Educating the Child with Spina Bifida and Hydrocephalus

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Introduction
Integration of Children with Disabilities
Spina Bifida Today
What are Spina Bifida, Hydrocephalus and the Arnold Chiari Malformation?
Physical and Medical Management
Learning Abilities
In The Classroom
Attention
Language
Memory and Learning
Visuo-Motor Integration Skills
Planning and Organisational Skills
Further Information and Assistance

Introduction
The purpose of this article is to provide essential information to assist educators provide better quality educational programs and general assistance for students with spina bifida and hydrocephalus (SBH). The article contains the following information:

• a brief description of spina bifida and hydrocephalus
• the factors involved in their ongoing management
• detailed information about specific learning difficulties associated with spina bifida and hydrocephalus
• general strategies to address these difficulties that will be of assistance to school communities

Integration of Children with Disabilities
A major review of specialist educational services commissioned in 1982 produced the report: Integration in Victorian Education (1984). The report's recommendations concerned the school community's rights, responsibilities and resources.
All children with disabilities today have the right to attend their local school. To achieve successful educational outcomes for these students, a range of factors need to be addressed by schools. These include employment of integration staff and aides, management of the physical environment and provision of paramedical assistance. In addition, busy staff need access to information and contacts in order to plan and deliver appropriate programs for students with particular disabilities. This article aims to meet this need for staff in their interaction with students with SBH.
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Spina Bifida Today
Spina bifida and cerebral palsy are the most common congenital disabilities in the world. The incidence of spina bifida is approximately one per 1600 births. This means that it is quite likely that a child with SBH will enrol, at some time, in your school.
The last two decades have seen many changes in the treatment and understanding of children born with SBH. The number of children surviving has increased and they are healthier and usually within the normal range of intelligence. Improvements in their physical status and changes in community attitudes have enabled more of these children to attend regular schools. However, most will experience subtle learning difficulties which will require systematic intervention by experienced educators. If these difficulties are not properly addressed, students will be at risk of unnecessary failure in education.
Educators need to be made aware of the medical and cognitive problems that these students may present. Those most likely to impact on educational performance include hydrocephalus, the Arnold Chiari malformation of the lower brain, varying degrees of paralysis to the lower limbs, and bowel and bladder incontinence.
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What are Spina Bifida, Hydrocephalus and the Arnold Chiari Malformation?
Spina bifida is a serious abnormality of the spinal cord. The spinal cord fails to form properly at some point along its length resulting in impaired nerve signals between the brain and the rest of the body below this point. As with other complex defects, there are variations in the severity and the educational consequences of the condition. Of the different types, myelomeningocele, as depicted (see diagram below) is the most common and severest form.

Myelomeningocele

Myelomeningocele is evident at birth as an open cyst or lesion on the back which contains the damaged spinal cord and nerves. The degree of disability varies according to where the lesion occurs. Approximately ninety per cent of children affected have hydrocephalus, the Arnold Chiari malformation, some degree of paralysis of their legs, some degree of incontinence of bowel and bladder, skin sensitivity problems and a variety of orthopaedic abnormalities.
Although a specific cause is not known, research has shown that a complex interaction of genetic and environmental factors contribute to the occurrence of spina bifida. An important recent finding has established that folic acid supplements prior to, and during, pregnancy can significantly help reduce the incidence of this condition.
Hydrocephalus is the accumulation of cerebrospinal fluid (CSF) which is caused by an imbalance in the production and drainage of fluid within the brain. The condition normally requires the surgical insertion of a shunt as shown. A shunt is an internal device which diverts accumulated CSF from the obstructed pathways and allows it to drain. A blocked or infected shunt can relate to physical and cognitive changes in performance at school.

Brain with shunt in place

The Arnold Chiari malformation occurs when the lower part of the brain is positioned abnormally causing hydrocephalus. Many subtle learning difficulties are believed to result from this malformation. In addition, other consequences may be apparent including problems with fine motor skills, visual-spatial perception, auditory processing and breathing.
Further information about these conditions is available in various publications, some of which are listed in the Further Information section of this article.
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Physical and Medical Management
Management of spina bifida depends on its severity. Medical specialists, nurses, physiotherapists, speech and occupational therapists, social workers and neuropsychologists all contribute to manage the condition. As there is damage to the central nervous system, there is currently no cure.
Generally, limitations in mobility can result in poor skin and muscle tone, and weight gain and declining mobility in adolescents.
The child's normal social development may be hampered because of frequent hospital stays, outpatient appointments, and periods in plaster.
The spinal cord: An operation is performed immediately after birth to close the lesion on the back. For the majority of children, no further treatment of the spinal cord is required. For a minority, further complications including spinal curvature and spinal cord adhesion will require additional surgery.
Incontinence: Varying degrees of incontinence are a result of the nerve signals between the brain and bowel/bladder being impaired. Surgery of various types may be helpful in some cases. Bladder management usually involves Clean Intermittent Catheterisation. Initially parents, and then the individuals, learn to insert a catheter to empty the bladder a number of times daily. Social continence is thus achieved.
Bowel management requires additional training and dietary considerations. However, by the time the child reaches the age of seven, incontinence problems are usually under control, although occasional 'accidents' will occur.
Shunt complications: Once inserted, the modern shunt causes few problems. Sometimes, however, a shunt may need to be replaced if it becomes blocked or infected. Symptoms of shunt malfunction are diverse and varying. Early signs can be detected in a gradual deterioration of the child's overall performance. Sometimes, symptoms are severe and include headaches and vomiting. A suspected shunt malfunction should be immediately communicated to parents and requires medical intervention.
Mobility: Weakness and paralysis of the lower limbs is helped by special braces (eg. Ankle Foot Orthosis: AFOs), crutches or wheelchairs. Surgery may be performed to enable functioning muscles to work more effectively. Some children will be involved in ongoing physiotherapy programs to maximise muscles and joint function.
Skin care: Children with spina bifida often have a lack of sensation due to nerve damage and poor circulation. They are prone to injury from prolonged pressure, friction, heat or cold. To prevent sores and burns, conscientious skin care, frequent position changes and careful monitoring of the child's environment is essential.
Testing: Professional neuropsychological assessment of the child to identify visual perception, auditory processing and cognitive differences is also essential in overall management. A range of specific interventions may be required from an early age.
It is important that the school does its best to manage the physical environment for students with SBH. Impaired mobility requires appropriate modifications to buildings and grounds. Incontinence requires access to toilet and washroom facilities which provide additional space, provision for support and privacy.
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Learning Abilities
Although most children with SBH are within the normal range of intelligence, most experience specific learning difficulties. These problems are primarily related to the neurological partners of spina bifida: hydrocephalus and the Arnold Chiari malformation.
The Australian Spina Bifida and Hydrocephalus Association, by providing comprehensive information about the condition and its often hidden manifestations, aims to assist educators provide carefully targeted educational programs for students with SBH. In the past these students were sometimes labeled incorrectly as lazy, careless or clumsy.
Teaching a student with SBH requires all the normal teaching skills. Teachers must possess good communication skills and a willingness to work as a team with parents, therapists and students. They must also have the ability to use cooperative learning techniques and to modify teaching strategies and curriculum where necessary.
The recognition of problems and a fulfilling educational experience for these children will involve:
Observation: Parents are often the most skilled in this as they spend most time with their children. It is essential that parent's observations are noted and considered.
Specialist Medical and Therapy Reports: These may reveal certain problematic areas and, therefore, need to be carefully read and analysed with regard to the impacts at school and home.
Discussion: Difficulties may be revealed or identified by parents and professionals talking together. Discussions should include doctors, therapists, psychologists, other teachers and other professionals involved with the child's wellbeing.
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In The Classroom

• John does not remember today what he appeared to know last week.
• Susie talks a lot but is often off the point. She does not listen effectively and the other students find this irritating.
• Toby seems interested and starts tasks willingly, but rarely completes anything. His writing is awful.
• Peter works well when the teacher or aide sits beside him but he gets distracted and produces little if left to work on his own.
• Melissa forgets due dates for work requirements.
• Sean finds it difficult to grasp fundamental mathematical and spatial concepts.
• George can learn effectively but this often takes longer than his peers.

The above statements often apply to students with SBH. Although they may sound like difficulties that can affect any student every once in a while, it must be realised that for SBH students these problems are pathological in origin and need to be addressed accordingly.
The problems can be grouped under the following headings: attention, language, memory and learning, visuo-motor integration skills, planning and organisational skills. What follows is a short description of how they manifest and some recommended strategies for educators when confronted with them. Remember that though most students with SBH will exhibit similar learning difficulties, the range and their severity in individuals will vary widely. The teacher's knowledge and experience of their students will be the best guide to the distinctive pattern of cognitive strengths and weaknesses exhibited by individual students.
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Attention
Most SBH students appear interested and motivated to learn. However they are easily distracted and find it difficult to sustain attention until the completion of work. Some have difficulty identifying the most salient aspect of a task and focusing their attention. Instead they tend to get distracted to less relevant aspects.
Students with attention problems usually function best when:

• the work environment is quiet, well organised and clearly structured;
• a single activity is set and competing distractions are minimised;
• high demand working periods are brief and interspersed with more relaxing activities;
• instructions are clear and step by step and repeated when required;
• adult assistance is available to redirect the student after lapses in concentration.

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Language
Students with SBH are often described as sociable and talkative with good vocabulary skills. However, they may have difficulty monitoring what they say for logic, relevance or appropriateness. This poor comprehension may be difficult to identify when associated with articulate presentation. Some 'over talk', perhaps to compensate for their limited mobility or to conceal their inability to do what is asked, may be evident.
Teachers can help by:

• encouraging the student to use language for communication of meaning rather than only to manipulate others or to conceal areas of difficulties;
• insisting that the student maintains a shared topic of conversation and redirecting them if they wander;
• routinely checking the student's understanding of the language they are using (for example, by asking the student to paraphrase what is said to them, particularly instructions).

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Memory and Learning
Immediate memory for auditory/verbal information may be intact, i.e. the student has an age appropriate capacity to remember instructions or explanations immediately after they are given. However, there is a rapid loss of information over time and difficulty in retrieving the appropriate bit of information from long term memory when it is needed. Visual memory is weak and concepts grasped last week are later lost. Students with SBH can learn effectively however often take longer to learn and struggle with abstract concepts, for example, mathematics. During bad periods they may appear lazy or 'inert' and simply cannot function at the level they are capable of.

Students with memory learning deficit are helped by:

• reducing the amount of information presented at one time and allowing extra opportunity for rehearsal;
• emphasising key points in a logical sequence - information is remembered most effectively when it is processed in an organised and logical way. Extraneous information should be minimised;
• reinforcing conceptual learning through practical activities related to the student's interests and life experience.

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Visuo-Motor Integration Skills
Most students with SBH have difficulty with tasks requiring eye-hand coordination and motor planning skills. They may have difficulty with accurately interpreting what they see in terms of shape, size, space, distance and then correctly matching their movements (gross or fine). Some students may experience confusion differentiating between left and right. Complaints about slow and untidy handwriting are common and written tasks are often not fully completed.
Assistance can be given by:

• allowing extra time for written work or assignments;
• providing alternatives, for example using an audio tape for creating writing activities;
• encouraging early and frequent use of a word processor for the presentation of written work;
• providing activities which allow the above skills to be practised.

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Planning and Organisational Skills
Most students with SBH find it hard to organise themselves, plan ahead and think flexibly. In addition, some may experience difficulty in understanding the passage of time or understand when matters are urgent. They may be unable to generate strategies for solving problems or to alter their approach if the first attempt is unsuccessful. They seem lost when confronted by a novel or multi-staged task and their work output falls off when they are expected to work independently.
Teachers can help by:

• breaking down complex tasks (eg. assignments, projects) into smaller steps, helping the student generate a plan of approach before they commence, reviewing progress after each component step has been completed and, in brief, providing signposts to guide the student's progress;
• encouraging organised work habits, eg. set homework times, the use of a diary, focusing on time management, use of written or pictorial check lists, use of colour coding to assist planning;
• encouraging the student to check and proofread their work.

The levels of educational achievement of students with SBH are in a wide range, from completion of university and vocational training programs to non-completion of secondary schooling. Whatever the potential of individual students, through awareness of the specific learning difficulties listed above and consistent strategies to intervene where necessary, educators will assist them reach their full potential.
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Further Information and Assistance
For additional information on the specific learning difficulties associated with SBH and for detailed teaching strategies for primary and secondary levels, you are referred to the following publications:

• Rowley-Kelly & Riegal (eds.), Teaching the Student with Spina Bifida, Paul H. Brookes Publishing Company, Baltimore, 1993.
• Llewellyn, G & Green, L., Living with Spina Bifida - Shared Experiences, Cumberland College of Health Sciences, University of Sydney, 1987.
• Department of Education, Queensland, Children with Special Needs...Spina Bifida, Queensland Department of Education, Division of Special Education, 1982.

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Please visit out contacts page for details of local SBH associations, and our links section for further information.

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