activity, smoking, blood pressure), burden (e.g. inci-
dence, prevalence, mortality), and management (e.g.
screening, treatment) were included in the search strategy.
Data collection and analysis
Titles and abstracts of search results were screened, and
studies that did not meet the inclusion criteria were
excluded. If a study seemed to meet the inclusion criteria
or if there was any doubt about the inclusion of the study,
the full text of the article was retrieved. Data were
extracted on participant characteristics (age, sex,
What this paper adds
Adults with cerebral palsy (CP) have an increased risk of non-communicable
diseases (NCDs) and increased risk of death because of NCDs.
Evidence is inconsistent about the elevated prevalence of metabolic risk
factors for NCDs.
Evidence is consistent that people with CP participate in reduced physical
activity.
Only one study reported on management of NCD among people with CP.
Available evidence suggests people with CP are less likely to receive
preventive medicine.
2Developmental Medicine & Child Neurology 2018
ambulatory status, presence of intellectual disability);
prevalence of risk factors among people with CP and com-
parative values in people without CP if reported; measures
of burden of NCDs among people with CP and compara-
tive values in people without CP if provided, including
prevalence, risk, and rates; measures of management of
NCDs among people with CP and comparative values in
people without CP if provided, such as prevalence or inci-
dence of screening.
RESULTS
A summary of the number of records identified in each
database is presented Table I. The search of databases
identified 116 182 records. An additional 13 records were
identified from reviewing reference lists. After removal of
duplicate records, 67 247 records were screened by title
and abstract. Of these, 67 166 records were excluded.
Seventy-nine full-text articles were obtained and reviewed.
A further two full-text articles could not be obtained
despite contacting the authors.
47,48
Thirty-five reports were
excluded after review of the full-text article for the follow-
ing reasons: they were not applicable to any of the objec-
tives of the review (n=16);
4964
they did not report results
for people with CP separately where those with CP were a
subgroup of the sample (n=8);
45,46,6570
they did not
include people with CP or did not state whether people
with CP were included (n=8);
7178
they were not an origi-
nal research report (n=2);
79,80
and there was a second
report of a study already included (n=1).
81
Overall, 44
reports were included in the review (Fig. 1).
Risk of NCDs among people with CP
The search of the literature yielded 38 reports of risk fac-
tors for NCDs among people with CP. All were cross-sec-
tional studies. A summary of each study is presented in
Table SI (online supporting information). The number of
people with CP included in each study ranged from eight
to 1397, although most studies (n=24) included fewer than
100 people with CP. Most studies included females and
males with CP (n=37). Twenty-two studies included chil-
dren aged up to 18 years, one study included children aged
up to 19 years, one study included children aged up to
20 years, two studies included young adults aged 16 to
24 years, and 12 studies included adults aged 18 years and
above.
Metabolic risk factors
Only five studies reported the prevalence of hypertension
in people with CP. The prevalence of hypertensive blood
pressure values among adults with CP was 20.0% in Ire-
land,
82
25.5% in the Netherlands,
83
and 30.0% in the
USA.
84
The prevalence of prehypertension and hyperten-
sion was 50% among adults with CP in the USA
44
and
10.5% among children with CP in Ireland.
85
Only three
studies provided comparative values in the general popula-
tion; one reported that the prevalence of hypertension was
lower among adults with CP
83
and two reported that it
was higher.
84,85
Four studies reported the prevalence of hyperlipidaemia
among adults with CP, but only one compared the preva-
lence with the general population. The prevalence of
hyperlipidaemia ranged from 0% to 30.9%.
44,8284
Adults
with CP had a lower prevalence of hyperlipidaemia com-
pared with the general population.
86
Two studies reported
the prevalence of hyperglycaemia among adults with CP as
0% and 5.7% respectively, but neither compared this with
the prevalence in the general population.
82,83
Seventeen studies conducted in 13 countries reported
the prevalence of overweight and obesity according to BMI
among people with CP. Seven of these included
adults
44,8284,8688
and 10 included children.
85,8997
There
was large variation in the prevalence of obesity reported
(7.3%41.4% in adults and 3.3%18.2% in children). Only
10 studies compared the prevalence of obesity among peo-
ple with CP to the prevalence reported in the general pop-
ulation. Most studies reported that the prevalence of
obesity was lower among children and adults with
CP,
82,85,87,88,90,96,97
although two reported that the preva-
lence was higher among children with CP.
89,93
Two stud-
ies that directly compared the prevalence of obesity among
adults with CP with that in a reference group of people
without CP found that the prevalence of obesity was
higher among adults with CP.
84,86
The prevalence of central obesity, as indicated by waist
circumference, ranged from 24% to 36.4%.
82,83,86
The
only study that compared central obesity with a reference
group of people without CP found that the prevalence was
similar.
86
Modifiable behavioural risk factors
A low prevalence of smoking (2%) and alcohol consump-
tion (23%) was reported among 63 females with CP in the
USA.
35
However, a larger study reported that the preva-
lence of smoking among adults with CP was comparable
to the general US population (19.7% vs 20.4%).
84
This
was similar to that reported among Dutch adults with CP
(20.9%).
83
Approximately 49% of Dutch adults with CP
reported consuming alcohol, which was lower than the
general population.
83
Twenty-two studies assessed participation in physical
activity among people with CP,
35,83,94,98116
most of which
(54.5%) only included people who walked independently
with or without a walking aid.
101108,111114
There was
Table I: Search results obtained from each database
Database Search date
Database
date range
Number
of
records
Number of
records after
removal of
duplicates
Ovid
MEDLINE
August
13th, 2017
1946 to August
week 1, 2017
34 815 21 150
Embase
Ovid
August
13th, 2017
1974 to August
11th, 2017
74 375 44 286
CINHAL
Plus
August
13th, 2017
1937 to August
13th, 2017
6992 5964
Review 3
consistently strong evidence that people with CP partici-
pated in less physical activity and spent more time in
sedentary behaviour than people without CP throughout
the lifespan.
83,94,98,101,103105,108,109,111113,115,116
The num-
ber of children with CP who met guidelines for time spent
in physical activity varied significantly between studies,
from 7% to 94%.
94,99,102,105,107,110
Some of this variation
may be explained by the different methods used to measure
physical activity and the different guidelines that children’s
activity levels were compared with. When directly com-
pared with children having typical development, two out of
three studies reported that a lower percentage of children
with CP met guidelines.
94,102,105
Similarly, fewer adults
with CP met published physical activity guidelines com-
pared with adults without CP (24.4% vs 53.7%).
106
Burden of NCD among people with CP
Seven studies reporting on the burden of NCDs among
people with CP were identified (Table II): three cross-
sectional studies
44,84,117
and four cohort studies.
118121
Three examined the risk of NCDs
44,84,118
and four
examined the risk of death due to NCDs among people
with CP.
117,119121
Sample sizes ranged from 341 to
40 482 people with CP. All studies included females and
males; three included adults only
44,84,119
and four included
adults and children.
117,118,120,121
All studies reporting on the risk of NCDs found an
increased risk among adults with CP. A cohort study of
1975 adults with CP and 9875 age- and sex-matched
adults without CP from Taiwan found strong evidence
that adults with CP had an increased risk of cerebrovas-
cular disease (hazard ratio 2.17, 95% confidence interval
[CI] 1.742.69, p<0.001).
118
Similarly, a cross-sectional
survey of adults with CP in the USA found that they had
greater odds of cerebrovascular disease, hypertension,
emphysema, asthma, and other heart conditions compared
with adults without CP (odds ratios ranged from 1.32
[95% CI 1.041.67] for hypertension, to 2.03 [95% CI
1.392.97] for emphysema).
84
The prevalence of multi-
morbidity (i.e. the presence of at least two chronic condi-
tions) among adults with CP in the USA was found to be
57.8%.
44
116 182 records identified through
database screening
67 247 records after duplicates removed
67 247 records screened
by title and abstract
67 166 irrelevant records excluded
2 records excluded because
full-text articles unavailable
79 full-text articles
assessed for eligibility
44 studies included in
qualitative synthesis
35 full-text articles excluded with
reasons:
16 not applicable to any of the
objectives of the review
8 did not report results for people
with CP separately
8 did not include people with CP
2 not an original research report
1 a second report of a study
already included
13 records identified by reviewing
reference lists
Figure 1: Study flow diagram.
4Developmental Medicine & Child Neurology 2018
Table II: Summary of studies reporting on burden and management of non-communicable diseases
Reference
Country
of origin Study design
Sample
size Description of participants Summary of results
Burden of non-communicable diseases
Cremer et al.
44
USA Cross-sectional 435 Females (53.8%)
Aged 4060y
Ambulatory with/without aids: 54.2%
Intellectual disability: not reported
25.7% OB (according to body mass index)
11.5% were smokers
50% with prehypertension/hypertension
19% with hyperlipidaemia
57.8% with multimorbidity (at least two chronic conditions)
Day et al.
120
USA Cohort 40 482 Females (45%)
Aged 2y
Severe CP (23%)
Comparison group:
Age-specific mortality rates in the
California population (1988 and 2002)
People with CP have increased risk of mortality due to all malignant
neoplasms (SMR: 1.31, 95% CI 1.141.51)
People with CP have increased risk of mortality due to neoplasms of
oesophagus (SMR: 5.40, 95% CI 3.098.77), colon (SMR: 2.16, 95% CI
1.353.27), liver (SMR: 2.21, 95% CI 1.064.06), breast (SMR: 1.93,
95% CI 1.302.75), bladder (SMR: 4.57, 95% CI 2.098.68), brain
(SMR: 2.54, 95% CI 1.484.06), and lymphatic and haematopoietic
tissue (SMR: 1.61, 95% CI 1.092.30)
People with CP have reduced risk of mortality due to neoplasms of
the trachea, bronchus, lung (SMR: 0.22, 95% CI 0.090.43)
No evidence of difference in mortality between people with and
without CP due to neoplasms of male genitourinary organs (SMR:
0.78, 95% CI 0.212.01) or female genitourinary organs (SMR: 0.68,
95% CI 0.251.48)
Durufle-
Tapin et al.
118
France Cross-sectional 3031 deaths 3031 deaths in people with CP were
identified:
Females (41.9%)
Aged 014y (4.3%), 1584y (91.8%),
85y (3.8%)
Comparison group:
Deaths in general population (number
not specified):
Females (48.8%)
Aged 014y (0.9%), 1584y (64.9%),
85y (34.0%)
Deaths due to diseases of the respiratory system:
19% among people with CP
6% in the general population
Deaths due to diseases of the circulatory system:
15% among people with CP
29% in the general population
Deaths due to neoplasms:
7% among people with CP
29% in the general population
Hemming
et al.
119
UK Cohort 341 Females (44%)
Age at baseline not reported (cohort
selected conditional on surviving20y)
4% with severe ambulatory disability,
81% with non-severe ambulatory
disability, 15% with missing data
Intellectual disability: not reported
Comparison group:
Expected survival proportions for the
UK population
Among people aged 2029y:
Deaths due to neoplasms:
11% among people with CP
10% in the general population
Deaths due to diseases of the circulatory system:
6% among people with CP
5% in the general population
Deaths due to diseases of the respiratory system:
50% among people with CP
3% in the general population
Among people aged 3039y:
Deaths due to neoplasms:
0% among people with CP
16% in the general population
Deaths due to diseases of the circulatory system:
17% among people with CP
9% in the general population
Deaths due to diseases of the respiratory system:
Review 5
Table II: Continued
Reference
Country
of origin Study design
Sample
size Description of participants Summary of results
42% among people with CP
3% in the general population
Among people aged 4049y:
Deaths due to neoplasms:
24% among people with CP
27% in the general population
Deaths due to diseases of the circulatory system:
19% among people with CP
19% in the general population
Deaths due to diseases of the respiratory system:
10% among people with CP
4% in the general population
Among people aged 5059y:
Deaths due to neoplasms:
21% among people with CP
40% in the general population
Deaths due to diseases of the circulatory system:
21% among people with CP
27% in the general population
Deaths due to diseases of the respiratory system:
0% among people with CP
5% in the general population
Peterson
et al.
84
USA Cross-sectional 1015 Females (31%)
Mean age: 58y 2mo
Minor or no disability (48.7%),
moderate physical disability (8.9%),
severe physical disability (40.6%)
Intellectual disability: not reported
Comparison group:
206 600 adults without CP (48% female)
Mean age: 45y 5mo
Minor or no disability (82.0%),
moderate physical disability (4.9%),
severe physical disability (11.3%)
OW/OB according to body mass index:
People with CP:
31.2% OW
41.4% OB
People without CP:
34.2% OW
29.7% OB
Smokers:
19.7% of adults with CP
20.4% of adults without CP
Age-adjusted prevalence in people with CP vs people without CP:
diabetes (9.2% vs 6.3%)
asthma (20.7% vs 9.4%)
hypertension (30.0% vs 22.1%)
other heart conditions (15.1% vs 9.1%)
stroke (4.6% vs 2.3%)
emphysema (3.8% vs 1.4%)
People with CP have increased odds of all non-communicable
diseases except for diabetes (odds ratios range from 1.32, 95% CI
1.041.67, for hypertension, to 2.03, 95% CI 1.392.97, for
emphysema)
Strauss
et al.
121
USA Cohort 45 292 Females (44.7%)
Aged 0 to >55y
Ambulatory with/without aids: 51.9%
Intellectual disability: not reported
Comparison group:
For each cause of death, age- and sex-
People with CP have increased risk of death due to all cancers (SMR:
2.5 for people with severe CP; SMR: 2.1 for people with not-severe
CP; p<0.001 for both)
People with CP have increased risk of death due to breast cancer,
cancer of digestive organs, cancer of genitourinary organs, and
brain cancer (p<0.01)
6Developmental Medicine & Child Neurology 2018
Table II: Continued
Reference
Country
of origin Study design
Sample
size Description of participants Summary of results
specific mortality rates (deaths per
100 000 person-years of exposure) in
the general Californian population
People with CP have reduced risk of death due to lung cancer
(p<0.01)
People with CP have increased risk of death due to chronic
obstructive pulmonary disease (SMR: 13.8 for people with
severe CP; SMR: 2.3 for people with not-severe CP; p<0.01 for both)
People with CP have increased risk of death due to ischaemic heart
disease (SMRs range from 1.8 to 13.5 across age groups for people
with severe CP; SMRs range from 2.2 to 5.5 across age groups for
people with not-severe CP; p<0.01 for all)
People with CP have increased risk of death due to cerebrovascular
disease (SMRs range from 2.3 to 38.5 across age groups for people
with severe CP; SMRs range from 2.1 to 11.7 across age groups for
people with not-severe CP; p<0.01 for all)
People with CP have increased risk of death due to other heart
diseases (SMRs range from 3.3 to 21.8 across age groups for
people with severe CP; SMRs range from 2.9 to 13.5 across age
groups for people with not-severe CP; p<0.01 for all)
Wu et al.
117
Taiwan Cohort study 1975 Females (53.9%)
Age at baseline:
30y (38.5%)
3140y (11.5%)
4150y (18.4%)
5160y (15.4%)
6170y (9.0%)
>70y (7.0%)
Ambulatory disability: not reported
Intellectual disability: not reported
Comparison group:
9875 people without CP matched for
age and sex to people with CP in a 5:1
ratio
People with CP had greater risk of stroke compared with people
without CP (hazard ratio: 2.17, 95% CI 1.742.69, p<0.001)
Management of non-communicable diseases
Reference Country of origin Study design Sample size
a
Description of participants Summary of results
Sullivan et al.
122
Australia Cross-sectional 380 (62) Females with mild, moderate, and
severe intellectual disability (39.5%,
32.1%, and 28.4% respectively)
No description of subgroup of
participants with CP provided
85.5% of females with CP had not received a breast cancer screening
Females with CP had lower odds of receiving mammography
screening compared with females with other intellectual disabilities
(odds ratio 0.27, 95% CI 0.130.57)
a
Total sample (participants with cerebral palsy [CP]), where people with CP are a subgroup of the total sample. OB, with obesity; SMR, standardized mortality ratio; CI, confidence interval;
OW, with overweight.
Review 7
There was mixed evidence about the risk of death due to
NCDs among adults with CP. A descriptive cross-sectional
study conducted in France found that the percentage of
deaths due to respiratory diseases was higher among adults
with CP, but the percentage of deaths due to diseases of
the circulatory system and neoplasms was lower.
117
Simi-
larly, in a cohort study of 341 adults with CP in London, a
higher percentage of deaths due to respiratory diseases was
reported among young adults with CP compared with
adults without CP.
119
This difference did not exist between
older adults with and without CP.
119
The percentage of
deaths due to diseases of the circulatory system and neo-
plasms was similar between adults with CP and the general
population.
119
In contrast, two cohort studies of over 40 000 people
with CP in the USA reported that they had an increased
risk of death due to all cancers compared with the general
population.
120,121
Specifically, people with CP had
increased risk of death due to breast cancer, oesophageal
cancer, colon cancer, liver cancer, bladder cancer, and
brain cancer, but reduced risk of death due to lung can-
cer.
120,121
The studies found conflicting evidence about an
increased risk of death due to cancer of the genitourinary
organs.
120,121
Strauss et al.
121
also reported that adults with
CP had at least a twofold increased risk of death due to
chronic obstructive pulmonary disease, ischaemic heart dis-
ease, cerebrovascular disease, and other heart diseases,
compared with the general population.
Only two studies examined differences in the burden of
NCDs according to severity of disability.
44,121
Ambulatory
males with CP (i.e. Gross Motor Function Classification
System [GMFCS] levels IIII) had a higher prevalence of
diabetes compared with non-ambulatory males (i.e.
GMFCS levels IV and V) and ambulatory females with CP
had a higher prevalence of stroke compared with non-
ambulatory females.
44
However, non-ambulatory adults
with CP had a higher prevalence of multimorbidity com-
pared with ambulatory adults.
44
When examining causes of
death among adults with CP, Strauss et al.
121
defined sev-
ere CP as ‘a condition so substantial that it is exceedingly
difficult to find an appropriate placement for the client
and/or constant care/supervision is required’. Although in
this study people with severe CP of all ages had a greater
risk of death due to chronic obstructive pulmonary disease
compared with people with non-severe CP,
121
the differ-
ences in risk of death due to other NCDs were age depen-
dent.
121
The risk of death due to cancers, ischaemic heart
diseases, and other heart diseases was higher among people
with severe CP up to the age of 54 years, compared with
people without severe CP of the same age, but the risk was
similar between people with and without severe CP who
were older than 54 years. The risk of death due to stroke
was higher among people with severe CP aged 0 to
34 years and older than 54 years, compared with people
without severe CP, but was similar between people with
and without severe CP aged 35 to 54 years.
Management of NCD among people with CP
One study investigated the management of NCDs among
people with CP, and included 62 females with intellectual
disability only (Table II). Only 15% of females with CP
had received a screening for breast cancer in their life.
122
Moreover, females with CP were 73% (95% CI 4387%)
less likely to have received a mammography screening
compared with females with other causes of intellectual
disability.
122
DISCUSSION
In summary, people with CP are at increased risk of
NCDs, which may be a direct result of reduced participa-
tion in physical activity and screening programmes. There
is mixed evidence that people with CP have increased
prevalence of metabolic risk factors such as hyperlipi-
daemia and obesity.
This review highlights the consistent evidence that peo-
ple with CP participate in reduced levels of physical activ-
ity throughout their lifespan. The causal link between lack
of physical activity, metabolic risk factors, and NCDs sug-
gests that people with CP may be at increased risk of
NCDs as a result of chronic inactivity. Conversely, the few
studies that investigated the prevalence of metabolic risk
factors among adults with CP reported similar or lower
prevalence of risk factors compared with the general popu-
lation. These studies, however, mostly included small num-
bers of relatively young adults, and therefore potentially do
not give a representative depiction of the prevalence of
metabolic risk factors in the CP population.
While the prevalence of overweight and obesity was the
most widely reported metabolic risk factor, there was large
variation in the prevalence reported between studies. This
may partly be because studies used different methods to
classify overweight and obesity according to BMI in chil-
dren, such as the International Obesity Task Force or the
World Health Organization cut-offs.
123
Additionally,
prevalence of obesity was reported across several countries,
which may explain variation. Although many studies
reported a lower prevalence of obesity among people with
CP compared with the general population, BMI is not sen-
sitive to detect excess body fat in people with CP.
124
Adults with impaired mobility with high body fat may be
misclassified as normal weight, resulting in metabolic risk
factors not being identified.
125
Further, height is difficult
to ascertain in people with CP, particularly among those in
GMFCS levels IV and V because of lower limb contrac-
tures and reduced standing balance, making BMI poten-
tially unfeasible to assess in the clinic. Indeed, measures of
central adiposity are better predictors of metabolic risk fac-
tors than BMI among people with CP
53,82
and thus a more
accurate and feasible indicator of increased risk of NCDs.
Despite identifying an increased risk of NCDs among
adults with CP, this review also highlights the lack of epi-
demiological data on the risk of NCDs in this population
worldwide. Only two studies so far have directly compared
8Developmental Medicine & Child Neurology 2018
the risk of NCDs between adults with and without CP. Of
these, one only investigated the risk of stroke
118
and one
was a cross-sectional study using self-reported presence of
disease as the outcome.
84
Similarly, only four studies have
reported the incidence of death due to NCDs among
adults with CP compared with the general population, with
conflicting results. Although two large cohort studies con-
ducted in the USA did identify an increased risk of death
due to cancers, cardiovascular diseases, and chronic respi-
ratory diseases,
120,121
two smaller studies did not report a
difference in the risk of death due to NCDs between peo-
ple with and without CP.
117,119
Additionally, only two
studies examined the burden of NCDs according to dis-
ability severity. These studies used different categorizations
of disability severity and therefore the results are not com-
parable. However, the results of both studies suggest that
the burden may differ according to disability severity, with
more severe disability not necessarily indicating a higher
risk of NCDs. Disability severity as a predictor of the risk
of NCDs should be examined further to aid efficient iden-
tification of adults with CP most at risk.
There is a dearth of literature on preventive medicine for
adults with CP. Only one study examined the uptake of
screening, specifically breast cancer screening, among
females with CP and found a very low rate. Children and
adults with disabilities including CP have consistently
higher health care use, including more visits to clinicians,
more prescriptions dispensed, more hospitalizations, partic-
ularly elective and medical admissions, longer length of stay,
and higher numbers of procedures per admission.
55,126,127
Despite this increased use, people with disabilities report
difficulties accessing needed care.
126
Over 20% of children
with CP report unmet therapy services needs,
128
and adults
with CP report having inadequate access to the coordinated
services that they received as children when they experience
chronic health conditions during middle age.
129
These diffi-
culties with accessing health care may contribute to the
higher incidence of NCDs in this population, which may be
prevented if metabolic risk factors such as hyperlipidaemia
were identified and treated early.
21,130
Indeed, McPhee
et al.
131
demonstrated that age was significantly associated
with decreased endothelial function and arterial stiffness in
CP, independent of central adiposity and mobility status.
While this review indicates that chronic physical conditions
are prevalent among people with CP, a recent review also
highlighted the high prevalence of mental health conditions
among young people with CP.
132
Given an increasing
prevalence of CP, owing in part to the reduction in child-
hood mortality,
15,133
there is mounting evidence for the
need to approach health care delivery for persons with CP
within the context of a life-course health development
model.
134
Limitations
Although many studies investigating risk factors for NCDs
were identified, most included a small number of partici-
pants, making it difficult to generalize the findings to the
wider CP population. Additionally, many studies did not
directly compare the prevalence of risk factors between
people with and without CP. Although some studies pro-
vided information on the prevalence of risk factors in the
general population, it is possible that any differences
observed may have been due to confounding factors. Simi-
larly, three studies reporting the burden of NCD in people
with CP only reported descriptive statistics and did not
directly compare the burden between people with and
without CP adjusting for potential confounders.
44,117,119
Further, at a review level, we were unable to obtain full
texts for two articles and therefore may not have included
some relevant data.
Epidemiology and surveillance programmes are a key
component to prevention of NCDs worldwide, as they
provide essential data to identify and track high-risk popu-
lations, inform targeted solutions, and monitor trends of
health across the lifespan.
130
This review highlights the
need for further epidemiological research on the risk of
NCDs among adults with CP. Further research is also
required to understand current health promotion and
screening practices for people with CP worldwide. Under-
standing these areas, and the barriers people with CP expe-
rience to accessing integrated health services throughout
their lifespan, are key to identifying ways to reduce the risk
of NCDs in this population.
ACKNOWLEDGEMENTS
We thank Dr Grace Lavelle for her feedback on a draft of this
manuscript. The authors have stated that they had no interests
which might be perceived as posing a conflict or bias.
SUPPORTING INFORMATION
The following additional material may be found online:
Appendix S1: Search strategy.
Table SI: Summary of studies reporting on risk factors for
non-communicable diseases.
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12 Developmental Medicine & Child Neurology 2018
  • Article
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    Objetivo: Analisar a relação entre qualidade de vida (QV) e sobrecarga dos cuidadores relacionados ao grau de comprometimento neuromotor de crianças com paralisia cerebral (PC). Métodos: Estudo transversal realizado em 2014, com 38 cuidadores de crianças com paralisia cerebral atendidas pelo serviço de Fisiatria e Reabilitação do Hospital de Clínicas de Porto Alegre, Rio Grande do Sul, Brasil. Utilizaram-se os instrumentos World Health Organization Quality of Life Questionaire (WHOQOL – BREF), Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Inventário de Ansiedade Traço-Estado (IDATE) e Zarit Caregiver Burden Interview (ZCBI). Avaliou-se o grau de desenvolvimento motor das crianças pelo Gross Motor Function Classification System (GMFCS). Avaliaram-se as associações (coeficiente de Pearson) entre as variáveis descritas por média e desvio padrão, ou mediana, e amplitude interquartílica. Resultados: Encontraram-se 20 cuidadores (52,6%) com sobrecarga moderada. Encontraram-se associações no domínio social da WHOQOL-BREF em relação ao desenvolvimento motor das crianças (rs=0,355; p=0,029) e associação positiva entre a ZCBI e os escores do IDATE (rs=0,540; p
  • Article
    Background People with cerebral palsy (CP) are less physically active than the general population and, consequently, are at increased risk of preventable disease. Evidence indicates that low-moderate doses of physical activity can reduce disease risk and improve fitness and function in people with CP. Para athletes with CP typically engage in ‘performance-focused’ sports training, which is undertaken for the sole purpose of enhancing sports performance. Anecdotally, many Para athletes report that participation in performance-focused sports training confers meaningful clinical benefits which exceed those reported in the literature; however, supporting scientific evidence is lacking. The aim of this paper is to describe the protocol for an 18-month study evaluating the clinical effects of a performance-focused swimming training programme for people with CP who have high support needs. Methods This study will use a concurrent multiple-baseline, single-case experimental design across three participants with CP who have high support needs. Each participant will complete a five-phase trial comprising: baseline (A1); training phase 1 (B1); maintenance phase 1 (A2); training phase 2 (B2); and maintenance phase 2 (A3). For each participant, measurement of swim velocity, health-related quality of life and gross motor functioning will be carried out a minimum of five times in each of the five phases. Discussion The study described will produce Level II evidence regarding the effects of performance-focused swimming training on clinical outcomes in people with CP who have high support needs. Findings are expected to provide an indication of the potential for sport to augment outcomes in neurological rehabilitation.
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    This commentary is on the original articles by Heyn et al. and Peterson et al.
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    Cerebral palsy is a rare condition following injury of the developing brain and including nonprogressive neurological disorders, spasticity, intellectual impairment and others. Boys with cerebral palsy have a high incidence of undescended testis. Although the motives for treatment (infertility, cancer prevention, psychological aspects, testicular torsion) are not different in boys without neurological impairment, the decision-making process in boys with cerebral palsy is very difficult. Besides medical and surgical arguments the discussion involves challenging ethical issues.
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    People with brain impairments are less active than the general population and consequently have an increased risk of chronic disease. To increase activity requires interventions that are theory driven and evidence based. Here, we describe the adapted physical activity program (APAP), a physical activity promotion program with demonstrated efficacy in community dwelling adults with brain impairments. Distinguishing features of the APAP include the following: delivery in the participants home/or community environment and the utilisation of the principals of community-based rehabilitation; the assessment of each of the domains of the International Classification of Functioning, Disability and Health (ICF) (i.e., health conditions, impairments, activity limitations, participation restrictions and personal and environmental characteristics) to determine how they will impact physical activity adoption and maintenance; the incorporation of theory-based physical activity adoption and maintenance strategies; the utilisation of lifestyle physical activity programs (including client-centred selection of activities) and/or structured exercise programs (requiring principles of exercise prescription). It is anticipated that this program description will permit researchers and/or practitioners to implement the program, replicate its evaluation and/or translate the program into multi-professional rehabilitation settings.
  • Article
    Aim: This study examined cross-sectional population-based rates in reported need and unmet need for occupational, physical, and speech therapy services in children with autism spectrum disorder (ASD) compared with children with attention-deficit-hyperactivity disorder (ADHD) and cerebral palsy (CP). Method: The 2005-2006 and 2009-2010 (USA) National Survey of Children with Special Health Care data sets were used to compare therapy need and unmet need among children younger than 18 years with ASD (n=5178), ADHD (n=20 566), and CP (n=1183). Bivariate approaches and multivariate logistic regression using imputed data were used to identify associations between child and family characteristics, and access to therapy services. Results: After adjusting for other variables, children with ASD had a significantly greater likelihood of having an unmet therapy need compared with children with ADHD (odds ratio [OR] 1.66, 95% confidence interval [CI] 1.36-2.03), but a similar unmet need as children with CP (OR 1.30, 95% CI 0.97-1.74). Factors associated with unmet need included survey year, younger child age, no health insurance, and increased functional and behavioral difficulties. Interpretation: Children in our sample had greater unmet therapy needs in 2009 than in 2005. Caregiver-reported reasons for unmet need included cost and school resources. Research examining future trends in therapy access are warranted for children with ASD and CP.
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  • Article
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  • Article
    Full-text available
    Importance Cerebral palsy describes the most common physical disability in childhood and occurs in 1 in 500 live births. Historically, the diagnosis has been made between age 12 and 24 months but now can be made before 6 months’ corrected age. Objectives To systematically review best available evidence for early, accurate diagnosis of cerebral palsy and to summarize best available evidence about cerebral palsy–specific early intervention that should follow early diagnosis to optimize neuroplasticity and function. Evidence Review This study systematically searched the literature about early diagnosis of cerebral palsy in MEDLINE (1956-2016), EMBASE (1980-2016), CINAHL (1983-2016), and the Cochrane Library (1988-2016) and by hand searching. Search terms included cerebral palsy, diagnosis, detection, prediction, identification, predictive validity, accuracy, sensitivity, and specificity. The study included systematic reviews with or without meta-analyses, criteria of diagnostic accuracy, and evidence-based clinical guidelines. Findings are reported according to the PRISMA statement, and recommendations are reported according to the Appraisal of Guidelines, Research and Evaluation (AGREE) II instrument. Findings Six systematic reviews and 2 evidence-based clinical guidelines met inclusion criteria. All included articles had high methodological Quality Assessment of Diagnostic Accuracy Studies (QUADAS) ratings. In infants, clinical signs and symptoms of cerebral palsy emerge and evolve before age 2 years; therefore, a combination of standardized tools should be used to predict risk in conjunction with clinical history. Before 5 months’ corrected age, the most predictive tools for detecting risk are term-age magnetic resonance imaging (86%-89% sensitivity), the Prechtl Qualitative Assessment of General Movements (98% sensitivity), and the Hammersmith Infant Neurological Examination (90% sensitivity). After 5 months’ corrected age, the most predictive tools for detecting risk are magnetic resonance imaging (86%-89% sensitivity) (where safe and feasible), the Hammersmith Infant Neurological Examination (90% sensitivity), and the Developmental Assessment of Young Children (83% C index). Topography and severity of cerebral palsy are more difficult to ascertain in infancy, and magnetic resonance imaging and the Hammersmith Infant Neurological Examination may be helpful in assisting clinical decisions. In high-income countries, 2 in 3 individuals with cerebral palsy will walk, 3 in 4 will talk, and 1 in 2 will have normal intelligence. Conclusions and Relevance Early diagnosis begins with a medical history and involves using neuroimaging, standardized neurological, and standardized motor assessments that indicate congruent abnormal findings indicative of cerebral palsy. Clinicians should understand the importance of prompt referral to diagnostic-specific early intervention to optimize infant motor and cognitive plasticity, prevent secondary complications, and enhance caregiver well-being.
  • Article
    A critical issue in health-care reform concerns how to realign health-care delivery systems to manage medical care services for people with ongoing and costly needs for care. We examined the overlapping health-care needs of two such population groups among the U.S. working-age population (those aged 18–64 years): people with chronic medical conditions and people with disabilities.
  • Article
    Aim: To estimate the number of children living with cerebral palsy (CP) in England and Wales in 2013 by severity, and to extrapolate this figure to 2020. Method: Data from the North of England Collaborative Cerebral Palsy Survey for births during the period 1991 to 2000 were restricted to individuals aged at or above 3 years to estimate the prevalence of CP and to calculate 15-year survival by severity according to the number of severe impairments and lifestyle assessment score. The number of 3- to 15-year-olds with CP of different severity in England and Wales was estimated in 2013 and 2019 using actual and nationally projected births. Results: Cumulative survival estimates up to the age of 16 years in children with CP differ significantly by severity, ranging between 97 per cent and 100 per cent for children with non-severe CP, and between 64 per cent and 67 per cent for those with the most severe CP. By the end of 2013, the estimated number of children aged 3 to 15 years living with CP in England and Wales will be about 20 500 rising to approximately 22 100 by 2020, a 7.5 per cent increase. Interpretation: Owing to an increasing population, the number of children living with CP in England and Wales will increase by 2020. This will have significant implications for health and social care service planning.
  • Article
    Full-text available
    Importance Maternal overweight and obesity are associated with increased risks of preterm delivery, asphyxia-related neonatal complications, and congenital malformations, which in turn are associated with increased risks of cerebral palsy. It is uncertain whether risk of cerebral palsy in offspring increases with maternal overweight and obesity severity and what could be possible mechanisms. Objective To study the associations between early pregnancy body mass index (BMI) and rates of cerebral palsy by gestational age and to identify potential mediators of these associations. Design, Setting, and Participants Population-based retrospective cohort study of women with singleton children born in Sweden from 1997 through 2011. Using national registries, children were followed for a cerebral palsy diagnosis through 2012. Exposures Early pregnancy BMI. Main Outcomes and Measures Incidence rates of cerebral palsy and hazard ratios (HRs) with 95% CIs, adjusted for maternal age, country of origin, education level, cohabitation with a partner, height, smoking during pregnancy, and year of delivery. Results Of 1 423 929 children included (mean gestational age, 39.8 weeks [SD, 1.8]; 51.4% male), 3029 were diagnosed with cerebral palsy over a median 7.8 years of follow-up (risk, 2.13 per 1000 live births; rate, 2.63/10 000 child-years). The percentages of mothers in BMI categories were 2.4% at BMI less than 18.5 (underweight), 61.8% at BMI of 18.5 to 24.9 (normal weight), 24.8% at BMI of 25 to 29.9 (overweight), 7.8% at BMI of 30 to 34.9 (obesity grade 1), 2.4% at BMI of 35 to 39.9 (obesity grade 2), and 0.8% at BMI 40 or greater (obesity grade 3). The number of cerebral palsy cases in each BMI category was 64, 1487, 728, 239, 88, and 38; and the rates per 10 000 child-years were 2.58, 2.35, 2.92, 3.15, 4.00, and 5.19, respectively. Compared with children of normal-weight mothers, adjusted HR of cerebral palsy were 1.22 (95% CI, 1.11-1.33) for overweight, 1.28 (95% CI, 1.11-1.47) for obesity grade 1, 1.54 (95% CI, 1.24, 1.93) for obesity grade 2, and 2.02 (95% CI, 1.46-2.79) for obesity grade 3. Results were statistically significant for children born at full term, who comprised 71% of all children with cerebral palsy, but not for preterm infants. An estimated 45% of the association between maternal BMI and rates of cerebral palsy in full-term children was mediated through asphyxia-related neonatal morbidity. Conclusions and Relevance Among Swedish women with singleton children, maternal overweight and obesity were significantly associated with the rate of cerebral palsy. The association was limited to children born at full term and was partly mediated through asphyxia-related neonatal complications.
  • Article
    The life course health development (LCHD) model by Halfon et al. conceptualizes health development occurring through person–environment transactions that enable well-being and participation in desired social roles throughout life, areas that have not received adequate attention in healthcare. The aim of this ‘perspectives’ paper is to apply the six core tenets of the LCHD model and the concept of health development trajectories to individuals with lifelong neurodevelopmental conditions. We share the perspective that modifiable aspects of the environment often restrict health development; we then advocate that children, beginning at a young age, should engage in ‘real-world’ experiences that prepare them for current and future social roles. LCHD encourages future planning from the outset, continuity of care between pediatric and adult systems, and coordination of services and supports. We believe LCHD can be transformative in enabling healthy living of individuals with neurodevelopmental conditions.
  • Article
    This study examined the evolution of individuals with cerebral palsy (CP) from childhood to adulthood. Seventy-two adults with a diagnosis of CP born between 1934 and 1980 were studied. Individuals were recruited and data comprehensively collected using case notes and through direct assessments of the majority of participants from three rehabilitation units in Bologna, Padua, and Rovigo in Italy. The main findings can be summarized as follows: contact with health and rehabilitation services was radically reduced once individuals reached adulthood more individuals who were integrated into mainstream schools achieved and maintained literacy than those who had attended special schools; in a high number of participants, motor performance deteriorated once into adulthood. Independent walking or other forms of supported locomotion were lost in many on reaching adulthood. Of those who continued to walk, walking deteriorated in terms of distance. It was concluded that even though CP has been considered as predominantly a childhood pathological condition, the evolution of the effects of CP do not stop at 16 or 18 years of age. For this reason, the traditional child- (or infant-) oriented approach concentrating mainly or exclusively on the achievement of independent walking, may not be an ideal approach to children with CP. Instead a more independence-oriented therapeutic approach would be appropriate.