Information-seeking coping behaviors during painful procedures in African-American children with sickle cell disease.

This study examined the frequency of information-seeking coping behaviors in 37 African-American children (ages 5-17 years) with sickle cell disease during venipuncture. The relationships between coping behaviors and child- and parent-reported pain and observational distress were also assessed. The majority of children attended to the procedure, but did not seek information via questions. This pattern of coping was only partially effective at reducing distress and had no relation to pain. This pattern of coping is discussed within the context of cultural factors that may be important in understanding responses to procedural pain in pediatric sickle cell disease.

Responsiveness of the PedsQL to pain-related changes in health-related quality of life in pediatric sickle cell disease.

OBJECTIVE: To determine if caregiver report of the pediatric quality of life inventory (PedsQL) is responsive to changes in health-related quality of life (HRQL) associated with pain episodes in pediatric sickle cell disease (SCD). METHODS: 81 caregivers of children ages 2-19 years with SCD completed the PedsQL as part of routine psychosocial screenings at 2 time points, ranging from 6 to 18 months apart. Frequency of SCD-related pain episodes between time points was assessed using medical chart review. RESULTS: The frequency of pain episodes between time points was a significant predictor of decreases in physical, psychosocial, and total HRQL, even after controlling for time interval, demographic, and medical variables. CONCLUSIONS: The caregiver report of the PedsQL appears to be a useful tool for capturing changes in HRQL over time associated with pain episodes in SCD.

Use of handheld wireless technology for a home-based sickle cell pain management protocol.

PURPOSE: To evaluate use of a handheld electronic wireless device to implement a pain management protocol for participants with sickle cell disease (SCD). METHODS: Participants were 19 patients with SCD aged 9-20 who experienced vaso-occlusive pain. A single-session training on the use of cognitive-behavioral coping skills was followed by instruction on how to practice these skills and monitor daily pain experience using the device. Daily pain experience and practice of coping skills were collected for the 8-week intervention period using wireless technology. RESULTS: High rates of participation, daily diary completion and consumer satisfaction support the use of handheld wireless devices to implement this protocol. A comparison of the rates of self and device-recorded skills practice provides important information about the use of electronic monitoring for behavioral interventions. CONCLUSION: Wireless data transfer technology has significant potential to become a practical method to improve symptom monitoring and communication between patients and providers.

Time out based discipline strategy for children's non-compliance with cystic fibrosis treatment.

PURPOSE: To evaluate a time out based discipline strategy designed to decrease treatment avoidance in children with cystic fibrosis (CF) who displayed non-compliant behaviour to their parents' treatment requests. METHOD: A single-subject reversal design was used to compare baseline compliance when a time out based discipline strategy was in effect. Outcome measures included observational assessments of compliance, parent ratings of child treatment-related behaviour problems and parent reported preference for the time out strategy. RESULTS: Greater compliance was demonstrated when the time out strategy was in place. Parents reported fewer treatment-related behaviour problems and desired less professional help for these problems upon completion of the study and reported preferring the time out discipline strategy to their typical child management strategy. CONCLUSIONS: This discipline strategy represents a practical and appealing brief intervention that is shown to help parents maximise their children's participation in life-extending treatments. This intervention minimises the need for parent-therapist interaction, which may be of particular importance to families of patients with CF who already devote significant time to medical appointments and interventions. The preliminary nature of this data and lack of reversal for one of the participants emphasise the need for additional research to replicate the findings.

Validity of the Pediatric Quality Of Life Inventory for youth with sickle cell disease.

OBJECTIVE: Evaluate the validity of the Pediatric Quality of Life Inventory (PedsQL) for sickle cell disease (SCD). METHODS: Sixty-eight parent-child dyads (children 5-18 years) completed the PedsQL. Medical record review assessed history of specific morbidities. RESULTS: Internal consistency of the scales varied. The strongest reliability was for parent proxy-report for specific domains or for global functioning scores with either informant. Modest internal consistency was found for specific domains with child informants, particularly for younger children. Moderate convergent validity was found between informants. History of neurologic problems or major pain episodes indicated criterion validity for specific scales. CONCLUSIONS: The PedsQL appears to validly assess quality of life in youth with SCD. Domain-specific measurement of quality of life was limited by (a) low reliability for youth-report and (b) lack of discriminant validity. Choice of informant may be important when evaluating quality of life effects from pain or neurologic problems in SCD.

Neurodevelopmental screening in toddlers and early preschoolers with sickle cell disease.

Sickle cell disease is associated with an elevated risk for neurologic complications beginning in early childhood. Detecting higher-risk cases with developmental screening instruments may be a cost-effective method for identifying young children in need of more frequent or intensive assessment. We evaluated the validity of the Denver II test as a tool to detect lower levels of developmental attainment and their association with neurologic risk in 50 young children with sickle cell disease. Children with suspect Denver II outcomes showed lower scores for functional communication skills, had lower hematocrit percentage, higher mean velocities on transcranial Doppler ultrasound imaging, and were more likely to have had preterm birth. Validity of age equivalencies from specific Denver II areas was demonstrated for Language and Fine Motor scores, suggesting the instrument could be used to index children's developmental levels in these domains. The Denver II may be a useful behavioral screening tool for neurodevelopmental risk in sickle cell disease.

Changes in coping, pain, and activity after cognitive-behavioral training: a randomized clinical trial for pediatric sickle cell disease using smartphones.

OBJECTIVES: We examined the outcomes of a cognitive-behavioral therapy (CBT) intervention for pain in pediatric sickle cell disease (SCD) using smartphones as a novel delivery method. MATERIALS AND METHODS: Forty-six children with SCD received CBT coping skills training using a randomized, waitlist control design. The intervention involved a single session of CBT training and home-based practice using smartphones for 8 weeks. Pre-post questionnaires between the randomized groups were used to evaluate changes in active psychological coping and negative thinking using the Coping Strategies Questionnaire. Daily diaries completed by the full sample during the treatment period were used to assess whether CBT skill use was related to reductions in next-day pain intensity and increases in same-day functional activity. RESULTS: The pre-post group comparison suggested that the youth increased active psychological coping attempts with the intervention. Daily diary data indicated that when children used CBT skills on days with higher pain, there were reductions in next-day pain intensity. There was no such association between skill use and functional activity. DISCUSSION: CBT coping skills training supported using smartphones can increase coping and reduce pain intensity for children with SCD; however, additions to the study protocols are recommended in future studies. Advantages and caveats of using smartphones are also discussed.

Sensitization to acute procedural pain in pediatric sickle cell disease: modulation by painful vaso-occlusive episodes, age, and endothelin-1.

UNLABELLED: The impact of pain early in life is a salient issue for sickle cell disease (SCD), a genetic condition characterized by painful vaso-occlusive episodes (VOEs) that can begin in the first year of life and persist into adulthood. This study examined the effects of age and pain history (age of onset and frequency of recent VOEs) on acute procedural pain in children with SCD. Endothelin-1, a vaso-active peptide released during VOEs and acute tissue injury, and its precursor, Big Endothelin, were explored as markers of pain sensitization and vaso-occlusion. Sixty-one children with SCD (ages 2 to 18) underwent venipuncture at routine health visits. Procedural pain was assessed via child and caregiver reports and observational distress. Pain history was assessed using retrospective chart review. Three primary results were found: 1) younger age was associated with greater procedural pain across pain outcomes; 2) higher frequency of VOEs was associated with greater procedural pain based on observational distress (regardless of age); and 3) age was found to moderate the relationship between VOEs and procedural pain for child-reported pain and observational distress for children 5 years of age and older. Associations between the endothelin variables and pain prior to venipuncture were also observed. PERSPECTIVE: For children with SCD, the child's age and recent pain history should be considered in procedural pain management. The endothelin system may be involved in preprocedure pain, but additional research is needed to understand the role of endothelins in pain sensitization.

Criterion and convergent validity for 4 measures of pain in a pediatric sickle cell disease population.

OBJECTIVE: To evaluate the psychometric properties of 4 measures of acute pain in youth with sickle cell disease (SCD) during a medical procedure. METHODS: Heart rate, child self-report, parent proxy-report, and observable pain behaviors were examined in 48 youth with SCD ages 2 to 17 years. Criterion validity for acute pain was assessed by responsiveness to a standardized painful stimulus (venipuncture) in a prospective pre-post design. Convergent validity was evaluated through the correlation across measures in reactivity to the stimulus. RESULTS: Child self-reported pain, parent proxy-report, and behavioral distress scores increased in response to venipuncture (concurrent and convergent validity). In contrast, heart rate did not reliably change in response to venipuncture. Extent of change in response to venipuncture showed moderate intercorrelation across child and parent pain ratings, and behavioral distress. Preprocedure pain ratings correlated with pain experienced during the procedure. An item analysis of observable pain behaviors suggested differences in the presentation of pain in SCD compared with previous pediatric research. CONCLUSIONS: Criterion and convergent validity were demonstrated for child-report, parent-report, and observable pain behaviors. These measures seem to tap into distinct, yet overlapping aspects of the pain experience. Assessment of acute procedural pain responses in SCD requires evaluation of preprocedural pain due to the frequent presence of low-level, baseline pain.

Sickle cell disease as a neurodevelopmental disorder.

Sickle cell disease (SCD) is a blood disorder; however, the central nervous system (CNS) is one of the organs frequently affected by the disease. Brain disease can begin early in life and often leads to neurocognitive dysfunction. Approximately one-fourth to one-third of children with SCD have some form of CNS effects from the disease, which typically manifest as deficits in specific cognitive domains and academic difficulties. We discuss SCD as a neurodevelopmental disorder by reviewing the mechanisms of neurological morbidity in SCD, the timing of these mechanisms, the types of cognitive and behavioral morbidity that is typical, and the interaction of social-environmental context with disease processes. The impact of the disease on families shares many features similar to other neurodevelopmental disorders; however, social-environmental factors related to low socioeconomic status, worry and concerns about social stigma, and recurrent, unpredictable medical complications can be sources of relatively higher stress in SCD. Greater public awareness of the neurocognitive effects of SCD and their impact on child outcomes is a critical step toward improved treatment, adaptation to illness, and quality of life.

A child-focused intervention for coping with procedural pain: are parent and nurse coaches necessary?

OBJECTIVE: To examine the efficacy of training children to cope with immunization pain without the assistance of trained coaches and determine whether untrained parents or nurses are more effective at decreasing children's distress. METHODS: We compared the procedural coping and distress behavior of 31 3- to 7-year-old children trained in coping skills to 30 who did not receive training. The behavior of the untrained parents and nurses was evaluated as it related to child coping and distress. RESULTS: Children demonstrated understanding of the training, but they did not use the coping skills during the procedure. In general, the nurses' behavior was associated with child coping and parents' behavior with child distress. CONCLUSIONS: More extensive child training or the involvement of coaches for procedural distress might be necessary. Nurses' behavior appears to center on encouraging child coping, and parents tend to comfort child distress.

Pediatric procedural approach-avoidance coping and distress: a multitrait-multimethod analysis.

OBJECTIVE: To evaluate the construct validity of children's approach-avoidance coping and distress during immunizations, and to examine the instruments used to assess these domains. METHODS: We used a multitrait-multimethod matrix to examine the validity of the approach-avoidance coping and distress constructs for 62 4- to 6-year-old children receiving immunization injections. Assessment instruments of both constructs consisted of child, parent, and nurse ratings, and three behavior observation scales. RESULTS: Pediatric procedural distress demonstrated adequate convergent and discriminant validity, and it can be assessed in a valid manner. Whereas most approach-avoidance measures demonstrated good convergent validity, several measures did not adequately distinguish between avoidance and distress. CONCLUSIONS: It is possible that children's approach-avoidance coping may be qualitatively different than adult's approach-avoidance. Researchers should be cognizant of these differences when designing treatments for child procedural distress.