A Functional Approach to Serving Preschool and School Aged Children with Spina Bifida Presenter Fred Klingbeil, MD Medical Director Pediatric Rehabilitation Medicine

Role of pediatric rehabilitation medicine Functional assessment Primary disabling disorder Associated conditions and issues Impairments and functional limitations Managerial focus, objectives and expectant outcome

Primary Condition

Occurrence 0.2% of general population 5% if one child with defect 10% if one parent with defect 10% if two children with defect 21% if three children with defect More common if Irish ancestry

Spina Bifida Occulta (5 percent of population) Incomplete vertebral closure without meninges or cord protrusion, and no signs of neurologic impairment

Spina Bifida Occulta Clinical L5-S1 (most common) Dermal hyperpigmentation Hairy patch Lump Dermal sinus Initially asymptomatic   Complications Hinders spinal cord mobility with growth

Protrusion of sac through unfused vertebral arch defect Spina Bifida Cystica Protrusion of sac through unfused vertebral arch defect Meningocele Incomplete vertebral closure with meninges protrusion and no sign of neurologic impairment Myelomeningocele Incomplete vertebral closure with meninges and cord protrusion, and signs of neurologic impairment 47% lumber 26% thoracic 20% sacral 2% cervical

Latex Allergy Risk factors Toys containing latex, supplies, equipment Avocado Banana Water chestnut   Clinical Sudden anaphylactic reaction Subtle hives or rash Management Prevention Education Prevent exposure Intervention Diphenhydramine (Benadryl) Epinephrine (EpiPen)

Hydrocephalus Occurrence 85 percent Clinical Enlarged head Bulging forehead and fontanel Widespread cranial sutures Sunset eyes Decreased activity Poor feeding Somnolence Irritability Headaches Decreased eye-hand coordination Decline in school performance

Chiari Malformation Chiari refers to malformation and displacement of the hindbrain into cervical vertebral canal

Chiari Malformation Occurrence Occurs in all children with hydrocephalus but is symptomatic in only 20-30 percent of cases Clinical Nystagmus, strabismus Difficulty swallowing, slow to tolerate solid foods Apnea spells, stridor, aspiration Spasticity Face, neck, extremity weakness Loss of fine motor control Sensory loss Growth delay

Tethered Cord Clinical Deterioration of strength Changing reflex level Change in tone and reflexes Change in bowel and bladder function Forming deformities Change in sensation    Intervention Observe for asymptomatic cord and stable neurologic impairments Symptomatic cord release

Flaccid (“Keeper”) Type Neurogenic Bladder Flaccid (“Keeper”) Type Pathophysiology Failure to empty due to ineffective bladder contractility and/or ineffective urethral relaxation Complications Urinary tract infection Vesicoureteral reflux, hydronephrosis Overflow incontinence Management Clean intermittent catheterization Prophylactic antibiotics

Spastic (“Leaker”) Type Neurogenic Bladder Spastic (“Leaker”) Type Pathophysiology Failure to adequately store urine due to uninhibited bladder detrusor contracting and/or ineffective urethral resistance Complications Incontinence Intervention Clean intermittent catheterization Decrease bladder contraction using (antecholinergic) medications Increase urethral resistance using (adrenergic) medications Surgery

Neurogenic Bowel Pathophysiology Failure to adequately control bowel usually due to involuntary relaxation and/or contraction of internal anal sphincter

Neurogenic Bowel Clinical Absent anal sensation Incontinence Constipation Intervention Dietary suggestions Postprandial routine education Fiber diet  Rectal suppositories Enema

Obesity Causes Decreased metabolic rate Increased body fat Decreased lean body mass Increased caloric intake as awards   Intervention Caloric intake 50-75 percent of normal Low cholesterol and unsaturated fat diet Maximize activity

Cardiovascular Disease Risk factors Obesity Decreased activity Hypertension due to renal dysfunction   Evaluation Lipid profiles Diet and weight Exercise routine Intervention Encourage exercise Modify diet Cholesterol lowering medications

Orthopedic Deformities Spine Kyphosis Scoliosis Lordosis   Knee Flexion contracture Extension contracture Foot/ankle Equinovarus Rocker bottom Hind foot valgus

Cocktail Personality Verbose, irrelevant conversation Poor pragmatic use of language Increased use of routine social phrases

Learning Disorders Clinical Achievement tests reveal deficits in arithmetic more than reading or spelling Visuomotor dysfunction more common and severe in higher level lesions Math and visual-spatial skills fall more behind normal peers with age Evaluation Neuropsychologic testing

Psychological Issues Family stress Increased divorce rate Increase substance abuse Altered relationships Overprotected Decreased socialization   Sexual dysfunction Penile erection partial and unsustained Ejaculation may be absent or retrograde Females usually fertile Marriage and parenting 1 in 10 risk of parenting infant with neural tube deficit

Projected Mobility

S2 Level (S2 intact, S3 absent) Motor activity Intact Hip flexion, extension, adduction, abduction Knee extension, flexion Foot dorsiflexion, plantarflexion, adduction, abduction  Absent Foot intrinsics Forces and deformities Foot claw, cavovarus   Mobility Walking with or without bracing and crutches

S1 Level (S1 intact, S2 absent) Mobility Walking with or without bracing and crutches   Intervention Hinged FRAFO   Supramalleolar orthoses (SMO)  Athletic shoes Lightweight Stable Designed for performance

L5 Level (L5 intact, S1 absent) Motor activity Intact Hip flexion, adduction, abduction Knee extension, flexion Foot dorsiflexion, inversion Absent Hip extension Foot planter flexion, eversion Mobility Walking with or without bracing and crutches

L4 Level (L4 intact, L5 absent) Motor activity Intact   Motor activity Intact Hip flexion, adduction Knee extension Foot dorsiflexion, inversion  Absent Hip extension, abduction Knee flexion Foot plantar flexion, eversion Mobility Walking with or without bracing and crutches

L3 Level (L3 intact, L4 absent) Motor activity Intact   Motor activity Intact Hip flexion, adduction, rotation (weak) Knee extension (antigravity or greater) Mobility Limited walking with significant bracing, crutches, and walker Intervention Knee-ankle-foot orthoses (KAFO)  Floor-reaction-ankle-floor orthoses (FRAFO)

L2 Level (L2 intact, L3 absent) Motor activity Intact Hip flexion, adduction Knee extension (weak) Forces and deformities Mobility Limited walking with significant bracing, crutches, and walker Intervention Hip abduction brace Thoraco-hip-knee-ankle-foot orthoses (THKAFO)  Reciprocating gait orthoses (RGO)

Thoracic Level Motor activity Absent lower extremity action Mobility Wheelchair Intervention Thoraco-lumbar-sacral orthoses   Parapodium

Focus and Desired Outcomes

General Desired Outcomes Overall outcome Maintain health status and prevent secondary conditions and complications Specific outcomes Control hydrocephalus Optimize neurological functioning Preserve renal function Obtain urinary continence Obtain bowel continence Progress in growth and development Obtain and maintain mobility Progress in psychological and sexual development

General Desired Outcomes Up to date with current immunizations Maintain ideal body weight Maintain good general health Absence of repeated infection Stable cardiovascular and respiratory status Intact skin Balance patterns of eating, sleeping, and exercise Absence of orthopedic deterioration Obtain and maintain positive attitude and self esteem Follow latex precautions

Toddlerhood 1-3 Years First to third year of life is referred to as the toddler stage. During this time the child experiments with holding on and letting go, (control issues), and begins to attach enormous value to will power. Extensive exploration of environment occurs. This is the beginning of independence. For growth and development to progress, child needs to have appropriate opportunities to express autonomy. Parents play vital role in encouraging safe, appropriate independence

Toddlerhood 1-3 Years Normal intracranial pressure maintained Optimal mobility achieved Bladder drainage and renal function remain normal Urinary tract infections controlled Bowel continence program initiated Normal range for height and weight Child enrolled in early intervention school program Parents receiving adequate information and support Mother aware of importance of taking folic acid Latex precautions being followed

Preschool 3-5 Years Third year through firth year of life is considered the preschool stage. During this stage the child want to know about and do everything. A beginning understanding of boy/girl roles, body image, and body boundary develops. Much is explored through imagination. Parent can do a lot to encourage child’s need to now and understand so that growth and development will progress. Learning self-care skills becomes important

Preschool 3-5 Years Normal intracranial pressure maintained Optimal mobility maintained Bladder drainage and renal function remain normal Urinary tract infections controlled Social continence of bowel and bladder achieved Normal range for height and weight Child enrolled in appropriate preschool program Child’s overall development is progressing Parents receiving adequate information and support Necessary durable medical equipment, including wheelchair obtained for mobility and school bus transportation No deterioration of skills evident Latex precautions being followed

School Age 6-11 Years   Sixth year through eleventh year is referred to as the school-age stage. It is the time when the child need to feel mastery and completion of tasks and projects and be recognized for them. This will contribute to self-esteem and self-confidence. Peers become increasingly important. Parents can do a lot to facilitate peer socialization and a feeling of recognition for what is accomplished

School Age 6-11 Years Normal intracranial pressure maintained Optimal mobility maintained Secondary disability due to tethered cord due to preadolescent growth spurt prevented Bladder drainage and renal function remain normal Urinary tract infections controlled Child becoming independent in bowel and bladder management, brace application, skin checks, and other self care activities Child follows regular exercise program Normal range for height and weight Child enrolled in appropriate grade school with full inclusion in classroom and extracurricular activities Parents receiving adequate information and support Mother aware of importance of taking folic acid Child following and able to discuss latex precautions and prevention

Questions?