Implications of Resolving the Diagnosis of PKU for Parents
and Children
Bruce Lord,1,2 PHD, Judy Ungerer,2 PHD, and Colin Wastell,2 PHD
1
The Children’s Hospital at Westmead and 2Macquarie University
Objective To examine resolution of the diagnosis among parents of children with phenylketonuria (PKU) as
a mechanism of adjustment for parents and children. Methods Reaction to diagnosis interviews were
conducted with 52 mothers and 47 fathers of 55 children with PKU aged 2–12 years. The parents also
completed questionnaires assessing their personal adjustment (stress symptoms), their child’s adjustment
(behavior problems), and coping variables (personal hopefulness and coping strategies). Results Most
mothers (69%) and fathers (77%) were resolved to their child’s diagnosis. Lower levels of parent stress were
explained by higher personal hopefulness (14% of the variance for mothers and 21% for fathers) and
resolution of the diagnosis (15% of the variance for mothers and 6% for fathers) after taking account of
demographic variables and severity of the child’s PKU. Parent resolution, however, did not contribute
independently to the variance explained in child behavior problems after taking account of coping variables
and severity of PKU. Conclusions Resolution of the diagnosis of PKU is a strong indicator of parent
adjustment, and assessment of parent reactions should be considered an integral component of clinical care.
Further research is warranted in relation to the implications of parent resolution for the child’s response to
PKU through different development stages and the effectiveness of interventions in aiding parent resolution.
Key words adjustment; child; coping; PKU; parent; resolution of the diagnosis.
The Impact of PKU
Parents play a pivotal role in the management of childhood
phenylketonuria (PKU), but few studies have examined
parent reactions and their adjustment to this condition.
PKU is a rare inborn error of metabolism, detected by
newborn screening (Waisbren, 1999). Children with PKU
have an enzyme deficiency that affects the metabolism of
phenylalanine, a specific amino acid contained in normal
diets. If untreated, PKU causes brain damage and developmental delay. However, the detrimental effects of PKU are
largely controllable through a protein-restricted diet, which
is crucial during early childhood and recommended for
the whole of life. Most children with PKU require blood
testing, at least monthly, to monitor phenylalanine levels.
In developed countries, children with PKU are usually
managed by major pediatric centers, and most achieve
normal physical and intellectual development. For a small
minority, poor adherence to the diet leads to learning and
behavioral difficulties (Sullivan & Chang, 1999). A central
issue for parents is how they deal with the emotional impact
of the diagnosis, which occurs abruptly soon after the birth
and represents a serious threat to the child’s development.
Moreover, the child’s diet is highly restrictive and exacting.
Thus, PKU tends to have an intrusive impact on family
life (Eiser, 1985), and both mothers and fathers tend to
worry about their child’s development (Lord, Wastell, &
Ungerer, 2005).
Resolving the Impact of the Diagnosis
While there have been few studies of parent reactions to
PKU, common reactions to other serious childhood
conditions have been well described. Numerous studies
have highlighted the intensity of parental grief that is
often associated with the diagnosis, as well as individual
differences in reactions and coping styles (Beresford,
1994; Shapiro, 1983).
All correspondence concerning this article should be addressed to Bruce Lord, Clinical Programs Office,
The Children’s Hospital at Westmead, Locked Bag 4001, Westmead 2145 NSW, Australia.
E-mail: [email protected]
Journal of Pediatric Psychology 33(8) pp. 855–866, 2008
doi:10.1093/jpepsy/jsn020
Advance Access publication March 13, 2008
Journal of Pediatric Psychology vol. 33 no. 8 ß The Author 2008. Published by Oxford University Press on behalf of the Society of Pediatric Psychology.
All rights reserved. For permissions, please e-mail: [email protected]
856
Lord, Ungerer, and Wastell
A grief response is usually triggered by the painful
realization that the child has a condition that changes
expectations of normal healthy development (Trout,
1983; Waisbren, 1980). Although the intensity of grief
varies, initial reactions usually involve strong feelings of
disappointment, sadness, or anger (Ormerod & Huebner,
1988; Thernlund, Dahlquist, Ivarsson, & Ludvigsson,
1996) and may include fear, numbness, and difficulties
in retaining information (Alonzo, 2000; Jedlicka-Kohler,
Gotz, & Eichler, 1996). Persistent trauma effects have
been recognized among parents of children surviving
life-threatening conditions such as cancer (Barakat et al.,
1997), and similar reactions have recently been found
in relation to PKU (Lord et al., 2005).
How parents process the emotional impact of the
diagnosis has been a debated issue (Marvin & Pianta, 1996).
An emphasis on pathological responses in the early
literature (Shapiro, 1983) was followed by models depicting
‘‘stages’’ through which parents typically move toward
adaptation (Clubb, 1991; Drotar, Baskiewicz, Irvin,
Kennell, & Klaus, 1975; Trout, 1983), with the common
theme of progression over time towards eventual adjustment (Blacher, 1984). While most parents appear to move
through an initial crisis to a state of relative equilibrium, the
notion of stages has been criticized as too linear and
prescriptive (Allen & Affleck, 1985). The ‘‘stages’’ approach
also implies the normal pathway is toward completion
of grief, which has not accorded well with many parents
whose experience has been described as chronic sorrow
(Teel, 1991) or episodic grief (Hewson, 1997).
An important advance in this area has been the
development of the concept of ‘‘resolution’’ of the child’s
diagnosis, based on attachment theory, in a way that
recognizes individual differences in coping styles and
adjustment outcomes (Marvin & Pianta, 1996; Pianta,
Marvin, Britner, & Borowitz, 1996). Marvin and Pianta
(1996) define resolution as the relative cessation of active
grieving and a reorientation to current realities and future
possibilities. This is characterized by acknowledgment of
feelings, a change in the quality and intensity of emotional
response, and an assertion of coping and moving on in life.
Unresolved parents display an absence of these characteristics and a coping style that reflects the ongoing effects of
trauma. Common features include cognitive distortion,
emotional disorientation, and a continuing search for an
explanation or existential reason for the child’s condition.
The concept of resolution has similarities to notions of
‘‘rebuilding world assumptions’’ (Janoff-Bulman, 1992)
and ‘‘redefining situations’’ (Tunali & Power, 1993),
which involve the realignment of beliefs, values, and living
patterns following a traumatic event to accord with new
realities.
Research on resolving the diagnosis has been conducted with mothers of children diagnosed with cerebral
palsy or epilepsy, at 2–4 years postdiagnosis (Marvin &
Pianta, 1996; Sheeran, Marvin, & Pianta, 1997). Resolved
and unresolved parents were reliably identified through a
brief standardized assessment known as the Reaction to
Diagnosis Interview (RDI) (Pianta & Marvin, 1993).
Approximately equal numbers of resolved and unresolved
mothers were found. Given the challenges presented by
PKU, it is likely that a substantial proportion of parents of
children with PKU will also experience difficulty in resolving
the diagnosis. (Pianta et al., 1996) found no association
between the parent’s resolution classification and the time
since the diagnosis. This was interpreted as indicating that
resolution occurs soon after the diagnosis and that parents
move into a relatively stable coping style. However, there has
been relatively little research into implications of resolution
versus nonresolution for longer term adjustment of children
and parents.
Resolution of the Diagnosis and
Child Adjustment
The concept of resolution has parallels with the attachment
theory notion of secure caregiving internal working models,
which provide the foundation for secure parent–child
attachment relationships (Bowlby, 1988). Secure caregiving
internal working models are defined by an acknowledgment
and integration of positive and negative thoughts and
feelings about the parent as caregiver and about the child in
the attachment relationship. In contrast, the concept of lack
of resolution captures features of insecure caregiving
internal working models where thoughts and feelings are
not integrated and where, in the most insecure form,
thoughts and feelings are sufficiently distorted to make it
difficult for parents to adequately care for their child
(George & Solomon, 1999). Marvin and Pianta (1996)
suggest that resolving the diagnosis involves the integration
of internal working models in a way that allows acceptance
of the condition and enables the parent to organize their
caregiving behavior. This implies that resolved parents are
more likely to respond sensitively to their child’s emotional
needs and to display confidence, clarity, and consistency in
setting behavioral limits.
There is limited empirical research supporting an
association between parent resolution and positive child
adjustment. In the Marvin and Pianta (1996) study
of mothers of preschool children with cerebral palsy,
Resolving the Diagnosis of PKU
a strong association was found between parent resolution
and security of mother–child attachment. Incidentally, no
association was found between resolution classification
and the severity of the child’s disability. These findings
are supported by Sheeran et al. (1997), who examined
resolution among parents of children with cerebral palsy
or epilepsy and found that resolved mothers reported
significantly lower levels of parenting stress than
unresolved mothers. Thus, parent resolution has implications for the child, but further study is needed to identify
the impact on the child’s adjustment.
Resolution of the Diagnosis and
Other Coping Processes
Resolving the diagnosis can be viewed as a resilience factor
for dealing with the stressful impact of the child’s condition
within a stress and coping framework based on risk and
resilience factors, such as that proposed by Wallander and
Varni (1998). As a resilience factor, the construct of resolution has similarities to recognized coping processes described in the wider literature. First, the ‘‘coping styles’’
described within the Marvin and Pianta (1996) model have
similarities to ‘‘coping strategies’’ that mediate the impact of
stress on health (Carver, Scheier, & Weintraub, 1989;
Lazarus & Folkman, 1984). Resolved coping styles may be
emotion-, cognition-, or action-based. These coping styles
have similarities to adaptive coping strategies, such as
support seeking, reappraisal, and problem-solving, which
have been identified in wider research on childhood chronic
conditions (Beresford, 1994). Some of the unresolved
coping styles, such as those classified as ‘‘emotionally
overwhelmed’’ or ‘‘neutralizing’’, have similarities to
palliative coping strategies that involve self-blame and
avoidance (Beresford, 1994). Second, resolution is characterized by a balanced outlook with positive expectations
of being able to manage the challenges that lay ahead
(Marvin & Pianta, 1996). In this regard, resolution implies
the formation of a hopeful outlook, which has been
identified as an important coping resource in recovery
from traumatic events (Nunn, 1996).
Although resolution implies the use of adaptive coping
strategies and a hopeful outlook, the construct is essentially
based on emotional processing of an event that is deemed to
be traumatic. Marvin and Pianta (1996) suggest that this
occurs through cyclical phases of the stress response
(Horowitz, 1997) that enable the parent to incorporate
painful new information and move forward. Resolved
parents are seen as able to recall events surrounding the
diagnosis and acknowledge distress associated with it,
but are more oriented to the present and future needs
of their child. In contrast, unresolved parents are described
as emotionally ‘‘stuck in the past’’ and more preoccupied
with negative aspects of their child’s condition (Marvin
& Pianta, 1996). Lack of resolution indicates that the
diagnosis continues as a major source of stress and is likely
to be a strong predictor of stress symptomology. Further
study is needed to clarify the relationship between resolving
the diagnosis, coping strategies, and hopefulness, and
to determine whether resolving the diagnosis contributes
independently to the explanation of parent and child
outcomes.
Aims of the Study
The present study had three aims. The first was to examine
the relationship between resolution of the diagnosis and
adjustment outcomes for parents (stress symptoms) and
children (behavior problems) in families of children with
PKU. The second was to examine relations between parent
resolution of the diagnosis and other coping variables,
specifically coping strategies, and personal hopefulness.
The third aim was to compare the variance in parent and
child outcomes explained by resolution of the diagnosis
with that explained by personal hopefulness and coping
strategies. It was hypothesized that resolved parents will
have fewer stress symptoms and that children with resolved
parents will have fewer behavior problems. It was also
hypothesized that resolved parents will have higher levels of
personal hopefulness and tend to use active rather than
palliative coping strategies. It was further hypothesized that
parent resolution classification will predict variance in
parent and child outcomes over and above measures of
personal hopefulness and coping strategies.
Method
Participants
Participants were recruited through the PKU Clinic at an
Australian pediatric referral hospital, which is linked to
a newborn screening service and provides management
for all children with PKU in the home state. The sample
consisted of one or both parents from 55 two-parent
families with a child with PKU aged 2–12 years.
If families had more than one child with PKU within
the age range, data were collected in relation to the older
child with PKU. Participating families represented 83%
of the clinic population. The nonparticipating families
(n ¼ 11) did not differ significantly from the study sample
when compared on available demographic data (child age
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Lord, Ungerer, and Wastell
and gender), and their family names indicated they were
from a range of cultural backgrounds. Reasons for refusals
were mostly either the time commitment required for the
study or the personal nature of the information sought.
The Parent Sample
From 55 families, data were available for 52 mothers
and 47 fathers. The mean age of mothers was 35.3 years
(SD ¼ 5.2, range 24–51 years), and the mean age of
fathers was 38.0 years (SD ¼ 5.7, range 25–51 years).
Parents were from various cultural backgrounds; 31%
(n ¼ 16) of mothers and 26% of fathers (n ¼ 12) had
migrated to Australia, mainly from South East Asia,
the Middle East, and Northern and Southern Europe.
A minority of families (13%, n ¼ 7) spoke a language
other than English at home, and 76% (n ¼ 42) were
residing in metropolitan areas. Most fathers (94%, n ¼ 44)
were in the paid workforce, 60% (n ¼ 28) had completed
tertiary education, and 51% (n ¼ 24) were in occupations
requiring technical or professional qualifications. More
than half of the mothers (56%, n ¼ 29) were in the paid
workforce, 50% (n ¼ 26) had completed tertiary education, and 23% (n ¼ 12) were in technical or professional
occupations.
The Child Sample
Of the 55 children, 28 were male and 27 were female.
The mean age of the children was 6.6 years (SD ¼ 3.2,
range ¼ 2.0–12.7 years). All children had a form of
PKU. Most (93%, n ¼ 51) had a diagnosis of classic PKU
requiring dietary treatment and regular blood tests. Three
children had a mild form of PKU requiring limited dietary
adjustment and monitoring, and one had a diagnosis of
BH4 Synthesis Defect, requiring medication and monitoring. In most families there were three or fewer children
(95%, n ¼ 52), and the child with PKU was either the
first- or second-born (89%, n ¼ 49).
Measures
Resolution of the Diagnosis
The RDI (Pianta & Marvin, 1993) is a brief, structured
interview that aims to elicit the affective and cognitive
responses of parents to receiving their child’s diagnosis,
changes in responses over time, and the meaning the
parents attribute to the experience. The RDI is comprised
of five questions and takes 15 min to administer.
Interviews are video-recorded and later coded (resolved
vs. unresolved) using the RDI Classification System
(Pianta & Marvin, 1993). Resolution is determined by
identification of key characteristics (acknowledgment of
feelings, change in emotional response, and assertion
of moving on).
In the present study, all except three interviews were
video recorded. In these cases, either an audio recording
or hand-written notes were made. Coders were the first
and second authors, both of whom obtained formal
certification (from R. Marvin, University of Virginia) in the
use of the classification system. Reliability was established
by independently coding 20 interviews. Initial agreement
was 90%, and differences were conferenced to agreement.
All RDI classifications were assigned while the coders
were blind to the questionnaire data.
Parent Adjustment
The Malaise Inventory (MI) (Rutter, Tizard, & Whitmore,
1970) assessed emotional and physical stress symptoms.
The MI is self-administered and consists of 24 items, with
a ‘‘yes/no’’ response format. The score is derived by totalling
the ‘‘yes’’ responses. A score of six or more is considered
to represent a clinical level of distress. Internal reliability
(Cronbach’s a) was .82 for mothers and .87 for fathers. The
MI correlates well with other stress scales, and test–retest
reliability over short periods has been reported at r ¼ .91
(Hirst & Bradshaw, 1983). The measure has been extensively used with parents of children with chronic conditions
(Sloper & Turner, 1993).
Child Adjustment
The Child Behavior Checklist (CBCL) Problem Scale
(Achenbach, 1991) assessed the emotional and behavioral
adjustment of the children. The relevant age-related scale
versions were used, and T-scores were derived to allow
comparison across age and sex groups. Achenbach (1991)
reports a high test–retest reliability (r ¼ .93) for the
Problem Scale. T-scores above 63 represent a clinical level
of emotional disturbance, while T-scores of 60–63 are in
the borderline range. Some reports use T 70 as the
clinical range, and the results are reported for both cut-off
points.
The CBCL has been extensively used in developmental and clinical research (Achenbach, 1991). Items
capturing somatic symptoms have been shown not to
confound behavioral assessment in children with chronic
medical conditions (Holmes, Respess, Greer, & Frentz,
1998). The Problem Scale was, therefore, used.
Coping Variables
The Hunter Opinions and Personal Expectations (HOPE)
Scale (Nunn, Lewin, Walton, & Carr, 1996) assessed the
parents’ personal hopefulness. The measure consists of
20 items, each scored on a 5-point Likert scale, and
Resolving the Diagnosis of PKU
comprises two subscales, Hope and Despair. Internal
reliability (Cronbach’s a) for the Hope subscale was .82
for mothers and .76 for fathers, and for the Despair
subscale was .84 for mothers and .77 for fathers.
A Global Personal Hopefulness (GPH) score is computed
from combining the subscales, and a higher GPH score
means a more hopeful and less despairing outlook. The
GPH score was used in the present study. GPH has been
shown to have good test–retest reliability over a period of
64 weeks (r ¼ .71) and to correlate well with measures
of trait anxiety (r ¼ .64) and depression (r ¼ .54)
(Nunn et al., 1996).
The Ways of Coping Questionnaire (WCQ) (Folkman
& Lazarus, 1988) assessed the parents’ coping strategies.
Parents completed the measure in relation to a challenging situation associated with their child’s PKU that
occurred in the previous month. The WCQ has 66-items,
each with a 4-point Likert scale indicating the frequency
with which the strategy was used. The measure comprises
eight scales representing theoretically different coping
strategies: Confrontative Coping, Distancing, SelfControlling, Seeking Social Support, Accepting Responsibility, Escape-Avoidance, Problem Solving, and Positive
Reappraisal. In the present study, the relative score
method was used. Internal reliability (Cronbach’s a)
ranged from .65 to .84 in the mothers’ data and .71 to
.86 in the fathers’ data. The WCQ has been widely used
in studies of childhood conditions (Beresford, 1994).
Demographic Variables
Data were collected in relation to the child’s age, gender,
birth order, number of siblings, and number of children
in the family with PKU. Parent demographic data were
collected in relation to their age, education level, type of
occupation, country of birth, and language background
(English speaking vs. non-English speaking).
Severity of PKU
Information about the child’s type of PKU was collected
from the treating doctor for use as a control variable.
Severity of PKU was treated as a binary measure reflecting
whether or not the child required a special diet.
Procedures
The research ethics committees of the hospital and the
associated university approved the study. Participating
parents provided written consent and attended a single
assessment session lasting 60–90 min in which they were
interviewed and completed a set of self-report questionnaires. Assessments were conducted by the first author
or a qualified social worker at either the hospital or
the parent’s home. Parents were seen separately, and
language interpreters were used on three occasions with
parents who were not fluent in English.
Data Analysis
The mothers’ and fathers’ data were analyzed separately,
and the distributions of scores for all measures were
examined. To test the first and second hypotheses, the
bivariate relationship of resolution classification with MI,
CBCL Problem Scale, HOPE Scale, and WCQ coping scales
was examined using t-tests. To test the third hypothesis,
a hierarchical regression strategy was used to examine
coping variables (HOPE Scale and WCQ scales) and
resolution classification as predictors of parent and child
outcomes, with the Malaise score as the dependent variable
in the parent models and the CBCL Problem Scale as
the dependent variable in the child models. Preliminary
analyses were conducted to examine the bivariate relationship of the predictor and dependent variables with each
other and with the severity of PKU and demographic
variables (i.e., child’s age, gender, birth order, number
of siblings, parent’s age, parent’s language background,
parent’s education, and parent’s occupation), using t-tests,
ANOVA, chi-square, or Spearman correlations as appropriate. None of the predictor variables was confounded with
the severity of PKU or demographic variables (rs .61).
Demographic and coping variables were retained for the
multiple regression analyses where they were related to the
dependent variable at an a-level of .05. The severity of PKU
was entered first as a control variable, and the retained
demographic variables were entered second. The retained
coping variables were then tested in the model using the
stepwise procedure with a at .05. In the fourth step,
resolution classification was entered as a dummy variable.
Finally, an exploratory mediation analysis was conducted to
examine whether the influence of resolution on Malaise and
CBCL scores was mediated by the other coping variables.
Results
Resolution of the Diagnosis and
Adjustment Outcomes
Resolution Classification and MI
Coding of RDIs indicated that 69% of mothers (n ¼ 36)
and 77% of fathers (n ¼ 36) were resolved to the
diagnosis. Although a larger percentage of fathers than
mothers was resolved, the difference between the groups
in the ratio of resolved to unresolved cases was not
significant, [w2 (1, 45) ¼ .79, p > .05]. No association was
found between resolution classification and the child’s
age in either the mothers’ or fathers’ data.
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Lord, Ungerer, and Wastell
Table I. Malaise Inventory and CBCL Problem Scale by Resolution Classification for Mothers and Fathers
Resolution classification
Resolved
M
SD
Unresolved
n
M
SD
n
t
95% CI
d
Mothers (N ¼ 52)
Malaise Inventory
CBCL Problem Scale
Fathers (N ¼ 47)
Malaise Inventory
CBCL Problem Scale
3.39a
3.06
36
7.75a
5.31
16
3.74
c
12.17
36
56.06c
9.57
16
1.99
2.83b
2.76
36
5.64b
9.73
36
49.22
49.17
55.36
6.70 to 2.02
13.75 to .07
1.01
.62
6.17
11
2.15
5.44 to .17
.59
12.71
11
1.72
13.46 to 1.06
.55
a
Resolved < Unresolved, p < .001.
Resolved < Unresolved, p < .05.
c
Resolved < Unresolved, p ¼ .05.
b
The mothers’ scores on the Malaise Inventory ranged
from 0 to 20, with a mean of 4.73 (SD ¼ 4.34). Although
most mothers reported a relatively low number of stress
symptoms, 33% (n ¼ 17) scored 6 or more, indicating a
high level of distress. Resolved mothers had significantly
lower Malaise scores, t (50) ¼ 3.74, p < .001, although
there was greater variability in scores among the unresolved group (Table I).
Fathers’ Malaise scores ranged from 0 to 18 with
a mean of 3.49 (SD ¼ 3.94), and 17% (n ¼ 8) scored 6 or
above. The difference in scores for mothers and fathers
was not significant when compared using a paired t-test.
Resolved fathers had significantly lower scores than
unresolved fathers, t (45) ¼ 2.15, p < .05, and like
mothers there was greater variability in scores among the
unresolved group (Table I).
Resolution Classification and Child Behavior
The T-scores for the CBCL recorded by mothers were
normally distributed (M ¼ 51.33, SD ¼ 11.78), with 13%
(n ¼ 7) in the borderline clinical range (T ¼ 60–63), 11%
(n ¼ 6) in the clinical range (T 64), and three T-scores
>70. The CBCL T-scores recorded by fathers were also
normally distributed (M ¼ 50.62, SD ¼ 10.68), with 16%
(n ¼ 8) in the borderline clinical range, 10% (n ¼ 5) in the
clinical range, and with two T-scores above 70. The
correlation between the mother’s and father’s T-scores
was .64 (p < 0.001).
Resolved parents tended to report fewer behavior
problems with their child. However, the difference in
CBCL T-scores recorded by resolved and unresolved
parents approached significance only in the mothers’
data, t(50) ¼ 1.99, p ¼ .05 (Table I).
Resolution of the Diagnosis and Parent Coping
Resolution classification was not significantly related
to the HOPE Scale score in either the mothers’ or
fathers’ data. Resolved mothers had significantly lower
scores for Escape-Avoidance coping, t(50) ¼ 3.37,
p < .01. However, resolution classification was not significantly related to the other coping strategy scales in either
the mothers’ or fathers’ data.
Resolution of the Diagnosis, Parent Coping,
and Stress Symptoms
Mothers
Mothers’ Malaise scores were unrelated to the severity
of PKU or demographic variables other than their language background. Mothers born in a non-English
speaking country had significantly higher Malaise scores,
t(50) ¼ 2.93, p < .01. The correlation of Malaise scores
with the coping variables and resolution classification is
presented in Table II.
In the hierarchical regression analysis, the severity
of PKU was entered first, and the retained demographic
variable (mother’s language background) was entered
second. In the third step, the retained coping variables
(HOPE Scale, Problem Solving scale, and Escape-Avoidance
scale) were tested using the stepwise procedure and the
HOPE Scale entered the model (p < .01). The mother’s
resolution classification was then entered and found to be
significant (p < .01). In the final equation, non-English
speaking background (b ¼ .28), lower levels of personal
hopefulness (HOPE Scale, b ¼ .38) and lack of resolution
of the diagnosis (b ¼ .40) were associated with higher
Malaise scores. The model explained 44% of the variance
in mothers’ Malaise scores, with language background
accounting for 15%, the HOPE Scale adding 14%, and
resolution classification adding a further 15% (Table III).
Fathers
Fathers’ Malaise scores were unrelated to the severity of
PKU or demographic variables other than their occupation level. Fathers in lower occupation groups had
Resolving the Diagnosis of PKU
Table II. Correlations of Malaise Inventory, CBCL Problem Scale, and Resolution Classification with Coping Measures for Mothers (N ¼ 52)
and Fathers (N ¼ 47)
Malaise Inventorya
CBCL Problem Scalea
Resolution classificationb
Coping measures
Mothers
Fathers
Mothers
Fathers
Mothers
Fathers
HOPE Scale
Coping strategies scales
.44**
.52**
.35*
.36*
.10
.09
.05
Confrontative coping
.16
.12
.01
.13
.11
Distancing
.03
.09
.02
.15
.01
.16
Self-controlling
.10
.04
.03
.13
.04
.22
Seeking social support
.03
.22
.03
.21
.16
.17
.20
.08
.21
.18
.12
.06
.37*
.43**
.11
.11
.15
.02
.09
Accepting responsibility
Escape-avoidance
Problem solving
Positive reappraisal
.38**
.43**
.30*
.24
.12
.07
.19
.21
.10
.02
.03
a
Pearson correlations.
Spearman correlations.
*p < .05.
**p < .01.
b
Table III. Final Step of Hierarchical Regression Analyses for Variables
Predicting Malaise Scores for Mothers and Fathers
Variables
B
Cumulative
R2 (%)
SE B b
Mothers (N ¼ 52)
Step 4
21%, and resolution classification adding a further 6%
(Table III).
Resolution of the Diagnosis, Parent Coping,
and Child Behavior Problems
*p < .05.
**p < .01.
þ
p ¼ .05.
Mothers
Mothers’ CBCL Problem Scale scores were significantly
related to the severity of PKU, t(50) ¼ 2.34, p < .05,
but unrelated to the demographic variables. The correlation of mothers’ CBCL scores with the coping variables
is presented in Table II.
In the hierarchical regression analysis, CBCL Problem
Scale scores were predicted by the severity of the child’s
PKU (b ¼ .38) and the mother’s level of personal
hopefulness (HOPE Scale, b ¼ .41). These two variables
together explained 28% of the variance in CBCL Problem
Scale scores recorded by mothers. Mothers’ resolution
classification, however, was not a significant predictor of
their child’s behavior problems (Table IV).
significantly higher Malaise scores, t(45) ¼ 2.13, p < .05.
The correlation of fathers’ Malaise scores with the coping
variables and resolution classification is presented in
Table II.
In the hierarchical regression analysis, higher
Malaise scores for fathers were predicted by lower
levels of personal hopefulness (HOPE Scale, b ¼ .43)
and lack of resolution of the diagnosis (b ¼ .25). With
these variables in the model, the fathers’ occupation
level lost significance. The model explained 37% of the
variance in fathers’ Malaise scores, with occupation
level accounting for 10%, the HOPE Scale adding
Fathers
Fathers’ CBCL Problem Scale scores were unrelated to the
severity of PKU or the demographic variables. The correlation of fathers’ CBCL scores with the coping variables is
presented in Table II.
In the hierarchical regression analysis, CBCL Problem
Scale scores were predicted by the severity of the child’s
PKU (b ¼ .36), and the father’s Escape-Avoidance coping
(b ¼ .25) and level of personal hopefulness (HOPE
Scale, b ¼ .43). These three variables together explained
33% of the variance in CBCL Problem Scale scores.
Fathers’ resolution classification was not a significant
predictor of children’s behavior problems (Table IV).
Severity of PKU
2.00 2.55
.09
0
Mother’s language
2.99 1.20
.28*
15
background
HOPE Scale
Mother’s resolution
classification
.14
.05 .33** 29
3.68 1.04 .40** 44
Fathers (N ¼ 47)
Step 4
Severity of PKU
Father’s occupation level
HOPE Scale
.12 2.44
.01
0
1.49 1.02 .19
10
.19 .06 .43** 31
Father’s resolution classification 2.30 1.16 .25+
37
861
862
Lord, Ungerer, and Wastell
Table IV. Final Step of Hierarchical Regression Analyses for
Variables Predicting Child Behavior Problem Scores reported
by Mothers and Fathers
Variables
B
SE B
Cumulative
R2 (%)
b
Mothers (N ¼ 52)
Step 3
Severity of PKU
22.83
7.56
.38**
10
HOPE Scale
Mother’s resolution
.46
4.22
.14
3.11
.41**
.17
28
31
18.64
50.70
6.77
21.54
.36**
.32*
7
24
classification
Fathers (N ¼ 47)
Step 4
Severity of PKU
Escape-Avoidance coping
HOPE Scale
Father’s resolution
.35
.16
.29*
33
3.28
3.19
.13
35
classification
*p < .05.
**p < .01.
Coping Variables as Mediators of Resolution
of the Diagnosis
Only one coping variable in the mothers’ and fathers’
data met the criteria for examination as a mediator of the
influence of resolution on the outcomes of interest. In the
mothers’ data, the Escape-Avoidance scale had a similar
correlation to both resolution classification (rs ¼ .43,
p < .01) and Malaise score (rs ¼ .43, p < .01). When
tested in a regression analysis, while controlling for the
severity of PKU and mother’s language background,
Escape-Avoidance reduced the variance in Malaise scores
explained by resolution classification from 20% to 13%,
thus, indicating partial mediation.
Discussion
The study examined the proportion of resolved versus
unresolved cases and the association of resolution classification with parent adjustment (stress symptoms) and
child adjustment (behavior problems). The study also
compared the strength of association of resolution classification with coping-related variables (hopefulness and
coping strategies) to parent and child adjustment. The
coping variables were selected because of their conceptual
similarity to the notion of resolving the diagnosis.
The proportions of resolved cases found in the
present study (69% for mothers and 77% for fathers)
were higher than the proportions of resolved cases
(46–48% for mothers) found in previous studies
(Marvin & Pianta, 1996; Sheerin et al., 1997).
The higher proportion of resolved cases in this study
did not appear to be a function of time since birth,
despite the wide age range of the children (2–12 years).
This finding supports the assumption that the affective
and cognitive responses of parents to their child’s
diagnosis tend to remain constant over time (Pianta et
al., 1996). The results raise the question of whether some
childhood disorders are intrinsically more difficult for
parents to resolve than others. It has been argued that the
impact varies according to features such as the duration
and course of the condition, intensity of treatment, and
level of functional impairment (Silver, Westbrook, &
Stein, 1998). However, controllable conditions also
present particular adjustment challenges. For PKU,
these include not only the underlying threat to the
child’s brain development and the rigorous dietary
management required, but also the difficulties of living
with an ‘‘invisible’’ condition that might not be socially
recognized or validated. On the other hand, the
predictable management and relatively positive outlook
for PKU might aid parents in resolving the emotional
impact of the diagnosis.
The results for the MI were consistent with those
for the RDI and with previous studies of distress among
parents of children with chronic conditions (Sloper &
Turner, 1993; Wallander & Varni, 1998). A minority of
parents had Malaise scores in the clinical range, and a
significant association was found between Malaise scores
and resolution classification for both mothers and fathers.
The results suggest that lack of resolution contributes
to stress symptamology over time, although the crosssectional design of this study prevents any conclusion
regarding the direction of influence. It is also possible
that parents with more stress symptoms were more
impacted by sources of stress other than PKU, either
prior to or following the diagnosis, which may have
impeded efforts to resolve the diagnosis.
Contrary to expectations, resolution classification was
not significantly associated with personal hopefulness.
Personal hopefulness is an integrative construct that
combines several elements of personal functioning,
including self-efficacy, capacity to control events, and
purpose in life (Nunn, 1996). The HOPE Scale captures
a broad range of life expectations, and GPH is seen to
represent a relatively enduring trait (Nunn et al., 1996).
Although resolution also involves a positive future orientation, it appears to be a distinct process that is relatively
independent of the parent’s general outlook on life.
Resolution reflects expectations that are specific to the
child’s future and also has a strong focus on emotional
Resolving the Diagnosis of PKU
experience, which is linked to its roots in attachment
theory.
Resolution classification was also unrelated to parent
coping strategies other than mothers’ use of EscapeAvoidance. This scale includes items that focus on
wishful thinking, anesthetizing negative feelings, and
avoidance of people (Folkman & Lazarus, 1988). The
association of Escape-Avoidance with lack of resolution
for mothers is consistent with previous research on
palliative coping strategies (Sloper & Turner, 1993;
Thompson, Gil, Burbach, Keith, & Kinney, 1993) and
the notion that unresolved parents tend to use more
avoidant strategies to defend against painful feelings
associated with the diagnosis. Fathers’ resolution of the
diagnosis was not associated with their coping strategies,
which most likely reflects the less prominent role played
by fathers in managing childhood chronic conditions
(Beresford, 1994). Overall, the results in relation to
coping strategies most likely reflect the broad range in the
type of stressful events selected by parents when
completing the WCQ. Coping strategies generally vary
according to both the perceived degree of threat and
situational appraisal of control (Folkman & Lazarus,
1988), and the results are consistent with previous
studies indicating that parents of children with chronic
conditions tend to use a wide range of coping strategies
in response to varying problems (Beresford, 1994). Future
research might investigate whether resolved and unresolved parents respond differently to specific kinds of
challenges in managing their child’s condition.
The models predicting parent adjustment indicated
that resolution classification was a relatively strong predictor of level of stress symptoms compared with the
coping variables. Resolution classification independently
explained 15% of the variance in Malaise scores for
mothers and 6% for fathers. These findings highlight the
relevance of the issues explored in the RDI to parent
wellbeing and the importance of resolving the diagnosis
in the adjustment process. They also support the validity
of the RDI in capturing diagnosis-related distress.
Personal hopefulness (GPH) was also a strong predictor
of mothers’ and fathers’ level of stress symptoms. Hopefulness represents expectations of being able to deal
positively with future events, based on a sense of value
and purpose in one’s activities (Nunn, 1996). This result
is consistent with a number of previous studies that
have found hope to be an important resilience factor
for parents in enduring the demands of childhood
chronic conditions (Horton & Wallander, 2001; Mednick
et al., 2007).
The association of parent resolution with stress
symptomology appears to be relatively independent of
other coping processes, as indicated by the exploratory
mediation analysis. Escape-Avoidance coping by mothers
was the only coping variable found to partially mediate
the influence of resolution on stress symptomology. This
suggests that coping strategies may not be the mechanism
by which resolution is associated with parent and child
adjustment.
Language and cultural factors appeared to play a role
in mothers’ adjustment, as mothers of non-English speaking background tended to report more stress symptoms.
This might relate to perceptions of PKU, the cultural
significance of the diet, or difficulties accessing support
services. However, this result needs to be treated with
caution given the small sample sizes and without further
evidence of the reliability of the MI with parents of nonEnglish speaking background. Fathers’ level of stress
symptoms was more strongly associated with their occupation level than cultural or language factors. This might
represent a variety of influences, including self-appraisal,
income, and occupational stress.
The models predicting child adjustment examined
parent resolution and other coping variables as predictors
of child behavior problems. It should first be noted that
90% of children were functioning in the normal range,
and the percentage of children with clinical levels of
behavior disturbance was similar to that in the general
community (Achenbach, Hensley, Phares, & Grayson,
1990). This suggests that metabolic control was satisfactory for most of the children, as the neuro-toxic effects of
high phenylalanine (PHE) levels can give rise to problems
in intellectual and behavioral functioning (Sullivan &
Chang, 1999). Resolved mothers tended to report fewer
child behavior problems. However, resolution classification accounted for only 7% of variance in mothers’ CBCL
Problem Scale scores, and the trend was weaker in
the fathers’ data. The parent’s level of personal hopefulness (GPH) was a stronger predictor of child behavior
problems reported by both mothers and fathers. As well
as supporting personal resilience, hopefulness may contribute to more effective parenting and more positive
perceptions of the child’s behavior. Fathers’ engagement
in the management of their child’s condition was also
highlighted, as fathers who relied on Escape-Avoidance
coping tended to report more child behavior problems.
It is important to recognize that the wide developmental range of the children and the reliance on parent-report measures represent limitations to the study. For
parents, the challenges of managing PKU change as the
863
864
Lord, Ungerer, and Wastell
child develops, and lack of parental resolution may be
reflected in different behavioral issues at different developmental stages. In early childhood, for example, the
challenges include helping the child accept the special
formula, restricted diet, and blood testing. Through middle
childhood, managing the PKU diet in the context of school
and peer relationships becomes important. As resolution
appears strongly linked to attachment style (Marvin &
Pianta, 1996), the implications of parent resolution for the
child’s behavioral adjustment warrant further exploration.
Future studies should also be based on assessment of more
specific behavioral issues associated with the condition,
such as eating or demanding prohibited foods and refusal to
take the prescribed dietary formula. It is also important
to note that the cross-sectional design and the small sample
size limit the interpretation and generalization of the
findings. The outcomes examined may have also been
influenced by other variables, such as the parent’s appraisal
of PKU and the support available through the partner and
social networks. Nevertheless, the study adds to the limited
available data on adjustment to PKU.
The results have implications for intervention with
families. This study highlights the importance of acknowledging the emotional impact of the diagnosis on parents and
supporting hopefulness by recognizing positive aspects of
the child’s condition. Pediatricians and dieticians in PKU
clinics need to be aware of indicators of lack of resolution,
such as persistent distress, avoidance of discussion of
the diagnosis and related problems, and a continuing
preoccupation with the cause or reason for the child’s
condition. An understanding of these issues will assist
clinicians in referring parents appropriately for psychosocial
support. Ideally, psychological assessment of parent reactions to the diagnosis should be an integrated component
of clinical care. Although the RDI is not suitable for routine
use in clinical settings, due to the recording and coding
required, the interview can be readily adapted for incorporation into psychosocial assessments with parents.
Parents assessed as unresolved should be offered counseling
aimed at relieving diagnosis related grief and promoting
balanced expectations of the child’s future (Ormerod &
Huebner, 1988). An important avenue for future research is
the evaluation of counseling and other supportive interventions in assisting unresolved parents in coming to terms
with the diagnosis.
Conflicts of interest: None declared.
Received April 21, 2007; revisions received February 19,
2008; accepted February 20, 2008
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