1. By Jennifer Ryan Sodexo Dietetic Intern

2. Why Spina Bifida? 1949- Steve Ryan was born with spina bifida 1994- Steve became paralyzed from the waist down Began living in a nursing home 2009- Steve died from “spina bifida complications”

3. What is Spina Bifida? “Split spine” Neural tube defect (NTD) The most common type Tissue that forms into the spinal cord and brain of fetus inside mother’s womb Only takes about one month into pregnancy to form and properly close

5. Types of Spina Bifida 1. Spina bifida occulta 2. Closed neural tube defects 3. Meningocele 4. Myelomeningocele

6. Spina Bifida Occulta Mildest form Few symptoms (“hidden spina bifida”) Small opening in the vertebrae of spinal column but no damage to the spinal cord Affects 10-20% of spina bifida cases Diagnosis: X-ray Signs: dark-colored skin, dimples, or small patches of hair Treatment: None!

7. Closed Neural Tube Defects Diverse group of defects in which the spinal cord is marked by malformations of fat, bone, and meninges Some cases: Can have few to no symptoms Can have bowel/urinary dysfunction due to malformations

8. Meningocele A sac contains some spinal fluid along with coatings that protect the spinal cord that pokes out of the open spine Mild but requires surgery Most rare type Typically minimal disabilities Some with bowel/bladder dysfunction

9. Myelomeningocele Most common and serious type The babies back is exposed -”Open spina bifida” -Increases risk of infection 90% of cases Surgery is necessary

13. Common Complications of Myelomeningocele Progressive tethering- spinal cord becoming abnormally stretched due to the spinal cord wrapping around immovable structures, (vertebrae and membranes) Occurs in 20-50% of cases Causes loss of leg muscle function, control of bladder/bowel movements

14. Common Complications of Myelomeningocele Leg paralysis-loss of muscle function in the legs Due to the nerves of the spinal cord being damaged Severity of paralysis depends on the location of the spinal opening Assistant devices for traveling (crutches, braces, wheelchair) Neurogenic bladder -having no control over the bladder Due to inability to use those specific muscles and nerve damage

15. Myelomeningocele Brain Malformations The only type of spina bifida where brain malformations are common Cerebellum and hindbrain 90% of cases Chiari II malformation- The cerebellum is in an abnormal position Can lead to breathing, eating, and swallowing difficulties

16. Myelomeningocele Brain Malformations Meningitis- inflammation of the membranes or meninges that surround the brain and spinal cord Can be severe and could possibly cause fatal brain injury Hydrocephalus- “water in the brain” Causes increased pressure inside the head due to cerebral spinal fluid buildup in the brain Can cause seizures or mental retardation if left untreated

18. Myelomeningocele Surgical History Early surgeons did not completely understand the disease Trial and error

19. DateSurgeonMethodOutcome 1610Peter Van ForestDrained the fluid- filled sac on a two year olds neck Patient did not survive 1641Nicholas TulpDissection of sacPatient died a few days later from infection 1811Sir Astely CooperSerial tapping to draw off fluid from the sac, and external compression Success! Patient survived 1877James MortonNon invasive treatment: sclerosing injections Sometimes successful 1892C. BayerImproved the method by rotating the skin and flaps in order to perform a closed spina bifida procedure Surgeon Antoine Chipault had successful outcomes using the closure method by forming multiple layers over the open area

20. Diagnosing Spina Bifida Prenatal diagnosis is a commonly used practice In order to detect these defects early Maternal serum alpha fetoprotein (MSAFP) screening Screening for a protein called alpha fetoprotein Elevated levels of this protein in the mother’s blood may point to the child having an open neural tube defect

21. Prenatal Diagnosis Amniocentesis Exam used to diagnose a neural tube defect Fluid from the amniotic sac that surrounds the fetus is tested for elevated levels of alpha fetoprotein Able to diagnose spina bifida, but not the severity of the malformation

22. Postnatal Diagnosis Mild closed spina bifida cases are able to be diagnosed from an X-ray More serious open spina bifida cases are usually apparent just from appearance MRI’s (magnetic resonance imaging) and CT (computed tomography) scans are used in order to see in more detail the deformities and the vertebrae damage

23. Treatment Advancements Shunt: a tool invented in 1952 Drained the fluid from the brain, reducing the pressure in babies with hydrocephalus By the 1990’s, shunts are able to be placed in babies while still in the mother’s womb Risks to mother: weight gain, gestational diabetes, infection Risks to baby: premature delivery

24. The Good News The Bad News The survival rate for spina bifida went up to 90% by the 1990s Between 1995-1996 and 2003-2004, there was a 23% decrease in the occurrence of spina bifida Spina bifida occurred in just 2 out of 1000 births in 2005 in the United States There is no cure for spina bifida The cause is still unknown Damaged nerves cannot be restored, and the nerve tissue cannot be repaired Estimated 7-10% of spina bifida cases diagnosed prenatally are terminated

25. Risk Factors Maternal diabetes Maternal obesity Pesticide exposure Low intake of folic acid Certain drugs with folate antagonist effects Family history of spina bifida

26. Obesity, Diabetes, and NTD’s The etiology of this relationship is not completely understood Studies show obesity affects the level of serum folate Obese women need to consume more folate than the recommended amount to have the same effect

27. Genetics Family history increases risk of spina bifida 3-5% increased risk for a first degree relative having spina bifida 1-2% increased risk for a second degree relative However, the majority of families have just one family member with spina bifida

28. Dioxins Dioxins are made from incomplete burning of hospital or solid waste from byproducts of industrial processes Can end up in meat and dairy foods, and will accumulate in human fat tissue Increases risk of mutations Spina bifida

29. Medications Examples: Methotrexate and Aminopterin They block the absorption of folic acid in the body causing folate deficiency Exposed the relationship between folate and spina bifida in 1950s

30. Inadequate intake/absorption of folic acid Folate is a B vitamin Job: cell division and DNA synthesis Necessary for embryo to properly grow Intervention: In 1998, the Food and Drug Administration (FDA) declared that grain foods and cereals be fortified with folic acid This use of fortifying food and supplements could reduce the risk of neural tube defects by 50-70%.

32. Folate Recommendations For all women able to become pregnant (even if they are not currently planning for pregnancy) is 0.4 mg daily before conception Women with an increased risk of neural tube defects should increase their folate daily intake to 4 to 5 mg starting at least one month prior to conception

33. Ways to Meet Folate Needs 1. Eat foods that are naturally high in folate 2. Folate supplement/multivitamin 3. Folic acid fortification

35. Vitamin B12 Some people are not able to absorb folate effectively leading to malabsorption Vitamin B12 is a vitamin mostly found in animal food sources like meat and dairy products Deficiency is common in vegetarians, the elderly, and is becoming common in women of child-bearing age Deficiency in vitamin B12 affects the metabolism of folate Can increase chances of spina bifida in pregnancies even with adequate folate intake

36. Spina Bifida and Family Studies show families of children with spina bifida: Overall have higher levels of stress due to their child’s condition Typically are close and even resilient Are overprotective

37. Adults with Spina Bifida These common character traits in children with spina bifida often follow them into adulthood Depression and anxiety are common Adults with spina bifida are less likely to go to college compared with other adults 41-49% of spina bifida adults versus 61% of typical developing youth

38. Employment Employment depends on the individual’s situation and physical abilities Research shows the more shunt revisions and the higher the spinal lesion level, the more likely a patient will be on disability or unemployed Studies show young men with spina bifida are more likely to become employed Women were shown to be more independent overall

39. Diet Recommendations for Adults with Spina Bifida No specific diet Healthy balanced eating May need increased protein and fluids if patient has wounds Studies have shown that unhealthy eating habits and physical inactivity are common in young adults with spina bifida Explains common occurrence of obesity

40. Patient Background 40 year old white male 63 inches; 133 pounds BMI: 23.7 Single; lives alone in apartment On disability Admitting diagnosis: “Multiple Deformity”, Colostomy Stage 4 ischial pressure ulcer and a stage 4 sacral pressure ulcer

41. Past Medical History PMH: Spina bifida, bronchitis, urinary tract infections (UTI), neurogenic bladder, self catheters 4 to 5 times per day, along with sacral and ischial wound debridement Surgical history: Surgery of the spine, right hip, numerous wound debridement's Family History: Non- contributory

42. Medical/Surgical Hospital Course Patient’s length of stay: 5 days Pre-operative electrocardiogram (ECHO) Diverting colostomy (secondary to ischial and sacral wounds) Wound debridement of right ishium Placed on a telemetry floor for postoperative management Nasogastric tube insertion Pressure reduction mattress Quick diet advancement to regular diet Home health care for continued colostomy care

43. Nutrition Care Risk assessment: No reason specified Education on colostomy care Low residue/low fiber diet Small frequent meals Unpleasant odor/ “gassy” foods Increased protein education

44. Diet Progression and Nutrition Needs Clear liquids  full liquids  regular house diet Recommended low residue, high protein diet Nutrition Needs Calories: 2135-2440 kcal (35-40 kcal/kg) Protein: 79-91 grams (1.3-1.5 g/kg) Fluids: 2135 ml (35 ml/kg)

45. Abnormal Labs Red Blood Cells- 4.18 (low) Hemoglobin- 11 (low) Hematocrit- 35.2 (low) Carbon dioxide- 21 (low) Glucose- 48 (low) to 193 (high) Calcium- 8.5 (low) Albumin- 3.9 (low) Total protein- 8.3 (high)

46. Medications Heparin Sodium Hydromorphone Oxycodone Ketorolac Zofran Gas-X

47. Resource For more information, go to spinabifidaassociation.org

48. Thank You!