Information and content provided courtesy of

The Children's Tumor Foundation

Ending Neurofibromatosis Through Research

www.ctf.org

US based foundation focussing on research, public education and patient support.

Largest non-government funding source of NF research.

Reviewed and edited by Professor Kathryn North, April 2008

To view the full PDF, please click here.  All material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.

 

 

IMAGINEHOWCONFUSINGITWOULOBEIFEVERYTHINGYOUREADLOOKEDLIKETHIS! ORI FTH EWOR DSBE GINA NDEN DI NPLAC ESTH ATDON’TM AKES ENSET OY OU? TAHW FI EHT SRETTEL EREW DESREVER, or OTU FO ODRER?

 

Now imagine being called on to read aloud and the words seem to dance all over the page:
w r e o n al o pa T oset aelveg e. e ds m c er h h d t

 

What if on top of that people called you lazy, dumb, or retarded, and you know you're not? These are just a few of the difficulties children with learning disabilities experience every day at school.

 

These problems make learning difficult, but NOT impossible! It is important to remember that children with learning disabilities are more LIKE their peers than unlike them. What is different is HOW they learn. Too often children with learning disabilities are accused of not trying hard enough or not paying attention when in reality they are doing the very best they can and working many times longer and harder than their classmates. Most people are surprised to learn that children with learning disabilities have average or above average intelligence, and many are gifted as well. That is difficult to understand.

 

Too often we equate reading and writing ability with intelligence. A child with learning disabilities may have deficits in one or more areas, but may excel in others. No assumptions can be made about a child with learning disabilities. They are fascinating to work with because they will always surprise you! It is important to focus on their strengths and not their disability. They will amaze you with their ability to compensate for their weaknesses.


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Understanding what a learning disability is

    The first step in helping the child with LD is to understand what a learning disability is and how it relates to the learning process. Four steps are required for learning to take place:
     

    1. INPUT (information is entered into the brain via the senses—visual input, auditory input, tactile)
     

    2. INTEGRATION (the information that is received is processed and interpreted)
     

    3. MEMORY (the information must be used or stored and later retrieved)
     

    4. OUTPUT (the information must be sent out through language or motor activities)

     

    A learning disability is a 'short-circuit' or dysfunction in one or several of the channels to the brain. A dysfunction in any step may interfere with subsequent steps in the learning process and may result in a discrepancy between the child's potential ability and his or her academic performance. Any learning task involves more than one process and any learning disability can involve more than one area of dysfunction. For example, a child's visual-perceptual disabilities may likely result in fine/motor and writing difficulties, as well as difficulties with social perceptions.

     

    Disabilities at the Input Stage

     

    During the INPUT stage, a learning disability results when information from the environment is "misperceived." These misperceptions do not pertain to visual or auditory acuity. Thus, a child with perfect vision or hearing may still have a "visual or auditory perceptual disability." It's not "what" you see or hear, but "how" you perceive it. Perceptual disabilities often leave the child feeling confused, anxious and/or frustrated. Self-doubts set in when one cannot trust what he/ she is seeing or hearing. The child whose perceptions are inaccurate, inconsistent, and misleading lives in an unstable and unpredictable world. A tremendous amount of conscious effort is required to override distorted visual and auditory information. And it takes a great deal of persistence and intelligence to overcome them.

     

    Visual Perceptual Disabilities

     

    A child with a Visual Perceptual Disability has difficulty organizing the position and shape of what is seen. The child may:


    1. Reverse or rotate letters, numbers, words, and even sentences when he/she is reading, copying, or writing ("E" is seen as "3"; "w" as "m"; "dog" as "god"; "+" sign as a times sign);


    2. Or the child may have difficulty with figure-ground (focusing on a significant figure instead of the rest of the background) causing him/her to be unable to track left to right, line to line, or to skip words, read the same line twice, see two words as one, one word as two, or skip lines. When doing a math sheet, the child might put the answer under the wrong problem or add part of another problem to the one he/she is doing.

    3. Children with visual perceptual disabilities also may misjudge distance, depth or position in space, bumping into things, falling off their chairs, or knocking things over when reaching for them. These children are often labelled "clumsy" or uncoordinated when the real problem is one of visual perception.

     

    Auditory Perceptual Disabilities

     

    Auditory Perceptual Disabilities are those where the child has:


    1. Difficulty distinguishing the subtle differences in sounds, confusing words that sound alike. The child might answer your question about how he or she is by giving you his/her age.

    2. Or, these children have trouble picking out sounds from the rest of the background (auditory figure ground}. Understanding and following directions, particularly those with several steps, is a strenuous task for children with auditory perceptual difficulties. They are often thought to not be paying attention or listening. Actually, they are paying attention to TOO much!

    3. Children who ask you to repeat questions or directions over and over again may not be able to process the information as fast as most people can (auditory tag}. They "stall" for more time to think about and respond to what they are being asked. Or, they may be only hearing part of what is said.

     

    Social Perceptual Disabilities

     

    Children with a perceptual disability may also misperceive social cues and body language. They may misinterpret gestures, facial expressions, and tone of voice. Or they may not notice them at all. These are the children who go too far and don't know when to stop at home and in the classroom because they do not pick up that someone is annoyed or frustrated with them.

     

    Children with social perceptual disabilities are often shunned by their classmates because of their inappropriate behaviour. They have trouble making and keeping friends, although they desperately want and need others to like and accept them. Without friends, a child feels isolated and many times withdraws from social situations, including school. Social perceptual disabilities are the most devastating type of learning disability a child can have. Many children with LD say, "it is bad enough having to have a learning disability, but the worst thing in the world is not having friends."

     

    Disabilities at the Integration Stage

     

    The next step in the learning process is to put together or process the information that has come in through the senses, i.e. Integration. The information that has been taken in has to be understood before it can be remembered and be useful to the child. There are at least three parts in this step:

     

    Sequencing—organizing information into an order that makes sense; Abstraction—inferring meaning from the words or symbols;

     

    Organization—information must be integrated with new incoming information, and it must also be related to previously learned information.

     

    A child with a sequencing disability might have trouble retelling a story in order, or spell words with all the correct letters, but in the wrong order. He or she may be able to memorize the days of the week or numbers in correct order, but be unable to tell you what comes after Tuesday, or 19, without starting from the beginning.

     

    These children also have a poor concept of time. When a child is unable to understand jokes and humour based on a play of words, he/she is exhibiting an abstraction disability. In a way, the child is thought to be somewhat narrow minded with his/ her understanding of words, particularly those with more than one meaning, as well as concepts.

     

    Many children with learning disabilities have organizational disabilities. These children are able to take in information, such as a series of facts, but are unable to answer questions using the facts. They are unable to pull all the newly learned information and previous information together to make a whole concept. The signs of an organizational disability are clearly evident when one observes the child. His/her desk, notebook, reports, bedroom, etc. are in disarray. These children leave their homework at home or work needed at home at school. Time management is a major issue with these children.

     

    Memory Disabilities

     

    The next step in the Learning process is to take the information that has been received and integrated and store it for later use—in other words; we must remember what we have learned. There are two types of memory—short-term memory and long-term memory.


    Short-term memory has been defined as anywhere from a few minutes to 24 hours and involves retaining information for a short time while attending to and concentrating on it. Long-term memory can be anywhere from a few minutes to over 24 hours. Children with learning disabilities usually don't have much difficulty with long-term memory. If they have learned something well, they most likely retain it, particularly if the information is interesting and meaningful.

     

    Children with learning disabilities have excellent memories when it comes to remembering their past failures! Most likely a memory disability is a short-term one. Children with a short-term memory disability may need 10-15 repetitions to retain what the average child retains after just a few repetitions. Short-term memory disabilities can occur with information received both visually and/or aurally. A child may understand his/her homework until it's time to do it at home. Then he/she can't remember how to do it. These children practice and practice for a spelling test at home, and get them all right, only to flunk the test the next day at school Timed tests, particularly those involving math facts such as multiplication tables, are sheer torture for children with memory problems. It is unreasonable and unfair to put these children under the pressure of having to retrieve information and respond under time constraints. Children with memory problems are often frustrated and tempted to give up. We would be too! Trying to retrieve information you know can be energy and time consuming.

     

    Disabilities at the Output Stage

     

    This final step in the learning process is the proof that we have learned something—output. It involves being able to express in some way what has been learned. Information is expressed either through language—by means of words; or through writing, drawing, gesturing—motor output.

     

    Language Disabilities

     

    There are three forms of language output:


    Spontaneous—where one initiates whatever is said and has the opportunity to select the subject, organize his/her thoughts, and choose the correct words before saying them.

    Demand—a language situation where the child is asked to respond to a question or is required to communicate. It is necessary to simultaneously organize, find the right words, and answer appropriately in a brief amount of time.

     

    For children with a language disability, it is like being in a pressure cooker. Social—social language skills are needed when carrying on a conversation with peers and others, or when asking for help or getting his/her needs met. Language is perhaps the most complex and difficult of all learning tasks. Language disabilities put a child at risk for failure in school, work and social situations. Most children with learning disabilities have problems with "demand language." These are the children who can talk on and on with a great deal of intelligence and expression about a wide range of topics, and then freeze when asked a question. The difference is remarkable.

     

    Children with a "demand language" disability will often mumble, ask you to repeat questions to gain time, or not answer at all. If forced to answer, the response many be so confusing and jumbled that you are not able to understand it. It's hard to believe that this is the same child who was speaking so fluently a moment ago.

     

    Motor Disabilities

     

    Motor Disabilities are those involving coordination of the large muscles (gross motor) and small muscles (fine motor). The child with gross motor difficulties may appear to be clumsy, falls, bumps into things, and has trouble with gym activities. Usually more complex {and more frustrating) are fine motor disabilities. These show up when the child begins to write and has to get the muscles in the dominant hand to work together in a cooperative and coordinated way. Children with a written language disability have slow and poor handwriting. The writing task also requires a tremendous amount of energy and stamina. These are the children with the awkward pencil grip and white knuckles.


    Quite often the child with visual perceptual problems has motor problems as well—referred to as a visual motor disability. If the brain receives information that has been misperceived visually, then incorrectly processes and records it, it may misinform the muscles that require eye-hand coordination. Written Language tasks are made even more difficult because they require using correct grammar, punctuation, spelling, and vocabulary at the same time. Children with learning disabilities who can tell creative, involved, and detailed stories are often unable to get any of their thoughts onto paper. The words are often in the wrong order, usually are misspelled or unintelligible or completely omitted. And they can usually manage to write only a few words or sentences. Writing definitely stifles these creative children. Free them of the burden of writing by allowing them to dictate, tape record or use a word processor to get their thoughts down. They'll be forever grateful!


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Behavioural Characteristics of Children with Learning Disabilities

    Learning is a complex process. A learning disability can occur at any or several of the steps. If what you are seeing or hearing is confusing or distorted, and you cannot trust your brain to understand or store the information you need and then be able to express it, then it's little wonder that you might begin to doubt yourself, or become frustrated! Many of the behavioural characteristics exhibited by children with learning disabilities that interfere with their ability to learn are the result of the confusion and insecurity they feel because of their difficulties.

     

    Some of these behavioural characteristics include:

Impulsivity—the difficulty of controlling impulses. Children with learning disabilities, when faced with uncertain situations, tend to respond quickly without evaluating alternative solutions.

 

Inattention or Short Attention Span—the inability to focus on one activity for reasonable lengths of time.

 

Distractibility—where attention is disturbed by noise, movement, visual stimuli, or one's thoughts.

 

Perseveration—inability to shift easily from one activity to another.

 

Social Misperception—immature or inappropriate responses in social encounters.

 

Inflexibility—overly excited or unsettled by changes in routine. Hyperactivity—unusually high rate of purposeless motor activity.

    Several of the behaviours—hyperactivity, distractibility, and impulsivity—are also characteristic behaviours of children with Attention Deficit Disorder or ADD, with or without hyperactivity. Until October 1991, children with ADD/ADHD were classified under the label "learning disabilities." Now ADD/ ADHD are recognized as being separate and distinct disorders— related to LD but different. Many children with LD also have attention deficit disorder. An even larger number of youngsters with ADD have learning disabilities. It is important to note that not all kids who exhibit hyperactivity, distractibility, and/or impulsivity have ADD. It is important to rule out other conditions that could cause these behaviours, such as stress, anxiety, depression, and learning disabilities, because the treatment for each is very different.


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Learning Disabilities & children & Adolescents with NF1

     This section will explain what we currently understand about LD in children and adolescents with NF1 and give some suggestions to foster healthy development.

     

    VARIATION IN DEVELOPMENT

     

    First, it may be helpful to talk about LD in general.  Some brain functions required for learning include attention, language and memory. These brain functions normally vary, or are different, from person to person. Each of us has patterns of individual strengths and weaknesses. This developmental variation becomes significant when it limits skill development. When brain functions are delayed and skill development is weak, there is developmental dysfunction. When one or more dysfunctions, such as dysfunction in development of motor skills, result in a child's poor performance on a particular type of task, a disability (for example, writing) is described for those particular tasks. A child with disabilities is handicapped when those tasks are critical and the child has no strategies to compensate for the disability.


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Special Education

    SPECIAL EDUCATION In the school system, LD are indicated when a child's academic achievement is significantly below what is expected for his or her intellectual, or cognitive, ability. Other factors, such as many absences in school or need for glasses, must be ruled out as the reason for low achievement. Although your child may have dysfunction in developmental skills that affect learning, he or she may not meet the specific criteria for special education classification of LD in the school system. However, if special education is needed, your child may receive specially designed services through the classification of "Other Health Impairment" because of having NF1.


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Characteristics of Children & Adolescence with NF

    The following is a description of a profile of characteristics we identified in children and adolescents with NF1. Our findings will need to be confirmed by future research. General cognitive (IQ) Average skills are most common. Above average skills do occur. Language Language dysfunction is common but may not be identified. Memory Memory for stories is stronger than memory for pictures, shapes and other visual forms. Attention Further research on attentional skills in NF1 is needed. Visual-perception Average skills for simple visual discrimination and visual perception of figure-ground relationships. Visual-spatial Dysfunction occurs in complex, problem solving for visual-spatial information. Motor Dysfunction is common and may affect development of motor based skills (such as daily living, work or recreational skills) expected for one's age. Visual-motor integration Dysfunction is common and may affect fluency and efficiency of writing, copying and drawing skills. Reading Average skills are common. Math LD are likely to occur. Written expression LD are common but may not be identified. Learning disabilities LD or low academic achievement are more likely found in those students with more than one area of dysfunction in development.


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Problem Behaviour

    Behaviors associated with being anxious or depressed are common in children and adolescents with NF1. Average social skills are common. Psychosocial Within a family, the child with NF1 is likely to be less competent in many skills than a brother or sister who does not have NF1. Social problems are common. Children and adolescents with NF1 often have difficulty being accepted by other children at school and in the community.


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Learning Disabilities

    Developmental dysfunctions and LD occur more frequently in children and adolescents with NF1 than in the general population. Developmental dysfunctions and LD also occur more frequently in children and adolescents with NF1 than in their sisters or brothers who do not have NF1. Our research suggested that more than 50% of children and adolescents with NF1 need special education services. Early research described nonverbal LD as the typical LD of NF1. Current studies suggest the NF1 gene produces broader effects on development. Disabilities are not limited to nonverbal skills. In NF1, dysfunction also occurs in the development of language. Language dysfunction can affect many different skills and lead to language-related learning problems. Here are some of the problems a child with language dysfunction might experience.


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Learning Disabilities

    Weak attention to verbal information Poor listening skills and distractibility in situations such as school or in groups where much talking occurs. Difficulty listening for a long period of time. Weak verbal memory Poor memory for rules of language or sequences of words. Weak understanding of word meanings Limited vocabulary, poor reading comprehension, trouble with word problems in math. Weak interpretation of language Difficulty with multiple meanings of words such as in ambiguity, irony, and metaphor. Weak verbal reasoning Poor verbal problem-solving skills. Poor understanding of language in social situations Trouble with interpreting a person's meaning or intentions in social situations. Trouble using language appropriate to a social situation (verbal social skills). Difficulty distinguishing sounds Problems with sound-symbol association in reading and phonics. Weak word finding Limited vocabulary. Slow word retrieval. Disorganization in communication Problems starting and organizing spoken or written language for describing events or telling stories. Limited speaking or making statements that don't make sense. What about nonverbal LD? Developmental dysfunctions in NF1 may contribute to language based LD, a cluster of nonverbal problems that are part of nonverbal LD, or both. What are nonverbal LD? Although there is no common definition of nonverbal LD, these involve brain functions such as motor, attention and perception that are not language based. Nonverbal skills include what is known as simultaneous processing; that is, the perception of an entire array of visual-spatial information together, at the same time, rather than in a step-by-step manner. Nonverbal LD are not a recognized disability for classification in special education. If your child has characteristics of nonverbal LD, he or she would have to meet other criteria, such as "Other Health Impairment" due to NF1, to gain specially designed services. Our study suggested that children and adolescents with NF1 have some aspects of nonverbal LD but not the full profile of nonverbal LD. A cluster of nonverbal dysfunctions in NF1 was identified. Learning disabilities in math and written language may result from the effects of both nonverbal and verbal dysfunctions. Here are some of the problems a child with NF1 might experience. DYSFUNCTION/DESCRIPTION Weak visual-spatial skills Difficulty with skills of interpreting position or direction and orienting oneself to the surroundings. Weak simultaneous processing of visual-spatial information Difficulty interpreting, organizing or working precisely with spatial information such as of maps, diagrams graphs, and complex charts, music and mathematics. Poor spatial planning and visual organization for writing and drawing and organizing material spatially on a page. Weak visual-motor integration Slow, uncoordinated and imprecise copying, writing numbers and words or drawing. Poor mechanical and construction skills for arts and crafts or for building or fixing things. Poor athletic skills for catching, hitting or kicking a ball.


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What about Attention-Deficit Hyperactivity Disorder (ADHD)?

    To date, firm conclusions about ADHD in NF1 cannot be made. No study has emphasized the comprehensive evaluation of ADHD in children and adolescents with NF1. Future studies will need to consider attentional problems as possibly secondary; that is, problems due to other developmental causes. Although, our study suggested that symptoms of ADHD are found more often in children and adolescents with NF1 than in the general population, future research specific to ADHD is needed. INTERVENTIONS The same recommendations that are made for children and adolescents with the types of developmental dysfunctions and LD described previously should also be made for children and adolescents with NF1. An excellent resource has been written for parents, teachers and others with specific interventions for developmental and learning disorders entitled "Developmental Variation and Learning Disorders."1 More information can be found on page 28. What is different about children or adolescents with NF1 is that they may have BOTH learning and developmental problems PLUS the many physical signs and medical complications of NF1. It is these multiple problems that contribute to the "burden" of NF1 and may lead to anxiety or depression. Because of multiple problems, the child and adolescent with NF1 may be more vulnerable to feeling discouraged. Perhaps the most basic intervention we can offer to each and every child with NF1 is the suggestion that parents, teachers and others develop a hopeful attitude of acceptance and respect for the developing child. Our goal is to promote the child's successes, to foster his or her talents, and to prevent an ongoing sense of inadequacy. Specifically, as child advocates, we want to avoid feeling disappointed by the child's struggles. Most of all, we want to prevent children with NF1 from feeling that they are disappointing their families, teachers or themselves. Here are some specific recommendations for children and adolescents with NF1.
    1. Assessment of Skills The first step in solving problems is to accurately describe the problem. What are your child's problems and weaknesses? What are your child's talents and strengths? A formal assessment of skills can give you this description. Talk to your child's teacher or the school psychologist about psychoeducational assessment in the schools or talk to your child's physician about a developmental evaluation and other available options for this in your community. For children with NF1 we recommend a neurodevelopmental evaluation in the first year of life to allow for early intervention. 2. "Demystification" This is a helpful process of describing your child's strengths and weaknesses clearly, honestly with as little mystery or fantasy as possible. Students with LD frequently do not understand what is wrong with them. A child should know that he or she has a real problem that is specific and can be described. A child needs to know that he or she is not "dumb." Based on an assessment of your child's skills, your observations and knowledge of your child, learn to talk openly to your child about strengths and strengths and weaknesses, without labels or technical jargon, for teachers, family members or other important people in your child's life. Discussions should begin and end positively with frequent references to your child's strengths. An excellent resource for the process of demystification is a readable child's guide describing a variety of learning problems in words that make sense to children is entitled, "All Kinds of Minds: A Young Students Book About Learning Abilities and Learning Disorders."2 More information can be found on page 28. 3. "Bypass Strategies." Teach your child ways to bypass, or get around learning problems. Develop methods in home and school to bypass areas of difficulty for the child. Specific strategies can be included in an intervention plan or an individualized educational plan (IEP) and may involve modifications of expectations, curriculum or procedures. For example, to bypass language dysfunctions, the child can sit close to the teacher in the classroom and directions can be repeated multiple times throughout the day. Visual aids can be provided. In group discussions, the child with language dysfunction can be prompted in advance to allow time for preparation before being called upon; questions can also be asked that only require a yes or no answer. 4. Actively teach problem solving skills. Children and adolescents with NF1 need skills to solve problems. The following set of questions can be used to teach your child a practical approach to solving simple problems of daily living as well as complex life challenges. The best way to teach a new skill is by your own example. STOP-THINK 1. What is the problem? 2. What are ALL the things I could do? 3. What might happen if I do this? What might happen if I do that? 4. Choose the best thing to do and try it. 5. Promote Individual Success. Children need to experience personal accomplishment in at least one area of their own specialty; that is, some special skill that the child practices overtime and in which the child develops proficiency. To learn to strive for excellence, success must be experienced firsthand. Children with LD or developmental dysfunctions may need special attention to ensure that they experience success. Support your child in finding interests for personal accomplishment that he or she will stay with overtime. It is through such practiced accomplishments that respect can be gained from peers, siblings and adults. The child,
    through direct experience of personal mastery, can develop confidence to meet other life goals.

 
   
 

Questions about NF

This booklet is intended to answer your questions about neurofibromatosis (NF). It is published by The Children's Tumor Foundation in response to multitudinous requests from individuals with NF, their families, and the professionals who care for them.  Download the booklet here.

How common is NF2?

Neurofibromatosis type 2 (NF2) is much less common than NF1 with an estimated birth frequency of 1 in 33-40,000. Signs of NF2 usually develop in late adolescence but may not be obvious until adult life. Some people do not develop problems until their 40's and 50's.
 

A guide for Educators

This 12 page booklet is written in question and answer format and provides concise and practical information  about the cognitive, behavioral and physical manifestations of the disorder. It was developed by Dr. Bruce Korf, a neurologist at Harvard Medical School.  Download NF1 Educator's booklet here.

   

(c) Neurofibromatosis Association of Australia, Inc, 2007.  ABN 37 211 072 458     Privacy Policy     |      Terms of Use