a functional approach to serving adolescents with spina bifida presenter fred klingbeil, md medical...
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A Functional Approach to Serving Adolescents with
Spina Bifida
PresenterFred Klingbeil, MD
Medical Director Pediatric Rehabilitation Medicine
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Primary
Condition
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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
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Spina Bifida Occulta (5 percent of population)
5 percent of population
Incomplete vertebral closure without meninges or cord protrusion, and no
signs of neurologic impairment
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Spina Bifida Occulta
Clinical L5-S1 (most common) Dermal hyperpigmentation Hairy patch Lump Dermal sinus Initially asymptomatic
Complications Hinders spinal cord mobility with growth
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Spina Bifida Cystica
Protrusion of sac through
unfused vertebral arch defectMeningocele Incomplete vertebral closure with meninges protrusion
and no sign of neurologic impairmentMyelomeningocele Incomplete vertebral closure with meninges and cord
protrusion, and signs of neurologic impairment 47% lumber 26% thoracic 20% sacral 2% cervical
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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)
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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
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Chiari Malformation
Chiari refers to malformation and displacement of the hindbrain
into cervical vertebral canal
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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
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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
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Neurogenic Bladder
Flaccid (“Keeper”) TypePathophysiology Failure to empty due to ineffective bladder contractility
and/or ineffective urethral relaxation
Complications Urinary tract infection Vesicoureteral reflux, hydronephrosis Overflow incontinence
ManagementClean intermittent catheterizationProphylactic antibiotics
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Neurogenic Bladder
Spastic (“Leaker”) TypePathophysiology 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
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Neurogenic Bowel
Pathophysiology• Failure to adequately control bowel
usually due to involuntary relaxation and/or contraction of internal anal sphincter
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Neurogenic Bowel
Clinical Absent anal sensation Incontinence Constipation
Intervention Dietary suggestions Postprandial routine education Fiber diet Rectal suppositories Enema
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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
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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
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Orthopedic Deformities
SpineKyphosisScoliosisLordosis
KneeFlexion contractureExtension contracture
Foot/ankleEquinovarusRocker bottomHind foot valgus
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Cocktail Personality
Verbose, irrelevant conversation
Poor pragmatic use of language
Increase use of routine social phrases
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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
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Reproduction
Clinical Females
Most are fertile Normal labor, delivery
Males Unsustained, partial erection Absent, retrograde ejaculation Abnormal sexual stimulation
Management Education of alternative sexual stimulation Obstetric consultation Artificial penile implant
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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
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Projected Mobility
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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
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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
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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
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L4 Level
(L4 intact, L5 absent)
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
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L3 Level
(L3 intact, L4 absent)
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)
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L2 Level
(L2 intact, L3 absent)
Motor activity Intact Hip flexion, adduction Knee extension (weak)
Forces and deformities Hip flexion, adduction
Mobility Limited walking with significant bracing, crutches, and walker
Intervention Hip abduction brace Thoraco-hip-knee-ankle-foot orthoses (THKAFO) Reciprocating gait orthoses (RGO)
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Thoracic Level
Motor activity Absent lower extremity action
Mobility Wheelchair
Intervention Thoraco-lumbar-sacral orthoses Parapodium
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Focus and Desired Outcomes
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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
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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
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Adolescent stage 12-18 years
Twelve through eighteenth year of life is referred to as the adolescent stage. It is a time when a child seeks his/her own identity, (Who am I? Where am I going? What do I want to be? What can I be?), and independence. To achieve this, conflicts with parents are both natural and necessary. Peers are extremely important throughout this stage. Close parent/child communication helps the child grow and develop into responsible adulthood
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Adolescent stage 12-18 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 Urinary and fecal continence maintained Awareness of sexual issues Independent in all self care, including dressing, bathing, elimination,
wheelchair transfers, brace application, and driving Teen becoming independent in bowel and bladder management,
brace application, skin checks, and other self care activities
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Adolescent stage 12-18 years
Teen follows regular exercise program Normal range for height/weight ratio Teen receiving adequate information and support Teen has participated in adaptive driver’s education course Teen understands importance folic acid supplementation Teen enrolled in appropriate grade school with full inclusion in
classroom and extracurricular activities Teen following and able to discuss latex precautions and prevention Teen aware of safe sex and contraceptive options Parents receiving adequate information and support
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Questions?