1. 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

2. Neural Tube Defect 101 Myelomeningocele - most common, yet most serious type of spina bifida (Lipomyelomeningocele) Myelomeningocele - most common, yet most serious type of spina bifida (Lipomyelomeningocele) Meningocele – rare, requires intervention, typically better outcome Meningocele – rare, requires intervention, typically better outcome Anencephaly – rare, prognosis uniformly grim for those not stillborn Anencephaly – rare, prognosis uniformly grim for those not stillborn Encephalocele – Usually associated with Chiari II malformation, may also occur with a spina bifida Encephalocele – Usually associated with Chiari II malformation, may also occur with a spina bifida

3. Neural Tube Defect 101 Lipoma / Lipomeningocele – A collection of fatty tissue impacting the spina bifida 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 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.

4. Spina Bifida: A Neurosurgical View Closure 24 – 72 hours 80% shunt dependent Hydrocephalus 90 % Arnold Chiari II Malformation Tethered Cord Syringomyelia/Syrinx

5. Spina Bifida: A Urological View Reflux (secondary) Reflux (secondary) Hydronephrosis Hydronephrosis Bladder capacity and post void residuals Bladder capacity and post void residuals Neurogenic Bladder Neurogenic Bladder Kidney / Bladder Infections sensation Kidney / Bladder Infections sensation Kidney / Bladder Stones sensation Kidney / Bladder Stones sensation Sphincter Issues Sphincter Issues

6. Spina Bifida: A Urological View Clean Intermittent Catheterization (80 – 90%) Clean Intermittent Catheterization (80 – 90%) Medications Medications Neurogenic Bowel Management Neurogenic Bowel Management Surgical Interventions Surgical Interventions –Deflux –Bladder Augmentation –Ureteral Reimplant –Urethral Slings / Bladder Neck Suspension –Mitrofanoff –A.C.E. / Cecostomy

7. Spina Bifida: An Orthopedic View Club Feet Club Feet Hip Dislocation / Subluxation Hip Dislocation / Subluxation Fractures and Contractures Fractures and Contractures Spinal Curvatures 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

8. Spina Bifida : A Rehab View Spina Bifida Level Spina Bifida Level –S2/S4 –L5/S1 –L4 –L2/3 Prognosis for Walking Prognosis for Walking Frequently walk w/o aides, may need shoe inserts Usually need short leg braces for foot position and push off. May need crutches or cane. Usually need braces, above or below knee; crutches/cane…some to wheelchair at older age Long leg bracing (thigh/waist) w/ crutches. Often walk for exercise only when older, w/c main mode

9. Spina Bifida : A Rehab View Spina Bifida Level Spina Bifida Level –L1 –T12 and above –Cervical Prognosis for Walking 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) “A RARE BIRD!!!”

10. Spina Bifida : A Rehab View ADL’s ADL’s Bracing / Seating Bracing / Seating Nutrition Nutrition Skin Integrity Skin Integrity Gross Motor Gross Motor Fine Motor Fine Motor Education / Learning Disabilities Education / Learning Disabilities Sensory Impairment Sensory Impairment Motor Impairment Motor Impairment Obesity Obesity

11. Spina Bifida: A General Overview Average lifespan now well into adulthood, “normal or near normal Average lifespan now well into adulthood, “normal or near normal lifespan”. (Children With Spina Bifida, Woodbine House, 1999) 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 I.Q. average range 80 to 90, studies show this may be lowered due shunt infections and multiple revisions Learning, Cognitive & Language Disabilities Learning, Cognitive & Language Disabilities Psychosocial Impact Psychosocial Impact School School Parent: work, family, transport, relationship, finance Parent: work, family, transport, relationship, finance Sexual function Sexual function Latex allergy Latex allergy Transition Programs Transition Programs Healthcare Healthcare Funding Funding Transportation Transportation Employment Employment Mental Health Mental Health

12. 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 health- related 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

13. 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

14. 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

15. Prenatal Visit: A Team Effort!! Referral from OB Services Seen in SB Clinic Introductions to staff, Education & Tour (Clinic/NICU)

16. 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

17. 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

18. Be A Part of OUR TEAM !!! Pre-Natal Referrals 501-364-1806 Spina Bifida Program