Neural Tube Defect A Lifetime of Implications Becky

Neural Tube Defect: A Lifetime of Implications Becky Watkins-Bregy, R. N. , C. Arkansas Children’s Hospital Bifida Program Coordinator Friday, April 14, 2006 Spina

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 – – 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 – S 2/S 4 • Prognosis for Walking Frequently walk w/o aides, may need shoe inserts – L 5/S 1 Usually need short leg braces for foot position and push off. May need crutches or cane. – L 4 Usually need braces, above or below knee; crutches/cane…some to wheelchair at older age – L 2/3 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 – L 1 – T 12 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 • 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, • • 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 • • 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 Qo. L 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 Qo. L 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 Qo. L, the parent’s had a much higher concern on the scale of emotional aspects of Qo. L. 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 HRQo. L. The researcher concluded that there was a high level of satisfaction with HRQo. L 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 • • • 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