An Introduction to Spina Bifida and the Spina Bifida Resource Center

1
An Introduction to Spina Bifida and the Spina
Bifida Resource Center

• Spina Bifida Resource Center
• Regional Center for Independent Living
• 497 State Street
• Rochester, NY 14608
• 585-442-6470
• www.rcil.org/SBRC

2

• Presentation Objectives
• Provide an overview of spina bifida
• Medical and learning issues
• History of Spina Bifida
• Explain the role of the Spina Bifida Resource
  Center

3
What is Spina Bifida?

• Spina bifida is often referred to as
• the most complex birth difference compatible with
  life.

4
What is Spina Bifida?

• Spina bifida is one of a group of congenital
  disorders known as neural tube defects.
• Most common types of NTDs
• Anencephaly
• Encephalocele
• Spina Bifida
• The term spina bifida means open spine

5
Types of Spina Bifida
Myelomeningocele
Occult Spinal Dysraphism
Spina Bifida Occulta
Meningocele
There are many other closely related conditions.
6
Neural Tube Closure
Complete closure 18-25 days of
gestation.
7
Myelomeningocele
8
Prevalence

• 6 out of 10,000 births
• The occurrence of spina bifida has decreased in
  recent years. (Down from 1 out of 1,000 births
  in the 1980s and 90s)
• Lowered incidence thought to be a result of
  various factors, prenatal testing and dietary
  folic acid supplementation.

9
What causes spina bifida?

• Exact cause not certain.
• Thought to be a result of a combination of
  genetic and environmental factors.

10
A word about folic acid

• We know, the vitamin, folic acid can prevent some
  (but not all) cases of spina bifida.
• Estimates of prevention rates are as high as 70.
• 0.4 mg/day , or 4.0 mg/day if there is family
  history.
• Since January 1998 enriched grain products have
  been fortified with some folic acid

11
Medical and Learning Issues

• Some medical issues require long term support
  physical supports.
• Some learning issues can be compensated through
  educational support during the school age years.
• Other learning issues require adapted equipment
  or learning to do something in an alternative
  manner.

12
Related Conditions and Impairments

• Paralysis
• Loss of sensation
• Orthopedics
• Ambulation
• Hydrocephalus
• Chiari II Malformation
• Other structural differences
• in the brain.
• Tethered Spinal Cord
• Bowel Dysfunction
• Neurogenic Bladder Dysfunction
• Respiration/Sleep Apnea

• Seizures
• Vision
• Strabismus
• Visual Perceptual
• Endocrinology
• Obesity
• Latex Allergy
• Skin Breakdown
• / Pressure sores
• Sexuality
• Learning
• Social/Emotional Issues

13
Related Conditions and Impairments

• Paralysis
• Loss of sensation
• Orthopedics-Most have structural and functional
  abnormalities of the musculoskeletal system
• Ambulation
• Level of involvement is often dependent on size
  and location of the lesion

14
Related Conditions and Impairments Hydrocephalus
15
Related Conditions and Impairments Hydrocephalus-
Signs of Shunt Failure

• Infant/Toddler
• Head enlargement
• Soft spot on head is bulging /tense (infant)
• Vomiting
• Irritability
• Lethargy
• Downward displacement of eyes
• Seizures
• High pitched crying
• Children and Adults
• Lethargy
• Headache
• Vomiting
• Irritability
• Change in personality
• Decline in school/work performance
• Increased weakness of the arms of legs

16
Related Conditions and Impairments Chiari II
Malformation

• Symptoms /signs of spinal cord compression
• Difficulty swallowing
• Choking
• Hoarseness
• Breath-holding
• Apnea
• Stiffness in the arms
• Tendency to hold the head arched backward

17
Related Conditions and Impairments Tethered
Spinal Cord

• Signs and symptoms
• Increased weakness in legs or arms
• Change in gait
• Changes in Bowel/bladder function

18
Related Conditions and Impairments

• Other differences in development and structure of
  the brain.
• Bowel Dysfunction
• Neurogenic Bladder Dysfunction
• Respiration/Sleep Apnea
• Seizures (15)
• Vision
• Strabismus- 20 require surgical correction
• Visual Perceptual Difficulty

19
Related Conditions and Impairments

• Endocrinology
• Growth Hormone Deficiency
• Precocious puberty
• Obesity
• Latex Allergy
• Skin Breakdown/ Pressure sores
• Sexuality
• Neurological, psychological and social issues

20
Related Conditions and Impairments Learning

• Learning Disability Unexpected difficulty or
  failure
• Nonverbal Learning Disability Higher verbal IQ
  than Performance IQ scores. Difficulty with gross
  and fine motor skills. Sloppy handwriting.
  Better word reading and spelling than arithmetic
  performance. Misinterprets social cues.
  Impulsive. Limited self-awareness unrealistic
  vocational choices.

21
Related Conditions and Impairments Learning

• Executive Function Deficits Difficulty with
  control processes- planning, prioritizing,
  sequencing, organizing time and space, and task
  completion. Other areas of difficulty include
  self-regulation and cognitive flexibility.

22
Understanding Memory, Attention and Executive
Function

• Memory relates to the past
• Attention relates to the present
• Executive Function relates to the future

Yesterday Memory
Today Attention
Tomorrow Executive Function
23
Related Conditions and Impairments Learning
Common Areas of Difficulty

• http//www.waisman.wisc.edu/rowley/sb-kids/stepha
  nie.jpg

• Math
• Comprehension
• Problem Solving
• Memory
• Attention
• Organization
• Visual-Perceptual Skills
• Fine Motor Skills
• Executive Function
• Perseveration

24
Related Conditions and Impairments Learning
IEP Checklist

• Assessment
• Health Issues
• Mobility/Accessibility
• Safety/Emergency Preparedness
• Social/Emotional Issues
• Related Services
• Daily Living/Self-help skills
• Transportation
• Transition

25
The first step toward understanding spina bifida
is understanding the heterogeneous nature of the
label.

Alex is six years old, he loves anything to do
with sports and airplanes. He walks with forearm
crutches and is starting to insistthat he do
things for himself. He has 2 sisters Bryana 8 and
Emily 4. http//www.waisman.wisc.edu
26
The History of Spina Bifida
27
The History of Spina BifidaFocus on Learning

• 40 years ago emphasis was on survival
• Gradually, care focused on issues of long-term
  health and mobility.
• Parents and teachers began to report difficulties
  with learning. Professionals attributed problems
  to frequent hospitalizations and school absence,
  over-protective parenting, lack of motivation,
  etc. (1970-1995)
• Today, there is widespread agreement that the
  neurological disruption associated with spina
  bifida is the underlying cause of learning
  difficulty.

28
The History of Spina BifidaParent Invented the
Shunt

• John Holter engineer/ machinist
• Son "Casey (1955) born with
• spina bifida and hydrocephalus.
• Holter, along with Philadelphia surgeons,
  designed the shunt.
• Although he was unable to save his son, his
  design, the Spitz-Holter valve/shunt has helped
  millions around the world since the late 1950s.

29
The History of Spina BifidaTulp and Rembrandt

• Professor Nicholas Tulp gave spina bifida its
  name. In his book, Observationes Medicae,
  published in 1641, he included several written
  and sketched (thought to be Rembrandts work)
  descriptions of patients with spina bifida.
• The Anatomy Lesson of Dr Tulp (1632)

30
The Spina Bifida Resource Center

• Mission
• The Spina Bifida Resource Center is dedicated to
  making a positive difference in the lives of all
  individuals with spina bifida and their families,
  especially those living in the Finger Lakes,
  Central, and Western Regions of New York State.
  The SBRC seeks to accomplish this goal through
  educational materials and resources, advocacy,
  outreach services and programs, and collaborative
  efforts.
• Vision
• The Spina Bifida Resource Center is dedicated to
  fostering communities in which
• All individuals with spina bifida are prepared
  and empowered to fully participate in society.
• All individuals with spina bifida have access to
  education, health care and employment options
  that are equitable to those of non-disabled
  people in their community.
• The associated impairments and complications of
  spina bifida are universally understood and
  effectively addressed.

31
The Spina Bifida Resource Center

• The Spina Bifida Resource Center is a program of
  the Regional Center for Independent Living.
• The SBRC is funded by a grant from the Family
  Consumer Council of the Finger Lakes
  Developmental Disabilities Service Office.
• The Spina Bifida Resource Center seeks to
  increase the availability of support services for
  individuals with spina bifida and their families
  by providing consumers with much needed
  information and referral services. While primary
  focus is placed on individuals and families
  living in Monroe County, SBRC programs provide
  benefits (such as a regional conference) that
  reach a wide range of consumers.