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C 2002) Journal of Clinical Psychology in Medical Settings, Vol. 9, No. 3, September 2002 (°
Treatment Adherence in Children With Sickle Cell Disease: Disease-Related Risk and Psychosocial Resistance Factors1 Lamia P. Barakat,2,4 Kim Smith-Whitley,3 and Kwaku Ohene-Frempong3
To identify disease-related risk factors and psychosocial resistance factors that impact adherence to prescribed treatment in the context of admission to a Hematology Acute Care Unit (HACU) designed to provide acute care for children with sickle cell disease (SCD) presenting with pain or fever. A total of 73 primary caregivers and 24 children (if age 8 or older) completed standardized forms during the HACU admission. Treatment adherence variables (medical staff rating, SCD-related care activities, percentage of agreement between treatment recommendations made and care activities, and attendance at hematology clinic) indicated moderate-to-high adherence. Based on regression analyses, the risk variable of disease-related stress and the resistance variables of family flexibility and less reliance on passive coping accounted for significant portions of the variance in treatment adherence. Empirical evaluation of interventions designed to improve communication regarding expectations for the care of children with SCD and to support active family problem solving during times of SCD-related stress must be ongoing. KEY WORDS: treatment adherence; pediatric sickle cell disease.
exacerbations of anemia, retarded growth, splenic changes, and strokes (Hurtig, Koepke, & Park, 1989; Morgan & Jackson, 1986). Treatment may involve administration of analgesic medication at home, during outpatient visits or hospitalizations, prophylactic antibiotics to reduce susceptibility to infections, folic acid supplementation to support red cell production, regular follow-up, early identification and treatment of symptoms, and blood transfusions. On average, school-age children with SCD experience at least one to two pain episodes a month, and one to two hospital admissions and/or emergency department visits a year (Hurtig & White, 1986). The unpredictable nature of SCD complications and their treatment, including frequent hospitalizations and school absenteeism, potentially disrupt psychosocial development and threaten quality of life (Lemanek, Buckloh, Woods, & Butler, 1995). Treatment adherence in pediatric populations has received attention in the literature in light of the findings that nonadherence to prescribed treatment for chronic conditions has been reported as between
Sickle cell disease (SCD) is a group of inherited, chronic, hematological disorders in which sickle hemoglobin predominates. The incidence of SCD in the United States is 1 in every 400–500 live births for the African American population in which SCD is most prevalent. SCD is characterized by hemolytic anemia, vasoocclusion, and chronic organ damage. Complications are unpredictable and include recurrent episodes of severe pain in the lower extremities, back, abdomen, and chest, pneumococcal infections, 1 Portions
of this paper were presented at the 24th annual meeting of the National Sickle Cell Program, Philadelphia, PA, and the 8th Florida Conference on Child Health Psychology, Gainesville, FL. 2 Department of Psychology, Drexel University, Philadelphia, Pennsylvania. 3 Division of Hematology, Comprehensive Sickle Cell Center, The Children’s Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania. 4 Correspondence should be addressed to Lamia P. Barakat, Department of Psychology, Drexel University, 3141 Chestnut Street, Philadelphia, Pennsylvania 19104; e-mail: lamia.p.barakat@ drexel.edu.
201 C 2002 Plenum Publishing Corporation 1068-9583/02/0900-0201/0 °
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202 11 and 89% in pediatric populations (Dunbar, 1983). Despite serious consequences of nonadherence to prescribed medical regimens for children with SCD (vaso-occlusive episodes, life-threatening infection, stroke), treatment adherence of these children has not been well studied. It has been suggested that children with SCD, particularly as they reach adolescence, are at risk for nonadherence because of stresses related to their ethnic minority status in the United States, the burden of poverty that disproportionately affects African Americans, and the increased risk of internalizing behavior problems and academic problems related to SCD (Baskin et al., 1998). Examination of treatment adherence is hindered by difficulty in developing standard, reliable, and valid measures (La Greca & Schuman, 1995; Parrish, 1987). Most researchers agree that treatment adherence lies on a continuum with variation depending on the behaviors involved in adherence to complex regimens for chronic pediatric conditions. As a result, the range of adherence measures used in the previous literature is broad and includes ratings made by health care providers, parent and child report in interviews, selfmonitoring, pill counts, biological indices, and health status. Each method has its strengths and weaknesses, for example, parent, child, and health care provider reports and pill counts tend to overestimate adherence, objective measures such as pill counts do not account for the many skills and behaviors involved in adherence, and biological indices and health status reflect many factors other than adherence. For SCD in children, measuring adherence is complicated further by the nature of the disease and its treatment: (1) treatment recommendations vary depending on prior occurrence of SCD symptoms and overall severity of the illness, such as in the case of transfusion therapy in the presence of or risk for stroke; (2) standard recommendations are typically limited to cases involving the management of pain or fever at home and in the hospital; (3) some treatment recommendations (e.g., rest when fatigued, remain well-hydrated) are considered preventive, do not require high levels of skill and training, and are highly subjective; (4) use of prescription medication is required primarily to treat infection associated with fever or for prophylactic purposes for younger children; otherwise, use of prescription medication is variable and used for some instances of pain and for treatment on an experimental basis, that is, hydroxyurea, and (5) many families of children with SCD seek treatment in emergency rooms leading to a lack of consistency in health care providers, lack of
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Barakat, Smith-Whitley, and Ohene-Frempong adequate information about the disease, and its treatment and mistrust of the health care system (Hurtig & Viera, 1986). Christiaanse, Lavigne, and Lerner (1989) contended that treatment adherence may be viewed as one aspect of adjustment to chronic illness, and the association between factors related to adjustment and those related to adherence has been supported in the general pediatric literature. We will use the risk-resistance adaptation model (Wallander, Varni, Babani, Banis, & Wilcox, 1988) as a guide in outlining factors associated with treatment adherence as this model has proven useful in identifying factors associated with adaptation in children and their parents with SCD (Brown, Doepke, & Kaslow, 1993). Wallander et al. (1988) suggest that condition parameters, including severity of the condition, functional independence, and stress related to the condition are risk factors that impact adaptation. The evidence is mixed regarding the role of these risk factors in adaptation of children with SCD specifically, but there is some evidence to suggest that stress associated with SCD impacts the adaptation of children and their mothers (Brown et al., 2000; Casey, Brown, & Bakeman, 2000; Thompson, Gustafson, Gil, Kinney, & Spock, 1999). In the general pediatric literature, illness severity has not been reported as a predictor of adherence, however disease chronicity and treatment complexity have (La Greca & Schuman, 1995). Regarding the proposed resistance factors of stress processing (coping), social–ecological factors (family functioning), and intrapersonal factors (competence; Wallander et al., 1988), there is evidence that less use of disengagement coping, more flexible and cohesive family functioning, and child competence impact the adaptation of children with SCD and their parents with some exceptions (Brown et al., 2000; Casey et al., 2000; Hurtig & Park, 1989; Thompson et al., 1999). Regarding adherence, Gill, Williams, Thompson, and Kinney (1991) found, in a sample of 72 children and adolescents with SCD, that child and parent use of passive coping strategies was associated with less activity and greater use of health care services during pain episodes. Conversely, use of active coping by parents and children was associated with greater activity and less health care service use. Treatment adherence has also been associated with parental knowledge of SCD (Day, Brunson, & Wang, 1992). In the general pediatric literature, child adaptive coping strategies (Jacobson et al., 1990), child adjustment (Christiaanse et al., 1989; Korsch, Fine, & Negrete, 1978), parental knowledge
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Treatment Adherence in Children With Sickle Cell Disease of the illness (Bender, Milgrom, Rand, & Ackerson, 1998), parental problem-solving skills (Fehrenbach & Peterson, 1989), and family functioning (Bender et al., 1998) have been related to greater adherence. The primary goals of this study were twofold. First, clarification of the definition and assessment of treatment adherence in SCD was addressed by utilizing four measures aimed at assessing adherence level and adherence behaviors. Second, working within the risk-resistance adaptation model, we identified disease-related risk factors and psychosocial resistance factors that were expected to predict adherence to prescribed treatment in the context of admission to the Hematology Acute Care Unit (HACU) for pain or fever. The HACU was designed to provide intensive education and follow-up to families of children with SCD in order to improve adherence to recommendations for management of pain and fever and parent and child quality of life through shorter admissions and telephone follow-up following admission. Conducting the assessment of child and parent coping with SCD during a stressful SCD-related situation (pain or fever and admission) allowed for more ecologically valid measures of these variables. The following hypotheses were made based on the literature: (1) the risk factors of disease severity and diseaserelated stress would be associated with poorer adherence and (2) psychosocial resistance factors (parental knowledge of SCD, child and parent coping, child and parent adjustment, and family functioning) were expected to be associated with greater adherence to treatment recommendations.
METHODS Participant Recruitment Parent(s) accompanying each child (birth through 18 years) or 18-year-old adolescents presenting without a primary caregiver admitted to the HACU for fever or pain were approached regarding participation. Forms were completed by the parent(s) and the children themselves (if age 8 years or older) with the assistance of a supervised graduate research assistant. Of the over 500 children followed actively by The Comprehensive Sickle Cell Center at a major pediatric hospital, approximately 2 are admitted daily to the HACU. Of 104 families who agreed to participate and signed the informed consent form, 81 (77.9%) completed the study. All participants had at least one prior admission to the HACU in the past
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203 year. Twenty-three families signed consent, were unable to complete the questionnaires while the child was admitted to the HACU (because of demands of medical intervention and external family demands), and did not return the forms by mail. Twenty-eight families refused participation at the time of initial contact. Reasons for refusal included lack of interest and requests for the research assistant to return when families were more rested and less busy (when assistant returned, families had been discharged). The overall participation rate was 61% (81/132). Measures Demographic variables were obtained by completion of a General Information Form by the primary caregiver. Knowledge of SCD and its treatment was assessed through a rating from 1 (not at all) to 7 (extremely) to the question, “How comfortable do you feel with your knowledge of your child’s SCD and how to care for SCD-related problems?” Diseaserelated variables were obtained through review of patients’ medical files; the past year was the time frame. File review for each patient was completed at minimum 3 weeks after HACU discharge. The following disease-related variables were used: SCD genotype to reflect disease severity (as is commonly used in the literature, e.g., Brown et al., 2000) and number of pain episodes, number of HACU admissions, and number of telephone calls to clinic to reflect disease-related stress. The KIDCOPE (Spirito, Stark, & Williams, 1988) is a 15-item screening measure assessing 10 common cognitive and behavioral coping strategies: distraction, social withdrawal, cognitive restructuring, self-criticism, blaming others, problem solving, emotional regulation, wishful thinking, social support, and resignation. It was developed for use with children with chronic illness. There are two forms, one for children (ages 7–12 years) and one for adolescents (ages 13–18 years). Children were asked to respond to the coping items on the basis of how they were dealing with their current fever or pain and the resulting HACU admission. Active and passive coping subscales were formed based on recommendations made in the scale manual. The COPE (Carver, Scheier, & Weintraub, 1989) is a 60-item scale that assesses the degree to which parents use coping strategies scored along 13 theoretically and empirically derived scales describing problem-focused (e.g., planning), emotionfocused (e.g., positive reinterpretation), and less
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204 functional (e.g., behavioral disengagement) coping styles. Validity and reliability are adequate. Parents responded to the coping items on the basis of how they were dealing with their child’s current fever or pain and the resulting HACU admission. Active and passive coping subscales were formed based on recommendations in the scale manual. The McMaster Family Assessment Device (FAD; Epstein, Baldwin, & Bishop, 1983) is a 53-item, Likert-type measure that assesses family functioning across seven scales: General Functioning, Problem Solving, Communication, Roles, Affective Responsiveness (expression of positive emotion), Affective Involvement, and Behavior Control. It was designed for use as a screening instrument to indicate problems in family functioning. There is evidence of adequate validity and reliability (Miller, Epstein, Bishop, & Keitner, 1985). All scales were used. Children and parents completed this scale. General psychological adjustment for children with SCD was assessed with the Child Behavior Checklist (CBCL). The CBCL (Achenbach, 1991) assesses social competence and behavior problems of children aged 4–18 as reported by parents or other caregivers. The CBCL has considerable evidence for its reliability and validity in both clinical and nonclinical populations. Scores based on parent report on the Total Behavior Problems Scale were used. The Brief Symptom Inventory (BSI; Derogatis & Melisaratos, 1983) is a 53-item self-report measure assessing the presence and the intensity of symptoms relating to psychopathology in the parent. The BSI has been shown to have good reliability and validity with adult populations (Tennen, Affleck, & Herzberger, 1984). The Global Severity Index was used as an estimate of parent adjustment. Treatment adherence was measured in four ways, using medical staff report, parent report, and documentation of attendance of follow-up clinic to gain a broad representation of adherence recommendations and behaviors specific to SCD and to reflect the individualized nature of treatment recommendations for each patient: (1) Medical staff rating of overall adherence. The nurse practitioner responsible for each participant in the HACU rated overall treatment adherence on a 7-point Likert-type scale from 1 (not all adherent) to 7 (extremely adherent). Information required to make this rating was gathered during routine telephone follow-up with families in the week following the HACU admission during which forms were completed. This measure was considered the most subjective, prone to overestimating adherence
Barakat, Smith-Whitley, and Ohene-Frempong (La Greca & Schuman, 1995). (2) Number of SCDrelated care and treatment activities engaged in by families as reported by the primary caregiver on the General Information Form. Thirteen categories of activities were formed to reflect the open-ended responses provided by primary caregivers to the question, “Please list all activities engaged in by you and your child as part of your child’s sickle cell disease care and treatment.” Categories included attending hematology clinic for follow-up appointments, other medical visits for routine care, pain management, provision of medication as required, daily hydration, taking patient to hospital/contacting clinic when ill or in pain, information seeking/education. (3) Percentage of agreement between medical staff report of recommendations for SCD treatment (noted on same form with rating of overall adherence) and primary caregiver report of SCD-related activities engaged in by families was computed by examining overlap in responses after they were placed in the categories noted in SCD-related care and treatment activities. (4) Attendance at follow-up clinic in the past year as noted in medical chart and obtained through file review. For the four adherence variables, higher scores reflected greater adherence. Procedure All parent(s) of children with SCD or 18-yearold adolescents with SCD admitted to the HACU for fever or pain were identified by medical staff of the HACU and approached by a graduate research assistant to explain the study and obtain consent/assent. Following consent/assent, the graduate research assistant administered in interview format the questionnaires, first to the parents and then to the child. It took approximately 1.5 h to complete the questionnaires. If the child or parent or both were unable to complete the questionnaires because of severity of the current illness or demands of treatment, they were asked to complete them at home and return them by mail in a postage-paid return envelope. No forms were completed in this manner. Data were collected from October 1998 through September 2000. The protocol met approval of the appropriate Institutional Review Boards. Data Analyses In preliminary analyses, to identify demographic variables to include in the regression analyses, correlation coefficients (Pearson and Spearman as
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Treatment Adherence in Children With Sickle Cell Disease appropriate) were used to examine the association of demographic (child age, child sex, and family income) and treatment adherence variables. These demographic variables were chosen based on the extensive literature supporting their association with adaptation in samples of children with SCD (see Baskin et al., 1998, for a review). Also, correlation coefficients were computed for disease-related risk factors and psychosocial resistance variables with treatment adherence as outlined in Hypotheses 1 and 2. Following this, multiple regression equations were constructed (with the measures of treatment adherence as the dependent variables) to assess the contribution of the demographic, disease-related risk, and psychosocial resistance variables to explaining the variance in adherence. In a manner consistent with prior research on adherence (Ricker, Delamater, & Hsu, 1998) and with children with SCD (Gill et al., 1991), the independent variables were included based on significant findings in the correlation analyses, and demographic variables were entered on the first step, disease-related risk factors on the second step, and psychosocial resistance variables on the final steps (in this order: coping, adjustment, and family functioning). Power to detect a medium effect given p < .05, the sample size, and the regression design approached .80.
RESULTS Participants Of the 81 participants, 8 were 18-year-old patients and 73 were primary caregivers (63 mothers, 2 female guardians, 7 fathers, and 1 male guardian; see Table I). Additionally, 16 children older than 8 years and younger than 18 years completed forms. Fiftyeight percent of the children with SCD were male; the mean age for children was 7.54 years (SD = 5.49, range = newborn to 18). Most of the participants (89%) were African American. Family income was less than $49,000 for 68.9% of families, and 2.8% had family income over $100,000. Almost half the mothers (43.6%) had graduated from high school, 35.9% had some college/vocational school, and 16.7% had graduated from college. Findings were similar for fathers. Only 16.2% of families did not own or have access to a car. For SCD genotype used as a measure of severity: SCD-SS (63.7%), SCD-SC (26.3%), SCD-SB0 thal (10%), SCD complications reflected disease-related
205 Table I. Demographic and Disease-Related Variables Variable Child Age (years) Sex (male) Grade Ethnicity African American Latino Other Mother Age (years) Education 9–12th grade Some college/ vocational school College graduate Professional/graduate school Father Age (years) Education 9–12th grade Some college/ vocational school College graduate Professional/graduate school Income
% of sample
M
SD
Range
7.54
5.49
0.39–18.97
5.15
5.42
0–12
33.72
7.98
18–50
34.14 12.46
20–75
58.0
88.8 1.2 10.0
43.6 35.9 16.7 3.8
51.3 23.7 22.4 2.6
31.2 37.8 24.3 6.7 83.8 16.2 63.7 26.3 10.0 3.00
2.49
1–17
3.18
3.59
0–19
3.49 4.62 5.51 12.40
0–28 1–7
stress and were distributed as follows. Mean number of pain episodes in the past year was 3.49 (SD = 4.62, range = 0–28). Mean number of telephone calls to clinic in the past year was 3.18 (SD = 3.59, range = 0–19). Mean number of inpatient admissions in the past year was 5.10 (SD = 4.32, range = 1–23); of these there was a mean of 3.00 for HACU admissions (SD = 2.49, range = 1–17). SCD knowledge had a mean rating of 5.51 (SD = 12.40, range 1–7).
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206 Treatment Adherence The mean total number of SCD-related care and treatment activities reported by primary caregivers was 3.18 (SD = 1.25, range = 1–7). The most common activities reported were medication provision as required (84.8%), hematology clinic follow-up appointments (72.2%), presenting to the hospital when patient was ill or in pain (49.4%), and active pain management (31.6%). The least reported activities were daily hydration (10.1%) and fever care (have thermometer and check temperature when fever evident; 8.9%). For percentage of agreement of staff recommendations with caregiver-reported SCD-related care activities, there was less than 50% agreement for 91.6% of the sample. In contrast, but as expected, medical staff gave a mean adherence rating of 5.32 (SD = 0.94) indicating moderate adherence. All but 4 participants (95.1%) attended hematology clinic in the past year; mean number of hematology outpatient clinic visits in the past year was 3.16 (SD = 1.82, range = 0–9). Because of the lack of variance in the attendance at clinic variable, it was not included in further analyses. Examination of the correlations among the adherence variables indicated that SCD-related care activities were significantly associated with percentage of agreement, r = .67, p = .000. This significant correlation was expected as the SCD-related care activities variable was used in computing percentage of agreement. Demographic Variables and Treatment Adherence Family income was significantly associated with medical staff rating (r = .28, p = .023) and showed a trend to significance with percentage of agreement (r = −.20, p = .10). No significant associations were found for child age or child sex and the adherence variables. Disease-Related Variables and Treatment Adherence No significant correlations were found for SCD severity with treatment adherence. For disease-related stress due to complications, HACU admissions were significantly associated with SCDrelated care activities (r = .37, p = .001) and percentage of agreement (r = .24, p = .040). Pain episodes (r = .29, p = .009) and number of phone calls to hematology clinic in the past year (r = .24, p = .037)
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Barakat, Smith-Whitley, and Ohene-Frempong were significantly associated with SCD-related care activities.
Psychosocial Variables and Treatment Adherence SCD knowledge was significantly associated with medical staff rating (r = .25, p = .035) in the expected direction. The parent passive coping subscale was significantly negatively associated with SCD-related care activities (r = −.26, p = .030) and approached significance with percentage of agreement (r = −.21, p = .086). An association of parent and child active coping with better adherence was not supported. For the family variables, the following significant correlations were found: parent FAD problem solving with medical staff adherence rating (r = .28, p = .026) and parent FAD behavior control with SCD-related care activities (r = −.25, p = .036). Child and parent general psychological functioning was not significantly associated with the adherence variables; only child total behavior problems showed a trend to significance in its correlation with percentage of agreement (r = .27, p = .092).
Prediction of Treatment Adherence Based on results of the correlation analyses, linear regression equations were computed for medical staff rating and SCD-related care activities (see Table II). A regression equation was not computed for percentage of agreement because only the
Table II. Regression Analyses for Demographic, Disease-Related, and Psychosocial Variables With Treatment Adherence Adherence variables
Total R2
df
F ratio
Medical staff adherence rating Family income SCD knowledge Parent FAD problem solving SCD-related care activities HACU admissions Pain episodes No. of phone calls to clinic Parent passive coping Parent FAD behavior control
.196
3, 63
4.88
∗p
< .01. ∗∗ p < .05.
β
p .004∗
.225 .059 .256 .032∗∗ .249 .037∗∗ .209
5, 70
3.43
.008∗ .266 .161 .122 .455 −.051 .742 −.218 .071 −.125 .296
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Treatment Adherence in Children With Sickle Cell Disease HACU admissions variable was significantly associated with it. For medical staff rating, family income was forced to enter on the first step, SCD knowledge on the second step, and parent FAD problem solving on the third step with the total model showing significance, F(3, 63) = 4.88, p = .004. Family income contributed to the explanation of 4% of the variance in medical staff rating, which approached significance ( p = .053). SCD knowledge ( p = .032) and parent FAD problem solving ( p = .037) accounted for significant proportions of the variance in medical staff rating. Greater knowledge of SCD and treatment on the part of the primary caregiver and higher ratings of family effort at solving family problems were associated with higher medical staff ratings of adherence. For SCD-related care activities, the full model with disease-related stress (number of HACU admissions in the past year, pain episodes in the past year, and phone calls to clinic in the past year) entered on the first step, parent passive coping entered on the second step, and parent FAD behavior control entered on the third step, was significant, F(5, 70) = 3.43, p = .008. The stress variables ( p = .020) and parent passive coping ( p = .031) accounted for significant proportions of the variance in SCDrelated care activities. Parent FAD behavior control did not account for a significant proportion of the variance in SCD-related care activities. Examination of Beta for each variable indicated that HACU admissions showed the strongest association with SCDrelated care activities with more admissions contributing to more reported activities. Parent passive coping also showed a strong but negative association with higher use of passive strategies associated with fewer reported activities. DISCUSSION Examination of the findings provides partial support for the expectation that disease-related risk factors and psychosocial resistance variables would contribute to understanding treatment adherence in children with SCD and their families. Although disease severity was not associated with adherence, other disease-related risk factors, particularly frequency of HACU admissions, were associated with treatment adherence with the unexpected finding of more HACU admissions related to greater adherence in terms of SCD-related care activities and percentage of agreement between medical recommendations and activities undertaken. In addition, resistance factors
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207 reported by parents were shown to play an important role in adherence in this sample. Family problemsolving ability, which measures effort to solve and follow up on family problems, and lower reliance on passive coping strategies by the parent were the resistance factors associated with greater adherence as measured by medical staff rating and SCD-related care activities. Despite these important findings supportive of the application of the risk-resistance adaptation model to understanding treatment adherence in children with SCD, a number of resistance factors (child report variables, child and parent active coping, child and parent general functioning) were not correlated significantly with adherence. Disease-related risk factors, parent coping, and family functioning were associated with adherence with some variation in findings across measures of adherence, pointing to the challenge of defining and measuring adherence in SCD in an objective and consistent manner. This issue is of relevance with the high demands associated with new and more frequently used treatments for SCD, such as chronic transfusion, hydroxyurea, and bone marrow transplant. In this study, notations made in the patient’s chart independent of the study as well as matching specific and standard recommendations made to each patient with SCD-related care activities engaged in by the family were used. This approach had the advantage of taking into account patient-specific variations in recommendations in SCD (often depending on the age of the child and illness-risk factors) as well as standard recommendations associated with pain and fever. Findings indicate that families are adherent to concrete recommendations such as regular followup care in hematology clinic, yet adherence seemed to be less strong for recommendations that are less concrete (maintain hydration) or require on-the-spot decision-making (if fever, take temperature with thermometer). Because there was no objective, observational measure of the extent to which families actually provided prescribed medication, the skill and consistency with which they engaged in pain management, and the extent to which fever and pain symptoms were attended to at home, future research will need to develop more objective and efficient, patient and disease-specific measures of adherence. The lack of findings for disease severity is consistent with recently published reports (Brown et al., 2000; Casey et al., 2000) and suggests that there may be problems with using genotype to reflect disease severity. In our comprehensive sickle cell center, pain, fever, and other SCD complications are managed
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208 in the same manner, with a standard set of treatment recommendations, regardless of genotype so that children with SCD with similar presenting symptoms receive the same education regarding the treatment of SCD. Therefore, genotype may be less relevant to adherence than disease-related complications (stress). Of relevance to the goal of intensive education in the HACU and other acute care settings are findings regarding increased contact with medical staff and its association with adherence. SCD knowledge was associated with higher medical staff ratings of adherence, which is consistent with the expectation that the more one understands an illness and the relevance of specific recommendations, the more likely one is to engage in adherence behaviors (Bender et al., 1998; Boroffice, 1992). However, knowledge alone may not be sufficient to maintain adherence. The finding that more HACU admissions were associated with greater adherence may provide a link between knowledge and adherence. It may be that children with more disease-related stress (reflected in more frequent pain episodes, HACU admissions, and phone calls to clinic) have greater contact with medical staff and thus, more opportunities for education and reminders that support adherence. This supposition was supported with a positive and significant association between SCD knowledge and HACU admissions. In addition, families of children with more disease-related stress may believe they have a great deal to gain from adherence, whereas families of children with few symptoms may not perceive the importance of treatment recommendations aimed at prevention of SCD symptoms. This interpretation of the findings coalesces with the health beliefs model, which posits that perceived susceptibility to illness or illness symptoms, perceived severity of symptoms, and costs (perceived barriers) versus benefits of engaging in treatment interact to predict adherence (Janz & Becker, 1984). The weakness of the psychosocial variables in consistently explaining the variance in adherence is perplexing and counterintuitive based on the prior literature (see La Greca & Schuman, 1995, for a review). Some psychosocial variables were significantly correlated with adherence in the expected direction; better family problem solving and less reliance on passive coping strategies by parents were associated with better adherence suggesting parent coping and family functioning as appropriate targets for interventions to improve adherence. Nonetheless, the psychosocial variables did not always maintain their predictive power when disease-related variables were entered first into the regression equations and many were not
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Barakat, Smith-Whitley, and Ohene-Frempong correlated initially. For the child-reported variables, the sample was small enough (n = 24) that power may have been an issue; however, this critique is not relevant to the parent data. The scales have been used with minority samples previously, but they may not be adequately standardized with minority groups and therefore do not assess relevant psychosocial aspects, even though families were asked to respond in the framework of SCD. In the case of SCD, issues related to ethnic minority status interacting with the medical system and disease-specific concerns may outweigh the role of personal adjustment and family functioning in understanding adherence. The association of family income with adherence supports this interpretation. Obstacles to data collection may point to unidentified factors that impact adherence, and certainly representativeness of the sample must be considered. Few caregivers remained in the hospital throughout their child’s HACU admission because of work and family requirements. They often stayed with their child long enough for the admission and then returned at the time of discharge. Although this indicates some trust in the quality of nursing care their child received, it suggests also a perception that engagement in the medical admission of their child need not be the priority. Moreover, these families had a number of stressful life circumstances associated with their urban dwelling environment. Although this might explain the low-to-moderate adherence found for some measures for this sample, it is possible that these were actually the most adherent families (relative to others) as they were the ones who made themselves available to medical staff in the HACU and therefore were present and willing to participate in this study. Finally, the alliance among families and medical staff has been shown to impact adherence to prescribed treatment (Gavin, Wamboldt, Sorokin, Levy, & Wamboldt, 1999). Given findings of the importance of contact with medical staff in this sample of children with SCD, for future research, it will be important to examine the ways in which demographic and disease-related variables impact the development of treatment alliance and how treatment alliance may expand our understanding of how to improve adherence to treatment in SCD.
ACKNOWLEDGMENTS The authors thank the following students who contributed to data collection and analysis:
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Treatment Adherence in Children With Sickle Cell Disease Nilmini Klur, Meredith Lutz, Reem Tarazi, Suzanne Braniecki, Marissa Carey, and Jennifer Hetzke. REFERENCES Achenbach, T. M. (1991). Manual for the Child Behavior Checklist/ 4-18 and 1991 profile. Burlington, VT: University of Vermont, Department of Psychiatry. Baskin, M. L., Collins, M. H., Brown, F., Griffith, J. R., Samuels, D., Moody, A., et al. (1998). Psychosocial considerations in sickle cell disease (SCD): The transition from adolescence to young adulthood. Journal of Clinical Psychology in Medical Settings, 5, 315–341. Bender, B., Milgrom, H., Rand, C., & Ackerson, L. (1998). Psychological factors associated with medication nonadherence in asthmatic children. Journal of Asthma, 35, 347–353. Boroffice, O. B. (1992). Fostering medical adherence in some Nigerian sickle cell disease patients. Journal of Applied Rehabilitation Counseling, 23, 33–37. Brown, R. T., Doepke, K. J., & Kaslow, N. J. (1993). Risk-resistance adaptation model for pediatric chronic illness: Sickle cell syndrome as an example. Clinical Psychology Review, 13, 119–132. Brown, R. T., Lambert, R., Devine, D., Baldwin, K., Casey, R., Doepke, K., et al. (2000). Risk-resistance adaptation model for caregivers and their children with sickle cell syndromes. Annals of Behavioral Medicine, 22, 158–169. Carver, C. S., Scheier, M. F., & Weintraub, J. K. (1989). Assessing coping strategies: A theoretically based approach. Journal of Personality and Social Psychology, 56, 267–283. Casey, R., Brown, R. T., & Bakeman, R. (2000). Predicting adjustment in children and adolescents with sickle cell disease: A test of the risk-resistance—adaptation model. Rehabilitation Psychology, 45, 155–178. Christiaanse, M. E., Lavigne, J. V., & Lerner, C. V. (1989). Psychosocial aspects of compliance in children and adolescents with asthma. Journal of Developmental and Behavioral Pediatrics, 10, 75–80. Day, S., Bruson, G., & Wang, W. (1992). A successful education program for parents of infants with newly diagnosed sickle cell disease. Journal of Pediatric Nursing, 7, 52–57. Derogatis, L. R., & Melisaratos, N. (1983). The Brief Symptom Inventory: An introductory report. Psychological Medicine, 13, 595–605. Dunbar, J. M. (1983). Compliance in pediatric populations: A review. In P. J. McGrath & P. Firestone (Eds.), Pediatric and adolescent behavioral medicine (pp. 210–230). New York: Springer. Epstein, N. B., Baldwin, L. M., & Bishop, D. S. (1983). The McMaster Family Assessment Device. Journal of Marital and Family Therapy, 9, 171–180. Fehrenbach, A. M. B., & Peterson, L. (1989). Parental problemsolving skills, stress, and dietary compliance in phenykletonuria. Journal of Consulting and Clinical Psychology, 57, 237–241. Gavin, L. A., Wamboldt, M. Z., Sorokin, N., Levy, S. Y., & Wamboldt, F. S. (1999). Treatment alliance and its association with family functioning, adherence, and medical outcome in adolescents with severe, chronic asthma. Journal of Pediatric Psychology, 24, 355–365.
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Style file version June 24th, 2002
209 Gill, K. M., Williams, D. A., Thompson, R. J., & Kinney, T. R. (1991). Sickle cell disease in children and adolescents: The relation of child and parent pain coping strategies to adjustment. Journal of Pediatric Psychology, 16, 643–663. Hurtig, A. L., Koepke, D., & Park, K. B. (1989). Relation between severity of chronic illness and adjustment in children and adolescents with sickle cell anemia. Journal of Pediatric Psychology, 14, 117–132. Hurtig, A. L., & Park, K. B. (1989). Adjustment and coping in adolescents with sickle cell disease. Annals of New York Academy of Sciences, 565, 172–182. Hurtig, A. L., & Viera, C. T. (1986). Sickle cell disease: Psychological and psychosocial issues. Chicago: University of Illinois Press. Hurtig, A. L., & White, L. S. (1986). Psychosocial adjustment in children and adolescents with sickle cell disease. Journal of Pediatric Psychology, 11, 411–427. Jacobson, A. M., Hauser, S. T., Lavori, P., Wolfsdorf, J. I., Herskowitz, R. D., Milley, J. E., et al. (1990). Adherence among children and adolescents with insulin-dependent diabetes mellitus over a four-year longitudinal follow-up: I. The influence of patient coping and adjustment. Journal of Pediatric Psychology, 15, 511–526. Janz, N. K., & Becker, M. H. (1984). The health belief model: A decade later. Health Education Quarterly, 11, 1–47. Korsch, B. M., Fine, R. N., & Negrete, V. F. (1978). Noncompliance in children with renal transplants. Pediatrics, 61, 872–876. La Greca, A. M., & Schuman, W. B. (1995). Adherence to prescribed medical regimens. In M. C. Roberts (Ed.), Handbook of pediatric psychology (2nd ed.). New York: Guilford Press. Lemanek, K. L., Buckloh, L. M., Woods, G., & Butler, R. (1995). Diseases of the circulatory system: Sickle cell disease and hemophilia. In M. C. Roberts (Ed.), Handbook of pediatric psychology (2nd ed.). New York: Guilford Press. Miller, I. W., Epstein, N. B., Bishop, D. S., & Keitner, G. I. (1985). The McMaster Family Assessment Device: Reliability and validity. Journal of Marital and Family Therapy, 11, 345–356. Morgan, S. A., & Jackson, J. (1986). Psychological and social concomitants of sickle cell anemia in adolescents. Journal of Pediatric Psychology, 11, 429–440. Parrish, J. M. (1987). Parent compliance with medical and behavioral recommendations. In N. A. Krasnegor, J. D. Arasteh, & M. F. Cataldo (Eds.), Child health behavior: A behavioral pediatrics perspective (pp. 453–501). New York: Wiley. Ricker, J. H., Delamater, A. M., & Hsu, J. (1998). Correlates of regimen adherence in cystic fibrosis. Journal of Clinical Psychology in Medical Settings, 5, 159–172. Spirito, A., Stark, L. J., & Williams, C. (1988). Development of a brief coping checklist for use with pediatric populations. Journal of Pediatric Psychology, 13, 555–574. Tennen, H., Affleck, G., & Herzberger, S. (1984). SCL-90-R. In D. J. Keyser & R. C. Sweetland (Eds.), Test critiques (Vol. 3, pp. 583–594). Kansas City, MO: Test Corporation of America. Thompson, R. J., Jr., Gustafson, K. E., Gil, K. M., Kinney, T. R., & Spock, A. (1999). Change in psychological adjustment of children with cystic fibrosis or sickle cell disease and their mothers. Journal of Clinical Psychology in Medical Settings, 6, 373–392. Wallander, J. L., Varni, J. W., Babani, L., Banis, H. T., & Wilcox, K. T. (1988). Family resources as resistance factors for psychological maladjustment in chronically ill and handicapped children. Journal of Pediatric Psychology, 14, 157–173.
