CHILDREN WITH SHWACHMAN-DIAMOND SYNDROME
At the 2006 SDS Family Conference, Dr. Elizabeth Kerr gave a very
interesting talk focusing on the learning patterns of children (ages
6-17) with Shwachman-Diamond Syndrome. Based on the findings of her
research, Phonological Processing, Visual Processing, Attention, Flexible
Problem Solving, and Behavior were among the types of difficulties
that can be experienced. As such, the following recommendations were
made:
If you notice specific difficulties, the earlier your child receives
support or intervention the better. With a “wait and see”
approach, you may miss many months of valuable support.
Phonological Processing/ Reading/Spelling:
Phonological processing is related to the use of sound structures
in processing written and oral language and involves rapid naming,
phonological awareness (i.e., the sound structures), and phonological
memory. Chronic middle ear infections almost always infer with hearing.
In turn, delays in language development may be evident earlier on
with continued delays in phonological processing. Weaknesses in phonological
processing are related to delays in reading and spelling achievement.
Research at the Hospital for Sick Children and their colleagues (e.g.,
“Putting struggling readers on the PHAST track” m. Lovett
et. al., Journal of Learning Disabilities, 2000 Vol 33 (5), pgs 458-476)
have discovered three essential components to remediation:
 |
Development of pre-requisite skills
which includes letter sounds associations, identification of
sight words, recognition of vowel, variant vowel sounds, and
affixes. These skills are best developed if a specific block
of time is set aside each to focus on them. Direct Instruction
can be extremely beneficial. Lessons are highly structured and
scripted. Responses are modelled for the student. The structure
and repetition of the lessons leads to on-line processing of
information. Teaching is reciprocal, in that, the instructor
is constantly checking in with the student to ensure that he
or she is learning. “Direct Instruction Reading”
by Douglas Carine, Jerry Silbert, and Edward Kameenui (Prentice
Hall, 1996) is one resource. Other suggestions include the grapheme
level activities outlined in “Phonological Awareness Kit-Intermediate”
and activities such as deletions (i.e., Rosner activities) and
substitutions (i.e., Lindamood activities). |
 |
Practice in word identification strategies.
Specific strategies include (a) Rhyming or being taught a list
of relatively simple words containing common spelling patterns
(e.g., look) and then being taught how to compare new, unfamiliar
words to the list (e.g., book, nook, shook, brook, etc); (b)
Vowel alert or being taught that each vowel sounds has a short
and a long sound and how to use different sound to make a real
word (e.g., “I see the vowel ‘o” in this word.
First I’ll try ”o” as in “go”.
Next I’ll try ‘o’ as in “dog”.
That sounds like a read word.”); (c) I Spy or being taught
how to look for small words or parts for words in longer, more
difficult words (e.g., identify ‘bad’, ‘in’,
and ‘on’ in the word “badminton”); and
(d) Peeling off, or being taught how to identify and remove
prefixes and suffixes before using another strategy to read
the root word. |
 |
Use of learning to learn strategies. These
strategies include verbal scripts that an individual can use
when approaching a new task or word. The highly scripted approach
to direct instruction exposes a child to this as do the examples
outlined in number 2 above. |
Visual Processing:
Visual processing is related to the ability to process, interpret,
and organize visual and visual-spatial information. When an individual
has weaknesses in these areas, it can cause difficulties at school
and sometimes in the social realm. For a given individual, they might
display some (but not necessarily all) of the following: poor internal
and external organization, difficulty coping with changes in routine,
difficulty with generalizations, making literal translations, being
overwhelmed, having difficulty with directional concepts and co-ordination,
and being readily distractible.
“The Source for Non-Verbal Learning Disorders” by Sue
Thompson (lingisystems) and “Educational Care: A system for
understanding and helping children with learning problems at home
and in school” by Mel Levine (Educators Publishing Service,
Inc) are two resources which provide information for understanding
and assisting with weaknesses in visual processing. If a child’s
language skills are stronger, then teaching them to put labels on
what they are seeing and doing can help solidify information.
Attention:
Attention is multifaceted. It refers to a number of processes including:
(1) how much information a person can hold in his mind and process
at one time (i.e., attention span and working memory); (2) whether
an individual is able to focus on, or search for, a specified target
(i.e., selective attention); and (3) how well s/he can pay attention
during a mundane or boring task (i.e., sustained attention). A child
who has difficulties with attention may have trouble listening when
someone talks, waiting her or his turn, completing a task, or returning
to a task if interrupted. By the age of 5, a child needs to be able
to pay attention for a least 25 minutes in order to perform adequately
in school.
TeachADHD (www.teachadhd.ca) is a new resource developed by researchers
at the Hospital for Sick Children and their colleagues for use by
teachers at school. It instructs on various types of attention and
helps the teacher develop strategies to harness attention within the
classroom.
At school and at home it will be important to:
 |
Follow a structured daily routine..
School and household routines help the inattentive child to
accept order. Keep the times for wake-up, meals, snacks, chores,
naps, and bed as regular as possible. Try to keep your environment
relatively quiet because this encourages thinking, listening,
and reading at home. In general, leave the radio and TV off.
Predictable daily events help your child’s responses become
more predictable. |
 |
Maintain firm discipline. Children
with attention difficulties need more carefully planned discipline
than the average child. Rules should be formulated mainly to
prevent harm to your child and to others. Aggressive behavior,
such as biting, hitting, and pushing, should be no more accepted
in an inattentive or hyperactive child than in the normal child.
|
At home:
 |
Stretch your child’s attention
span. Encouraging attentive behavior is the key
to preparing your child for school. Increased attention span
and persistence with tasks can be taught at home. Be sure to
praise your child when he plays independently. Set aside several
brief periods each day to teach your child listening skills
by reading to him. Teach games to your child, gradually increasing
the difficulty by starting with building blocks and progressing
to puzzles, dominoes, card games, and dice games. Later, consequence
games such as checkers or tic-tac-toe can be introduced. When
your child becomes restless, stop the activity and return for
another session later. Plan to have your child do homework and
other tasks that require concentration in short blocks of time
with breaks in between. Try having your child study with low-level
background sound such as white noise or instrumental music.
Do homework and studying away from the sounds of television,
radio, or others talking but where adults can supervise. |
Flexible Problem Solving:
This skill typically develops with age and is required more at school
when a child reaches his or her teen years. Flexible problem solving
requires the ability to generate ideas, as well as to maintain and/or
shift thought processes. Individuals with difficulties in this area
may have trouble dealing with ambiguity or getting “unstuck”
from an automatic way of responding.
Teachers and parents can help students in this area by making the
steps involved in an assignment or daily activity more explicit
(e.g., provide “cheat” sheets or templates that outline
the steps required to write the essay, to analyse and solve math
problems etc). Embed questions designed to prompt the individual
into using analytical skills. Questions can include “How did
you solve that problem?” “Can you think of another way
of doing that?”, “What can you do to help remember that
information”. Teach the individual a set of questions to ask
him/herself when confronted with a problem, such as: “What
is my problem? What is my plan? Am I following my plan? How did
I do?”. In other words have the individual identify the problem,
develop a solution strategy, self-monitor his or her performance
and evaluate the outcome.
The Learning Toolbox website (http://coe.jmu.eud/Learn¬ing Toolbox/)
is designed for secondary students with specific learning challenges
(e.g., organization, problem solving, attention etc) as well as
for their teachers and their parents. The student section includes
tools to help the student improve in many areas (e.g. Study skills,
test taking, advanced thinking, organization) by providing templates
to follow.
Behavior:
Children and adolescents who are not doing well in school may not
feel good about themselves. If they feel they can’t cope,
they may withdraw from their friends and social activities. Social
difficulties were raised by parents of children and adolescents
with SDS on ques¬tionnaires. Two resources to foster social
competence are:
 |
“No one to play with: Social problems of
LD and ADD Children” by Betty B. Osman, and |
 |
“Raise Your Child’s Social IQ” by Cathi
Cohen. |
|