friday, april 14, 2006

Neural Tube Defect: A Lifetime of Neural Tube Defect: A Lifetime of ImplicationsImplications

Becky Watkins-Bregy, R.N.,C.Becky Watkins-Bregy, R.N.,C.Arkansas Children’s Hospital Arkansas Children’s Hospital

Spina Bifida Program CoordinatorSpina Bifida Program Coordinator

Friday, April 14, 2006Friday, April 14, 2006

Neural Tube Defect 101Neural Tube Defect 101

• Myelomeningocele Myelomeningocele - most common, yet most - most common, yet most serious type of spina bifida serious type of spina bifida (Lipomyelomeningocele)(Lipomyelomeningocele)

• MeningoceleMeningocele – rare, requires intervention, – rare, requires intervention, typically better outcometypically better outcome

• AnencephalyAnencephaly – rare, prognosis uniformly grim – rare, prognosis uniformly grim for those not stillbornfor those not stillborn

• EncephaloceleEncephalocele – Usually associated with Chiari II – Usually associated with Chiari II malformation, may also occur with a spina bifidamalformation, may also occur with a spina bifida

Neural Tube Defect 101Neural Tube Defect 101

• LipomaLipoma // LipomeningoceleLipomeningocele – A collection – A collection of fatty tissue impacting the spina bifidaof fatty tissue impacting the spina bifida

• Spina Bifida Occulta Spina Bifida Occulta – Mildest form, a – Mildest form, a simple bony abnormality with minimal or simple bony abnormality with minimal or no nerve involvement. May be present in no nerve involvement. May be present in up to 25 % of the “normal” population, of up to 25 % of the “normal” population, of which may or may not become an which may or may not become an incidental finding.incidental finding.

Spina Bifida: A Neurosurgical Spina Bifida: A Neurosurgical ViewView

Closure 24 – 72 hours Closure 24 – 72 hours

80% shunt dependent80% shunt dependent

HydrocephalusHydrocephalus

90 % Arnold Chiari II 90 % Arnold Chiari II MalformationMalformation

Tethered CordTethered Cord

Syringomyelia/SyrinxSyringomyelia/Syrinx

Spina Bifida: A Urological Spina Bifida: A Urological ViewView• Reflux (secondary)Reflux (secondary)

• HydronephrosisHydronephrosis

• Bladder capacity and Bladder capacity and post void residualspost void residuals

• Neurogenic BladderNeurogenic Bladder

• Kidney / Bladder Kidney / Bladder Infections Infections sensationsensation

• Kidney / Bladder Kidney / Bladder Stones Stones sensationsensation

• Sphincter IssuesSphincter Issues

Spina Bifida: A Urological Spina Bifida: A Urological ViewView• Clean Intermittent Clean Intermittent

Catheterization Catheterization (80 – (80 – 90%)90%)

• MedicationsMedications

• Neurogenic Bowel Neurogenic Bowel ManagementManagement

• Surgical Surgical InterventionsInterventions– DefluxDeflux– Bladder AugmentationBladder Augmentation– Ureteral ReimplantUreteral Reimplant– Urethral Slings / Urethral Slings /

Bladder Neck Bladder Neck SuspensionSuspension

– MitrofanoffMitrofanoff– A.C.E. / CecostomyA.C.E. / Cecostomy

Spina Bifida: An Orthopedic Spina Bifida: An Orthopedic ViewView

• Club FeetClub Feet

• Hip Dislocation / Hip Dislocation / SubluxationSubluxation

• Fractures and Fractures and ContracturesContractures

• Spinal CurvaturesSpinal Curvatures– KyphosisKyphosis: sometimes : sometimes

present at birth, high present at birth, high lesionslesions

– Lordosis: Lordosis: usually usually result of hip flexion result of hip flexion contractcontract

– ScoliosisScoliosis: common, : common, effects 50 – 90 %, effects 50 – 90 %, may become life may become life threateningthreatening

Spina Bifida : A Rehab ViewSpina Bifida : A Rehab View

• Spina Bifida LevelSpina Bifida Level– S2/S4S2/S4

– L5/S1L5/S1

– L4L4

– L2/3L2/3

• Prognosis for WalkingPrognosis for WalkingFrequently walk w/o aides, may Frequently walk w/o aides, may

need shoe insertsneed shoe inserts

Usually need short leg braces for Usually need short leg braces for foot position and foot position and push off. May need crutches or push off. May need crutches or cane.cane.

Usually need braces, above or Usually need braces, above or below knee; crutches/cane…some below knee; crutches/cane…some to wheelchair at older ageto wheelchair at older age

Long leg bracing (thigh/waist) w/Long leg bracing (thigh/waist) w/crutches. Often walk for exercise crutches. Often walk for exercise only when older, w/c main modeonly when older, w/c main mode


• Spina Bifida LevelSpina Bifida Level– L1L1

– T12 and aboveT12 and above

– CervicalCervical

• Prognosis for Prognosis for WalkingWalkingLong leg braces with a band Long leg braces with a band around the waist, crutches. around the waist, crutches. Usually limited to walking for Usually limited to walking for exercise only. Wheelchair for exercise only. Wheelchair for

anyanydistance.distance.

Wheelchair for most activities Wheelchair for most activities even in childhood.even in childhood.(Answering Your Questions About Spina Bifida,(Answering Your Questions About Spina Bifida, Children’s National Medical Center 2003)Children’s National Medical Center 2003)

““A RARE BIRD!!!”A RARE BIRD!!!”


• ADL’sADL’s

• Bracing / SeatingBracing / Seating

• Nutrition Nutrition

• Skin IntegritySkin Integrity

• Gross MotorGross Motor

• Fine MotorFine Motor

• Education / Learning Education / Learning DisabilitiesDisabilities

• Sensory Sensory ImpairmentImpairment

• Motor ImpairmentMotor Impairment

• ObesityObesity

Spina Bifida: A General Spina Bifida: A General OverviewOverview• Average lifespan now well into Average lifespan now well into

adulthood, “normal or near adulthood, “normal or near normal normal

lifespan”. lifespan”. (Children With Spina Bifida, Woodbine House, (Children With Spina Bifida, Woodbine House, 1999)1999)

• I.Q. average range 80 to 90, I.Q. average range 80 to 90, studies show this may be studies show this may be lowered due shunt infections lowered due shunt infections and multiple revisionsand multiple revisions

• Learning, Cognitive & Language Learning, Cognitive & Language DisabilitiesDisabilities

• Psychosocial Impact Psychosocial Impact • SchoolSchool• Parent: work, family, transport, Parent: work, family, transport,

relationship, financerelationship, finance

• Sexual function Sexual function • Latex allergyLatex allergy• Transition ProgramsTransition Programs• HealthcareHealthcare• FundingFunding• TransportationTransportation• EmploymentEmployment• Mental HealthMental Health

Spina Bifida: A Researcher’s Spina Bifida: A Researcher’s ViewView29 spina bifida patients ages 4 – 14 (mean age 11.4) 29 spina bifida patients ages 4 – 14 (mean age 11.4)

and their parents were studied at SB Centre and their parents were studied at SB Centre Universita Cattolica, Rome, Italy. The study related Universita Cattolica, Rome, Italy. The study related to the health-related QoL surrounding the diagnosis to the health-related QoL surrounding the diagnosis of spina bifida. The multiperspective assessment of spina bifida. The multiperspective assessment showed a surprising finding that the children had a showed a surprising finding that the children had a higher focus related to deterioration of physical higher focus related to deterioration of physical aspects of QoL due to continence issues and aspects of QoL due to continence issues and number of catheterizations, rather than other number of catheterizations, rather than other physical limitations. It also showed while the physical limitations. It also showed while the children were more focused on the physical aspects children were more focused on the physical aspects of their QoL, the parent’s had a much higher of their QoL, the parent’s had a much higher concern on the scale of emotional aspects of QoL.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 2005Spinal Cord (2005) 43, 230-235. doi: 10.1038/sj.sc.3101707 Published online 18 January 2005

Spina Bifida: A Researcher’s Spina Bifida: A Researcher’s ViewViewA descriptive study, conducted by University of Connecticut A descriptive study, conducted by University of Connecticut

Health Center, using a convenience sample of 60 youth (15 Health Center, using a convenience sample of 60 youth (15 – 25 yr. old) w/ SB. This study was designed to look at the – 25 yr. old) w/ SB. This study was designed to look at the relationship among health status (severity of illness), relationship among health status (severity of illness), functional status and HRQoL. The researcher concluded functional status and HRQoL. The researcher concluded that there was a high level of satisfaction with HRQoL in this that there was a high level of satisfaction with HRQoL in this group of young people who are succeeding at college, group of young people who are succeeding at college, participating in recreation, sporting activities and other participating in recreation, sporting activities and other aspects of young adult living. It is important to note that aspects of young adult living. It is important to note that despite the fact that many continued to face a number of despite the fact that many continued to face a number of secondary health conditions that leave them with concerns secondary health conditions that leave them with concerns for their future.for their future.

Rehabilitation Nurse, 2005, Sep-Oct;30(5):180-7;discussion 188Rehabilitation Nurse, 2005, Sep-Oct;30(5):180-7;discussion 188

A.C.H. Spina Bifida Program• Only multidisciplinary

program in the state• 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 !!!Be A Part of OUR TEAM !!!

Pre-Natal ReferralsPre-Natal Referrals

501-364-1806501-364-1806

Spina Bifida ProgramSpina Bifida Program

friday, april 14, 2006

Documents