unit. 15 Care of Child with Cerebral and Neuro Muscular Disorder.pptx

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1. Care of Child with Cerebral and Neuro Muscular Disorder

2. Objectives  Cerebral palsy  Muscular dystrophy  Gillian–Barre Syndrome  Spina bifida  Meningomyelocele  Nursing care, rehabilitative care and other medical and surgical management for the children with the above disorders  Overview of institutes i -e, NGO’s and Government law organization working in Pakistan for the rehabilitation of children with the above dysfunctions

3. Cerebral Palsy Cerebral Palsy is disorder of movement caused by a premature, non-progressive lesion in the developing brain. Incidence 1.9-2.3 in every 1000 live births. Etiology Could be Congenital or Acquired Prenatal 44%, Postnatal 8%, Labor and delivery 19%, Childhood 5%, Not obvious 24%

4. Types of Cerebral Palsy Spastic (Commonest) – Spastic Diplegia - either both arms or both legs are involved – Spastic Hemiplegia - one side of the body is affected, usually the arm more than the leg - Spastic Triplegia 3 limbs are involved, usually one leg and both arms – Spastic Monoplegia- 1 limb is affected – In some cases, spastic cerebral palsy follows a period of poor muscle tone (hypotonia) in the young infant. Dystonic Ataxic Mixed type/dystonic.

5. Pathology : Cerebral Palsy It is by caused damage to one or more specific areas of the brain (pyramidal system- cerebrum, cerebellum, basal ganglia) due to: – Gross malformation – Vascular occlusions – Loss of neuron – Low brain weight (Microcephaly) – Anoxia d/t significant cause of pathologic state of brain damage , but secondary to other causes

6. Clinical Manifestations : CP Appear before 18 months of age Parents first suspect (not developing motor skills normally). Delayed gross motor development Universal manifestation Discrepancy between motor ability and expected achievement with successive development (developmental delay) such as learning to roll over, sit crawl, walk, smile. Delay in one ability affects the other mile stone. Abnormal motor performance p reference for unilateral hand use at 6 months abnormal crawling Walk on toes Poor sucking and feeding difficulties

7. Clinical Manifestations Alteration of muscle tone Hypotonia/ hypertonia (stiff and rigid) Floppy and relax baby H ypotonia Unusual posture or use one side of the body Difficulty in handling and dressing Difficulty in diapering when pulled in sitting position extend his entire body Abnormal posture when in prone position maintain hip higher than the trunk. Scissoring Reflex abnormalities Persistence of primitive reflexes ( moro , planter, palmer and grasp)

8. Cerebral Palsy Associated disabilities and problems: Visual impairment Hearing impairment Communication problems, drooling, constipation Speech difficulties Problems with breathing because of postural difficulties. Skin disorders because of pressure sores. Seizures Intellectual impairment Diagnostic evaluation Assessment and history taking ( warning sign)

9. Therapeutic management Cerebral Palsy Physiotherapy Speech therapy Behavioral therapy Vocational therapy Recreation and leisure activities . Special education programs ( to improve abilities) Surgery---for contractures Medications – Anti anxiety, skeletal muscle relaxant i.e. baclofen, valium, dentrium , robaxin – Anti epileptic medications esp. Phenobarbital, and phenytoin

10. Nursing Considerations: CP AIMS/ plans Establish locomotion Establish self help Facilitate acquisition of educational opportunities according to capabilities Promote positive self image Ensure balance diet Support family in their efforts in meeting child needs

11. Implementation ROM exercises Provide safe environment Use restraints when child on chair or vehicle Encourage sitting, crawling, walking at appropriate age Encourage using aids that facilitate locomotion (crunches, braces) Provide extra calories for the activities but monitor wt Adopt utensils and clothing to facilitate self help (front buttons, special utensils Assist parents in toilet training Use articles / pictures that reinforce speech Speak slowly so he can understand

12. ROM exercises Provide safe environment Use restraints when child on chair or vehicle Encourage sitting, crawling, walking at appropriate age Encourage using aids that facilitate locomotion (crunches, braces) Provide extra calories for the activities but monitor wt Adopt utensils and clothing to facilitate self help (front buttons, special utensils Assist parents in toilet training Use articles / pictures that reinforce speech Speak slowly so he can understand

13. Teach verbal and non verbal communication ( sign language) Establish feeling of trust and independence Psychological support ( stress management ) Encourage parents to define their concerns Positive to enforce therapeutic plan/assist in normalization. Examples team work to modify utensils, modify cloths for self help Practical suggestion for feeding, moving, holding and encouraging to explore hand and feet to play. Positive reinforcement Support family Support groups of parents Referral to institutions ( eg : Al Shifa Trust)

14. Duchenne Muscular Dystrophy It is the commonest hereditary neuromuscular disease due to X-linked recessive trait. It is progressive disorder resulting in death in the early twenties Incidence Commonest hereditary neuromuscular disease Only boys are affected Etiology X-linked recessive trait Dystrophin gene defect

15. Diagnosis History Frequent falls Difficulty climbing stairs Delay in acquired walking Physical Examination Lordotic waddling gait Enlarged weak calf muscles Positive Gower’s sign Confirmatory Investigation Elevated Creatinine kinase EMG shows myopathic changes Muscle biopsy shows characteristic histology

16. Duchenne Muscular Dystrophy

17. Prognosis : DMD Progressive deterioration Confined to wheel chair by age 8-11yrs Involvement of chest muscles –respiratory infections Death usually occur at age 15-25yrs Management Physiotherapy Genetic Counseling (50% of the sons would be affected) Early detection Family support system Supportive therapy Stress management and coping

18. Guillain-Barre Syndrome Guillain-Barre syndrome (GBS) is a rare disorder of the nervous system that causes rapidly progressing muscle weakness that, in severe cases, can result in paralysis. Incidence May occur at any age Children are less affected than Adults High risk age b/w 4-10 yrs Affects both sexes Etiology Unknown Associated with number of viral infections and vaccines

19. Pathophysiology Unknown cause (after a mild infection, surgery, or immunization) Body’s immune system becomes confused Myelin around the nerves becomes its target for destruction Nerve impulses slow down or stop altogether

20. Clinical Manifestation : GBS Begins with muscle weakness, tingling, and loss of sensation in the legs (periphery) Then progress to the arms bilaterally and then to the face, Within a short time, normal reflexes are lost. The symptoms can increase until the muscles cannot be used at all and the patient is almost totally paralyzed. In the majority of patients, the most intense weakness occurs within the first two weeks. In severe cases, the muscles needed for breathing and swallowing become so weak that the patient cannot perform these functions unassisted.

21. GBS Diagnostic Test Spinal tap (reveals high protein levels in the cerebrospinal fluid) Electro diagnostic studies --to test the function of the nerve endings. Treatment Respiratory assistance I/V fluids Plasmapheresis Intravenous solution of autoimmune globulin Physical therapy

22. Nursing Management : GBS Educating Family Activities of Daily Living and Self-Care includes bathing dressing, and feeding Braces and Splints - may be used after surgeries or to prevent muscle tightness Communication - practicing ways to communicate and working on thinking and memory Lifestyle - adaptive recreation programs and community/school reentry Mobility and Transfers - ambulation, wheelchairs, and transfers Passive Range of Motion - stretching exercises performed to prevent muscle tightness

23. Nursing Management Positioning in Bed and Wheelchair education on positions in the bed and/or wheelchair and changing positions Psychology - assist with coping and adapting to illness or disability School - continues school work and contact school to set up services when your child can return Social Work - assist with resource applications and referrals to community agencies

24. Spina bifida Refer to abnormal development of spinal cord in the embryonic life (lack of union between the laminae of the vertebra). The defective closure usually occur lower in the spinal column Cause/ risk Genetic Folic acid deficiency Maternal viral infections Anti epileptic drugs (pregnancy) Diabetes Prevention Folic acid before and during in early pregnancy

25. Spina Bifida Occulted The incomplete closure at one level of spinal column with no apparent damage (L5-S1) Clinical manifestation Usually goes unrecognized A tuft of hair and Skin dimple may be present Manifestations may become evident when child starts walking or toilet trained – Foot Weakness gait changes – Enuresis – Incontinence

26. Nursing Management: Spina Bifida Pre surgery care of Sac place the child in warmer or incubator Apply moistened, non adherent sterile dressing over the sac to prevent leak, irritation or infection Keep the child in prone/ lateral position support with sheet, diaper roll or other support device Gentle handling Post operative care Monitor vitals, I/O, Head circumference Prone position Catheterization neurological assessment (extremity movements ) prepare parents for rehabilitation -self catheterization , use of upper limbs if paraplegia