Cerebral Palsy: The Stigma, The Fears and The Reality

1. 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

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.

3. The Brain

4. 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.

5. 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).

6. 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.

7. Important Factors Intrinsic • Genetics • Age • Neurotransmitters (brain chemicals) • Gender Extrinsic • Exposure to drugs (cocaine, phenytoin, alcohol) • Stress • Hypoxia/ischemia, • Viral infection • Gonadal steroids • Nutrition

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?

12. 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

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.

16. GMFCS

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 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.

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.

20. Approach to Management 3What 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.

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

22. Issues in Management • The Stigma • The Fears • The Reality

23. 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.

24. 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.

25. 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

26. 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).

27. 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.

28. 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.

29. 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.

30. 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.

31. 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): doi10.5463/DCID.v22i2.24. • LesiFEA, 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 • EzeakaVC,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): 120-124.

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