Cerebral Palsy: The Stigma, The Fears and the Reality - Benola

Report
Cerebral Palsy: The Stigma, The
Fears and The Reality
Professor F E A Lesi
Consultant Paediatrician,
Lagos University Teaching Hospital
Benola: Cerebral Palsy Initiative
20th July 2013
1
Outline and Objectives
• Understand normal development and describe
normal motor milestones.
• Define and describe the causes and pattern of
Cerebral Palsy.
• Outline discuss the issues in the management of
Cerebral Palsy.
• Influence a new reality.
2
The Brain
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Structure
• An amazing organ that appears grey made up of
protein and fat. Looks and feels like jelly. Its
shape is given by the skull bones that protect it.
• Made of units/cells (neurones), connecting wires
(dendrites/axon) and switches (synapses). It is a
complex electrical circuit board.
• It is nourished by blood that flows through
vessels that arise from the internal carotid artery.
• It is cushioned by fluid (called cerebrospinal fluid)
which prevents the brain from damage when we
shake or bump our heads.
4
Function
• Motor Control (Movement).
• Sensory Processing/Coordination (Sensation,
Feeling)
• Higher Centre (Personality, Intellect,
Behaviour, Memory, Thought, Emotions).
• Endocrine/Hormones (Pituitary, Growth,
Prolactin, Melatonin [sleep hormone], etc).
5
The Developing Brain
• Critical Periods of Brain growth
– 1 month – neural tube
– 4th month – All the lobes and major divisions complete
– 1 year post-natal – 2/3 adult size
– 2 years age – 75% adult size
– 5 years – 90% adult size
• Potential for Neurogenesis [new brain cell formation] (peaks in
utero) and Synaptogenesis [new connection formation] (peaks by 5
years) continues throughout life.
6
Important Factors
Intrinsic
– Genetics
– Age
– Neurotransmitters (brain chemicals)
– Gender
Extrinsic
– Exposure to drugs (cocaine, phenytoin, alcohol)
– Stress
– Hypoxia/ischemia,
– Viral infection
– Gonadal steroids
– Nutrition
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Normal Development
–
–
–
–
Motor
Social
Cognitive/IQ
Language
Maturation of the nervous system underlies
development and it progresses cephalocaudally (head downward). It is also a
smooth continuous process.
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Motor
•
•
•
•
•
•
•
•
Primitive reflexes (disappear by 3-4 months)
Neck control 3-4 months (earlier in African children)
Sitting 5-6 months
Rolls 7 months
Crawls 7-8 months
Stands with support 10 months
Walks 12 months
Climbs up and down stairs 20 months
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Cerebral Palsy (CP)
• A group of disorders of the development of
movement and posture, causing activity
limitation that are attributed to nonprogressive disturbances that occurred in the
developing or infant brain.
• This is often accompanied by disturbances of
sensation, cognition, communication,
perception, behaviour or by a seizure disorder.
• It is reported to be the most common cause of
motor deficiency in childhood both in
developing and developed countries.
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How Common is CP?
Country
Prevalence
Developed
USA
UK
4/1000
2/1000
Developing (Nigeria)
Lagos
Port-Harcourt (Frank-Briggs et al 2011)
Enugu (Izuora et al 1989)
10/1000
10.3/1000
5/1000
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The Burden: By the Numbers
170 m - Nigeria’s population*
700,000 - Children with CP
60-80% will have co-morbidities like
Seizures, Intellectual disabilities etc,
3.5 m (incl. 1.4 m parents) - People directly
affected by
CP
* CIA World Factbook
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Causes of CP
Pre-natal (Before Delivery), Perinatal (Around
Delivery), Postnatal After Delivery)
Commonest Causes
Nigeria and Developing Countries
– Severe Perinatal Asphyxia (difficulty in establishing breathing at
birth)
– Kernicterus (excessively high bilirubin/jaundice)
– Infections (Intrauterine and Perinatal)
– Metabolic (Hypoglycaemia-Low blood sugar)
Developed Countries
– Extreme prematurity
– Inborn Errors of Metabolism
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Classification (CP)
• Clinical (spastic [too stiff], flaccid [too soft], extra-pyramidal
[moving without control or abnormally positioned] and
mixed).
• Anatomical (number body parts [limbs] affected)
• The Gross Motor Function Classification System (GMFCS) is a
recently developed system which classifies children with CP by
their age specific motor activity.
– It is based on the assessment of severity of CP in children 0-12 years of
age based on their functional abilities rather than their limitations.
– The GMFCS describes the functional characteristics in five levels, from
I to V, level I being the mildest.
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GMFCS
Before 2 years
2-4 years
4-6 years
6-12 years
Level I
Manipulate objects with
hands and walk
independently
Gets up from sitting
without holding unto
something
Can climb stairs
Walk indoors and
outdoors, climb stairs.
Level II
Belly crawls, pull to stand
on furniture and cruise
Can assume sitting
position without
assistance, walk with
assistive device
Sitting with both
hands free, walk
short distances
without assistive
device
Walk indoors or
outdoors on level
surface only
Level III
Can roll and creep forward
on stomach
‘w’ sit and require adult
assistance to assume
sitting
Walk with assistive
device
Walk indoors or
outdoors on level
surface with an
assistive mobility
device.
Level IV
Can roll independently
Able to roll and creep,
can sit when placed,
but need both hands on
the floor.
Sit independently in a
chair but minimal
hand function
Rely on wheeled
mobility, may achieve
self-mobility using
assistive device
Level V
Limited voluntary
movements, no head
control
Requires adult
assistance to roll
All areas of motor
functions are limited.
Functional limitations
in sitting and standing
are not fully
compensated for
through the use of
assistive device.
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Diagnosis of CP
Delayed motor milestones
• Fisting after 5 months of age
• Not sitting with support by 8 months
• Not walking by 15-18 months
• Discrepancies between intellectual and motor development
Persistent or evolving increased or decreased muscle tone
• Head lag beyond 6 months of age
• Poor trunk control and balance
• Opisthotonic posturing and extensor thrusting
• Dystonic
• Toe walking/scissoring
• Abnormal motor or gait patterns
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Approach to Management 1
What will the doctor do?
•
•
History: Explore parental concerns (mum is right
90% of the time), development, progression of the
problem, interaction and feeling of other family
members. Various histories will be taken e.g.
history during the antenatal period, labour and
delivery, neonatal history).
Physical examination for some chromosomal
disorders and syndromes. It is important to do
head circumference, check eyes, hearing, and skin
and organ enlargement.
18
Approach to Management 2
•
Investigations. No single investigation is
appropriate. In places where facilities are available
the following are advisable:
– Chromosomal studies
– Radiological imaging of the brain (CT or MRI)
– Metabolic studies
– Others include enzyme assays, tissue biopsies,
toxicological screening, TORCHES screening.
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Approach to Management 3
What should parents do?
• Get a diagnosis from the appropriate specialists.
• Get informed (separate fact from myths).
• Identify local interventions.
• Get involved or start a support group.
• Get counselling.
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General principles of treatment
• Determine severity of the disorder (it helps in assessing
how aggressive the intervention should be)
• Determine clear indications and goals of traditional medical
therapies
• Therapists and intervention programs should be informed
about the child and they in turn should inform the
physician of their activities
• Clinicians should be familiar with local intervention
programs with details of eligibility, access and payment
• Identify needs and appropriate intervention options
• Include parents in any therapy which they should be able to
incorporate into their everyday lives
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Issues in Management
• The Stigma
• The Fears
• The Reality
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Issues: The Stigma
• Children are highly cherished in our culture for
many reasons including the hope of future
advancement or prosperity of the family. Thus
when a child has a condition that reduces the
family’s expectation of this hope they often go
through the processes often associated with
grieving.
• Culture: annoyance of the gods, evidence of
infidelity, the village witch, etc.
• Hereditary Problem: It runs in their family.
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Issues: The Stigma
Consequences
• Denial of child: Not counted as an individual
with a unique identity.
• Neglect: A significant number of children are
malnourished (under- or over-).
• Social isolation: Hiding away of child from
family, friends and community. Shipping off
child to relative.
• Infanticide: Murder.
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Issues: The Fears
• Will this happen again?: Depends on the
circumstances and improvement in care of the
newborn.
• Cost of care: High
• Duration of care: Long Term
• Quality of Life: Depends on Severity (Tella et al 2011)
• The Label: Impairment, Disability, Handicap
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Issues: The Fears
Consequences
• Issues are often beyond the individual or
family and requires advocacy leading to
feelings of powerlessness.
• Issues are often overwhelming leading to
depression (often maternal).
• Reduction of family income.
• Child and spousal abuse (often emotional).
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Issues: The Reality
• There are no quick fixes or magic cures. Care is
multi-disciplinary.
• The process of caring for children with CP is very
long and requires determination, patience and
faith in the in-born (often times undiscovered)
abilities of the child.
• Most therapies would often involve prolonged
periods before appreciable differences can be
seen.
• It is very difficult to predict response to therapies.
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Issues: The Reality
• Most families will go through the different stages
of grieving before finally accepting the diagnosis.
This is normal.
• In Nigeria, without social security, the care of this
children is at a great cost.
• Most causes are preventable and can be reduced
with the improvement of basic health care
services (PHC).
• We are all stakeholders and need to be change
agents and advocates for children living with
cerebral palsy and their families.
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Conclusion
• CP is the commonest cause of movement
disorders in children.
• Some causes can be prevented with adequate
care of pregnant women and babies.
• Families are critical in the care of affected
children and they need financial, social and
emotional support they can get.
• Concerted efforts should be made to discover
the abilities within children with disabilities.
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Next Steps………….
• Professional need to listen and provide adequate as
well as appropriate information to families.
• Consultation, Collaboration, Advocacy.
– Appreciation to the Gbadebos and Benola Initiative for
having the burden, from personal experience, to raise
awareness and ensuring that the discussions at all levels of
collaboration take place.
• Families and NGOs must come together.
• Governments at all levels must respond to the cry of
these children and their families.
• An inclusive National Policy/Legislation on all NeuroDevelopmental conditions.
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References
•
•
•
•
•
•
Izuora GI, Iloeje SO. A review of neurological disorders seen at the Paediatric
Neurologic Clinic of the University of Nigeria Teaching Hospital, Enugu. Ann Trop
Paediatr 1989; 4: 185-190.
Frank-Briggs AI, Alikor EAD (2011). Pattern of Paediatric Neurological Diseases in
Port Harcourt, Nigeria. Int. J. Biomed Sci 2011; 7 (2): 145-149.
Tella BA, Gbiri C A, Osho O A, Ogunrinu A E. Health-Related Quality of Life of
Nigerian Children with Cerebral Palsy. Disability, CBR and Inclusive Development
2011; 22 (2): doi 10.5463/DCID.v22i2.24.
Lesi FEA, Onifade EU, Egri-Okwaji MTC, Danesi MA. The Social Environment of
Children with Cerebral Palsy in Lagos. Nigerian Quarterly Journal of Hospital
Medicine 1998; 8: 245-47
Ezeaka VC, Lesi FEA, Onifade EU, Grange AO. Childhood Neurological Disabilities in
Lagos: Clinical and Socio-demographic factors. Nigerian Medical Journal 2004:
45(1); 1-4.
Ogunlesi T, Ogundeyi M, Ogunfowora O Olowu A. Socio-clinical issues in cerebral
palsy in Sagamu, Nigeria. South African Journal of Child Health 2008; 2 (3): 120124.
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THANK YOU FOR YOUR
ATTENTION
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