For a person with CdLS, being normal may include behaving differently. Why is this true? At a workshop at the 1994 Arizona Convention Dr. Douglas Stockwell answered that question as he briefed parents on behavior patterns and problems normal to children with CdLS.
Dr. Stockwell, a member of the Foundation's Scientific Advisory Council and a neuropsychiatrist from Houston, Texas, listed the suspected causes of behavior variations. First, he described a difference at the biochemical level, the most basic level for persons.
It is believed that the genetic makeup for someone with CdLS is not typical, "We know there may be a genetic difference in a person with CdLS. This could produce a difference in the way the brain actually grows. It is like a 'hard-wired' difference in computer design," he said.
Throughout the workshop Dr. Stockwell compared this biochemical difference to the way a computer's basic components are wired and arranged in a certain unalterable way. Even software will not change that basic design. "In the same way," he explained, "the brain of a person with CdLS may be structured differently from the start." Their hard-wiring, or bio-chemical design, may lead them to behave differently as they grow up.
Children with CdLS May React to Things around them Differently
"Not only are there hard-wired differences," said Dr. Stockwell, "but there are also developmental differences like the software or programming in a computer." Sometimes parents of children with the syndrome are comforted by the thought that their child is different because of something very specific in the brain that cannot be changed. But, that is not the whole story. An interaction occurs between the person's genetic or biochemical makeup and the environment. That interaction can change behavior.
"This is where parenting becomes so important. This is where experience becomes so important," reminded Dr. Stockwell. "The child's interaction with the environment actually builds structure into his or her brain."
Children with CdLS may react to things around them differently. Each time they do so, their brain grows and accommodates those actions or reactions. This is called "learning." The doctor explained, "If we were to use sophisticated brain scanning techniques, we might observe differences in the functioning of parts of the brain of children with CdLS, reflecting their biological and experiential differences."
So, along with the bio-chemical differences come acquired differences, the most obvious one being developmental delay. Persons with CdLS learn and grow more slowly. They may demonstrate observable and measurable delays in their chronological, emotional and functional ages. These delays are accompanied by behaviors that might not show up, apart from the syndrome. Dr. Stockwell pointed to slower development in speech, language and hearing. Pain and other medical problems may also contribute to this delayed rate of development. "It is important to understand your child in terms of what stage (relative age) of development they actually are in," said the doctor. But he warned that "children with CdLS may have additional quirks or peculiarities."
Psychology: The Way a Person's Mind Interprets His or Her World
Sometimes there are atypical psychological behaviors for the person with CdLS. In his explanation Dr. Stockwell defined psychology as "the way a person's mind interprets his or her world." Although not a lot is known about the psychology of CdLS, he pointed to differences here also.
A child with the syndrome experiences the psychological stages of life similar to other children. As all children grow, Dr. Stockwell explained, they develop more and more layers of understanding their world. An early stage occurs when they first become aware of themselves. Another layer is added when children start to interact and compare themselves with their parents or other children. "This is called individuation," he said.
Through individuation people recognize their own identity and personality. When they are able to say "No," they begin to make choices about what they do and don't want to do. This normally begins at one and one half to two years of age. The child with CdLS may become aware over the next few years of differences between him and his siblings or playmates. Physical limitations may generate lots of frustration, even pain.
Puberty: Another Time of Change
During puberty there will be more individuating and more comparison with peers. "This time of change may be a challenge for the child with CdLS and the caregiver," Dr. Stockwell warned. If puberty occurs, a person with less developed social skills will be confronted with all the same hormonal changes as other adolescents yet be ill-equipped to cope with them.
Though weighing only 55 pounds and having a cognitive functioning level of seven or eight years, an adolescent of 12 or 13 may begin to behave differently. The different behavior may signal that this physically small child is going through puberty with all its normal and sometimes uncomfortable emotional and hormonal changes. Most if not all these behaviors are an effort to cope with the feelings and thoughts generated by those changes.
Psychological Issues Associated With CdLS
1. Attention Deficit Hyperactivity Disorder
Dr. Stockwell then identified some psychological problems common to children with CdLS. ADHD (Attention Deficit Hyperactivity Disorder) affects both high and low functioning children at a higher incidence rate than in the population as a whole. More commonly known as hyperactivity, ADHD is a fairly new phenomenon first described in 1970. Children with ADHD are distractable and fidgity; their behavior is unfocused.
Initially depressant drugs were used to control hyperactivity. They did not work. Today other drugs are available that can help children with ADHD. With notable exceptions, children with CdLS who have attention deficit may respond to medicines or treatments like other children do.
2. Obsessive-Compulsive Behavior
Another psychiatric phenomenon observed in some children with CdLS is obsessive-compulsive behavior, a tendency to do things over and over in a driven fashion. In this type of behavior, the child may fight anxiety or discomfort by repetitive thoughts or actions which are comforting in their sameness. Constant repetition may develop an almost permanent functional change in the brain, and obsessive-compulsions are sometimes very hard to eradicate.
Back in the 20's the psychiatrist Freud suggested that people probably seek sameness to fight anxiety. Dr. Stockwell believes likewise. "Our children with CdLS, too, can get locked into a repetitive track to stimulate or calm themselves."
3. Perseveration
Perseveration or pedantic behavior is a common behavior in persons with CdLS. This behavior, typical in mental retardation, is characterized by a person not being able to switch gears or alter the frame of thought. Nothing or no one can distract the individual from what this one has set out to do.
Dr. Stockwell gave this example of perseveration. "A child with CdLS may set out to play a game of cards but not be able to start until the entire deck has been sorted to make sure every card is present. No short cut to this process seems to satisfy the child."
4. Mood Disorders
As children get older they are affected by stress-related mood disorders. Children with CdLS are easily frustrated and this frustration may even show up as depression. According to Dr. Stockwell, depression and the problem of chronic pain go hand in hand. Children who feel stress, pain, rejection or frustration may become depressed. There are medical and psychological treatments for depression. And the good news is that if a medication is tried and the child does not improve, the treatment can be discontinued and the child almost always will not be harmed.
5. Self-Injurious Behavior
Probably the most difficult behavior occurring in persons with CdLS is self-injurious behavior. Commonly known as SIB, this behavior may range from nail biting to breakage of the skin to more severe head banging. A first period of SIB may occur in younger children. Dr. Stockwell believes this SIB may relate to chronic pain.
For example, he described a response common for someone who has severe pain -- joint pain, for instance -- where rubbing the spot produces more pain briefly but ultimately makes it feel better. This is called "gaiting." Gaiting is a way to control pain by producing additional sensation. Some SIB may be an attempt to do this.
Later, in teenage years a second period of SIB may occur which is less explainable. Serious SIB may occur at this point and treatment by a professional is frequently appropriate and necessary.
In summary, children with CdLS behave differently than their nonaffected peers for many reasons. Dr. Stockwell reminded that "they may have differences in their brains, either genetic or ones which develop later." These differences may change the very way they perceive and deal with their world. Also, they may have delays in their maturation as well as physical and emotional development. These delays may lead to psychological issues like easy frustration, depression, aggressiveness or SIB. A parent 's first task is to be reminded of the comparative intellectual, emotional and physiologic ages (often each one different) in their child and to perform the challenging job of relating to the child on the appropriate level in all three of these spheres of life.