Rev. 2008; originally published 2000
Cognitive abilities involve how a person obtains information from the environment, processes and remembers it, and uses it for learning and problem solving.
Information comes primarily from what is heard (i.e., language) and from what is seen (i.e., visual/perceptual material). Cognitive abilities form the “building blocks” of learning academic skills, acquiring new concepts, and understanding social situations.
Children with A-T, just as all children, have variability in their cognitive abilities. There is variability from child to child, and each individual child may have specific areas of strength and weakness. Performances on tests of cognitive abilities have produced results that fall within all ranges of intelligence. However, children with A-T often have a general slowing in their cognitive development and learning ability over time, resulting in lower test scores than those of other children of the same age. As a group, children with A-T do not have a loss of specific cognitive skills as much as they are slower to acquire new knowledge. Thus, with passing time children with A-T may have more problems with academic work. They also may seem to be younger than their age in terms of understanding and interests. This is important to monitor so that academic and social expectations can be modified appropriately and the child receives as much help and support as needed to ensure success. Children often are sensitive to their areas of weakness. Thus, it is particularly important to provide ample opportunity to utilize areas of strength and have positive experiences. Success is crucial for good self-esteem and overall emotional adjustment.
PSYCHOLOGICAL EVALUATION PROCESS
A comprehensive psychological evaluation may be worthwhile to determine the child’s individual profile of strengths and weaknesses. A typical evaluation begins with a parent interview to learn about any concerns or areas of difficulty noted by the family. Information is obtained regarding school performance, information processing skills, social involvements, and adjustment issues. Psychological areas that can be assessed include language reasoning, visual/perceptual problem solving, memory, attention, academic skills, and social/emotional functioning. The child may be given a series of standardized tests to provide information regarding cognitive strengths and weaknesses and to address areas of specific concern. Various tests may be selected or modified to ensure optimal performance. The results of these tests are useful for setting reasonable goals and planning effective interventions. A child’s success is achieved through this combination of appropriate expectations and adequate support.
SCHOOL LEARNING: CLASS SETTING
Children with A-T may require modifications in goals and accommodations in teaching strategies to help them achieve at their optimal capability level. There are many reasons for these needs. Abnormal eye movements impair reading. Tremor and ataxia interfere with writing and use of the computer keyboard. Slurred speech and the increased time that most children with A-T need to process information may give a false impression that the child is unable to answer questions. The energy and attention that are required to maintain sitting posture may detract from the concentration needed for classroom instruction. Superimposed on these difficulties are a slowing in the rate of development of cognitive abilities with age and the accompanying learning problems.
Many children with A-T are able to be successful in a regular class setting with additional support and often with the services of a one-to-one assistant. At times, though, classroom accommodations are not sufficient to allow the child to be competitive with peers and achieve at his or her optimal capability level within the regular class setting full-time. Resource help or other services outside of the class can provide more individualized attention. Some children have had difficulty being successful in a regular setting and have benefited from placement in a special school that can tailor programs more effectively to meet the needs of the child. The emphasis in placement decisions should be on providing an environment that can best promote success in relation to peers and facilitate positive experiences.
As they get older, most children with A-T find that a one-to-one assistant is needed in class. Such help is useful for physical safety concerns and to help with motor-related activities. However, an assistant’s role can be much broader and help the child generally “keep up” with class activities. The assistant can facilitate schoolwork by taking notes or dictation, reading directions, explaining new material, and presenting written information orally. Of course, it is important that the assistant strives to facilitate learning and classroom involvement without simply doing the work for the child. Classroom assistants also can play a central role in reducing demands that contribute to fatigue.
If the child has areas of functioning that fall below age level, it is important to determine his or her current level of understanding. Behavioral expectations, management strategies, directions, explanations, social interactions, and activities in general need to be set accordingly. Academic goals and teaching strategies also need to be determined with respect to a child’s own cognitive profile. It is important to recognize that this profile most likely will change as the child becomes older. Thus, teaching goals and strategies may need to change as well. Goals that are set beyond a child’s capability level will only prove to be frustrating and may contribute to poor self-esteem or loss of motivation. Modifications of goals often can be made successfully within a regular class setting. A good measure of the appropriateness of goals is the success that the child is experiencing in class, regardless of the level of the work.
Information regarding a child’s strengths and weaknesses can be used to determine the type of accommodations needed to ensure optimal learning. For example, although eye movement problems can make the visual tracking of letters and words in reading difficult, some children show strengths in specific types of visual/spatial organization. In this case, the use of certain kinds of demonstrations or visual input can be helpful in accompanying verbal material. In contrast, if the child has difficulty processing spatial information, it can be helpful to accompany visual presentations with language. In other words, the child can benefit from being “talked through” the material. Other typical classroom accommodations include additional time allowances, the use of computers, and oral rather than written testing.
The child may become somewhat slower in grasping new concepts. Thus, the child is able to learn information but may require repetition and the opportunity to go over material several times to fully digest it. Material may need to be rephrased, explained, or presented slowly. It can be helpful to demonstrate new tasks and allow the child to practice several times. Some children particularly have trouble understanding concepts that are abstract and thus can benefit from concrete examples. Often it can be helpful to break down more complex material into smaller units. Not only can this facilitate processing but it also can reduce the chances of becoming overwhelmed. Such techniques also are useful when the child has difficulty organizing and integrating information. It can be beneficial to obtain frequent feedback to ensure that the child has understood the material or whether more help is necessary.
RATE OF PROCESSING INFORMATION
Many children are slow in processing not only new concepts but also information that is familiar to them. They may need additional time in order to answer a problem in school or reply to a comment in a social conversation. It can be frustrating for a child to feel pressured or to be cut off before giving a response. Creating a safe environment in which the child can set the pace is important. This can be accomplished by establishing a method of communication that allows the child to convey when extra time is needed. This can provide the child with more opportunity to be successful and interactive, as well as gain some control over the environment. There are times when a child may need information repeated or elaborated. However, there are other occasions during which the child only needs additional time to process the information, and receiving other help can be interfering. Established cues can help differentiate these situations.
Reading can become very effortful due to difficulty with eye movements. Enlarging print and covering lines above and below can make reading easier. Pointing to individual words can help in following text. Computer technology also is available to read text. Handwriting that is done with a dark pen or marker can be easier to read than that done with pencil. Some children prefer reading material that is presented at eye level, and a reading stand can be beneficial. Other children find it easier to read material that is placed on a flat surface. Large-print “talking” calculators or watches also have been found to be helpful.
These reading aids can be useful, but circumvention strategies become increasingly important as the child becomes older. Although it is important to acquire and maintain reading skills, it is equally important to utilize other avenues for obtaining information, in part to reduce time, fatigue, and frustration. These compensations are especially relevant when the child makes the transition from “learning to read” to “reading to learn.” Reading is a primary avenue for learning in other subject areas, and the child needs to be able to have easy access to such information. It may be more productive to read to the child or use audio books or videos rather than require the child to read long passages. Books-on-tape and CD have become increasingly available and can be used for pleasure and specific interest areas as well as academics. Many children show a relative strength in their ability to listen carefully and obtain information through the auditory modality. Thus, it is good to foster the development of listening skills in young children to help prepare them to use this avenue for learning if needed.
Some children with A-T have trouble with memory and word-finding skills. Specific strategies can be effective in improving the child’s ability to encode and retrieve information. Such techniques could involve using visual imagery, grouping information into smaller units (“chunking”), organizing information into categories, using verbal rehearsal or practice, developing associations using cues of similar meaning or similar sound, using environmental cues and prompts, relating information to previous experience, linking events temporally, concentrating on key aspects of communication, or using multiple modalities when presenting and processing information. If the child has a particular weakness with rote information, such as lists of specific facts, it can be helpful to try to turn such information into organized material that is meaningful to the child.
Fatigue usually is a significant problem for children with A-T and can interfere with learning, as well as social interactions and daily family life. Many activities become increasingly effortful for the child. It is necessary to work around this problem in order to ensure that the child is functioning at his or her optimal level. When setting expectations and planning interventions, attempts need to be made to conserve energy and prevent the child from becoming overly tired. Reducing fatigue can lead to more energy for both productive “work” activities and social “fun” activities. It is important for the child not to use all of his or her energy during school time and thus not be able to participate in activities with family or friends. Involvement in extracurricular and social activities is important to a child’s self-esteem and overall emotional adjustment.
Modifications and accommodations usually are necessary at school. As described, specific techniques can be used in class to help the child perform, and a one-to-one assistant can help to reduce demands and make tasks easier to complete. However, more support often is necessary. Work loads may need to be reduced. For example, assignments can be shortened to ensure that all concepts are sampled but that additional work and repetition are avoided. Also, most children are not required to do homework in addition to classwork. Although it is reasonable to strive for full-time attendance at school, many children have shortened school days and/or periodic work-at-home days or rest days. It is good to have a plan in place so that these arrangements can be made when the child is first observed to need them. Helping the child to recognize his or her energy limitations and to indicate the need for breaks also can increase the child’s control over the environment.
TRANSITIONING FROM HIGH SCHOOL
Assessment of the child’s cognitive profile and educational program also should lead to planning for a successful transition beyond high school. It is appropriate to encourage many young adults with A-T to pursue avocational and vocational goals. Some patients have been successful in college with a program that includes reduced class loads and adequate support to handle the demands. Others have been successful in appropriate job settings. Similarly, some patients have adjusted well to independent living situations outside the family when adequate support has been available.
SOCIAL/EMOTIONAL ADJUSTMENT AND ADJUSTMENT ISSUES
Handling the multiple demands of coping with A-T can be very stressful for children and families and can lead to difficulties with social/emotional adjustment.
Families often adjust very well, but many have reported that counseling services have helped them work through difficult issues. Some families have used counseling regularly for a time to assist in dealing with specific issues and then only on an “as needed” basis or for periodic monitoring. Frequently, concerns need to be revisited at different ages as the child handles “normal” developmental issues within the context of the diagnosis. At times children have difficulty recognizing and expressing their emotions. Further, they may be sensitive to any stress being experienced by other family members and be reluctant to add “problems.” Therapy can help the child understand these emotions and express them appropriately.
Over time, the child with A-T is faced with the loss of certain skills, the loss of independence, and a progressive inability to “keep up” with peers. Handling these losses can be frustrating for the child and impact self-esteem. The child may feel powerless, which can result in reduced motivation and/or depression. Whenever possible, it is good to allow the child the opportunity to make choices and exert control over the environment.
Children may encounter a variety of problems with peer relationships. Children with A-T may be misunderstood, identified as different, subjected to ridicule, or isolated from group activities. Individual friendships may change as children develop and those with A-T become more limited in their ability to participate in certain activities. For example, many parents have reported that children become frustrated and feel “left out” when they can no longer engage in activities such as bicycle-riding with their friends. Symptoms of anxiety or depression, as well as loss of self-esteem, are not uncommon. The dynamics of the entire family unit may change. Many issues must be confronted and can be extremely stressful to the child, siblings, other family members, and close friends. A variety of adjustment problems may become evident as the family works to accept the diagnosis, to adjust to changes in the child’s abilities, and to cope with the implications of the prognosis.
It is helpful to handle such concerns openly but with developmentally appropriate information. Thus, answers to questions need to be tailored to the child’s level of understanding. A young child will be much more concrete than an older child and require different explanations. Also, children at different developmental levels will have different concerns and questions. It can be helpful to allow the child to take the lead in asking questions in order to avoid over-interpreting the depth of meaning intended by the child. Long-range implications of the diagnosis often are more troubling to the parents than the child. This is true particularly for young children, who are more focused on immediate concerns, such as why they require help with a task or why they are not allowed to participate in certain activities with peers. Our experience has shown us that many children with A-T are gratified with small improvements that are made possible with new techniques or aids. They often focus on what they can do rather than what they cannot do. Parents may gain comfort from recognizing the positive effect of concentrating on immediate, concrete matters rather than the long-range, “larger” concerns.
The primary difficulty with the young child is adjusting to “being different” from peers, especially when he or she is not able to engage in usual activities or begins to use adaptive equipment such as a wheelchair. Although frequently it is not possible to locate counselors with experience in treating families with A-T, counselors who are experienced in working with children with other handicaps, such as cerebral palsy, are well trained to deal with such peer-related issues. However, it must be remembered that there is one major difference between A-T and cerebral palsy. The child with A-T will suffer from progressive loss of function; the child with cerebral palsy may or may not achieve new milestones over time but will not lose skills. At times it becomes necessary to discuss the situation openly with classroom peers to ensure that they are aware of the child’s problems and have the opportunity to be supportive.
Older children may well become concerned about issues related to prognosis and the uncertainty that goes with the diagnosis of A-T. Again, symptoms of depression or anxiety are not unusual. Counselors who deal with other life-threatening illnesses, such as cancer, can provide appropriate help in coping with these issues.
In the midst of all of these A-T issues, it is important to remember that children with A-T also will experience “normal” developmental issues, such as separation, independence, and sexuality. Children periodically need to re-negotiate their relationships with parents and strive to develop a strong sense of identity. For some, issues of developing or delayed sexuality become paramount.
Socialization also plays a role in the child’s emotional adjustment. Successful peer relationships can offer stimulation, provide a distraction from problems, enhance self-esteem, and improve the overall quality of life for the child. Thus, it is important to facilitate social interactions and participation in extracurricular group activities. Of course, group activities need to be selected carefully to ensure that they provide adequate opportunity for success and positive experiences. For example, a child with interests that are more similar to those of a younger child may do best in groupings that include some younger children.
Building relationships and experiencing a good social foundation while the child is relatively young may be helpful in maintaining social involvement later. As the child grows older, peers often engage more in activities that are difficult for the child with A-T to manage. New interest areas may need to be developed, and various activities that can offer success and positive involvement need to be explored. For example, a child who can no longer actively participate in a sport could become a manager or assistant. Activities that offer the opportunity to mentor or work with younger children can provide the experience of being a “helper” rather than the one always being “helped.” Such an experience can be extremely gratifying and enhance a child’s confidence and overall self-esteem. The computer can be useful in developing new interests, such as graphic design or chess, and in facilitating socialization. Computers can provide access to games and activities that can be enjoyed with friends. Electronic mail can enhance socialization opportunities and facilitate the development and maintenance of friendships and contacts with family members. Also, the opportunity for children to communicate with other children with A-T can provide a network of support and a sense of relatedness.
Information regarding a child’s profile of cognitive strengths and weaknesses is necessary to develop an educational and social program that will contribute to success. Such information can be helpful in setting appropriate academic goals, developing effective interventions, selecting extracurricular activities, and fostering positive social interactions with family and friends. Appropriate expectations and adequate supports can ensure that a child achieves at his or her optimal capability level and experiences life to the fullest extent possible.
FATIGUE AFFECTS ALL ASPECTS OF FUNCTIONING
Fatigue comes from:
- Visual scanning
- Ability to cope with problems
- Overall emotional well-being
- Completing homework
- Participating in school
- Length of school day
- Involvement in extracurricular activities
- Interactions with friends
- Family activities
- Physical health
READING ACCOMMODATIONS AND COMPENSATIONS
- Additional time allowances
- Enlarged print
- Positioning of text
- Covering lines above and below
- Pointing to words
- Computerized text reader
- Having books read
- Tape-recording material
- Books-on-tape or CD
The information provided on this website should NOT be used as a substitute for seeking professional medical diagnosis, treatment or care. You should not rely on any information in these pages to replace consultations with qualified health professionals