LONG TERM OUTCOMES IN
CEREBRAL PALSY
WORKSHOP ON ADULT CEREBRAL PALSY
IN HONOR OF ARNOLD WERNER
EAST LANSING, May 12th, 2008
Nigel Paneth MD MPH
Michigan State University
http://www.epi.msu.edu/faculty/paneth.htm
Department of Epidemiology, Michigan State University
LATEST DEFINITION
OF CEREBRAL PALSY
“Cerebral palsy describes a group of permanent
disorders of the development of movement and
posture causing activity limitation that are
attributed to non-progressive disturbances that
occurred in the developing fetal or infant brain.
The motor disorders of cerebral palsy are often
accompanied by disturbances of sensation,
perception, cognition, communication and
behavior, by epilepsy, and by secondary
musculoskeletal disorders”
Rosenbaum P et al: Dev Med Child Neurol (Suppl.) 2007;109:8-14
Department of Epidemiology, Michigan State University
CP PREVALENCE IN 12 NATIONAL
REGISTRIES
2.4
CP/1,000 2.33
2.2 2.23
Linear 2.12
2.08 2.08
(CP/1,000) 2 2.02 2.02
1.94
1.87
1.8
1.6 1.63
N of observations
1950 - 1; 1955 - 3
1960 - 4; 1965 - 3 1.4
1970 - 6; 1975 - 5
1980 - 8; 1985 -14
1990 - 7; 1995 - 2
1.2
1
1945 1950 1955 1960 1965 1970 1975 1980 1985 1990 1995 2000
Department of Epidemiology, Michigan State University
ONLY POPULATION-BASED
TIME TREND DATA IN US
Data from the 3
Metropolitan Atlanta
Congenital Disabilities 2.5
and Developmental
Disorders Surveillance 2 2
Program. 1.7
CP per
1.5
thousand
Winter et al Pediatrics live births
2002; 110:1220-25 1
0.5
0
1975-77 1986-91
Department of Epidemiology, Michigan State University
Cerebral Palsy Outreach Network (CPON)
www.epi.msu.edu/cpon
An Internet resource for children with cerebral
palsy (CP) and their families.
CPON’s mission:
– help families connect to needed services
– provide scientific information to families on
new treatments for CP
– develop research on the causes and
management of CP
– link families experiencing CP in our area and
provide a way for them to support each other
Department of Epidemiology, Michigan State University
PROBLEMS IN ASCERTAINING ADULT
OUTCOMES IN CEREBRAL PALSY
CP is highly variable in severity; sample
selection can greatly influence results
Much of the literature is anecdotal
Few studies have what is required:
– Large sample size
– Representative population
– Non-CP controls
Department of Epidemiology, Michigan State University
WHAT KINDS OF OUTCOMES?
Mortality/Life expectancy
Quality of Life
Functioning and Activity
Department of Epidemiology, Michigan State University
LIFE EXPECTANCY FOR CP IN
GREAT BRITAIN
Survival to age 30
– no severe disabilities – 99%
– one severe disability – 95%
– two severe disabilities – 78%
– three severe disabilities – 59%
– four severe disabilities – 33%
Hutton JL; Clin Perinatol 2006; 33:545-555
Department of Epidemiology, Michigan State University
20 YEAR SURVIVAL FOR CP IN CALIFORNIA
28,513 children aged 4 – 14 in 1983
– “not severe” CP – 98.2% survival
– “severe” CP – 85% survival
30,185 adults aged 15 or above in 1983
– “ not severe” CP – 93.2% survival
– “severe” CP – 71.6% survival
Survival for very severe CP improved over time
– 1983 – 1995: Life expectancy 14 years
– 1995 - 2002: Life expectancy 20 years
Strauss et al; Dev Med Child Neurol 2007;49:86-92
Department of Epidemiology, Michigan State University
DECLINE IN MOTOR FUNCTION
California sample (n = 7,550 at age
10; 5,721 at age 25; 904 at age 60)
– 77% of 10 year olds and 76% of 25
year olds who walked and climbed stairs
without difficulty still did so 15 years
later
– However, between ages 60 – 75, a high
fraction lost ambulatory ability; speech
and self-feeding less affected
Department of Epidemiology, Michigan State University
PAIN and FATIGUE
NORWAY (N = 406; mean age 34)
– 30% chronic pain vs 15% in the general
population
– 30% substantial fatigue vs 22% in the
general population
Department of Epidemiology, Michigan State University
WHAT SEEMS TO BE
HAPPENING
Life expectancy in CP is about the same as for
the general population for individuals without
severe cognitive impairment or multiple
disabilities. For the severely impaired, survival
rates are improving
Decline in motor abilities can occur in adults with
CP. This is found occasionally in early and mid-
adulthood, but quite commonly in older
individuals.
Chronic pain and fatigue are important features
of adult CP
Department of Epidemiology, Michigan State University
International Classification of
Functioning, Disability and Health
The ICF puts the notions of 'health' and
'disability' in a new light. It acknowledges
that every human being can experience a
decrement in health and thereby
experience some degree of disability.
Disability is not something that only
happens to a minority of humanity. The
ICF thus 'mainstreams' the experience of
disability and recognizes it as a universal
human experience.”
Department of Epidemiology, Michigan State University
ICF categories
1. Body structure and functions and
structures (anatomy and physiology)
2. Activities (carrying out daily living tasks)
3. Participation in home, work and
community
4. Interactions with
– personal factors (e.g., age, motivation,
desires) and
– environmental factors (e.g., settings of home
or community, building modifications)
Department of Epidemiology, Michigan State University
DESCRIBING FUNCTIONS IN CP
Mobility
– Gross Motor Function Classification System
(GMFCS)
www.canchild.ca/Portals/0/outcomes/pdf/GMFCS.pdf
Handling Objects
– Manual Ability Classification System (MACS)
www.macs.nu/
Communication
– Communication Function Classification System
(CFCS) in validation and reliability phases
– See Mary Jo Cooley Hidecker
Department of Epidemiology, Michigan State University
THE NEED FOR RESEARCH
Practitioners have a moral obligation to
have solid evidence that a treatment
works before providing it to patients
It follows from the above that there is a
moral obligation to do research to find out
what works
P–R=W
(Practice minus Research = Witchcraft)
Department of Epidemiology, Michigan State University