Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Spina bifida


Published on

Published in: Health & Medicine
  • Be the first to comment

Spina bifida

  1. 1. Spina Bifida : An overview of the most common permanently disabling birth defect in the United States Anne Marie Webster, APRN, MSN, CPNP, PMHS
  2. 2. Test your knowledge… <ul><li>Pre-test </li></ul>
  3. 3. Question #1 <ul><li>Why is the rate of Spina Bifida and other associated Neural Tube Defects declining? </li></ul><ul><ul><li>A. Implementation of folic acid fortification in cereals and recommendations for prenatal folic acid supplementation </li></ul></ul><ul><ul><li>B. Early detection of NTDs with elective terminations of pregnancy </li></ul></ul><ul><ul><li>C. Underreporting on birth certificates </li></ul></ul><ul><ul><li>D. All of the above </li></ul></ul>
  4. 4. Question #2 <ul><li>Taking daily folic acid in a multivitamin before getting pregnant can reduce the chances of having a child with Spina Bifida by what percent? </li></ul><ul><ul><li>A. 50 </li></ul></ul><ul><ul><li>B. 90 </li></ul></ul><ul><ul><li>C. 30 </li></ul></ul><ul><ul><li>D. 70 </li></ul></ul>
  5. 5. Question #3 <ul><li>Which of the following are proven risk factors that increase the chance of having a pregnancy with a NTD? </li></ul><ul><ul><li>A. Maternal obesity, smoking, high maternal temperatures early in pregnancy, alcohol abuse </li></ul></ul><ul><ul><li>B. Maternal obesity, anticonvulsant use, high maternal temperatures in early pregnancy, a previous NTD affected pregnancy </li></ul></ul><ul><ul><li>C. Smoking, anticonvulsant use, high maternal temperatures early in pregnancy, Hispanic ethnicity </li></ul></ul><ul><ul><li>D. Maternal obesity, alcohol abuse, smoking, a previous NTD-affected pregnancy </li></ul></ul>
  6. 6. Question #4 <ul><li>Which of the following is not a typical problem associated with Spina Bifida? </li></ul><ul><ul><li>A. Mobility Problems </li></ul></ul><ul><ul><li>B. ADHD </li></ul></ul><ul><ul><li>C. Hearing problems/deafness </li></ul></ul><ul><ul><li>D. Hydrocephalus </li></ul></ul><ul><ul><li>E. Urinary Tract Infections </li></ul></ul>
  7. 7. Question #5 <ul><li>The new fetal surgery for Spina Bifida has been shown to </li></ul><ul><ul><li>A. Reduce the rates of needing a shunt for hydrocephalus </li></ul></ul><ul><ul><li>B. Reverse damage that has already taken place in the spinal cord </li></ul></ul><ul><ul><li>C. Increase overall cognitive scores and intelligence </li></ul></ul><ul><ul><li>D. Decrease mortality rates for mothers and affected infants </li></ul></ul>
  8. 8. Question #6 <ul><li>An older individual with Spina Bifida is more at risk for all of the following except? </li></ul><ul><ul><li>A. Depression </li></ul></ul><ul><ul><li>B. Diabetes </li></ul></ul><ul><ul><li>C. Social Isolation </li></ul></ul><ul><ul><li>D. Decubitus Ulcers </li></ul></ul><ul><ul><li>E. Obesity </li></ul></ul>
  9. 9. Current Statistics <ul><li>One out of 2,500 newborns in the U.S. is born with SB </li></ul><ul><li>About 70,000 persons are in U.S. living with this condition </li></ul><ul><li>Occurs during 3 rd -4 th week of pregnancy before most women know they are pregnant </li></ul><ul><li>400 mcg Folic Acid EVERY DAY to help prevent-4,000mcg if have had previous NTD </li></ul>
  10. 10. Spina Bifida Terminology <ul><li>Spina Bifida “split or divided-spine” and is a general term for several different spinal cord defects and is a type of Neural Tube Defect (NTD) </li></ul><ul><li>Chiari II Malformation- abnormal development of lower part of brain (hindbrain)-can cause cervical cord dysfunction </li></ul><ul><li>Meninges -protective cover of the spinal cord </li></ul><ul><li>Meningocele -meninges protrude through spinal column </li></ul><ul><li>Myelomeningocele -meninges and spinal nerves protrude through spinal cord opening </li></ul><ul><li>Spina Bifida Occulta -small defect of some of the bones in the spine-no open abnormality at birth, usually asymptomatic </li></ul><ul><li>Lipomeningocele/Lipomyelomeningocele -fatty-defect in the spinal cord </li></ul>
  11. 11. Terminology Continued… <ul><li>Hydrocephalus -excessive spinal fluid in and around the brain </li></ul><ul><li>Syrinx -a fluid-filled space in the spinal cord that can cause neurologic symptoms </li></ul><ul><li>Neurogenic Bowel/Bladder -the condition where the bowel/bladder does not empty correctly, usually due to nerve damage and decreased sensation </li></ul>
  12. 12. How does this relate to me in my job? <ul><li>Yes, Spina Bifida is on the decline, however, people with SB are living longer and we are seeing new chronic problems that we have never seen before! </li></ul><ul><li>There are 1500 babies born each year in the United States, the 30 year survival rate is near 90% </li></ul><ul><li>Children with SB ages 1-17 have average medical expenditures 13 times greater than children without Spina Bifida </li></ul>
  13. 13. Not just the spine…Multisystem Disease <ul><li>Hydrocephalus </li></ul><ul><li>Tethered Cord </li></ul><ul><li>Club feet/limb abnormalities </li></ul><ul><li>Skin Ulcerations/breakdown </li></ul><ul><li>Scoliosis/Kyphosis </li></ul><ul><li>Mobility problems </li></ul><ul><li>Neurogenic Bowel </li></ul><ul><li>Neurogenic Bladder </li></ul><ul><li>Learning Disabilities </li></ul><ul><li>ADHD </li></ul><ul><li>Obesity </li></ul><ul><li>Depression </li></ul><ul><li>Decreased abilities for independent ADLs </li></ul>
  14. 14. Chronic Disease <ul><li>Level of defect often correlates to level of sensation/mobility-not always! </li></ul><ul><li>Symptoms progress over time, many surgeries </li></ul><ul><li>Daily maintenance of urine and bowel-CIC and regular Urology appts have decreased renal failure and improved life-span </li></ul><ul><li>Lack of transition and services for adults, many adult providers are not familiar with these needs in adults </li></ul><ul><li>Persons living longer-problems with chronic pain (hardware in back), depression, social-isolation, obesity, osteoporosis </li></ul>
  15. 15. Burden of Disease <ul><li>Many appointments </li></ul><ul><li>Frequent admissions- average admission 12.5 days </li></ul><ul><li>Transportation Issues-adaptive drivers licenses, lifts </li></ul><ul><li>Caregiver Issues- FMLA, daily ADLs, stress, affects on other children </li></ul><ul><li>School Issues- IEP </li></ul><ul><li>Therapists- PT/OT/ST </li></ul><ul><li>Cost of supplies/medications (i.e.. Adult diapers not covered, bowel supplies not covered) </li></ul><ul><li>Advocating for the child/family member </li></ul><ul><li>20% are able to maintain employment full-time as adults </li></ul><ul><li>Lack of support for families </li></ul>
  16. 16. Things are looking up!!! <ul><li>There are more resources today than ever before! </li></ul><ul><li>CDC is currently doing studies to establish a National Spina Bifida Patient Registry and a SB electronic medical record </li></ul><ul><li>CDC is funding 15 states to report birth defects (not SC) </li></ul><ul><li>With technology and routine Urologic care, we can prevent renal complications in most children </li></ul><ul><li>New fetal surgery </li></ul><ul><li>New options for shunts, 3 rd ventriculostomy </li></ul><ul><li>SBAA great resource </li></ul>
  17. 17. MOMS: Management of Myelomeningocele Study <ul><li>Funded by the National Institutes of Health, 2003-2010 </li></ul><ul><li>183 total participants </li></ul><ul><li>Published results 2/2011 </li></ul><ul><li>One group had fetal surgery between 19-25 weeks gestation and were monitored the remainder of the pregnancy; other group had surgery at birth—both groups delivered the infant at the high-risk fetal center </li></ul><ul><li>Exclusion criteria-maternal, fetal, obstetric reasons (obesity, other fetal malformations, hx preterm labor, etc) </li></ul><ul><li>Fetal surgery done at three hospitals, Children's Hospital of Philadelphia, Vanderbilt University Medical Center, and University of California-San Francisco </li></ul><ul><li>The children were examined and tested at 12 and 30 months of age </li></ul>
  18. 18. Findings <ul><ul><li>Primary Outcomes: </li></ul></ul><ul><ul><li>Death, placement of a shunt, or the need for a shunt </li></ul></ul><ul><ul><li>A composite test of mental development and an assessment of motor function </li></ul></ul><ul><ul><li>Secondary Outcomes: </li></ul></ul><ul><ul><li>Neonatal morbidity and mortality </li></ul></ul><ul><ul><li>Surgical/Obstetric complications </li></ul></ul><ul><ul><li>Locomotion capabilities </li></ul></ul><ul><ul><li>Assessment of disability </li></ul></ul>
  19. 19. Findings <ul><li>Prenatal group 68% met one of the primary outcomes (undesirable), 98% of the postnatal group met these </li></ul><ul><li>Less brain stem herniation in prenatal group (64/96%) </li></ul><ul><li>Prenatal 2 or more levels above predicted/postnatal 2 or more levels below in motor function </li></ul><ul><li>Postnatal with higher incidence of tethered cord </li></ul>
  20. 20. Findings Continued <ul><li>Prenatal Complications- (pre/postnatal %) </li></ul><ul><ul><li>preterm delivery (below 37 weeks)- 79%/15% </li></ul></ul><ul><ul><li>placental abruption- (26%/0%) </li></ul></ul><ul><ul><li>spontaneous membrane rupture- (48%/0%) </li></ul></ul><ul><ul><li>Oligohydramnios </li></ul></ul><ul><ul><li>average birth weight- 2383g versus 3039g </li></ul></ul><ul><ul><li>Uterine dehiscence and/or uterine rupture with subsequent pregnancies </li></ul></ul>
  21. 21. Take Home Points/Study Limitations <ul><li>Strict exclusion criteria in moms (obesity) </li></ul><ul><li>Experienced centers doing the fetal and postnatal surgery </li></ul><ul><li>Children have been followed only to 12, 30 months-no long-term data </li></ul><ul><li>No data on Urologic function </li></ul><ul><li>Cognitive scores not significantly different at 30 months, children may require a shunt later in life </li></ul>
  22. 22. What can I do? <ul><li>Review their medication list thoroughly, make no assumption </li></ul>