Download Spina Bifida Occulta - University of Arkansas for Medical Sciences

Neural Tube Defect: A Lifetime of Implications
Becky Watkins-Bregy, R.N.,C.
Arkansas Children’s Hospital
Spina Bifida Program Coordinator
Friday, April 14, 2006
Neural Tube Defect 101
• Myelomeningocele - most common, yet most serious
type of spina bifida (Lipomyelomeningocele)
• Meningocele – rare, requires intervention, typically
better outcome
• Anencephaly – rare, prognosis uniformly grim for those
not stillborn
• Encephalocele – Usually associated with Chiari II
malformation, may also occur with a spina bifida
Neural Tube Defect 101
• Lipoma / Lipomeningocele – A collection of
fatty tissue impacting the spina bifida
• Spina Bifida Occulta – Mildest form, a simple
bony abnormality with minimal or no nerve
involvement. May be present in up to 25 % of
the “normal” population, of which may or may
not become an incidental finding.
Spina Bifida: A Neurosurgical View
Closure 24 – 72 hours
Tethered Cord
80% shunt dependent
Hydrocephalus
Syringomyelia/Syrinx
90 % Arnold Chiari II
Malformation
Spina Bifida: A Urological View
• Reflux (secondary)
• Hydronephrosis
• Bladder capacity and
post void residuals
• Neurogenic Bladder
• Kidney / Bladder
Infections
sensation
• Kidney / Bladder
Stones
sensation
• Sphincter Issues
Spina Bifida: A Urological View
• Clean Intermittent
Catheterization
(80 – 90%)
• Medications
• Neurogenic Bowel
Management
• Surgical Interventions
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Deflux
Bladder Augmentation
Ureteral Reimplant
Urethral Slings /
Bladder Neck
Suspension
– Mitrofanoff
– A.C.E. / Cecostomy
Spina Bifida: An Orthopedic View
• Club Feet
• Hip Dislocation /
Subluxation
• Fractures and
Contractures
• Spinal Curvatures
– Kyphosis: sometimes
present at birth, high
lesions
– Lordosis: usually result
of hip flexion contract
– Scoliosis: common,
effects 50 – 90 %,
may become life
threatening
Spina Bifida : A Rehab View
• Spina Bifida Level
– S2/S4
• Prognosis for Walking
Frequently walk w/o aides, may need
shoe inserts
– L5/S1
Usually need short leg braces for
foot position and push off. May need
crutches or cane.
– L4
Usually need braces, above or below
knee; crutches/cane…some to
wheelchair at older age
– L2/3
Long leg bracing (thigh/waist) w/
crutches. Often walk for exercise only
when older, w/c main mode
Spina Bifida : A Rehab View
• Spina Bifida Level
– L1
– T12 and above
• Prognosis for Walking
Long leg braces with a band
around the waist, crutches.
Usually limited to walking for
exercise only. Wheelchair for any
distance.
Wheelchair for most activities
even in childhood.
(Answering Your Questions About Spina Bifida,
Children’s National Medical Center 2003)
– Cervical
“A RARE BIRD!!!”
Spina Bifida : A Rehab View
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ADL’s
Bracing / Seating
Nutrition
Skin Integrity
Gross Motor
Fine Motor
Education / Learning
Disabilities
• Sensory Impairment
• Motor Impairment
• Obesity
Spina Bifida: A General Overview
• Average lifespan now well into
adulthood, “normal or near normal
lifespan”. (Children With Spina Bifida, Woodbine House, 1999)
• I.Q. average range 80 to 90,
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studies show this may be lowered
due shunt infections and multiple
revisions
Learning, Cognitive & Language
Disabilities
Psychosocial Impact
School
Parent: work, family, transport,
relationship, finance
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Sexual function
Latex allergy
Transition Programs
Healthcare
Funding
Transportation
Employment
Mental Health
Spina Bifida: A Researcher’s View
29 spina bifida patients ages 4 – 14 (mean age 11.4) and
their parents were studied at SB Centre Universita
Cattolica, Rome, Italy. The study related to the healthrelated QoL surrounding the diagnosis of spina bifida.
The multiperspective assessment showed a surprising
finding that the children had a higher focus related to
deterioration of physical aspects of QoL due to
continence issues and number of catheterizations, rather
than other physical limitations. It also showed while the
children were more focused on the physical aspects of
their QoL, the parent’s had a much higher concern on
the scale of emotional aspects of QoL.
Spinal Cord (2005) 43, 230-235. doi: 10.1038/sj.sc.3101707 Published online 18 January 2005
Spina Bifida: A Researcher’s View
A descriptive study, conducted by University of Connecticut Health
Center, using a convenience sample of 60 youth (15 – 25 yr. old) w/
SB. This study was designed to look at the relationship among
health status (severity of illness), functional status and HRQoL. The
researcher concluded that there was a high level of satisfaction with
HRQoL in this group of young people who are succeeding at college,
participating in recreation, sporting activities and other aspects of
young adult living. It is important to note that despite the fact that
many continued to face a number of secondary health conditions
that leave them with concerns for their future.
Rehabilitation Nurse, 2005, Sep-Oct;30(5):180-7;discussion 188
A.C.H. Spina Bifida
Program
• Only multidisciplinary
program in the state
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Core Team
Neurosurgery
Orthopedics
Urology
Rehab Medicine
Neuropsychology
Occupational Therapy
Physical Therapy
Clinical Nutrition
Social Work
Currently following 473
active patients (prenatal
through lifespan)
Works with various outside
community agencies to
provide patient resources
and continuity of care
Community outreach
programs: teaching,
therapeutic recreation
activities
Prenatal Visit: A Team
Effort!!
• Referral from OB
Services
• Seen in SB Clinic
• Introductions to
staff, Education &
Tour (Clinic/NICU)
What Parents Have to
Say !!
“ It would have not been such a big shock if I
would have seen other kids in the Spina
Bifida Clinic. I would have been better
prepared.” Courtney, has a newborn son
“It was such a big help to have a pre-natal
visit! I knew exactly what to expect and
the doll was a wonderful teaching device.
It was nice to have a familiar face when
the baby came.” Carrie, mother of Hollie
What Parents Have to
Say !!
“We are so grateful to the SB Clinic and
A.C.H. The pre-consultation before our
daughter’s arrival was informative and
comforting. Knowing what to expect
ahead of time, from worse case to best
case scenario, is so important for your
child’s care. Meeting the doctors and the
nurses and getting a tour of the facilities
helped to relax my nerves on delivery day
knowing that Katie would be in good
hands.” Julie, mother of Katie
Be A Part of OUR TEAM !!!
Pre-Natal Referrals
501-364-1806
Spina Bifida Program