Cerebral Palsy: The Stigma, The

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23 févr. 2014 (il y a 3 années et 3 mois)

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Cerebral Palsy: The Stigma, The
Fears and The Reality

Professor F E A
Lesi

Consultant
Paediatrician
,

Lagos University Teaching Hospital



Benola
: Cerebral Palsy Initiative

20
th

July 2013


1

2

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.

The Brain

3

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

6

The Developing Brain


Critical Periods of Brain growth


1 month


neural tube


4
th

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.

Important Factors

Intrinsic


Genetics


Age


Neurotransmitters (brain chemicals)


Gender

Extrinsic


Exposure to drugs (cocaine, phenytoin, alcohol)


Stress


Hypoxia/ischemia,


Viral infection


Gonadal steroids


Nutrition

7

8

Normal Development


Motor


Social


Cognitive/IQ


Language


Maturation of the nervous system underlies
development and it progresses cephalo
-
caudally (head downward). It is also a
smooth continuous process.

9

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

10

Cerebral Palsy (CP)


A group of disorders of the development of
movement

and posture, causing activity
limitation that are attributed to
non
-
progressive
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.

11

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

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

12

13

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



14

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.

15

16

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.


17

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

18

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
.

19

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.


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
.

20

21

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

Issues in Management


The Stigma



The Fears



The Reality

22

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.


23

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.


24

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


25

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).

26

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.

27

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.

28

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.


29

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
Neuro
-
Developmental conditions
.

30

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
C
hild Health 2008; 2 (3): 120
-
124.

31

THANK YOU FOR YOUR
ATTENTION



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