Conditions Spina Bifida Flashcards

1
Q

Statistics and Demographics

  • myelomeningocele - birth rate
  • incidence in ireland and wales
  • males vs females
  • 3 reason for nural tube defectis decreasing world wide
  • % of children with Myelomeningocele surviving into adultgood in 2005
A

Myelomeningocele - 60 out of 100,000 births - US

Incidence is 4 times higher in Ireland & Wales

Females 3-7 times > males

Prevalence of neural tube defects are decreasing worldwide due to:
Maternal testing  abortions
Foods enriched with folic acid
multivitamins /folic acid with women trying to get pregnant

85% of children with myelomeningoceles survived to adulthood in 2005 (previously 10% in the 50s)

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2
Q

Causes

9 possible factors thought to contribute to Spina Bifida

A
  • 50% -nutritionally based deficiency of folate (folic acid, B vitamin)
  • Maternal alcoholism
  • Diabetes
  • Anticonvulsant medications
  • Anti-seizure medications (Depakene, Depakote, Tegretol)
  • Acne medication (Accutaine)
  • Maternal hypethermia (use of saunas, high fever)
  • Obesity
  • Race/Ethnicity
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3
Q

Spina Bifida Description

A
  • Occurs during fetal development (within the first 25 days of pregnancy) – neural tube does not form properly resulting in an open spine
  • A portion of the spinal cord protrudes outside of the body putting spinal cord, meninges, and nerves at risk for damage
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4
Q

3 types of Spina Bifida

A

1) Occulta:
no visible opening in the back, but vertebrae not completely closed – “hidden spina bifida”
spinal cord, meninges covered – no nerve damage
dimple or excess hair at site of opening on the back

2) Meningocele:
Outer part of some vertebrae do not close during development
Meninges damaged, protrude through opening as a sac outside of the skin
Usually no nerve damage
Least common

3) Myelomeningocele:

Outer part of some vertebrae do not close properly in utero
Spinal cord and meninges do not develop normally
Almost always nerve damage
Usually results in some form of paralysis
Most serious type
Causes most severe disabilities

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5
Q

Level of Lesions - Sacral Lesions

A

Resulting impairments

Mild weakness at ankle or toes; bladder and anal sphincters intact; able to walk with minimal support (bracing or adaptive devices)

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6
Q

Level of Lesion - Low Lumbar (L4, L5) Lesions

A

Resulting Impairments

Weak plantar flexion and hip extension (can flex hips and extend knees); able to walk with minimal support (bracing or adaptive devices)

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7
Q

Level of lesion - Mid Lumbar (L3) Lesions

A

Resulting Impairments

Paralysis at the ankles and toes (can flex hips and extend knees); may be able to walk with extensive bracing and/or adaptive equipment

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8
Q

level of Lesion - High Lumbar (L1, L2) and Thoracic Lesions

A

Variable weakness throughout the LE; bladder and anal sphincters and musculature are affected; may be able to walk with extensive bracing and/or adaptive equipment

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9
Q

Primary Neurological Impairments

Paralysis & loss of sensation

A
  • Depends on the location of the defect
  • Not always symmetrical
  • Always some sensation loss
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10
Q

Primary Neurological Impairments

Chiari Malformation

A
  • Almost all children with meningomyelocele above sacral lesion have Chiari type II Malformation
  • Symptoms: difficulty swallowing, choking, hoarseness, breath-holding spell, apnea, disordered breathing during sleep, stiffness in the arms, opisthotonos (holding the head arched backward)
  • Disordered breathing during sleep: “missed diagnosis” that can cause children to be tired during the day and affect school function
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11
Q

Primary Neurological Impairments

Hydrocephalus

A
  • 60-95% of children with meningomyeloceles
  • Symptoms of blockage include: lethargy, headache, vomiting, irritability, strabismus, double vision, paralysis of upward gaze, change in personality, decline in school performance, and weakness of arms and legs
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12
Q

Secondary Impairments

A
  • Mobility impairments
    • Ambulation impaired more significantly the higher the lesion
    • Use of braces, crutches, wheelchairs
  • Cognitive Impairments
    • ¾ have average IQ scores
    • Significant impairments in perceptual skills, organizational abilities, attention span, speed of motor response, memory and hand function
    • Impairment in executive functions that affect education, social, and self-help skills
  • Seizure Disorders
    • 15% have a seizure disorder
    • Usually tonic-clonic
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13
Q

More Secondary Impairments

A
  • Visual Impairments
    • Strabismus, double vision, paralysis of upward gaze due to increase in intracranial pressure
  • Musculoskeletal Abnormalities
    • Clubfoot
    • Ankle and foot deformities (use of extensive serial casting or bracing)
    • Hip deformities (due to muscle imbalance and lack of movement)
    • Predisposed to fractures (use of wheelchairs or weight bearing to strengthen bones)
    • Arthritis
    • Scoliosis, kyphosis, kyphoscoliosis
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14
Q

Even more Secondary Impairments

A
  • Urinary Dysfunction
    • Present in virtually all children
    • Incontinence
    • Lead to kidney damage or infection
    • Use daily intermittent catheterization (CIC) at least 4x/day
    • Many meds used to prevent infections and incontinence
  • Bowel Dysfunction
    • Present in virtually all children
    • Constipation, diarrhea, or soiling are socially devastating
    • Bowel management strategies
    • Use of laxatives
    • Surgical procedures to connect appendix to the colon
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15
Q

ugh more really? secondary Impairments

A
  • Skin Sores
    • Decubitus ulcers on weight-bearing surfaces not sensitive to pain
    • Feet, buttocks
    • Debridement (removal of dead tissue), plastic surgery, intravenous antibiotics
    • Prevention is key
  • Weight and Stature Abnormalities
    • Increased risk of obesity (need for aerobic and strength training)
    • Fewer calories needed due to paraplegia
    • Supplied with HGH to reduce short stature
  • Sexual Issues
    • Pregnancy risk due to urinary tract infection, ulcers, slipped discs, and difficult vaginal delivery (hip deformity)
  • Depression
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16
Q

detection

A
  • Diagnosed prenatally in the womb
  • Suspicion following an ultrasound
  • Amniocentesis: removal of some of the amniotic fluid that surrounds the fetus for analysis
  • Alpha fetoprotein (AFP) is a protein made by the fetus found in the amniotic fluid (When in excess, leakage is assumed)
  • Next analyze AChE levels, which surround nerve cells (When in excess, leakage is assumed)
  • Follow up with ultrasounds and sonograms to identify location of lesion, presence of hydrocephalus, and other congenital anomalies
17
Q

Immediate Action

A
  • Intervention and management begin at birth
  • If defect is leaking or open at birth, surgical closure is performed usually 24 hours after birth to prevent from infection and to prevent further loss of function
  • Surgical closure does not repair the damage
  • Can prevent from further loss of function due to further deformation of spinal cord and nerves
  • There will always be some impairment below the lesion, dependent on the location of the lesion
  • Also perform surgery to place a shunt in the ventricles of the brain if hydrocephalus is present
  • There have been several prenatal surgeries conducted; however, risk of maternal bleeding and infection lessen prevalence
18
Q

Medical, Allied, and Educational Professional Team

A
  • Immediately after discharge from the hospital
  • Facilitate optimal motor, self-care, and occupational performance
  • Physician: coordinate care and refer for appropriate services
  • Orthopedics: manage foot and hip deformities
  • OTs & PTs: facilitate motor milestone attainment & successful participation and performance in childhood occupations (especially after surgeries)
  • Neurosurgery: shunt management
19
Q

Evaluation/Assessment

A
  • Review: Medical and developmental histories, childhood occupations (parent report), psychological test of cognitive level and IQ
  • Physical Assessment conducted by OT and PT:
  • AROM & PROM
  • Muscle strength through antigravity postures and movements
  • Proximal and distal tone
  • Joint involvement
  • Sensation
  • Balance reactions
  • Motor control (fine and gross)
  • Developmental level
  • Asymmetry in UE and LE
  • Movement patterns
20
Q

Functional Assessment as conducted by OT

A
  • Feeding
  • Dressing
  • Grooming
  • Hygiene
  • Role as son or daughter, sibling, student, friend
  • Ability to function at home, school, or in the community
  • Overall independence level
21
Q

Equipment needs as conducted by OTs

A
  • Bath chair
  • Home modifications
  • School modification
  • Assistive technology
  • Adaptive equipment
  • Splinting
  • Bracing
  • Casting
  • Specialized seating systems (to build stability)
  • Walkers
  • Crutches
  • Wheelchairs (Manual ; Power)
22
Q

activities

A
  • Self-care and Occupational Roles
    • Posture: Adaptive seating (12 months)
    • Hand-eye coordination: Toys (1-2 y/o)
    • Dressing: Adaptive clothing (4-7 y/o)
    • Bladder control: Self-catheter (8 y/o)
    • Education: Writing skills (9-11 y/o)
    • Mobility: Wheelchair
    • Assistive Technology (Johnson and et al, 2007)
    • Psychosocial: Social and family support
  • Meaningful activities
23
Q

Llit Review

A

Autoethnographic – Dr. Ann Neville-Jan shares her own experiences as a person with impairments from spina bifida to connect to historical and medical views about spina bifida

Preventive efforts are discussed from a disability rights perspective

Challenges occupational therapists to examine their attitudes toward disability, act as advocates in their practice, and support or contest initiatives

24
Q

more lit review

A
  • Dr. Neville-Jan found and concludes the following alternative views of spina bifida:
  • “People with spina bifida and hydrocephalus live a full life with equal to that of any other citizen”
  • “Adults with spina bifida started very clearly that their quality of life is not a reason for abortion”
  • “Parents and adults state that impairment is not the burden. The burden is the constant struggle against inaccessible environments and inadequate systems, which should be allies and should be supportive of them”