Spina Bifida

2001 Linda Cox

Definition

Spina Bifida means cleft spine, and it is the most common neutral tube defect.  Spina Bifida results from the failure of the spine to close properly during the first month of pregnancy. In severe cases, the spinal cord protrudes through the back and may be covered by skin or a thin membrane.

Background

The exact cause of spina bifida are unknown at this stage, but are thought to be connected with both genetic and environmental factors. Therefore if there is a history of neural tube defect on either side of the family there is an increased risk of it occurring again. (SBA, 1998)   At present there is ongoing research into the environmental factors (dioxin as a contaminant of Agent Orange and other herbicides) which affected war veterans in Vietnam and the present genetic factors posed to their children. (VVAA – online, 2001)

Research has shown that taking folic acid for one month prior to conception and for the first 3 months of pregnancy can give up to 72% protection against neural tube defects. (Span – online, 2001)

Types and Characteristics

Spina Bifida Occulta

With Spina Bifida Occulta there is no opening in the back, but the outer parts of some of the vertebrae are not completely closed, thus the spinal cord and coverings are undamaged and there is usually no nerve damage. (SBA, 1998)  A common characteristic of this form of Spina Bifida may be hair or a dimple on the lower back, at the site.

The Spina Bifida Association of Western Australia (1998) suggests that as many as one in ten people may have this form of Spina Bifida, therefore it is the most common form.

The difficulties that may be associated with Spina Bifida Occulta may include foot deformity, weakness and reduced sensation of the legs, change in hand function, bladder infections and incontinence and bowel problems, however, many that suffer from this disorder show not apparent complications. (Span – online, 2001)

Spina Bifida Cystica – Meningocele

This form of Spina Bifida is the most uncommon of the three types.

The outer parts of some of the vertebrae are missing and the meninges are damaged and pushed out through the opening, appearing as a sac. The sac consists of Cerebrospinal fluid. (SBA, 1998)

Spina Bifida Cystica – Myelomeningocele.

This is the most serious and severe form of Spina Bifida due to a sac appearing consisting not only of tissue and cerebrospinal fluid but also nerves and part of the spinal cord. The spinal cord is damaged or not properly developed. As a result, there is always some degree of paralysis and loss of sensation below the damaged vertebrae. The amount of disability depends very much on where the spina bifida is and the amount of nerve damage involved. Many children and adults with this condition experience problems with bowel and bladder control. (Span – online, 2001)

Hydrocephalus

Approximately 80% of children born with spina bifida will develop a condition known as hydrocephalus, or commonly known as ‘water on the brain’.

In a healthy brain a watery fluid, known as cerebrospinal fluid is produced constantly inside each of the four spaces or ventricles inside the brain, flowing through narrow pathways from one ventricle to the next, then out over the outside of the brain and down the spinal cord. The fluid is then absorbed into the bloodstream, and thus the process repeating. However, in a child with Hydrocephalus there may be an obstruction in the narrow pathways and therefore the fluid can not drain properly thus accumulating in the ventricles, causing swelling resulting in compression of surrounding tissue. (SBA, 1998)

Most children with Hydrocephalus require surgical treatment, this consists of having a shunt device inserted into the brain.

What is a Shunt?

A shunt is a device which controls and diverts accumulated cerebrospinal fluid from the obstructed pathways and returns it to the blood stream. The device consists of a system of tubes with a valve to control the rate of drainage and prevent back-flow.

Shunt complications normally are caused by shunt blockage or infection, and rarely by mechanical failure. Complications may include;

  • The tubes becoming too short as the individual grows and therefore an operation to lengthen them may be necessary.
  • Shunt blockage – gradual deterioration in overall performance – symptoms may include headaches, vomiting, fever, drowsiness, vision disturbances or seizures. It is vital that medical assistance is sought urgently if blockage is suspected as the child may only have hours to live.
  • Shunt infection – shunt becomes infected and the lower catheter is often sealed off by tissue. symptoms resemble shunt blockage along with abdominal pains, anaemia and skin rashes.

Learning Difficulties

Many children with spina bifida and/or hydrocephalus have specific learning difficulties, even though most children fall within the normal range of intelligence. (SBA, 1998). It is important for teachers to identify these problems early by watching for restlessness, distractibility and forgetfulness. Difficulties may include;

Attention

Many students with spina bifida appear to be motivated and interested, however they can become easily distracted and therefore find it difficult to sustain their attention on a task until it is completed. Some students may have difficulties identifying the most important aspect of a task and continually react to unessential stimuli. (SBA, 1998)

The Spina Bifida Association of Western Australia (1998) outlines research supporting the belief that a number of students with spina bifida exhibiting distractibility, do in fact have an attention deficit disorder, without hyperactivity.

How can teachers assist with attention problems?

  • Set up a quiet, well organised and clearly structured work environment
  • Seat the child in the least distracting position of the classroom. At the front near the teacher is the most appropriate position.
  • Gain the attention and use eye contact before giving instructions
  • Give clear, simple, step by step instructions, repeating them if necessary, and also checking the child understands what is expected of them.
  • One activity at a time
  • Ensure high demanding activities are spaced with less taxing activities.
  • Ensure adult assistance is available to direct the students back to task if concentration lapses. (SBA, 1998)

Memory and learning

Students with spina bifida may display poor short term memory, and therefore forget instructions that have just been given. They also may have difficulties retrieving the appropriate piece of information from their long-term memory. Visual memory may also be weak and can be affected by visual perceptual deficits – children with this problem may find it difficult copying work from the blackboard, students may only be able to remember one word at a time, and instead of their eye returning automatically where it left off, they need to go back to the beginning each time. Therefore taking the student longer to complete a task. (SBA, 1998)

How can teachers assist with memory and learning?

  • Reduce the amount of information presented at any one time and allowing extra opportunities for consolidation.
  • Emphasising the key points in a logical sequence.
  • Minimising irrelevant information;
  • Giving brief, clear simple instructions to assist children with poor short-term memory.
  • Provide a sloping board or work table where visual memory is a problem
  • Write checklists or pictorial reminders with colour reinforcement;
  • Use students' interests and experiences to reinforce conceptual learning. (SBA, 1998).

Speech

Children with spina bifida frequently develop speech and language skills within normal limits, however some children have poorer receptive language skills than expressive language skills. Children with this problem may also have difficulties understanding of concepts such as beginning and end, under and over, or with time, and there may be difficulties answering problem solving and reasoning questions – the who, what, where, what happens if… questions. (SBA, 1998)

Children experiencing pragmatic difficulties may show reduced eye contact with other, or an inability to remain on topic in a conversation. The child may also have difficulties taking turns, or communicating in a whole group situation. Difficulties in this area can impact on reading skills and comprehension and understanding concepts such as sounds, letters, words, word meanings and sentence structure.

How can teachers assist with receptive language difficulties?

  • Keep instructions short and simple and repeat them if necessary.
  • Use gestures or pointing.
  • Emphasise and repeat key words
  • Break information into units that are easier to remember.
  • Provide visual reminders
  • When asking questions, repeat your request and allow additional time of a response.
  • Relate known information to the unknown.
  • Highlight important information. (SBA, 1998)

How can teacher assist with expressive language difficulties?

  • Use all the senses
  • Continually revise word definitions – explore synonyms and antonyms
  • Expand sentences that the children use.
  • Model complex language using different connectors and adjectives.

Children with speech difficulties should be referred to a speech pathologist. (SBA, 1998)

Perceptual skills

Children with perceptual difficulties take longer and find it harder to carry out certain tasks, for instance they may be slower at making visual judgements, they may take longer to get meaning from pictures or discriminating between shapes. Children may also have difficulties recognising and distinguishing between symbols and may find it even harder to reproduced symbols, such as letters and numbers correctly. (SBA, 1998) Children will also have difficulties with spatial awareness, for example judging size, distance or direction, and their organization of movement-space may be poor.

How can teachers assist with perceptual skill difficulties?

  • Allow extra time for written work
  • Use alternative means of recording – audio tapes
  • Encourage early and frequent use of word processors
  • Simplify worksheets so they are not 'busy’ (SBA, 1998) 

Motor Skill

Children with the serious form of spina bifida have impaired lower limb function, but along with this they also have impaired hand control. Children have problems with lateralization (preferred use of one hand) and the ability to distinguish between left and right may also be delayed.

Children with specific difficulties in sensory or motor organization tend to have poor reasoning skills, along with poor visual-perceptual and visual-motor ability. (SBA, 1998)

How can teachers assist with motor problems

  • Encourage the use of special grips on pencils or chubby pens
  • Encourage correct body posture – this help produce neater work.
  • Use systematic teaching – use colour cues to assist left and right discrimination
  • Observe which hand is preferred and then encourage consistent use of one hand. (SBA, 1998)

Planning and organization skills

Many children with spina bifida and hydrocephalus find it extremely hard to organise themselves, plan ahead and think flexibly. Children may not remember homework or reading tasks, and they may not have appropriate equipment or materials when needed. (SBA, 1998)

Children may also have a lack of understanding when it comes to passage of time, such as no inclination to hurry, or conversely they may become flustered when pressured to hurry. They may not be able to generate problem-solving strategies or alter their approach to problems if their first attempt fails. Some children appear to lack motivation and may wait for someone to tell them what to do – a simple verbal response can prompt them to start a task. (SBA, 1998)

How can teachers assist with planning and organisational difficulties?

  • Break down complex tasks into small manageable steps
  • Play games that encourage the awareness of passing of time.
  • Ensure all necessary books and materials are within easy reach.
  • Encourage children to make a list and keep diaries or a journal.
  • Encourage organised work habits
  • Encourage children to check and proofread their work. (SBA, 1998)

The teacher must realise that a child with spina bifida may have planning and organisational difficulties, it is not laziness on the child’s behalf.

Handwriting difficulties

Students with spina bifida find handwriting a laborious task, with the end product being barely legible. Poor handwriting can stem from poorly developed hand and fine motor control, problems with visual discrimination, and also spatial judgement – both between letters within a word and between words itself. Directional confusion can also hinder letter formation and fluency.(SBA, 1998)

How can teachers assist with handwriting difficulties

  • Systematic teaching of writing skills
  • Concentration of letter formation and the appearance of finished letters
  • Teach directionality of words and sentences
  • Provide the child with a keyboard or typewriter.

Children with severe handwriting problems may be directed to a occupational therapist.

Role of the teacher

Inclusion

The Spina Bifida Association (1998) describes inclusion as ‘the practice of treating all people as part of the whole, regardless of ability, race or religion’. It is important that teachers plan for the inclusion of children with disabilities as they have the same rights as able body children as far as their education is concerned. It is not acceptable for a teacher to disregard a student with a disability and to treat them like babies. The teacher must consider their strengths and weaknesses of each child in their class including able body children and plan for their inclusion in every possible activity and excursion. There is always support available to teachers that have students with disabilities in their classroom and parent communication is one of the most important.

The teacher’s role in supporting a child with Spina Bifida includes;

Toilet routine

For children with incontinence, it is important to speak the their parents and identify exact routines regarding toileting, and therefore it is imperative that the routine the parent has identified be maintained at school.

The vast majority of children with incontinence need to empty their bladder with a catheter on a very regular basis (as stipulated by the parents). These times should not be altered to fit in with lessons or other activities. As this routine often takes ten to fifteen minutes it is vital that the child does not miss out on recess time, as this time is necessary for social interaction, therefore it is the teacher role to organise his/ her timetable to minimise the effects on the child. An ideal time is during directed silent reading (usually after lunch) therefore the child does not miss out on the whole lesson or instructional content. (SBA, 1998).

It is important to note that if a child has incontinence problems there is a need for a teacher assistant to monitor and help in regards to this toileting routine.

School/ playground environment

It is important that the teacher assesses the school playground environment to make sure it is safe and easily accessible for a child in a wheelchair. Do not be shy in asking the child’s parents for their opinions of the playground set up, because they know their child best and their individual capabilities, however, if there is genuine concern about the suitability of the grounds then an occupational therapist may assist the teacher and advise of any adjustments that need to be made. (SBA, 1998).

Classroom Adaptations

It may be necessary to allocate desks, pigeonholes and bag hooks in an appropriate location also taking into consideration height requirements to ensure easy access for the special needs child. Always make sure aisles are clear of books, bags and any other materials that may impede mobility.

Each school should be designed to include children with special needs including those in wheelchairs, therefore doors should be wide enough, door handles, water fountains, sinks and cabinets should be low enough and access to each classroom and toilet should be of ease. It is important however to check the above before the child comes to their first day of school. (Span – online, 2001)

It is vital is the preparation of excursions to keep in mind students with special needs, especially those in wheelchairs. The follow need to be asked before engaging in an excursion.

  • How is the student going to be transported?
  • Will there be a teacher's assistant?
  • Are there ramps or lifts available?
  • Are there suitable toilet facilities?
  • Is the ground surface suitable for wheelchairs? (outside – grass)
  • Can the displays be seen from a wheelchair height?
  • Are there suitable fire escapes for a person in a wheelchair? (SBA, 1998)

It is important that children with spina bifida are not left behind because a venue is not suitable for their them or their wheelchair, in that case the venue should be advised, adjustment should be arranged otherwise a venue change is required for the whole class not just one child.

Benefits to other students

It is vital for the teacher to educate and inform other students about disabilities and the ways in which the class can work together to help children with special needs.

The benefits to able-bodied peers include;

  • More acceptance of individual differences;
  • More awareness of other children’s needs;
  • More comfortable feelings with people with disabilities;
  • More helpfulness in general to other children;
  • Less prejudice about people who are different;
  • More awareness of similarities shared by all people;
  • Enhanced self-esteem; and
  • Acquisition of leadership skills.

Even if there are no special needs students in the class, students can still benefit by going out into the community and helping other children, for instance an excursion can help build the students knowledge of ‘everyone is unique’ and ‘people are similar and different’ and also reinforce the values stated above.

Support

Parents are the best source of information, as they know their own children and the different capabilities they portray. It is important for the teacher to establish a positive relationship with the parents. Parents are interested in their child’s education and will do what ever it takes to help. (SBA, 1998)

Parents may not have a large amount of time to talk to the teacher, due to medical and specialist appointments, work commitments and other daily events therefore one way of communicating with parents without requiring ample time commitment is using a communication book, this goes to and from the classroom teacher and parent and provides reassurance of the child’s progress both at school and in the home.

Classroom activities

  • Allow the other students to use wheelchairs, braces and crutches to experience how it feel to move around in them.
  • Describe the role of the students in supporting an inclusive classroom
  • Discuss the difficulties they experienced when using the wheelchairs, braces or crutches.
child on a swing

Agencies in Western Australia

The Spina Bifida Association of Western Australia - (08) 9389 8311

Princess Margaret Hospital (08) 9340 8222

Rocky Bay (08) 9383 5111

Resource Unit for Children with Special Needs (08) 9221 5616

Disabilities and Learning Difficulties Branch (08) 9426 7111

Commonwealth Department of Health and Family Services (08) 9346 5111

he Disability Services Commission (DSC) (08) 9426 9200

References

Spina Bifida Association of Western Australia. (1998) Building Bridges: Teaching a student with Spina Bifida. Spina Bifida Association of Western Australia; Australia.

Spina Bifida Association of Western Australia. (1998) Building Bridges: Including a person with a disability. Spina Bifida Association of Western Australia; Australia.

Spina Bifida Association of Western Australia. (1998) Building Bridges: Parenting a child with Spina Bifida. Spina Bifida Association of Western Australia; Australia.

Web sites

http://www.span.com.au/

http://www.vvaa.org.au

Back to homepage Back to homepage Back to homepage