Dandy-Walker Syndrome By: Amanda - PowerPoint Presentation

Slide1

Dandy-Walker Syndrome

By: Amanda

McMurterSlide2

What is Dandy-Walker Syndrome?

The National Institute of Neurological Disorders and Stroke defines Dandy-Walker Syndrome as: “A congenital brain malformation involving the cerebellum (an area of the back of the brain that coordinates movement_ and the fluid-filled spaces around it” (NINDS).

Also know as Dandy-Walker Malformation (Dandy Walker Malformation)Slide3

History and Diagnosis

First described in 1887 by Sutton (International)

First clinical description described in 1914 by Dandy and

Blackfan

(International)

Current pathogenic model described in 1954 by Benda (International)

Dandy-Walker Syndrome is “diagnosed with the use of ultrasound, CT and MRI (Dandy Walker Malformation)Prenatal diagnosis is possible (Dandy Walker Malformation)The syndrome is usually identified in infancy (Dandy Walker Malformation) “In 10 to 20 percent of people with Dandy-Walker malformation, signs and symptoms of the condition do not appear until late childhood or into adulthood” (Genetics) 

Image from:

http://www.newhealthadvisor.com/Dandy-Walker-Syndrome.htmlSlide4

Key Features of Dandy-Walker Syndrome

Key Features of Dandy-Walker Syndrome include:

“Enlargement of the fourth ventricle … that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord” (NINDS)

“A partial or complete absence of the area of the brain between the two cerebellar hemispheres” (NINDS)

“Cyst formation near the lowest part of the skull” (NINDS)

“An increase in the size and pressure of the fluid spaces surrounding the brain (hydrocephalus) may also be present” (NINDS)Slide5

Symptoms

Early Infancy

Slow motor development (NINDS)

Progressive enlargement of skull (NINDS)

Older Children

Symptoms of increased intracranial pressure due to hydrocephalus including irritability, vomiting (NINDS)

Signs of cerebellar dysfunction including unsteadiness, lack of muscle coordination, jerky movements of the eyes (NINDS)Other SymptomsIncreased head circumference (NINDS)Bulging at the back of the skull (NINDS)Abnormal breathing problems (NINDS)Problems with the nerves that control the eyes, face, and neck (NINDS)

Vision and hearing impairment (Children’s)Slide6

Associated Symptoms and Issues

Hydrocephalus, blockage of normal spinal fluid flow resulting in excessive fluid build up in the brain, is experienced by 20-80% of individuals (Dandy Walker Malformation)

Other brain abnormalities can occur but are less common and include:

“Underdeveloped or absent tissue connecting the left and right halves of the brain” (Genetics)

“A sac like protrusion of the brain through an opening at the back of the skull” (Genetics)

“Failure of some nerve cells to migrate to their proper location in the brain during development” (Genetics)

Dandy-Walker can cause malformations of the heart, face, limbs, fingers and toes (NINDS)“Seizures occur in 15-30% of those affected” (Dandy Walker Malformation)Up to half of affected individuals have intellectual disability that ranges from mild to severe, and those with normal intelligence may have learning disabilities (Genetics)Slide7

Behavioural Characteristics

DWS affects the cerebellum, “the back part of the brain that controls movement, behavior, and cognitive ability” (Children’s)

The brain abnormalities that result from Dandy-Walker can affect mood (Genetics)

Among people who are diagnosed in late childhood or in adulthood some experience “mental and behavioral changes” (Genetics)

Image from:

http://chrissybernal.com/rare-diseasedisorder-day-2014/Slide8

Causes

Dandy-Walker Syndrome “is a brain malformation that occurs during embryonic development of the cerebellum and the 4

th

ventricle” (Dandy Walker Malformation)

There is research which has “found mutations in a few genes that are thought to cause Dandy-Walker malformation, but these mutations account for only a small number of cases” (Genetics)

The syndrome “has also been associated with many chromosomal abnormalities” (Genetics)

DWS “most often occurs in people with trisomy 18 (an extra copy of chromosome 18), but can also occur in people with trisomy 13, trisomy 21, or trisomy 9” (Genetics)DWS may be caused by prenatal exposure to toxins or medications or even viral infections experienced by the mother during pregnancy (Children’s)The syndrome could also “be caused by environmental factors that affect early development before birth” (Genetics)

Mothers with diabetes are “more likely than a healthy mother to have a child with Dandy-Walker” (Genetics)Slide9

Prevalence

There are varied estimates as to the prevalence of Dandy-Walker. I found the following estimates:

1 in 2,500 (Dandy-Walker)

1 in 10,000 to 30,000 newborns (Genetics)

1 per 25,000 to 35,000 live births and affects more females than males (Dandy Walker Malformation)

Image from:

http://dandy-walker.org/Slide10

Prognosis

A diagnosis of Dandy-Walker does not necessarily mean a shortened life span

Life expectancy depends on:

Severity of brain malformation, fluid buildups, and cysts

Existence and severity of other associated symptoms and issues

A diagnosis of Dandy-Walker also does not mean that the child will have cognitive problems

“The effect of Dandy-Walker Syndrome on intellectual development is variable, with some children having normal cognition and others never achieving normal intellectual development” (NINDS)Slide11

Medical Information

Images from:

http://ohiofetalmedicine.org/conditions-we-treat/dandy-walker-syndrome/Slide12

Therapy and Treatment

Treatment is generally focused on the symptoms and associated symptoms and issues (NINDS)

When hydrocephalus occurs, patients undergo

surgery

to insert a shunt (tube) to “redirect the fluid that surrounds the brain and to assist fluid drainage into other parts of the body that can absorb the fluid” (Dandy Walker Malformation)

Shunts will also “reduce pressure inside the skull” (NINDS)

Physical therapy may be required to support motor development (Dandy Walker Malformation)Occupational therapy may be required to “build self-care and mobility skills such as eating, getting dressed, and walking” (Children’s)Specialized education

may be required “for cognitive and learning problems” (Children’s)Speech therapy may be needed to help “speech and language development” (Children’s)

If seizures are experienced by the individual, seizure

medication

may be needed (Children’s)Slide13

Real People Living with Dandy-Walker

Abby Isakson

Click the picture to read Abby’s story

Caleb NeffSlide14

Support for Parents

Circle of Mom’s Community: Moms of Kids with Dandy Walker

http://www.circleofmoms.com/moms-of-kids-with-dandy-walker

Circle of Mom’s is a chat support group where mom’s talk about various issues related to their children. This particular group features discussions between mom’s of children with Dandy Walker

Dandy-Walker Alliance

http://dandy-walker.org/

Committed to supporting individuals and families of Dandy-Walker, connecting individuals and families to support, and researching the syndromeSpina Bifida and Hydrocephalus Association of Ontario

http://www.sbhao.on.ca/Committed to supporting individuals and families of children with hydrocephalus – one of the defining features of Dandy-Walker

Parenting Special Needs Magazine

Article: “What is Dandy-Walker Syndrome?”

https://parentingspecialneeds.org/article/what-is-dandy-walker-syndrome/

The above article outlines DWS in general in layman's termsSlide15

Educational Information

The following six slides contain charts produced by the Dandy-Walker Alliance

The charts were produced based on survey information gathered by the Dandy-Walker Alliance from families of children with Dandy-Walker

The aim of the survey and the resulting charts and article, was to provide parents with a guide on how to prepare the school for their child with Dandy-Walker, and to provide teachers with the kind of information they need to help students succeed

I’ve included these charts because they clearly show how varied educational programming can be for students with Dandy-Walker Slide16

Short and Long Term MemorySlide17

Subject StrugglesSlide18

Learns Best By …Slide19

School Programming TypesSlide20

School ServicesSlide21

Mobility Assistance in SchoolSlide22

Strategies for Teaching Students with DWS

Clear and specific learning objectives (Reynolds 282)

Complex learning objectives should be “broken down into small teachable steps” (Reynolds 282)

Repetition (Mauro)

Information processing can be delayed so allow time for processing (Mauro)

Fine and gross motor skills are affected so integrate technology to reduce stress on fine motor skills, ensure there is ample space in the classroom for the student and any equipment aids to move freely, and be considerate of the fact that the student may tire easily

Potential vision and hearing problems may call for preferential seating, visual cues and instructions, voice amplification technology, noise control Create hands on learning experiences (Dandy-Walker)

Create stable environment to prevent sensory overload and notify student in advance of changes to daily routine (Mauro)Accommodate and modify programming based on individual student needs

Student may require specialized education programs focused on speech, physical, and occupational therapySlide23

Technology

Fine and gross motor skills are affected by DWS so consider integrating technology that helps reduce overuse both these systems

iPads

Dragon

Google Read and Write

Apps, programs, or websites to help with speech and language development

Speech WizardArticulation Scenes Potential hearing loss may require voice amplification technologyVisual impairments may require preferential seating, increased font sizes or contrast, or Braille technology Slide24

Cautions for Educators

Know that every case of Dandy-Walker Syndrome varies significantly and that individual student needs should be taken into consideration

Make sure to do ongoing assessments to ensure strategies, programs, accommodations, and modifications put in place to help the student are working and actually helping the student

Keep open lines of communication with parents or guardians of the student, and other professionals so that all parties can work as a team to help the student succeed

Know the medical background of your student and what to do if there is an emergency (i.e. shunt, seizures, medications, etc.)

Image from:

http://

dandy-walker.org/Slide25

Role of Community Agencies: Supporting Family and School Professionals

Dandy-Walker Alliance

:

http://dandy-walker.org/

Connects individuals and families of Dandy-Walker to local support services, provides pertinent information to educational professionals about DWS, and researches DWS

Spina Bifida and Hydrocephalus Association of Ontario:

http://www.sbhao.on.ca/Provides programs “rooted in the principles of self-help and personal support” to individuals with hydrocephalus and provides support for educators of students with hydrocephalus (Spina) They note they are the “only organization in Ontario addressing the particular needs of our members” (Spina)

Community Living: http://www.communitylivingkingston.org

Provides support and programming to individuals and families with intellectual disabilities so that individuals can “participate fully in our community with dignity and independence” (Community)

Global Genes: Allies in Rare Disease

:

https://globalgenes.org/

Patient advocacy organization which promotes “the needs of the rare disease community” and aims to “eliminate the challenges of rare disease” (Global) Slide26

Specialized Training

Social Skills

Create a classroom atmosphere of respects, acceptance, and inclusion so that individuals with DWS can participate fully in all class and social activities

Foster the development of social skills with specialized social skill training and programs such as Social Stories

Speech and language therapy can build confidence with spoken language

Literacy and Numeracy Training

Provide technology to help student record their thinking and reduce overloading fine and gross motor skillsMake learning hands on whenever possible through manipulative and real life situationsBreak skills down into manageable, teachable chunks

Provide extra support via an EA, SST, or with a special education teacher if necessaryWorkplace PreparationProvide learning opportunities that are based on real life situations whenever possible

More specialized training and programming may come in the form of programming from community agencies, or programming provided by a special education teacher Slide27

How can teachers ensure that a student with this syndrome reaches their full potential?

Actively work to create a respectful, accepting, understanding, and inclusive classroom environment where all students feel cared for, welcome, and safe

Raise awareness of Dandy-Walker Syndrome by discussing it with your class

Consider having the student with DWS talk to the class about their personal experience with DWS

Talk with the student about this first to make sure it is something they are comfortable with

Recognize and understand the individual learning needs of the student with DWS and remember that the needs of one student with DWS may vary significantly from those of another with DWS

Create engaging opportunities for learning that are hands on and based in real life experiencesRecognize and be understanding of the use of fine and gross motor skills and provide technology to support the student to help prevent over working these skills

Create open lines of communication with the student, parents or guardians, and other professionals working with the student and work together as a team to support successSlide28

Discussion Questions

What would you do to create a caring, respectful, inclusive learning environment for a student with Dandy-Walker Syndrome?

What accommodations would help a student with Dandy-Walker be successful?

What types of alternative programs are available in your board that would help a student with Dandy-Walker be successful in school and in life?Slide29

Online Resources for Further Information

Dandy-Walker Alliance:

http://dandy-walker.org/

NORD: National Organization for Rare Disorders:

http://rarediseases.org/rare-diseases/dandy-walker-malformation/

National Institute of Neurological Disorders and Stroke:

http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htmGenetics Home Reference: https://ghr.nlm.nih.gov/condition/dandy-walker-malformation#diagnosisInternational Society for Pediatric Neurosurgery – Dandy-Walker Syndrome in Children:

http://ispn.guide/book/The%20ISPN%20Guide%20to%20Pediatric%20Neurosurgery/Hydrocephalus%20and%20Other%20Anomalies%20of%20CSF%20Circulation%20in%20Children/dandy-walker-syndSlide30

Works Cited

Bernal, Chrissy.

Support Rare Disease Day Image

. 28 Feb. 2014. Web. 31 July 2016. <

http://chrissybernal.com/rare-diseasedisorder-day-2014/

>.

Children’s National. “Pediatric Dandy-Walker Malformation.” Children’s National. Children’s National, 2016. Web. 30 July 2016.“Circle of Mom’s: Moms of Kids With Dandy Walker.” Circle of Mom’s. Circle of Mom’s, 8 June 2009. Web. 30 July 2016. Cole, Eric. “What is Dandy-Walker Syndrome?”

Parenting Special Needs Magazine. Parenting Special Needs. 2016. Web. 30 July 2016.Community Living: Kingston and District. Community Living: Kingston and District. 2016. Web. 30 July 2016.

Dandy-Walker

Alliance

.

Are There Certain Subjects Your Child Struggles With More Than Others? Chart

. 2009.

Dandy-Walker Alliance.

Web. 31 July 2016.

---. Are You Aware of Dandy-Walker? Image

. 2009.

Dandy-Walker Alliance.

Web. 31 July 2016.Slide31

Works Cited Continued

---. Dandy-Walker Alliance. 2009. Web. 30 July 2016.

---.

Does Your Child Attend Chart

.

Dandy-Walker Alliance.

Web. 31 July 2016.---. Does Your Child Have Challenges with any of the Following? Chart. Dandy-Walker Alliance. Web. 31 July 2016.---. Does Your Child Learn Best By Chart. Dandy-Walker Alliance. Web. 31 July 2016.

---. Does Your Child Require Assistance with Mobility Throughout the Day? Chart. Dandy-Walker Alliance.

Web. 31 July 2016.

---.

Prevalence Poster Image

. 2009.

Dandy-Walker Alliance.

Web. 31 July 2016.

---.

What, if any, Services Does your Child Receive in School? Choose all that Apply Chart

.

Dandy-Walker Alliance.

Web. 31 July 2016.

“Dandy Walker Malformation.”

National Organization for Rare Disorders

. National Organization for Rare Disorders. 22 Feb. 2015. Web. 30 July 2016.

DeLuca, Louis.

Abby Isakson in Swing Image

. 2013. Dallas News.

Mail Online

. Web. 30 July 2016.Slide32

Works Cited Continued

Genetics Home Reference

. “Dandy- Walker Malformation.”

Genetics Home Reference

. Genetics Home Reference, 26 July 2016. Web. 30 July 2016.

Gilednc

. “The Dandy-Walker Syndrome Info Video.” Online video clip. YouTube. YouTube. 8 Oct. 2012. Web. 30 July 2016.Global Genes: Allies in Rare Diseases. Global Genes. Web. 30 July 2016.International Society for Pediatric Neurosurgery. The ISPN Guide to Pediatric Neurosurgery: Dandy-Walker Syndrome in Children. Web. 30 July 2016. <

http://ispn.guide/book/The%20ISPN%20Guide%20to%20Pediatric%20Neurosurgery/Hydrocephalus%20and%20Other%20Anomalies%20of%20CSF%20Circulation%20in%20Children/dandy-walker-synd>.

Mauro, Terri. “Preparing the School for Your Child with Dandy-Walker Syndrome.”

Very Well

. Very Well. 30 June 2014. Web. 30 July 2016.

Neff, Caleb. “My Dandy-Walker Syndrome Story (with MY MOM).” Online video clip.

YouTube

. YouTube. 15 Aug. 2013. Web. 30 July 2016. Slide33

Works Cited Continued

“NINDS Dandy-Walker Syndrome Information Page.”

National Institute of Neurological Disorders and Stroke

. National Institute of Neurological Disorders and Stroke. 2 Feb. 2016. Web. 30 July 2016.

Normal and Dandy-Walker Malformation Image

. 2014. New Health Advisor. “Dandy-Walker Syndrome.” Web. 31 July 2016. <

http://www.newhealthadvisor.com/Dandy-Walker-Syndrome.html>.Ohio Fetal Medicine Collaborative. Dandy Walker Brain Image. n.d.

Ohio Fetal Medicine Collaborative. Web. 30 July 2016.---. Normal Brain Image

.

n.d.

Ohio Fetal Medicine Collaborative

. Web. 30 July 2016.

Oller

,

Dr

Carlo. “Dandy Walker.” Online Video clip.

YouTube

. YouTube. 21 Jan. 2012. Web. 31 July 2016.

Quigley, Rachel. “Meet the ‘Warrior Princess’ with Rare Syndrome who is Defying all Expectations After her Parents Were Advised to Terminate.”

Mail Online

. 17 June 2013. Web. 30 July 2016.Slide34

Works Cited Continued

Reynolds, Cecil R. and Elaine Fletcher-Janzen. Eds. “Dandy-Walker Syndrome (DWS).”

Concise Encyclopedia of Special Education: A Reference for the Education of the Handicapped and Other Exceptional Children and Adults

.2

nd

ed. Hoboken: Wiley, 2002. 282-3. Web.

Spina Bifida and Hydrocephalus Association of Ontario. Spina Bifida and Hydrocephalus Association of Ontario. 2011. Web. 30 July 2016.Sturt David, and Todd Nordstrom. Question Marks Image. 2014. Executive Placements: A Staffing Company. “3 Interview Questions to Start Asking Now, and 3 You Should Retire.” Web. 31 July 2015. <

http://www.executiveplacementsofva.com/2016/05/23/3-interview-questions-to-start-asking-now-and-3-you-should-retire/>.