Illness Specific Patterns of Psychological Adjustment

and Cognitive Adaptational Processes in Children

with Cystic Fibrosis and Sickle Cell Disease
Ä

Robert J. Thompson, Jr. and Kathryn E. Gustafson

Duke University Medical Center
Ä

Karen M. Gil
University of North Carolina at Chapel Hill
Ä

Jake Godfrey
Duke University
Ä

Laura M. Bennett Murphy

Otterbein College

Illness-specific patterns of adjustment and cognitive adaptational process

were identified in children (7–12 years of age) with cystic fibrosis (CF, n 5
40) or sickle cell disease (SCD, n 5 40). Anxiety diagnoses were most
frequent for both illness subgroups but children with CF had a higher rate
of oppositional disorder (27.5%) than did children with SCD (2.5%). Sig-
nificant portions of the variance in adjustment were accounted for by
stress appraisal (19%), expectations of efficacy (9%) and health locus of
control (9%) for children with CF and by stress appraisal (21%) and self-
worth (12%) for children with SCD. The interaction of general and specific
illness tasks and adaptational process with developmental tasks in delin-
eating intervention opportunities is discussed. © 1998 John Wiley &
Sons, Inc. J Clin Psychol 54: 121–128, 1998.

Correspondence to Robert J. Thompson, Jr., Ph.D. Box 3362, Duke University Medical Center, Durham, NC 27710.
This research was supported by National Institute of Health Grants HL 37548, HL 28391, and HL 39124.
We gratefully acknowledge the assistant of David A. Johndrow and the staff of the Duke-UNC Sickle Cell Center and
the Cystic Fibrosis Center at Duke University Medical Center and also Rhonda Strickland for manuscript preparation.

JOURNAL OF CLINICAL PSYCHOLOGY, Vol. 54(1), 121–128 (1998)

© 1998 John Wiley & Sons, Inc. CCC 0021-9762/98/010121-08
122 Journal of Clinical Psychology, January 1998

Children with chronic illness have a risk for psychological adjustment problems that is 1.5 to
3 times as high as their healthy peers (Pless, 1984). Theoretically and conceptually driven
research models are now guiding the search for processes that account for the variability in the
psychological adjustment of children with chronic illness (Wallander & Thompson, 1995).
The noncategorical model (Stein & Jessop, 1982) considers psychological adjustment not
as a function of specific chronic illnesses but as a function of common dimensions around
which specific illnesses vary such as visibility, threat to life, and intrusiveness of care routines.
In contrast, the transactional stress and coping model (Thompson, Gil, Burbach, Keith, &
Kinney, 1993; Thompson, Gustafson, George, & Spock, 1994) adopts a modified categorical
approach. In recognition that illnesses differ in the stresses and tasks presented to the child and
family in relation to age of symptom onset, type and severity of symptoms, treatment regimens,
and life expectancy, it is postulated that there may be important differences in the processes
associated with psychological adjustment as a function of illness type (Thompson & Gustafson,
1996). It is argued that investigating illness-specific patterns of adaptation could foster speci-
ficity of intervention programs (Thompson & Gustafson, 1996). Thus, the transactional stress
and coping model includes parameters that are illness specific as well as common demographic
and adaptational parameters that are assessed across illnesses.
In the transactional model, chronic illness is viewed as a potential stressor to which the
individual and family systems endeavor to adapt. The illness-adjustment relationship is a func-
tion of the transactions of illness parameters, demographic parameters, and adaptational pro-
cesses (Thompson & Gustafson, 1996). Illness parameters include type, severity, and age of
onset. Demographic parameters include patient age, gender, and socioeconomic status. Guided
by the work of Lazarus and Folkman (1984), the focus of the model is in the contribution of
processes hypothesized to mediate/moderate the illness–adjustment relationship over and above
the contribution of the illness and demographic parameters which can serve as potential inter-
vention targets. These adaptational processes include cognitive processes of stress appraisal,
expectations of efficacy and health locus of control, self-esteem, and causal attributions; coping
methods; and social support. It is postulated that illness parameters can account for some of the
variance in psychological adjustment and that the pattern of interrelationship among param-
eters, adaptational processes, and adjustment may differ with different illnesses.
The transactional model has been used to delineate the types and frequencies of psycho-
logical adjustment problems experienced by children/adolescents with different chronic ill-
nesses, and by their mothers, the role of the hypothesized adaptational processes in child and
maternal adjustment, and the change in adjustment, adaptational processes, and their interrela-
tionship over time (see Thompson & Gustafson, 1996, for a review). The model is dynamic and
still evolving in terms of specific adaptational processes investigated. To date, each of the
studies has been conducted with a single illness. However, use of a common model across these
studies enables subsequent contrasts between illness subgroups.
The current study has two objectives. First, to test the postulation of illness-specific
patterns of adjustment and interrelationship and adaptational processes by comparing children
with two different, genetically based, chronic illnesses: cystic fibrosis and sickle cell disease.
This is accomplished through analyses with two data sets obtained separately that utilized
the same measures of adjustment and adaptational processes. Second, to assess the contribu-
tion to children’s self-reported adjustment of children’s cognitive processes that have been
expanded to include stress appraisal and expectations of efficacy, which have not been exam-
ined previously, in addition to health locus of control and self-esteem. Two specific hypoth-
eses are addressed. Children with CF and SCD will demonstrate different patterns of self-
reported adjustment problems and interrelationships of adjustment with the cognitive adaptational
processes.
 Illness Specific Patterns 123

METHOD
Subjects
The subjects for this study were drawn from among children, 7–12 years of age, and their
mothers who had participated in stress and coping projects through the Duke Cystic Fibrosis
Clinic (n 5 43; 14 females, 29 males) and the Duke–University of North Carolina Sickle Cell
Disease Center (n 5 49; 22 females, 27 males). Matching on maternal education, as a measure
of socioeconomic status, and gender yielded a study sample of 40 White children (14 females;
26 males) with CF and 40 African-American children (14 females, 26 males) with SCD. There
was no significant difference (ANOVA) in the age of the children in these two subgroups (CF:
M 5 118 months; SCD: M 5 117 months; F(1,79) 5 .02).

Procedure
The Institutional Review Board-approved protocol included self-report questionnaires and struc-
tured interviews that were completed by children and their mothers, subsequent to providing
consent, at a regularly scheduled clinic visit.

Illness Parameters
Illness parameters included illness type and severity. Cystic fibrosis (CF) has a prevalence of 1
in 2,000 live births and is the most common genetic disease that affects Caucasians. Sickle cell
disease (SCD) affects about 1 of every 400 African-American newborns and refers to a group
of genetic disorders characterized by production of hemoglobin S (HbS), chronic hemolytic
anemia, and both acute and chronic tissue injury as a consequence of vascular occlusion.
Severity of cystic fibrosis was assessed through the Shwachman Clinical Evaluation Sys-
tem (Shwachman & Kulczycki, 1958). This rating system was completed by the medical staff
based on findings in four areas: activity, pulmonary function and cough, growth and nutrition,
and chest X-ray. Total scores range from 20 to 100, with higher scores indicating better func-
tioning and lesser severity.
Assessment of severity of SCD is complicated by the absence of an accepted measure or
index. Consequently, we have chosen to reflect severity of SCD in three ways: phenotype,
number of SCD complications, and frequency of painful episodes. Sickle cell anemia is usually
more severe than Hemoglobin SC disease and Sickle Beta Thalassemia Syndromes. Given that
patients use multiple local medical resources, mother report of complications and painful epi-
sodes was assumed to be more comprehensive than a review of medical records. This approach
is consistent with other approaches in the literature (Gil, Williams, Thompson, & Kinney, 1991;
Hurtig, Koepke, & Park, 1989). Each mother reported the total number of SCD complications
during the past year (e.g., leg ulcers, renal complications, and aseptic necrosis), and the number
of painful episodes due to vasoocclusion experienced by her child during the previous 9 months.

Demographic Parameters
Maternal education, in years, was used to reflect socioeconomic status.

Child Cognitive Processes

Self-esteem was assessed by the self-worth dimension of the Self-Perception Profile for chil-
dren (Harter, 1982).
124 Journal of Clinical Psychology, January 1998

Expectations of efficacy and appraisal of stress were assessed with two items formulated
similarly to the items used in the Self-Perception Profile for children (e.g., some kids think that
they will be able to manage their illness but other kids don’t think they will be able to handle it;
some kids see their illness as the most terrible thing, but other kids don’t see their illness as
such a big problem).
Expectations of control over health were assessed with the Children’s Health Locus of
Control Scales (Parcel & Meyer, 1979) which yields three subscales: Internal, Powerful Other,
and Chance.

Child Psychological Adjustment

The psychological adjustment of the children was assessed through the Child Assessment Sched-
ule (CAS: Hodges, Kline, Stern, Cytryn, & McKnew, 1982), a semistructured diagnostic inter-
view conducted by clinical child psychologists trained to a criterion of .70 (a) or greater for
five consecutive interviews. Each CAS item is phrased such that an affirmative response indi-
cates the presence of symptomatology. The CAS is scored by a computer program that matches
the CAS items to specific diagnostic criteria outlined in the third edition of the Diagnostic and
Statistical Manual of Mental Disorder (DSM-III; American Psychiatric Association, 1980).
The yield from the CAS in this study was a total symptom score and whether the symptom,
onset, and duration criteria were met for the major, common DSM-III diagnoses.

RESULTS
Adjustment Problems
Table 1 presents the illness subgroup comparisons of CAS self report symptoms. The illness
subgroups did not differ significantly (ANOVA) in CAS Total Symptom Score (CF: M 5 26.68,
SD 5 15.39; SCD: M 5 27.52, SD 5 12.23; F(1,78) 5 .07), but the MANOVA for overall
subgroup differences on the diagnostically related symptom scales was significant (Wilks’ l:
F(5,74) 5 2.67, p , .03). Subsequent ANOVA revealed that the children with CF had signif-
icantly higher levels of self reported oppositional behavior (CF: M 5 1.20, SD 5 1.49; SCD:
M 5 .38, SD 5 .77; F(1,78) 5 9.67, p , .003) and behaviors associated with attention deficit
disorder (CF: M 5 2.08, SD 5 2.10; SCD: M 5 .90, SD 5 1.52; F(1,78) 5 8.21, p , .005) than
did the children with SCD.

Table 1. Comparison of Self Reported Symptoms by Children with CF and SCD

CF SCD

M SD M SD F

Total Symptom Score 26.68 15.39 27.52 12.23 .07

Attention Deficit 2.07 2.10 .90 1.52 8.21**
Depression 4.75 3.87 4.60 3.47 .03
Conduct Disorder .20 .52 .05 .22 2.85
Oppositional 1.20 1.49 .38 .77 9.67**
Anxiety 4.25 2.53 4.68 2.79 .51

**p < .01.

 Illness Specific Patterns 125

Table 2. Frequency of DSM-III Diagnoses in Children with CF and SCD

Frequency (%)

Diagnosis CF SCD

DSM-III Dx 24 (60.0) 17 (42.5)

Specific Diseases
Depression 3 (7.5) 0 (.0)
Anxiety (any) 14 (35.0) 11 (27.5)
Separation Anxiety 10 (25.0) 11 (27.5)
Overanxious Disorder 6 (15.0) 4 (10.0)
Obsessive-Compulsive 2 (5.0) 4 (10.0)
Phobic 3 (7.5) 2 (5.0)
Attention Deficit Disorder 2 (5.0) 0 (.0)
Conduct Disorder 5 (12.5) 2 (5.0)
Oppositional 9 (22.5) 1 (2.5)
Enuresis 5 (12.5) 3 (7.5)
Encopresis 0 (.0) 1 (2.5)

Table 2 presents the frequency DSM-III diagnoses. The criteria for any diagnosis was met
by 60% (24/40) of the children with CF compared to 42% (17/40) of the children with SCD
(x 2 (1) 5 2.45, ns). Anxiety disorders were the most frequent diagnosis for both the children
with CF (35%) and the children with SCD (27.5%). However, the frequency of the externaliz-
ing disorders, particularly oppositional disorder and conduct disorder, were higher in the chil-
dren with CF (22.5% & 12.5% respectively) than in the children with SCD (2.5% and 5.0%,
respectively).

Adaptational Patterns
There were no significant illness subgroup differences across the six subscales of the perceived
competency scale (Wilks’ l: F(6,71) 5 .33, ns) or across the three children’s Health Locus of
Control Scales (Wilks l: F(3,76) 5 2.43, ns), or in perceptions of efficacy (ANOVA: F(1,78) 5
3.65, ns). However, children with SCD reported higher levels of stress (SCD: M 5 1.50, SD 5
1.32; CF: M 5 .75, SD 5 1.15; F(1,78) 5 7.34, p , .01) than did the children with CF.
Table 3 presents the Pearson correlations between the measures of children’s cognitive
processes and CAS total symptom score by illness subgroup. Low levels of perceived self-
worth and high levels of perceived stress were associated with high levels of self reported
symptoms in both illness subgroups. Low levels of perceived efficacy was associated with high
levels of self reported symptoms only among children with CF. Health locus of control was not
significantly related to self-reported symptoms for either illness subgroup.
Table 4 presents the summaries from the hierarchical multiple regression analysis of CAS
total symptom score conducted separately by illness subgroup. Maternal education, as a mea-
sure of SES, child age, and illness severity (Shwachman for CF and events total for SCD) were
forced to enter first. Then a forward, stepwise procedure was used in which variables were
allowed to enter if they accounted for a significant ( p , .05) increment in variance.
With the children with CF, 12% of the variance was accounted for by the control variables.
Higher levels of perceived stress, lower levels of perceived efficacy, and low levels of internal
health locus of control accounted for significant, and independent, increments in variance in
126 Journal of Clinical Psychology, January 1998

Table 3. Pearson Correlations of Child Cognitive Processes with Total

Symptom Score by Illness Subgroup

Illness Subgroup

Cognitive Process CF SCD

Self Concept
Self Worth −.46** −.39*
Appraisal
Stress .52*** .47**
Efficacy −.42** −.16
Health Locus of Control
Powerful Other −.22 −.10
Internal .08 .17
Chance −.30 .04

*p < .05; **p < .01; ***p < .001.

self reported symptoms. Together these cognitive processes accounted for 37% of the variance
in adjustment.
With the children with SCD, the control variables accounted for 2% of the variance. Higher
levels of perceived stress and lower levels of self-worth accounted for significant, and inde-
pendent, increments in the variance in self-reported symptoms. Together the cognitive pro-
cesses accounted for 34% of the variance.

DISCUSSION
The findings of this study provide support for the role of children’s cognitive processes in
psychological adjustment and for the hypotheses of illness specific patterns of adjustment and
cognitive adaptational processes in children with different chronic illnesses. Although anxiety

Table 4. Hierarchical Multiple Regression Analysis of Total Symptom

Score by Illness Subgroup

Variable B R2 R2 F

Cystic Fibrosis Cumulative

Maternal Education
Child Age
Severity .12 1.62
Stress 6.73 .19 .31 11.54**
Efficacy −4.32 .09 .40 6.57*
Internal Health LOC −24.73 .09 .49 5.64*
Sickle Cell Disease Cumulative
Maternal Education
Child Age
Severity .02 .28
Stress 3.99 .21 .23 6.56*
Self-Worth −8.28 .12 .36 9.18**

*p < .05; **p < .01.

 Illness Specific Patterns 127

disorders were most prominent for both groups, children with CF had significantly higher
levels of externalizing symptoms and higher frequencies of oppositional disorder and conduct
disorder than children with SCD. It is important to note that oppositional disorder according to
the DSM-III system (American Psychiatry Association, 1980) refers to negativistic, disobedi-
ent, and provocative behaviors and not hostility and defiance that are additional characteristics
of subsequent revisions of the Diagnostic and Statistical Manual.
Although appraisals of stress related to their illness accounted for significant portions of
variance in adjustment in both subgroups, there were different patterns of relationships among
the cognitive processes and adjustment within the illness subgroups. Significant increments in
adjustment variance were accounted for by expectations of efficacy and internal health locus of
control in children with CF and by self-worth in children with SCD. This is the first time that
children’s appraisal of stress and expectations of efficacy related to their illness have been
included in the model as adaptational processes. The relatively modest amount of variance in
adjustment being accounted for by the model indicates the need to continue to evaluate other
potential contributors including other cognitive processes, such as attributions, as well as cop-
ing methods and various dimensions of social support.
One reason to investigate for illness specific patterns of adjustment is to foster specificity
of intervention efforts to enhance adaptation. Cystic fibrosis and sickle cell disease differ in the
management demands placed on patients and families. The typical treatment regimen of chil-
dren with CF involves daily numerous medications and chest physical therapy whereas chil-
dren with SCD have fewer daily demands but experience periodic pain episodes. The daily
illness task demands associated with CF may serve to evoke oppositional behavior which in
turn may have particular relevance for compliance and health maintenance. Fostering an increased
sense of efficacy and internal health locus of control may be instrumental in promoting adap-
tation in children with CF whereas enhancing feelings of self worth may be a salient interven-
tion target in children with SCD. For both groups, the findings suggest the importance of
enhancing methods of managing cognitive appraisals of illness related stress.
This study provides support for the need to further investigate illness specific patterns of
adaptation in addition to general dimensions of adjustment that cut across illnesses. One limi-
tation of this study is the obvious confound of illness type with race. If illness specific patterns
emerge in other samples, then further support would be provided for specificity of intervention
efforts. It is also of importance to add a developmental perspective to the delineation of the
interrelationship of both general and specific illness tasks and adaptational processes with
adjustment. For example, another study in this project found the rate of oppositional disorder
among adolescents (age 13–17 years) also to be higher for children with CF (30%) than those
with SCD (14%) (Thompson, Gustafson, & Gil, 1995). However, the rate of oppositional dis-
orders for adolescents in both illness subgroups was higher than rates for children in the current
study (CF 5 22.5%, SCD 5 2.5%). This suggests that the normative adolescent developmental
trajectory toward independence and autonomy may interact with the demands and constraints
of illness tasks and may be manifested in opposition or noncompliance to illness tasks.
In attending to the psychosocial dimensions of the care of children with chronic illness it is
increasingly evident that multidimensional perspectives are necessary. The findings of this
study provide support for the potential usefulness of considering multiple interactions among
general and illness specific tasks and adaptational processes with developmental tasks in under-
standing adjustment problems and delineating intervention opportunities.

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