Spina Bifida :  An overview of the most common permanently disabling birth defect in the United States Anne Marie Webster,...
Test your knowledge… <ul><li>Pre-test </li></ul>
Question #1 <ul><li>Why is the rate of Spina Bifida and other associated Neural Tube Defects declining?  </li></ul><ul><ul...
Question #2 <ul><li>Taking daily folic acid in a multivitamin before getting pregnant can reduce the chances of having a c...
Question #3 <ul><li>Which of the following are proven risk factors that increase the chance of having a pregnancy with a N...
Question #4 <ul><li>Which of the following is not a typical problem associated with Spina Bifida? </li></ul><ul><ul><li>A....
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...
Question #6 <ul><li>An older individual with Spina Bifida is more at risk for all of the following except? </li></ul><ul><...
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 a...
Spina Bifida Terminology <ul><li>Spina Bifida “split or divided-spine”  and is a general term for several different spinal...
Terminology Continued… <ul><li>Hydrocephalus -excessive spinal fluid in and around the brain </li></ul><ul><li>Syrinx -a f...
How does this relate to me in my job? <ul><li>Yes, Spina Bifida is on the decline, however, people with SB are living long...
Not just the spine…Multisystem Disease <ul><li>Hydrocephalus </li></ul><ul><li>Tethered Cord </li></ul><ul><li>Club feet/l...
Chronic Disease <ul><li>Level of defect often correlates to level of sensation/mobility-not always! </li></ul><ul><li>Symp...
Burden of Disease <ul><li>Many appointments </li></ul><ul><li>Frequent admissions-  average admission 12.5 days </li></ul>...
Things are looking up!!! <ul><li>There are more resources today than ever before! </li></ul><ul><li>CDC is currently doing...
MOMS:  Management of Myelomeningocele Study <ul><li>Funded by the National Institutes of Health, 2003-2010 </li></ul><ul><...
Findings <ul><ul><li>Primary Outcomes: </li></ul></ul><ul><ul><li>Death, placement of a shunt, or the need for a shunt </l...
Findings <ul><li>Prenatal group 68% met one of the primary outcomes (undesirable), 98% of the postnatal group met these </...
Findings Continued <ul><li>Prenatal Complications- (pre/postnatal %) </li></ul><ul><ul><li>preterm delivery (below 37 week...
Take Home Points/Study Limitations <ul><li>Strict exclusion criteria in moms (obesity) </li></ul><ul><li>Experienced cente...
What can I do? <ul><li>Review their medication list thoroughly, make no assumption </li></ul>
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Spina bifida

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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>
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