CHAPTER 28: NEURAL TUBE

DEFECTS CHILDREN WITH DISABILITIES TEXT PAGES 419-435

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By: Jenna M. Filipone

June 2012

OBJECTIVES… Define the term Neural Tube Defects Identify the causes of NTD and the frequency

that it occurs Understand the effects of meningomyelocele

on the body and the effects on children's physical abilities

Explain the many ways for intervention, ways to provide care & support and ways to help establish independence.

Be aware of the teaching techniques to help guide you in your classroom instruction

NEURAL TUBE DEFECTS NTD are malformations that occur on the spinal

cord, brain and vertebrae. Types and Classifications of NTD

Encephalocel Malformation in the brain causing it to protrude. Side Effects of this NTD include…intellectual disabilities,

fluid in the brain and seizures. Anecephaly:

Cognitive malformation in the brain/skull preventing the neural development.

It is common to see still born and death soon after birth. Spina Bifida

MOST common form of NTD. Malformation that causes a split in the vertebrae arches

seen in either the bone or in the meningeal sac which contains a part of the protruding spinal cord. Occulta is the most benign form of spinda bifida effecting

only 10% of the population. There is no visual signs of this form

TYPES OF SPINA BIFIDA Occult-Spina Dysraphism (OSD):

Abnormality caused by a visual red mark on the lower back which is the result of the spinal cord getting connected to the surface

Meningocele: Characterized by exposing the sac

covering the spinal cord. The spinal cord is NOT trapped and

children with this NTD express no symptoms

Meningomyelocele: Characterized by having an exposed sac

covering the malformation. Side effects include paralysis, sensory

loss, hydrocephalus, and abnormalities of the brain which leading to learning difficulties.

PREVALENCE OF NEURAL TUBE DEFECTS

In the US there is a 60 out of 100,000 chance that a child will be born with Meningomyelocele Spina Bifida.

Caused from a genetic defect in a certain ethnic group however environmental factors, maternal age, and social economic status all have an impact on it.

Decrease due to better testing, prenatal screening, fortified food, and supplements provided.

Still prevalent because the overall survival rate for individuals with Spina Bifida has increased due to improved services and treatment available from them.

THE ORIGIN OF NEURAL TUBE DEFECTS

Defects being 26 days after fertilization of the egg. Central Nervous System is beginning to

develop Malformation occurs in the neural

Causes are both genetic andenvironmental.

Risk Factors include genetic errors,

chromosome disorders, exposures to antiepileptic drugs and acne medication, and excessive uses of alcohol. Maternal issues include exposure to

hyperthermia, having diabetes or being obese.

PREVENTION OF NEURAL TUBE DEFECTS

Folic Acid Supplements Daily supplement of Folic Acid has reduced

new cases of NTD by 50%.

Recommendations take .4 milligrams of folic acid before and after conception

1998 food staples in the united states became fortified with folic acid. Supplements should still be taken

PRENATAL DIAGNOSIS Around 16-18 weeks of pregnancy, it is important to test the levels of

AFP standing for alpha-fetoprotein in the mothers serum. AFP is a chemical found in fetal spine fluid, the brain and spinal cord.

Ultrasound will be given to detect the specific abnormalities of the fetal head and neck

Amniocentesis is preformed testing the levels of AFP and ACH both enzymes specific for NTD.

Chromosomal analysis of the amniotic fluid

If ALL test’s are positive then the parents have two choices to make. Therapeutic abortion Planning for delivery

Chose to deliver in a neonatal intensive care unit so that the back lesion can be closed immediately.

TREATMENT OF MENINGOMYELOCELE

Surgery

Close the opening in the back and a shunt is placed with in the brain to prevent CSF from leaking.

Prevent spinal cord infection

Prevent injury from occurring due to exposed spinal cord and other related exposed structures.

PRIMARY NEUROLOGICAL IMPAIRMENT IN CHILDREN

Chiari Malformations and Hydrocephalus Chiari Type II Malformation

Brain stem and cerebellum get displaced from the skull and end up closer to the neck.

SYMPTOMS include… trouble swallowing, choking, hoarseness, apnea, stiffness in arms, and disordered sleeping to name a few.

Disordered sleep is hard to detect but it can cause children o be tired during the day and effect their abilities to focus in school.

Hydrocephalus 60-95% of children with Meningomyelocele Seen in the central region of the spine and is caused by an error in CSF

alignment resulting in enlarged ventricles located in the brain. Treatment includes a shunt being placed with in the brain to helps drain

the extra fluid found with in the brain to the abdomen. Symptoms of Shunt Failure include…Abnormal head growth, headache, vomiting,

irritability, and lethargy

ANY change in personality, performance in school, or change in physical ability, an immediate evaluation of shunt blockage or failure should be performed.

ASSOCIATED IMPAIRMENTS & MEDICAL COMPLICATIONS

Associated Impairments Cognitive impairments Impairments of fine motor skills Impairments of gross motor skills Visual impairments Seizure disorders

Medical Complications Musculoskeletal abnormalities Spine curvatures/spinal humps Urinary and bowel problems Skin sores Latex allergies Weight/stature abnormalities Sexual issues dysfunction

IMPAIRMENTS OF SPINA BIFIDA

Mobility Impairments Higher the level meningomyelocele the greater the

degree of muscle weakness. Warning signs seeing during infancy

Cognitive Impairments ¾ of children with spina bifida have advanced IQ

scores and only ¼ have mild intellectual impairments.

Impairments are seen with specific skills like perception, organization, attention span, speed of motor response, memory and hand functions. Children usually have a non-verbal learning disability which means they have stronger reading than math skills.

IMPAIRMENTS WITH SPINA BIFIDA CONT.

Seizure Disorders 15% of all children with spina bifida. Seizures, like toxic-conic

seizures Controlled by anti-seizure medication

Visual Impairments 20% of the individuals w Meningomyelocele This is helped with visual corrects.

IMPAIRMENTS AND ABNORMALITIES Musculoskeletal Abnormalities

Deformed joints due to muscle imbalance or total paralysis Feet: Clubfoot which can be gradually straightened during the first 3-

4 months of life. Braces may need to be wore to help maintain proper foot

positioning Hips: Abnormalities caused by muscle imbalance and lack of

movement causing hips to become deformed. Orthopedic treatment Used

Spinal Curvatures and Humps 90% of children with spina bifida have spinal curvature's or

humps. There are 3 types of spinal abnormalities that can be seen. They include scoliosis, kyphosis, and phoscioliosis. Scoliosis: Spinal curvature if left untreated it can cause issues with

sitting, walking and lung functions. Surgery is needed to correct the curvatures in the spine.

Kyphosis: Spinal Hump Surgery can remove the hump once a child reaches school age

Phoscioliosis: This is a combination of both curvature and hump

IMPAIRMENTS AND COMPLICATIONS

Urinary Dysfunction Lack of control of Bladder Functions

Storing urine produced by the kidneys and emptying the bladder once it is full.

Intermittent catheterization (CIC) – used to help drain the bladder. Vesiostomy – used with infants to drain bladder

Bowel Dysfunction Caused by improper function of the kidneys, anus, and lack

of rectal sensation. Constipation followed by intense diarrhea.

Bowel Management 2-3 years old No Bowel Movement = 1. use of daily laxative, 2. fiber supplement,

3. nightly rectal suppository or enemas given Biofeedback Training administered to children who have

slight rectal sensation

IMPAIRMENTS AND COMPLICATIONS

Skin Sores Caused by an inability to feel pain in weight bearing surfaces like feet or buttocks. Rules to Follow:

Always examine the skin regulatory to check for sores, make sure tight shoes or braces are being replaced, avoid hot baths, provide protective footwear to swim with, don’t allow young to crawl on rough or hot surfaces, make sure wheelchair cushions continue to provide protection.

Individuals who are wheelchair bound should modify their wheelchairs with an adaptive seating system so they do not get sores from sitting.

Weight and Stature Abnormalities Weight

1/3 of children are overweight or obese Important for children to continue to participate in physical activity, strength training activities, and aerobic

conditioning activities. Limit the amount of fat and sugar they consume so they can prevent the risk of obesity.

Short statured human growth hormone can be given to select individuals.

Sexual Issues MALES:

¾ of males are able to have an erection but no control 2/3 of males have sufficient sperm in ejaculation permit for fatherhood using IVF or artificial insemination

FEMALES: Decrease in genital sensation and produce less sexually stimulated lubrication during intercourse Normal fertility and if sexually active they should make sure they take the correct safety measures to prevent

against pregnancy and STD’s. Early development in their breast

Pregnancy can pose a threat to females causing urinary tract infections, difficulty delivering vaginally, and hip displacement.

Usually minimal complication are seen

Allergies to Latex 50 % of children have a allergy to latex.

Due to frequent surgeries during childhood. All surgeries, dental work and medical work need to be

done with latex free material Avoid: balloons, rubber balls, Band-Aids and ace bandages

unless noted that they are latex free

Neurological Deterioration Visible or noticeable deterioration- immediate

medical attention should be provided. Evaluated the structure and function of the ventricular

shunt Evaluate the posterior fossa and spinal cord.

**It is important to be proactive with any issues that arise**

HELPFUL INFORMATION

Education Programs Referred to as intervention programs - started when the child in about 6 months old.

Sensory motor assessment evaluating the child’s Range of Motion, Muscle Tone, Sensation, Strength/bulk, and postural control.

Provide adaptive equipment to help the child and families. Psychoeducational test

Identifies the child's strength, needs, and helps develop realistic goals and expectations. Develop an IEP for the child and can allow for the school to provide Physical Therapy.

Psychological Issues Poor self image due to their physical inability, they have low levels of self esteem, poor

peer relationships in school and the community. Teens often are more socially immature, passive, less independent, less physically

active, more socially isolated and have a high then expected rate of depression and developing of learned helplessness.

Provide help to the child by… Being aware of the issues faced by the child, know the strengths and weaknesses of the child, develop strategies for dealing with issues, and provide support to help carry out the strategies

Multimodal approach through counseling, exercise programs, and medication

Interdisciplinary Management Provide therapy to children to correct any secondary emotional or physical problem that

can arise. Joint effort from family, health care provider, school personal Financial counseling- medical expenses can be overwhelming at times.

TEACHERS INFO Create a strong repore

Be an outlet/role model for child

Know Severity of NTD

Modify equipment, rules, activity space and activities.

NO Latex

Be aware of any change in personality, behavior or performance with in classroom, an immediate evaluation of shunt blockage or failure should be performed.

Be Proactive

QUICK SUMMARY A sac protruding from the spine creates the

most complex birth defect compatible with life

Loss of sensation/paralysis is seen below the defect (lesion)

Many disabilities can arise from a NTD

Early action and intervention is KEY!

WHAT HAVE YOU LEARNED…REVIEW

What is NTD? What causes of NTD and the frequency that

it occurs? What are the effects of NTD on the body and

physical abilities? What are ways for intervention, ways to

provide care & support and ways to help establish independence?

What are some things you can do in your classroom to provide support?

REFERENCES Batshaw, M. L., Pellegrino, L., Roizen, N.J. (2007). Children with

Disabilities (6th ed., 419-435). Baltimore, MD: Paul H Brooks

CDC Centers for Disease Control and Prevention. (2012, March 18). Spina Bifida Facts. Atlanta, Georgia. Retrieved July 16, 2012 from http://www.cdc.gov/ncbddd/spinabifida/facts.html

  Cure Byte: Information Bits Assembled Into Cure Bytes for Your Well-

being. 2011, August 22). What Does Neural Tube Defects Look Like? New York, NY. Retrieved July 16th, 2012 from http://trialx.com/curebyte/2011/08/22/what-does-neural-tube- defects-look-like/

Mayo Clinic Foundation for Medical Education and Research. (2012) Riverside Health System: Spina Bifida Retrieved July 16, 2012 from http://www.riversideonline.com/health_reference/Healthy-Baby/DS00417.cfm