Cerebral Palsy: The Stigma, The Fears and The Reality - PowerPoint Presentation

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Cerebral Palsy: The Stigma, The Fears and The Reality

Professor F E A LesiConsultant Paediatrician,Lagos University Teaching Hospital Benola: Cerebral Palsy Initiative20th July 2013

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Outline and ObjectivesUnderstand 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. Slide3

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. 4Slide5

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).5Slide6

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The Developing Brain Critical Periods of Brain growth1 month – neural tube4th month – All the lobes and major divisions complete1 year post-natal – 2/3 adult size2 years age – 75% adult size5 years – 90% adult sizePotential for Neurogenesis [new brain cell formation] (peaks in utero) and Synaptogenesis [new connection formation] (peaks by 5 years) continues throughout life. Slide7

Important Factors

IntrinsicGeneticsAgeNeurotransmitters (brain chemicals)GenderExtrinsic Exposure to drugs (cocaine, phenytoin, alcohol)StressHypoxia/ischemia, Viral infectionGonadal steroidsNutrition 7Slide8

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Normal DevelopmentMotorSocialCognitive/IQLanguage Maturation of the nervous system underlies development and it progresses cephalo-caudally (head downward). It is also a smooth continuous process.Slide9

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MotorPrimitive reflexes (disappear by 3-4 months)Neck control 3-4 months (earlier in African children)Sitting 5-6 monthsRolls 7 monthsCrawls 7-8 monthsStands with support 10 monthsWalks 12 monthsClimbs up and down stairs 20 monthsSlide10

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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 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. Slide11

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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

5/1000Slide12

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 Factbook12Slide13

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Causes of CPPre-natal (Before Delivery), Perinatal (Around Delivery), Postnatal After Delivery)Commonest CausesNigeria and Developing CountriesSevere 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 MetabolismSlide14

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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.Slide15

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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.Slide17

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Diagnosis of CPDelayed motor milestonesFisting after 5 months of ageNot sitting with support by 8 monthsNot walking by 15-18 monthsDiscrepancies between intellectual and motor developmentPersistent or evolving increased or decreased muscle toneHead lag beyond 6 months of agePoor trunk control and balance Opisthotonic posturing and extensor thrustingDystonic

Toe walking/scissoring

Abnormal motor or gait patterns Slide18

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Approach to Management 1What 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.Slide19

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Approach to Management 2Investigations. No single investigation is appropriate. In places where facilities are available the following are advisable: Chromosomal studiesRadiological imaging of the brain (CT or MRI)Metabolic studiesOthers include enzyme assays, tissue biopsies, toxicological screening, TORCHES screening.Slide20

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. 20Slide21

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General principles of treatmentDetermine severity of the disorder (it helps in assessing how aggressive the intervention should be)Determine clear indications and goals of traditional medical therapiesTherapists and intervention programs should be informed about the child and they in turn should inform the physician of their activitiesClinicians should be familiar with local intervention programs with details of eligibility, access and paymentIdentify needs and appropriate intervention optionsInclude parents in any therapy which they should be able to incorporate into their everyday livesSlide22

Issues in Management

The StigmaThe FearsThe Reality22Slide23

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. 23Slide24

Issues: The Stigma

ConsequencesDenial 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. 24Slide25

Issues: The Fears

Will this happen again?: Depends on the circumstances and improvement in care of the newborn. Cost of care: HighDuration of care: Long TermQuality of Life: Depends on Severity (Tella et al 2011)The Label: Impairment, Disability, Handicap25Slide26

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). 26Slide27

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. 27Slide28

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. 28Slide29

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. 29Slide30

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. 30Slide31

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

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THANK YOU FOR YOUR ATTENTION

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