A Case Study: Acceptance and Commitment Therapy for Pediatric
Sickle Cell Disease
Akihiko Masuda,1 PHD, Lindsey L. Cohen,1 PHD, Rikard K. Wicksell,2 PHD, Mike K. Kemani,2 MSC,
and Alcuin Johnson,3 PHD
1
Department of Psychology, Georgia State University, 2Behavior Medicine Pain Treatment Service, Karolinska
Institutet, and 3Aflac Cancer Center and Blood Disorders Services, Children’s Healthcare of Atlanta
All correspondence concerning this article should be addressed to Lindsey L. Cohen, PhD, Department of
Psychology, Georgia State University, Atlanta, GA, 30302, USA. E-mail: [email protected]
Received June 14, 2010; revisions received December 7, 2010; accepted December 8, 2010
Objective Sickle cell disease (SCD) negatively impacts patients’ functioning and quality of life. Acceptance
and Commitment Therapy (ACT) promotes acceptance of difficult sensations, emotions, and thoughts
when doing so facilitates living a values-based life. This study describes ACT for improving functioning and
quality of life for an adolescent with SCD and his parents. Methods A 16-year old with SCD and his parents attended an eight-session ACT program. Process (adolescent psychological flexibility, parent acceptance)
and outcome (adolescent social anxiety, pain, functioning, quality of life; parent distress) measures were
conducted prior to and following treatment and at 3-month follow-up. Results and Conclusions
Improvements were evident, especially at follow-up. Process measures suggest adolescent psychological flexibility and parent acceptance might explain positive effects. Anecdotal comments support these findings and
provide additional evidence that ACT might effectively promote functioning and quality of life in adolescents
with chronic diseases.
Key words
acceptance; commitment therapy; parents; sickle cell disease.
Sickle cell disease (SCD) is an inherited disorder of the
hemoglobin in red blood cells, affecting primarily people
of African descent. In the United States, 1 out of every 600
African-American newborns has SCD and approximately
72,000 people are currently living with the disease
[National Heart, Lung, and Blood Institute (NHLBI),
2006]. Despite advances in medical and psychosocial treatment, there is no cure for SCD. Medical complications of
SCD are common, including chronic pain, pulmonary and
cardiac problems, neurocognitive deficits, and stunted
growth (e.g., Edwards et al., 2005). Of those, unpredictable and severe pain is the most common reason for hospitalizations for patients with SCD (Woods et al., 1997).
SCD is found to be associated with a host of negative outcomes, such as functional disability (e.g., frequent medical
visits, school absences, poor peer relationships; Gil et al.,
2000; Palermo, Schwartz, Drotar, & McGowan, 2002),
negative affect (Alao & Cooley, 2001), social anxiety
(Wagner et al., 2004), and diminished quality of life
(QOL; Panepinto, Mahar, DeBaun, Rennie, & Scott, 2004).
The parents of pediatric patients with SCD are also
subject to greater psychological distress (Brown et al.,
1993; Wallander & Varni, 1998). This is in part due to
adjustments and sacrifices associated with parenting a
youth with SCD, such as missed days of work and neglected self-care. Considering the parents’ well-being is important in its own right and also because parents play a
significant role in pediatric patient care (Kazak, Rourke, &
Navsaria, 2009; Wicksell, 2007).
Given the multifaceted impact of the disease, pediatric
patients with SCD are typically treated in a multidisciplinary setting receiving a combination of medical and psychosocial interventions (e.g., Forseth & Gran, 2002). The
psychosocial strategies, such as relaxation, coping skills
Journal of Pediatric Psychology 36(4) pp. 398–408, 2011
doi:10.1093/jpepsy/jsq118
Advance Access publication February 15, 2011
Journal of Pediatric Psychology vol. 36 no. 4 ß The Author 2011. Published by Oxford University Press on behalf of the Society of Pediatric Psychology.
All rights reserved. For permissions, please e-mail: [email protected]
ACT for adolescent with SCD
training, and social support typically focus on symptom
management and treatment adherence (Chen, Cole, &
Kato, 2004). Unfortunately, literature reviews suggest
that these approaches provide varying success in symptom
management and treatment compliance, and that they do
not effectively address poor daily functioning and QOL of
patients or their parents (Anie & Green, 2002, updated
2009; Chen et al., 2004; La Greca & Bearman, 2001).
It should be noted that symptom management and adherence, when excessive and exclusive, might actually interfere
with the daily functioning (e.g., peer interactions) and
QOL of pediatric patients with SCD (Barakat, Lutz,
Smith-Whitley, & Ohene-Frempong, 2005).
Acceptance and Commitment Therapy (ACT, Hayes,
Strosahl, & Wilson, 1999) might be particularly effective
for youth with SCD. ACT emphasizes the promotion of
daily functioning and QOL while teaching a willingness
to experience difficult and possibly unavoidable private
events (e.g., pain, discomfort, fatigue, anxiety) without defense when doing so serves valued ends (Hayes, Luoma,
Bond, Masuda, & Lillis, 2006). Based on its behavioral
roots, ACT posits that functional disability and diminished
QOL is typically characterized by the domination of inflexible attempts to modify the form, frequency, or situational
sensitivities of unwanted private events (e.g., anxiety,
pain). For example, a pediatric patient with SCD might
be taught to engage in (e.g., hydrate) or avoid (playing
outside on a cold day) in order to minimize SCD symptoms. However, if these behaviors become dominant, excessive, or rigid, they might interfere with daily functioning
(making a friend, socializing with peers, studying, having
part-time work). Behaviorally, many of these negatively
reinforced behaviors can be rule-governed, which are
under the stimulus control of aversive private events
(e.g., fatigue) and verbal rules associated with these
events (e.g., ‘‘I can’t do anything when I am unmotivated.’’). It should be noted that these behaviors can be
reinforced by others (e.g., parents, physicians). In ACT, the
maladaptive behavioral pattern of attempting to alter the
form, frequency, or situational sensitivity of private events
(e.g., thoughts, emotions) are generally called experiential
avoidance (EA), and the stimulus control of verbal antecedents that excessively or improperly regulate EA are called
cognitive fusion (Hayes et al., 2006).
ACT does not negate the importance of symptoms
management (e.g., hydration, healthy diet, regular exercise,
medication adherence) in treatment of pediatric SCD.
Rather, ACT simply intervenes on the processes (e.g., EA
and cognitive fusion) when these lead to long term detrimental effects (e.g., interference of daily functioning) for
the individual. ACT posits that discomfort and pain might
be unavoidable and persistent in some contexts, and that
experiencing them openly without defense and engaging in
value-consistent behaviors are likely to promote greater
functioning and QOL. Thus, symptom management is important when it leads to living a more flexible and ultimately more meaningful life; however, symptom
management is not the end-goal and some forms of symptom management might in fact interfere with living a
values-based life.
Preliminary evidence indicates that ACT promotes
health behaviors and constructive living among adults
with a wide range of behavioral and health concerns
(Hayes et al., 2006), including Type II diabetes (Gregg,
Callaghan, Hayes, & Glenn-Lawson, 2007), epilepsy
(Lundgren, Dahl, Melin, & Kies, 2006), and idiopathic
pain (McCracken, Vowles, & Eccleston, 2005). ACT
might be beneficial to pediatric patients and their parents
(see Greco & Hayes, 2008), but research in this area is
sparse. In fact, Greco and Hayes (2008) write that ACT
for children and adolescents ‘‘has only recently begun to
be explored’’ (p. 4). A review of the literature reveals
one case study indicating that ACT is effective for an adolescent with anorexia (Heffner, Sperry, Eifert, &
Detweiler, 2004), a case study of ACT for an adolescent’s
chronic pain (Wicksell, Dahl, Magnusson, & Olsson,
2005), a small pilot study of ACT for 14 adolescents
with idiopathic chronic pain (Wicksell, Melin, &
Olsson, 2007), a randomized trial supporting ACT for adolescents with complex chronic pain (Wicksell, Melin,
Lekander, & Olsson, 2009), and a small sample within
subjects design study demonstrating that ACT is beneficial
for parents of children with autism (Blackledge & Hayes,
2006). Given these preliminary data that ACT can benefit
parents as well as adolescents with chronic conditions
as well as the importance of involving the entire family
system in children’s health care (e.g., Kazak et al., 2009),
a family-based ACT intervention might be particularly
useful to children struggling with the management of a
chronic illness.
Case studies allow researchers to highlight new
approaches for clinical populations in medical settings
(Drotar, 2009). Thus, the purpose of this case study
was to describe and obtain preliminary data for familycentered ACT therapy tailored to the needs of an adolescent with SCD and his parents. It was expected that ACT
would help improve the QOL and functioning of the patient and parents. Process measures were employed to explore whether ACT processes (e.g., psychological
flexibility) might be associated with any beneficial changes
in therapy.
399
400
Masuda et al.
Case Description
Participants and Setting
Appropriate institutional approval was obtained prior to
the initiation of the study. The study was conducted at
Aflac Cancer Center and Blood Disorders Service of
Children’s Healthcare of Atlanta (CHOA), which is one
of the largest centers in the country annually treating
over 1,600 pediatric patients with SCD. The two
ACT-trained cotherapists (AM, LC) presented the ACT
model to the SCD medical treatment team, who approved
of this therapeutic approach.
To protect the family’s confidentiality, the identifying
information was altered. The primary patients were ‘‘David
Smith,’’ a 16-year-old African American adolescent male
diagnosed with sickle-cell anemia (HbSS) since birth, and
his parents, ‘‘Mr and Mrs Smith.’’ Given work conflicts, Mr
Smith could only attend the third and final two sessions.
The family was referred to CHOA psychology because
of ongoing psychosocial concerns raised by Mr and Mrs
Smith, which included the patient having frequent complaints of pain and fatigue, apprehension about socializing
with peers, and poor study habits and grades. The patient
had not responded to prior approaches to these issues
(e.g., relaxation training for pain control). The medical
treatment team informed the therapists that the family
tends to minimize difficulties, especially with unfamiliar
people. The ACT approach appeared especially well-suited
to this patient because: (a) David did not respond to initial
therapeutic interventions (e.g., relaxation); (b) the physicians reported that acceptance would be a good approach
given that a number of David’s symptoms (e.g., fatigue,
pain) could not be further mitigated via available medical
interventions (e.g., medications); and (c) he reportedly was
engaging in EA (e.g., he tries to sleep when he feels pain)
and cognitive fusion (e.g., he stated, ‘‘Why should I ask my
teacher for help? She won’t be able to help me.’’).
David was a junior in high school, living with his parents and his older sister, who took classes at a local college.
The annual family income was approximately $75,000.
David and Mrs Smith attended the initial intake session.
David was reserved and reported that he had no difficulties
other than the SCD symptoms of pain and fatigue. Mrs
Smith reported that her primary concern was that David
was ‘‘irresponsible’’ and ‘‘too relaxed,’’ especially around
his schoolwork. She reported the recent deterioration of his
academic performance (i.e., ‘‘C’’ average) and said that she
had to ‘‘stay on top of him’’ to complete his homework.
According to Mrs Smith, David usually reported fatigue
when he came home from school and he often took a
long nap (e.g., 2 hr) before dinner. After waking and
having dinner, David typically watched TV or played on
the computer. David also reported poor sleep hygiene,
and he often stayed up past midnight. Mrs Smith had secondary concerns regarding David’s minimal peer relationships, and his low energy and fatigue. Mr Smith said that
he suspected that David’s low energy and motivation were
not related to sickle cell fatigue symptoms, and that David
needed to ‘‘take more responsibility for his life.’’
David’s presenting concerns were characterized by the
behavioral deficits of adaptive behaviors, such as studying,
socializing with peers, and having a part-time job combined
with the behavioral excesses of inattentiveness and inaction
(e.g., taking a long nap after school, playing on the computer). These inactions were hypothesized to be subtle
forms of EA (e.g., avoidance of the distress associated
with doing schoolwork) and related to cognitive fusion
(e.g., ‘‘If I am tired, I cannot do any homework’’), Given
David’s and his parents’ reports, the low levels of these
appropriate behaviors were likely to be due to a maladaptive stimulus control, rather than skills deficits. Finally, it
appeared that Mr and Mrs Smith unknowingly reinforced
the absence of proactive behaviors (e.g., not doing homework until told by Mrs Smith) and inactions.
Measures
Measures were administered 1 week prior to the beginning
of therapy (pre-treatment), 1 week following the last session (posttreatment), and 3 months following the last session (3-month follow-up). Assessment focused on both
process (David’s psychological flexibility; Mrs Smith’s acceptance) and outcome (David’s social anxiety, pain, functioning, and quality of life; Mrs Smith’s distress) indices.
Psychological Inflexibility
David completed the Avoidance and Fusion Questionnaire
for Youth (AFQ-Y; Greco, Lambert, & Baer, 2008), a
17-item questionnaire that assesses psychological inflexibility. The scale measures the degree of being fused with
the content of private events (e.g., ‘‘The bad things I think
about myself must be true’’), EA (e.g., ‘‘I push away
thoughts and feelings that I don’t like’’), and inaction or
behavioral ineffectiveness in the presence of unwanted internal experiences (e.g., ‘‘I can’t be a good friend when I
feel upset’’). Scores range from 0 to 68 with higher scores
indicating higher psychological inflexibility. The AFQ-Y has
good internal consistency with Cronbach’s coefficient a’s
ranging from .89 to .91 and has demonstrated validity via
associations in predicted directions with other measures of
similar processes, such as acceptance, mindfulness, and
thought suppression (Greco et al., 2008).
ACT for adolescent with SCD
Social Anxiety
David completed the Social Anxiety Scale for
Children-Revised (SASC-R, La Greca & Lopez, 1998; La
Greca & Stone, 1993), a 22-item measure of anxiety in
adolescents in the context of social interactions. The
scale uses a 5-point Likert scale, ranging from 1 (Not at
all) to 5 (All of the time). Eighteen items are used for a
composite score, ranging from 18 to 90. A study with pediatric patients with SCD (Wagner et al., 2004) revealed
internal consistency to be high for the composite score
(Cronbach’s a ¼ .86).
Pain
David and his mother completed the Varni/Thomson
Pediatric Pain Questionnaire (PPQ; Varni, Thompson, &
Hanson, 1987). Using the anchor from 0 (no pain) to 100
(a whole a lot of pain), the measure assesses the experiences
of current pain, worst pain, and average pain over a week.
The PPQ has been shown to be both a reliable and valid
measure of pediatric patient (Cohen et al., 2008).
Functional Disability
David and Mrs Smith (proxy report) completed the
Functional Disability Inventory (FDI; Walker & Greene,
1991) to measure David’s functioning. The measure contains 15 items that assess interference with daily activities
in youths with chronic pain, using a scale of 0 (no trouble)
to 4 (impossible), and greater scores suggest greater functional disability. The FDI has sound psychometrics
(Palermo et al., 2008) and has been shown to be internally
consistent with a population of adolescents with chronic
pain and their parents (a ¼ .85–.93) (Cohen, Vowles, &
Eccleston, 2010).
Quality of Life
The PedsQL (Varni, Seid, & Kurtin, 2001) was used to
measure David’s self-reported QOL. The PedsQL contains
23 items assessing a child’s functioning in physical (eight
items), emotional (five items), social (five items), and
school (five items) domains. Using a 5-point Likert scale,
with 0 indicating ‘‘never a problem’’ and 4 indicating
‘‘almost always a problem,’’ each subscale assesses how
much of a problem the child has had over the previous 1
month in these specific functional domains. The responses
for each item are reverse scored. The domain and total
scores are derived from summing the items and linearly
transformed to a 0–100 scale with greater scores reflecting
better QOL in specific domains. The PedsQL is a psychometrically sound instrument (Palermo et al., 2008) and has
been found to be valid and reliable with pediatric patients
with SCD (McClellan, Schatz, Sanchez, & Roberts, 2008;
Dampier et al., 2010).
Parent Acceptance of Child Chronic Illness
Mrs Smith completed the Parent Acceptance of Pediatric
Illness Questionnaire (PAPIQ), which was developed for
the study to measure parents’ acceptance of the child’s
chronic condition (e.g., ‘‘Despite my child’s illness, we
are able to do things that are important to us’’). The
PAPIQ is a 31-item self-report measure, which is based
on the Chronic Pain Acceptance Questionnaire (CPAQ;
McCracken, Vowles, & Eccleston, 2004). In the present
study, the items of CPAQ, which are designed to measure
an individual’s acceptance of one’s own pain experience,
were modified to reflect the degree to which a caretaker is
willing to accept the child’s chronic illness and also pursue
value-directed actions for him/herself and promote this in
the child. Conceptually, psychological acceptance is construed as the inverse of avoidance and cognitive fusion
(Hayes et al., 2006). Diminished acceptance is suggestive
of greater cognitive fusion (‘‘My child can live a good and
happy life only when his/her illness and symptoms are
controlled’’) and avoidance (e.g., ‘‘I try to avoid the difficult feelings that are related to my child’s illness’’). Using a
7-point Likert-scale ranging from 0 (never true) to 6 (always
true) for each item, participants’ responses to the 31 items
were summed, with greater scores indicating greater acceptance of the child’s chronic condition.
Parent Psychological Distress
The Brief Symptom Inventory 18 (BSI-18; Derogatis, 2000)
was used to measure Mrs Smith’s psychological distress.
The BSI-18 contains 18 items and employs a 5-point Likert
scale ranging from 0 (not at all) to 4 (extremely). The global
severity index score is derived from the sum of all item
scores, ranging from 0 to 72 with greater scores suggesting
greater psychological distress. The BSI has been shown to
be a reliable and valid instrument (Derogatis, 2000).
Acceptance and Commitment Therapy
Six processes of change are theorized to be at the core of
ACT (Hayes et al., 2006): acceptance of private events
(experiencing those events willingly and without defense),
cognitive defusion from the literal content of thoughts (not
necessarily believing them or acting on them), present
moment awareness of one’s experience, a sense of
self-as-context or perspective, clarification and induction
of valued directions for life, and committed action (building patterns of overt behavior in valued directions).
The treatment protocol was largely drawn from existing ACT manuals (e.g., Hayes & Smith, 2005).
401
402
Masuda et al.
Modifications were made to reflect issues specific to SCD
and the present case. Therapy consisted of eight 60-min
consecutive weekly family sessions. As mentioned above,
David and Mrs Smith attended all eight sessions and Mr
Smith attended the third and final sessions. Each session
typically started with a 5–10 min review of David’s daily
activities and medical concerns and the parent(s) concerns
about David and their own lives. ACT-specific content
(e.g., acceptance, mindfulness, and values clarification)
was typically introduced in a didactic fashion early in the
session and then used to address the family’s issues, with
this aspect of therapy spanning 30–40 min. During a few
sessions, David and the parent(s) were seen separately
when particularly personal issues were discussed. The
final 10–15 min of the sessions focused on helping David
and his parents set specific values-based goals.
Implementation and rationale for the four overarching
ACT intervention components are described below.
Consistent with the extant ACT literature, therapy was tentatively planned for eight sessions. As the eight session
approached, there appeared to be sufficient improvements
to warrant termination at that point.
Perspective Modification
Given the model of psychological flexibility, it was crucial
for David and his parents to shift their perspective from
excessive and exclusive attempts to avoid and control
unwanted psychological events (e.g., pain, discomfort, fatigue, anxiety) to values-focused living. This was a primary
focus during the early sessions. To help shift their perspective, the family discussed the inevitable, often unpredictable, and pervasive nature of SCD symptoms (e.g., pain,
fatigue) despite careful symptoms management efforts.
This was followed by a discussion of the futility and
costs (e.g., missed school; missed peer interactions) in
trying to live a ‘‘pain-free’’ and ‘‘stress-free’’ life. The discussion was particularly useful for Mrs Smith, who had
strong negative reactions to David’s experiences of SCD
symptoms and made great effort to reduce them (e.g., encouraging him to nap when fatigued). Behaviorally, goals of
this phase were to undermine the behavior regulatory function of rules associated with EA and cognitive fusion in the
context of SCD symptoms and associated events so that
alternative behaviors, such as value-consistent behaviors,
were likely to occur.
David and his parent(s) also briefly identified constructive and values-consistent activities (e.g., playing
with friends and studying for David; self-care for Mr and
Mrs Smith) that were inhibited by inaction occasioned by
SCD symptoms and associated private events (e.g., fatigue,
lack of motivation). Finally, the pursuit of value consistent
and vital living despite the presence of SCD symptoms in
some degree was established as the goals of therapy rather
than focusing on symptom control.
In the present case, we found it helpful to use a visual
illustration. Specifically, we drew a continuum line, which
indicated that David has a choice to focus his energy in
living a ‘‘stress-free’’ and ‘‘safe’’ life (e.g., staying home
from school when fatigued, avoiding being with friends
when experiencing pain) or a ‘‘vital’’ and ‘‘valuesconsistent’’ but ‘‘higher risk’’ life (e.g., accepting that
studying despite fatigue is important in achieving the
goals of a high school degree and consistent with the
value of education and learning, asking a girl on a date
despite the risk of rejection). We acknowledged that
some symptom reduction behavior (e.g., hydration)
would help him to move in the direction of living a vital
life. The exercise was modified from an ACT intervention
for pediatric patients with chronic pain (Wicksell, 2007,
pp. 14–15). As suggested by Wicksell (2007), the figure
was drawn collaboratively in session. Importantly, the aim
of the visual illustration was not immediate behavior
change, but to increase awareness of values-based living
and encourage motivation for engaging in values-congruent
behaviors. Another aim was to shift the families focus from
avoidance of negative psychological events to engaging in
values-based living in the presence of unavoidable distress
(e.g., pain, fatigue).
Values Clarification
Values were emphasized in Sessions 3 and 4 in order to
firmly establish ACT-congruent treatment goals. David and
his parents separately completed values-clarification exercises (e.g., projecting what they would like their life to be
like in 5, 10, 15, and 20 years), which are similar to activities described in ACT manuals (e.g., Hayes & Smith,
2005). In addition, the family identified emotional and
physiological obstacles that might interfere with
values-consistent activities (e.g., anxiety, pain, and fatigue
for David; fear of David’s getting sick or doing poorly in
school for Mr and Mrs Smith).
In order to promote the parent(s)’ value-consistent
behavior in the area of raising David, as well as more individual domains (e.g., hobbies), the values exercise with the
parents started with the validation of the emotional challenges and struggles with raising David (worry about his
future). Subsequently, the exercise explored the domain of
parenting by identifying their own desires for David’s
future (e.g., being autonomous, honest, independent,
caring, self-disciplined). The exercise allowed them to clarify the underlying values (e.g., being supportive of David)
and value-consistent actions they could take to promote
ACT for adolescent with SCD
David’s autonomy and personal growth. In the context of
these discussions, David and his parents seemed more
open to sharing their own personal challenges with the
therapists.
The values-clarification exercise helped David and his
parents agree upon concrete target behaviors and the arrangement of contingencies to promote the behaviors in
the context of family interaction. For example, David and
therapists discussed ways in which David might engage in
desirable activities (e.g., playing on the computer, drawing
pictures) and also behavior that would lead to future
values-based goals (obtaining better grades in school; attending college), such as playing 10 min of a computer
game or drawing for 10 min as a reward after completing
30 min of homework. Mr and Mrs Smith also discussed
ways of promoting David’s values-consistent behaviors
and independence (e.g., not repeatedly checking on this
homework progress; rewarding his good grades in school).
Acceptance and Mindfulness
The stance of acceptance and mindfulness was used
throughout all sessions, but emphasized in Sessions 2
and 5. Several exercises were implemented to facilitate
the family engaging in values-consistent behavior despite
SCD symptoms. For example, David identified that his
parents’ prompting and checking on his homework progress lead to him feeling ‘‘annoyed,’’ discouraged, and disempowered. In response, Mrs Smith said that she felt
anxious that David would not do his work if she did not
remind him and monitor his behavior. David and his
mother were encouraged to practice increasing their awareness of these thoughts and emotions—to experience these
private events—without acting on or trying to
change them, and to simultaneously engage in
values-based behavior (i.e., independent homework completion for David and encouraging independence and responsibility by not prompting or checking on David for
Mr and Mrs Smith). Behaviorally, the goals of acceptance
and mindfulness exercises were to alter the EA regulatory
function of their private events (e.g., thoughts, perceived
SCD symptoms) so that other behaviors, including
values-consistent activities, flexibly occurred in the presence of these events.
This same approach of encouraging acceptance of difficult thoughts, feelings, and physical sensations and mindfully engaging in behavior grounded in values was used in
other areas. For example, David was able to experience the
anxiety and reservation about applying for a job (which had
stymied him in the past) while simultaneously applying
and securing a job, which was consistent with his goals
of obtaining work to increase his social activity, strengthen
his college applications, and provide financial support for
other socializing. Another example is when David asked a
teacher for help while having the thoughts that she would
not be able to help him and that she would think he was
stupid and experiencing anxiety and apprehension. His
behavior was reinforced as the teacher provided supportive
assistance in the subject matter.
Commitment to Values-directed Life
Committing to values-directed living was infused throughout therapy, but highlighted in Sessions 5 through 8. This
was predominately accomplished by repeatedly identifying
David’s and the parent(s)’ values (e.g., education, rewarding profession, independence) and linking these values to
distal and proximal concrete goals (e.g., studying to improve grades, obtain a job). The psychological and physical
challenges (e.g., uncertainty, fear, pain) experienced by the
family when establishing behavioral goals were continuously discussed to normalize the experience, encourage acceptance, and place the primary focus on values-consistent
living.
At this point in the therapy, the family was encouraged
to revisit their thoughts and concerns about SCD and its
symptoms (‘‘I can’t stand this,’’ ‘‘I can’t pursue what I
want to be because of SCD’’) and to allow themselves to
have these thoughts without trying to alter or act on them.
These private events were revisited and discussed repeatedly and especially toward the end of therapy because they
were likely to remain and could interfere with daily functioning. The family was encouraged to continue to notice
these thoughts but to behave and live in accord with their
identified values, such as academic learning and socializing
with peers.
Results
The results of the present clinical case suggested that social
anxiety and pain scores remained relatively unchanged
from pre- to posttreatment (Table I). Given that therapy
did not directly target these areas, this is not surprising.
However, in ACT it is common to see a reduction in anxiety and pain—as a byproduct—when the patient is encouraged to willingly accept these difficult experiences and
instead focus efforts on living a more vital life (e.g., Vowles
& McCracke, 2008). Further, this type of change is often
slow to occur and not found until 6–12 months posttreatment (e.g., Hayes et al., 2006). There was preliminary evidence of this pattern in the 3-month follow-up reduction
in social anxiety (Table I). Comparing his scores to other
samples, David’s pre- and postintervention scores of social
anxiety fell within the average range and his follow-up
403
404
Masuda et al.
Table I. Scores of Study Variables
Pretreatment
Posttreatment
3-month follow-up
Adolescent self-report
Psychological inflexibility (AFQ-Y)
Social anxiety (SAS-A)
35
34
29
34
10
24
Pain (PPQ)
Current
0
0
0
Worst
20
0
65
Average
15
10
10
8
5
3
64.1
53.1
67.4
65.6
87.8
87.5
FDI
Quality of life (PedsQL)
Overall
Health and activities
Feelings
70.0
65.0
100.0
Getting along with others
80.0
75.0
95.0
School
60.0
65
55.0
Mother proxy report
Child’s pain (PPQ)
Current
1
0
40
Worst
Average
55
40
50
50
65
65
Child’s FDI
36
7
18
61
76
77
4
2
0
Mother self-report
Acceptance of child’s chronic illness (PAPIQ)
Psychological distress (BSI-18)
score was >1 SD below the mean of the anxiety scores for
another sample of pediatric patients with SCD (Wagner et
al., 2004). This change was supported by the family’s statements at the 3-month follow-up that David had become
more social, that he was spending more time with friends,
and that his attendance at school had improved. At
3-month follow-up, David remarked that he still noticed
difficult thoughts and emotions in the area of social and
peer interaction but that he did not let them dictate his
behavior. Given the unpredictable and pervasive nature of
SCD pain, it is not surprising to see fluctuating pain scores.
Scores on self-report measures completed by David
and his parents reflected improvement in David’s daily
functioning and quality of life (QOL) (Table I). The improvement was particularly salient at 3-month follow-up.
At postintervention, the mother reported ‘‘a little trouble’’
range of disability on average, followed by ‘‘a little trouble’’
and ‘‘some trouble’’ range of disability at 3-month
follow-up. While perceived greater disability in some
areas of activities remained unchanged throughout the
study, such as ‘‘walking the length of a football field’’
and ‘‘running the length of a football field,’’ a salient improvement was reported in other areas, such as peer relationships (i.e., ‘‘doing something with a friend’’), self-care
(e.g., ‘‘walking to a bathroom’’), and academic activities
(i.e., ‘‘being at school all day’’). This was consistent with
the foci of therapy on social and academic behavior.
Similarly, the improvement of David’s self-reported
QOL scores was observed at the 3-month follow-up. Preand posttreatment overall QOL scores of 64.1 and 67.4
were comparable with the 65.8 (SD ¼ 17.3) average score
found in a sample of 68 children and adolescents with SCD
(McClellan et al., 2008). However, David’s follow-up score
of 87.8 suggested a substantial improvement in QOL; this
score was >1 SD above the comparison sample. He had
similar improvements in QOL subscale scores (i.e., health
and activities, affect, and peer relationship) at the 3-month
follow-up.
Although David’s score on the QOL academic activities subscale was only slightly improved at 3-month
follow-up, David and his parents reported improvement
in grades from a ‘‘C average’’ to an ‘‘A average,’’ both
through the 8-week course of therapy and compared to
his grades the prior semester. In fact, David’s overall
GPA improved from a pre-treatment 2.8 to a posttreatment
3.1. At the posttreatment assessment and at the 3-month
follow-up, Mr and Mrs Smith remarked that this was one of
the most substantial changes, especially in light of the fact
ACT for adolescent with SCD
that they had discontinued prompting and checking on
him around homework completion.
Related to his goal of increasing independence, there
were a number of anecdotal data points. Specifically, David
obtained and maintained a job, began keeping a schedule
book to plan and monitor his homework, spoke to his
teachers more often for assistance, performed searches on
admissions criteria for colleges, and was beginning to
pursue obtaining a driver’s license. Along with these improvements, Mr and Mrs Smith continued to allow David
more freedom and reportedly had completely stopped
checking on his homework progress by the end of therapy.
It appeared that a positive feedback cycle had begun with
his parents providing more freedom and David demonstrating greater responsibility.
It is important to consider the mechanism of change in
treatment research, whether the study is of a single case or
a large group randomized controlled trial. In ACT, it is
assumed that acceptance and psychological flexibility
are key agents of change (Hayes et al., 1999). In this
case, David’s score on the AFQ (Table I) suggest that his
inflexible and avoidant behavioral pattern appeared to
be greater relative to his peers (Greco et al., 2008) at preand postintervention points. David’s psychological inflexibility, however, was found to substantially reduced at
3-month follow-up. Similarly, the increasing trend of Mrs
Smith’s PAPIQ scores from pre-treatment to follow-up suggests that she had increased her acceptance of David’s
medical condition and that her focus had shifted from
symptom management to encouraging David to have a
full and productive life with SCD symptoms. Mrs Smith’s
scores on the BSI-18 remained low, suggesting lower distress across the course of treatment. Whether this is a response bias or true perception of stress is difficult to
determine.
Although they should be interpreted tentatively, anecdotal comments recorded at the 3-month follow-up support the impact of ACT therapy. For example, Mr Smith
stated: ‘‘If you sit there just waiting for it to come [sickle
cell pain crises], you are just wasting your life away. Before
sessions, he waited for it, now he is living his life.’’
Regarding the commitment aspect of therapy, David said:
‘‘When I say something, I try to pursue it best I can. When
I make a promise, I try to keep it best I can.’’ Mr Smith said
that the ‘‘turning point’’ in therapy was when David ‘‘was
opening up, getting comfortable. He used to only talk to
his sister, now he is expressing himself to us . . . in front of
you [the therapists] . . . and we as parents had to take a look
at how we were doing things . . . we also learned some ways
to live from this too.’’
Discussion
The purpose of this case study was to evaluate a novel
approach for working with pediatric patients with SCD.
Specifically, the case report describes ACT for improving
the functioning and quality of life in an adolescent with
SCD and his parents. ACT is a behavioral treatment designed to directly enhance functioning and quality of life
via encouraging values-consistent living while willingly
experiencing difficult and often unavoidable internal experiences as they are without attempting to downregulate
them. The results of the case report suggest that ACT
might effectively promote functioning and quality of life
in adolescents with SCD and their parents and that it is
worthwhile to continue to investigate the application of
ACT to youths with SCD and the family.
It should be noted that the potential applications of
ACT are broad and that ACT might be applicable to youths
with a variety of chronic conditions. This is in part because
ACT is not an illness- or diagnosis-specific intervention.
Rather, the primary aim of ACT is to expand an individual’s
functioning and QOL regardless of the particular stressors,
difficulties, or situations. ACT teaches individuals to acknowledge, accept, and even embrace difficult private
evens (e.g., distress) if doing so facilitates living a life
that is closely tied to personally identified values (e.g.,
being a good friend). This conceptual and therapeutic
model of ACT can be applied to a wide range of pediatric
cases with diverse chronic illness. It is especially
well-suited to patients with chronic conditions because
chronic diseases often cannot be cured and there might
be ongoing or recurrent symptoms that cannot be controlled. In other words, accepting difficult and unchangeable facets of life while focusing on areas in need of
improvement is a framework that should resonate with
most any child struggling with a chronic condition.
A challenge of using an ACT-based approach with a
chronic illness is that the therapeutic stance acknowledges
that some symptoms will not remit or change. Families that
are waiting for a cure or a new treatment that will remedy a
chronic condition might experience this perspective as
asking them to give up hope. This is not the case. In
fact, ACT posits that the pediatric patient and the family
can have a vital and meaningful life with symptoms being
present; hoping for a new treatment would only be discouraged if this behavior interfered with quality of life. In
other words, the stance of ACT is in fact hopeful and encouraging. Another challenge of using ACT with adolescents is that it has been predominately developed and
used with adults and many concepts and exercises are abstract and sophisticated, often relying on metaphors, and
405
406
Masuda et al.
subtle uses of language. This is purposeful in that ACT
seeks to undermine some of the detrimental aspects of
language itself (Hayes et al., 1999). However, it will be
an ongoing challenge to modify this approach for younger
populations. Appropriate training in ACT can ameliorate
some of these challenges. Although there is no official credentialing body for ACT, competence might be acquired via
a variety of means, such as attending workshops, obtaining
supervision, and self-study. Additional information about
ACT training can be found on the Association for
Contextual Behavioral Science website (http://contextual
psychology.org/act).
Given that this is a case study, interpretations of
changes should be made cautiously. For example, therapy
might explain some of the positive changes, but other circumstances and factors (e.g., maturation of David, changes
in the school setting) might explain improvements. The
primary purpose of this study is to provide a description
of ACT therapy tailored to the difficulties experienced by
an adolescent with SCD and his parents.
In summary, this case study extends existing knowledge beyond what has been reported in the area of acceptance and mindfulness treatments for children and
adolescents, by suggesting that ACT intervention can be
a useful conceptual and therapeutic addition to the treatment of pediatric patients with SCD and the family. The
study provides preliminary but promising data that ACT is
a viable approach for improving functioning and QOL for
adolescents with SCD and their parents. The process measures suggest that the mechanism of change was the ACT
processes of increased youth psychological flexibility and
parent acceptance. As this is a case study, conclusions
about treatment effectiveness should be made cautiously.
Additional research is necessary to replicate these findings
across other individuals, examine the impact of the approach in a randomized trial, more closely highlight mechanism of change, identify the key ingredients of the
intervention, and determine which individuals and situations might be most appropriate for this treatment.
Although findings should be interpreted tentatively, this
report highlights a promising alternative approach to working with pediatric patients; rather than symptom control, a
focus on acceptance and vital living might prove a valuable
psychological method for improving functioning and quality of life in youth with chronic conditions. Given that
pediatric psychologists often help patients manage symptoms that are unresponsive to medical interventions, a
framework of enhancing functioning and focusing on
living a vital life with a chronic condition might prove to
be a promising mode of therapy.
Funding
International Association for the Study of Pain (IASP) and
the Scan|Design Foundation BY INGER & JENS BRUUN
awarded (to L. L. C. and R. K. W.).
Conflicts of interest: None declared.
References
Alao, A. O., & Cooley, E. (2001). Depression and sickle
cell disease. Harvard Review of Psychiatry, 9,
169–177.
Anie, K. A., & Green, J. (2002; updated 2009).
Psychological therapies for sickle cell disease and
pain. Cochrane Database of Systematic Reviews, 2,
Art. No.: CD001916.
Barakat, L. P., Lutz, M., Smith-Whitley, K., & OheneFrempong, K. (2005). Is treatment adherence associated with better quality of life in children with sickle
cell disease? Quality of Life Research, 14, 407–414.
Blackledge, J. T., & Hayes, S. C. (2006). Using acceptance and commitment training in the support of
parents of children diagnosed with autism. Child &
Family Behavior Therapy, 28, 1–18.
Brown, R. T., Kaslow, N. J., Doepke, K., Buchannon, I.,
Eckman, J., Baldwin, K., & Goonan, B. (1993).
Psychosocial and family functioning in children with
sickle cell syndrome and their mothers. Journal of the
American Academy of Child and Adolescent Psychiatry,
32, 545–553.
Chen, E., Cole, S. W., & Kato, P. M. (2004). A review of
empirically supported psychosocial interventions for
pain and adherence outcomes in sickle cell disease.
Journal of Pediatric Psychology, 29, 197–209.
Cohen, L. L., Lemanek, K., Blount, R. L., Dahlquist, L.
M., Lim, C. S., Palermo, T. M., . . . Weiss, K. E.
(2008). Evidence-based assessment of pediatric pain.
Journal of Pediatric Psychology, 33, 939–955.
Cohen, L. L., Vowles, K. E., & Eccleston, C. (2010).
Adolescent chronic pain-related functioning:
Concordance and discordance of mother-proxy and
self-report ratings. The European Journal of Pain, 14,
882–886.
Dampier, C., Lieff, S., LeBeau, P., Rhee, S.,
McMurray, M., Rogers, Z., . . . Wang, W. (2010).
Health-related quality of life in children with sickle
cell disease: A report from the comprehensive sickle
cell centers clinical trial consortium. Pediatric Blood
Cancer, 55, 485–494.
Derogatis, L. R., & Savitz, K. L. (2000). The SCL-90-R
and the brief symptom inventory (BSI) in primary care.
ACT for adolescent with SCD
In M. E. Maruish (Ed.), Handbook of Psychosocial
Assessment in Primary Care Settings (Vol. 236,
pp. 297–334). Mahwah: Lawrence Erlbaum
Associates.
Drotar, D. (2009). Editorial: Case studies and series: A
call for action and invitation for submissions. Journal
of Pediatric Psychology, 34, 795–802.
Edwards, L. E., Scales, M. T., Loughlin, C., Bennett, G.
G., Harris-Peterson, S., De Castro, L. M., . . .
Killough, A. (2005). A brief review of the pathology,
associated pain, and psychosocial issues in sickle cell
disease. International Journal of Behavioral Medicine,
12, 171–179.
Forseth, K. K., & Gran, J. T. (2002). Management of
fibromyalgia: What are the best treatment choices?
Drugs, 62, 577–592.
Gil, K. M., Porter, L., Ready, J., Workman, E.,
Sedway, J., & Anthony, K. K. (2000). Pain in children and adolescents with sickle cell disease: An
analysis of daily pain diaries. Children’s Health Care,
29, 225–241.
Greco, L., & Hayes, S. (2008). Acceptance and
Mindfulness Treatments for Children and Adolescents:
A Practitioner’s Guide. Oakland, CA: New Harbinger
Publications.
Greco, L. A., Lambert, W., & Baer, R. A. (2008).
Psychological inflexibility in childhood and adolescence: Development and evaluation of the Avoidance
and Fusion Questionnaire for Youth. Psychological
Assessment, 20, 93–102.
Gregg, J. A., Callaghan, G. M., Hayes, S. C., &
Glenn-Lawson, J. L. (2007). Improving diabetes
self-management through acceptance, mindfulness,
and values: A randomized controlled trial. Journal of
Consulting and Clinical Psychology, 75, 336–343.
Hayes, S. C., Luoma, J. B., Bond, F. W., Masuda, A., &
Lillis, J. (2006). Acceptance and commitment therapy: Model, processes, and outcomes. Behaviour
Research and Therapy, 44, 1–25.
Hayes, S. C., & Smith, S. (2005). Get out of your mind
and into your life: The new acceptance and commitment
therapy. Oakland, CA: New Harbinger.
Hayes, S. C., Strosahl, K. D., & Wilson, K. G. (1999).
Acceptance and Commitment Therapy: An experiential
approach to behavior change. New York: Guilford
Press.
Heffner, M., Sperry, J., Eifert, G. H., & Detweiler, M.
(2004). Acceptance and Commitment Therapy in the
treatment of an adolescent female with anorexia nervosa: A case example. Cognitive and Behavioral
Practice, 9, 232–236.
Kazak, A. E., Rourke, M. T., & Navsaria, N. (2009).
Families and other systems in pediatric psychology.
In M. C. Roberts, & R. G. Steele (Eds.), Handbook of
Pediatric Psychology (4th ed., pp. 656–671). New
York: Guilford Press.
La Greca, A. M., & Bearman, K. J. (2001). Commentary:
If ‘‘an apple a day keeps the doctor away,’’ why is
adherence so darn hard? Journal of Pediatric
Psychology, 26, 279–282.
La Greca, A. M., & Lopez, N. (1998). Social anxiety
among adolescents: Linkages with peer relations and
friendships. Journal of Abnormal Child Psychology, 26,
83–94.
La Greca, A. M., & Stone, W. L. (1993). The social anxiety scale for children-revised: Factor structure and
concurrent validity. Journal of Clinical Child
Psychology, 22, 17–27.
Lundgren, T., Dahl, J., Melin, L., & Kies, B. (2006).
Evaluation of acceptance and commitment therapy
for drug refractory epilepsy: A randomized controlled
trial in South Africa-A pilot study. Epilepsia, 47,
2173–2179.
McClellan, C. B., Schatz, J., Sanchez, C., & Roberts, C.
W. (2008). Validity of the pediatric quality of life inventory for youth with sickle cell disease. Journal of
Pediatric Psychology, 33, 1153–1162.
McCracken, L. M., Vowles, K. E., & Eccleston, C.
(2004). Acceptance of chronic pain: Component
analysis and a revised assessment method. Pain, 107,
159–166.
McCracken, L. M., Vowles, K. E., & Eccleston, C.
(2005). Acceptance-based treatment for persons with
complex, long standing chronic pain: A preliminary
analysis of treatment outcome in comparison to a
waiting phase. Behaviour Research and Therapy, 43,
1335–1346.
National Heart, Lung, and Blood Institute (2006).
Disease and Conditions Index. Sickle Cell Anemia.
Retrieved from: http://www.nhlbi.nih.gov/health/dci/
Diseases/Sca/SCA_WhatIs.html.
Palermo, T. M., Long, A. C., Lewandowski, A. S.,
Drotar, D., Quittner, A. L., & Walker, L. S. (2008).
Evidence-based assessment of health-related quality
of life and functional impairment in pediatric
psychology. Journal of Pediatric Psychology, 33,
983–996.
Palermo, T. M., Schwartz, L., Drotar, D., &
McGowan, K. (2002). Parental report of
health-related quality of life in children with sickle
cell disease. Journal of Behavioral Medicine, 25,
269–283.
407
408
Masuda et al.
Panepinto, J. A., O’Mahar, K. M., DeBaun, M. R.,
Rennie, K. M., & Scott, J. P. (2004). Validity of the
Child Health Questionnaire for use in children with
sickle cell disease. Journal of Pediatric Hematology and
Oncology, 26, 574–578.
Varni, J. W., Seid, M., & Kurtin, P. S. (2001). PedsQLTM
4.0: Reliability and validity of the pediatric quality of
life inventoryTM version 4.0 generic core scales in
healthy and patient populations. Medical Care, 39,
800–812.
Varni, J., Thompson, K., & Hanson, V. (1987). The
Varni/Thompson Pediatric Pain Questionnaire.
Chronic musculoskeletal pain in juvenile rheumatoid
arthritis. Pain, 28, 27–38.
Vowles, K. E., & McCracken, L. M. (2008). Acceptance
and value-based action in chronic pain: A study of
treatment effectiveness and process. Journal of
Consulting and Clinical Psychology, 76, 397–407.
Wagner, J. L., Connelly, M. A., Brown, R. T., Taylor, L.,
Rittle, C., & Cloues, B. (2004). Predictors of social
anxiety in children and adolescents with sickle cell
disease. Journal of Clinical Psychology in Medical
Settings, 11, 243–252.
Walker, L. S., & Greene, J. W. (1991). The Functional
Disability Inventory-measuring a neglected dimension
of child health-status. Journal of Pediatric Psychology,
16, 39–58.
Wallander, J. L., & Varni, J. W. (1998). Effects of pediatric chronic physical disorders on child and family adjustment. Journal of Child Psychology and Psychiatry,
39, 29–46.
Wicksell, R. K. (2007). Value-based exposure and acceptance in the treatment of pediatric chronic pain:
From symptom reduction to value living. Pediatric
Pain Letter, 9, 13–20.
Wicksell, R. K., Dahl, J., Magnusson, B., & Olsson, G. L.
(2005). Using acceptance and commitment therapy
in the rehabilitation of an adolescent female with
chronic pain: A case example. Cognitive and
Behavoural Practice, 12, 415–423.
Wicksell, R. K., Melin, L., Lekander, M., & Olsson, G. L.
(2009). Evaluating the effectiveness of exposure and
acceptance strategies to improve functioning and
quality of life in longstanding pediatric pain–A
randomized controlled trial. Pain, 141, 248–257.
Wicksell, R. K., Melin, L., & Olsson, G. L. (2007).
Exposure and acceptance in the rehabilitation of
adolescents with idiopathic chronic pain–a pilot
study. European Journal of Pain, 11, 267–274.
Woods, K., Karrison, T., Koshy, M., Patel, A.,
Friedman, P., & Cassel, C. (1997). Hospital
utilization patterns and costs for adult sickle
cell patients in Illinois. Public Health Reports, 112,
44–51.