Temporal relationship between daily pain and actigraphy sleep patterns in pediatric sickle cell disease

Limited research is available on the relationship between objective sleep patterns and pain in children with SCD. Research in other chronic pain populations suggests that the effect of sleep disruption on pain may be stronger than the effect of pain on sleep that night. To examine the bi-directional relationship between objective sleep patterns and daily pain in a pediatric SCD sample. Participants were 30 African American children with SCD 8–18 years (13?±?2.8 years; 66.7% female) with frequent pain. Children and parents completed questionnaires to assess pain, medications, and depression/anxiety. Over a 14-day period, children completed a pain diary and ambulatory actigraphy monitoring to assess nighttime sleep (duration, efficiency and WASO). Greater pain severity was associated with worse sleep efficiency and greater WASO that night, controlling for age, sex, opioid medication, and depression/anxiety symptoms. Worse sleep efficiency was associated with the occurrence of pain and more severe pain the next day. There was no relationship between WASO and pain. Similarly, sleep duration did not influence pain. Results lend support for a bi-directional relationship between sleep parameters and daily pain in pediatric SCD, and identify sleep as a potential target for future research and intervention.     

Impact of children's sickle cell history on nurse and physician ratings of pain and medication decisions.

The process of assessing and treating recurrent and unpredictable pain in children with sickle cell disease (SCD) is complex. A conceptual model is presented to aid in understanding the influence of mediating factors such as professional knowledge, attitudes and beliefs about pain, and learning history on the interpretation of objective data and resulting treatment decision. One aspect of this model, the effect of disease history on pain assessment and treatment decisions, is tested in an experimental study of SCD pain in children. Results suggest that nurses, but not pediatric residents, provide lower doses of narcotic analgesics to children with histories of frequent, as opposed to occasional, hospitalization for pain, although they do not differ in their ratings of the pain of children with these histories. Neither professional experience and training nor reported attitudes and beliefs about pain in children are related to this pattern of decision making. Results are discussed in terms of the aversive impact of repeated exposure to a noxious stimulus (pain behaviors) on caregiver interpretation of pain cues.     

Social information processing and magnetic resonance imaging in children with sickle cell disease

OBJECTIVE: To examine social information processing, social skills, and adjustment difficulties in children with sickle cell disease (SCD) as rated by caregivers, teachers, and the children themselves. Children were classified in two groups: cerebral vascular accidents (CVA) (n = 21) or without central nervous system (CNS) pathology (n = 20) on magnetic resonance imaging (MRI). Both groups had HbSS SCD. We compared these two groups and a third group of 11 children who had a milder type of SCD (HbSC). METHODS: Participants referred for evaluation of learning and behavior problems were administered MRIs to ascertain the presence of pathology and a series of measures designed to assess nonverbal emotional decoding abilities and ratings of social emotional functioning. RESULTS: Children with CVA displayed more errors on tasks of facial and vocal emotional decoding than did comparison controls without CVA. CONCLUSIONS: Acquired neurological impairments in children with SCD seemed to be associated with difficulties in the decoding of emotions of other children and adults. We recommend that future research integrate neuropsychological and psychosocial research programs for pediatric chronic illness groups.     

Sleep disturbances in school-age children with chronic pain

OBJECTIVES: To examine associations between pain, functional outcomes, and sleep disturbances in children with chronic pain, specifically juvenile idiopathic arthritis (JIA), sickle cell disease (SCD), and headache (HA). Sleep disturbances were tested as a risk factor for increased functional disability and decreased health-related quality of life (HRQOL). METHODS: One hundred children (JIA n = 30, SCD n = 26, HA n = 44; 8-12 years; 56% female) and their caregivers participated. Children completed questionnaires regarding pain, depression, and functional disability. Caregivers completed questionnaires regarding sociodemographics, child sleep habits, functional disability, and HRQOL. RESULTS: Levels of overall sleep disturbances were above the clinical cutoff for 53% of children with chronic pain. Sleep disturbances predicted lower physical HRQOL and higher functional disability, according to parent report. CONCLUSIONS: Sleep disturbances are common and associated with daytime functioning in school-age children with chronic pain, suggesting that assessment and treatment of sleep problems is clinically relevant.     

Comprehensive Assessment of Pain in Juvenile Rheumatoid Arthritis: An Empirical Model

A comprehensive assessment model of variables hypothesized toinfluence pediatric pain perception was empirically investigatedin 23 families who had a child with juvenile rheumatoid arthritis.To determine the effects of family environment, child psychologicaladjustment, and disease parameters on child pain perception,a developmentally appropriate model was developed. Childrenbetween the ages of 5 and 15 were found to be reliable judgesof their pain intensity. Several family environmental and childpsychological factors were found to interact with specific diseaseparameters in determining pediatric pain perception and report.A multidimensional age-appropriate assessment model is suggestedfor use in the further examination of pediatric chronic andrecurrent pain.     

Longitudinal relationships of depressive symptoms to pain intensity and functional disability among children with disease-related pain

OBJECTIVE: To examine the longitudinal relationship between depressive symptoms at study entry (T1) on pain intensity (PI) and functional disability over a 1-year period among children with either sickle cell disease (SCD) or juvenile idiopathic arthritis (JIA). METHODS: 119 children, ages 8-17 years, completed measures of depression at T1 as well as pain and functional disability at T1, 6-month (T2), and 12-month (T3) follow-ups. Caregivers also rated their child's pain and disability at each time point. General linear mixed modeling was employed to examine longitudinal relationships between study variables. RESULTS: For children with JIA, T1 pain significantly moderated the effects of T1-depressive symptoms on T2 and T3 pain where T1-depressive symptoms predicted future child-reported pain only when T1 pain was relatively mild. Similarly, T1-depressive symptoms predicted future child-reported disability only when initial reports of disability were relatively low. Only family income significantly predicted T2 and T3 pain in children with SCD. CONCLUSIONS: Study findings suggest that T1-depressive symptoms play a role in the longitudinal course of pain symptoms in children with JIA but not in children with SCD.     

A Qualitative Study of Chronic Pain and Self-Management in Adults with Sickle Cell Disease

Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is associated with substantial morbidity and impaired quality of life (QOL). The subgroup of adults with SCD who transition from recurrent, acute pain to chronic, persistent pain have even greater QOL impairment and higher rates of healthcare utilization. Self-management is central to SCD management; however, its role in chronic pain management is not established. This qualitative study was conducted to answer the following research questions: (1) What is the chronic pain experience of adults with SCD? (2) What self-management strategies do adults with SCD use for chronic pain? and (3) Do adults with SCD have any needs in the self-management of chronic pain? Eighteen Black adults with SCD completed a demographics questionnaire and an interview. The majority of the participants were 21–30 years of age (mean 33.5, SD 7.6), female (61.1%), employed at least part-time (61.1%), single/never married (72.2%), and had a SCD type of sickle cell anemia (55.5%). Interview analysis revealed three major themes: (1) the chronic pain experience; (2) strategies for managing chronic pain; and (3) challenges and needs in managing chronic pain. Study findings can be used to support chronic pain management among adults with SCD. Further research is needed to devise and implement effective strategies for the prevention and management of chronic SCD pain.     

Will developments in allogeneic transplantation influence treatment of adult patients with sickle cell disease?

With improvements in the treatment of children with sickle cell disease (SCD), there has been a significant increase in the number of patients with SCD in adult hematology practice. Quality of life and life expectancy continue to be severely compromised in adult patients; hydroxyurea is the only treatment currently available that could reduce the severity and frequency of painful episodes. Allogeneic stem cell transplantation (SCT) has been offered to children with SCD as a curative option. We discuss the implications of new developments in the field of allogeneic SCT in the treatment of adult SCD patients in light of the experience derived from pediatric transplantation. These developments include innovations in the conditioning regimens, GVHD prophylaxis, and alternative donor SCT and their possible effect on adult SCD patients. Finally, we discuss a nonmyeloablative conditioning protocol for adult SCD patients and the eligibility criteria for adult SCD patients undergoing allogeneic transplantation.     

Neurocognitive functioning and magnetic resonance imaging in children with sickle cell disease

OBJECTIVE: To examine neurocognitive functioning in children classified with overt cerebral vascular accidents (CVAs), silent infarcts, or without central nervous system (CNS) pathology on magnetic resonance imaging. METHODS: Participants were 63 children and adolescents with sickle cell disease (SCD). RESULTS: Children with overt CVAs and silent infarcts differed from their peers without CNS pathology on measures of attention and executive functioning. CONCLUSIONS: We consider these deficits the result of the high frequency of frontal lobe deficits incurred by children with SCD. Recommendations include the use of tests designed to measure attention and executive functioning as a way of screening children with SCD for possible CNS pathology. We also suggest that future research examine the mechanism underlying frontal lobe involvement for individuals with SCD.     

Brief report: sleep in children with sickle cell disease: an analysis of daily diaries utilizing multilevel models

OBJECTIVE: To investigate the pain-sleep relationship in children with sickle cell disease (SCD) and the influence of stress and pain medication use on this relationship. METHOD: Children with SCD (n = 20; aged 8-12 years) completed daily diaries assessing sleep, pain, stress, and pain medication use for up to 2 months. Data analyzed using multilevel modeling. RESULTS: High daily pain was related to poor sleep quality that night and poor sleep quality was related to high pain the following day. High stress was related to less sleep. High same-day pain and pain medication attenuated the impact of pain on sleep quality. CONCLUSION: Results highlight the importance of sleep in addressing functioning in children with chronic pain, knowledge which may help patients and their families better manage the child's pain. Behavioral pain interventions may be improved by the inclusion of strategies to encourage proper sleep hygiene and address sleep issues.     

Daily mood as a mediator or moderator of the pain-sleep relationship in children with sickle cell disease

OBJECTIVE: To investigate mood as a mediator or moderator of the pain-sleep relationship in children with sickle cell disease (SCD). METHOD: Children with SCD (n = 20; aged 8-12 years) completed daily diaries assessing mood, sleep, and pain for up to 2 months. Data was analyzed using multilevel modeling. Results Results indicate that negative mood partially mediates the relationship between high daily pain and poor sleep that night as well the relationship between poor sleep and high daily pain the following day. The impact of poor sleep on high pain the following day was weakened at increasing levels of positive mood. CONCLUSION: Research is needed to fully explore the ways positive and negative mood may relate to pain and sleep characteristics. This information may be beneficial for developing more effective pain management and sleep interventions.     

Effects of perceived stress on pediatric chronic pain

The dearth of theoretically driven research on the predictors of pediatric chronic pain may unwittingly contribute to needless suffering in children and adolescents by underinvestigating a potentially treatable condition. The objective of the present study was to investigate the hypothesized predictive effects of perceived stress on pediatric chronic pain intensity in 148 children and adolescents. Consistent with thea priori Biobehavioral Model of Pediatric Pain, higher perceived stress was predictive of greater pediatric pain intensity. The results are discussed with regard to the implications for cognitive-behavioral pediatric pain treatment.     

Psychosocial treatments in pain management of sickle cell disease

The principal symptom of sickle cell disease (SCD) is pain. Many studies have been conducted on pain management strategies for this illness. There is recognition that psychosocial factors influence clinical disease outcomes; therefore, more attention is being provided to behavioral interventions that address psychosocial problems. This review examines the psychosocial interventions that have been researched for children and adults with SCD, the limitations of these studies, and barriers to implementing the treatments. The intervention receiving the most empirical support was cognitive-behavioral therapy. Additional research is needed to define the efficacy and effectiveness of the other psychosocial treatments. Suggestions for future investigations include conducting research that has better methodology, and providing more education for health care providers about psychosocial treatments and the importance of considering cultural factors in health care delivery. In addition, individuals with SCD need to have more information about their illness and better access to psychosocial interventions.     

Tumor necrosis factor alpha in children with sickle cell disease in stable condition.

Tumor necrosis factor alpha (TNF-alpha) is known to induce wasting in humans and animals. This study was undertaken to determine TNF-alpha concentrations in children with sickle cell disease (SCD) and whether high TNF-alpha levels are more likely to be present in children with growth deficits, infection, or pain crisis. Tumor necrosis factor alpha was measured using enzyme immunoassay in 143 blood samples obtained from 101 children. Mean TNF-alpha levels were higher in patients (50 pg/mL) than in 21 control children (19 pg/mL) and in 26 laboratory employees (20 pg/mL). During the follow-up period, 35%, 38%, and 28% of children with SCD had infection, pain crisis, or a blood transfusion, respectively. Mean TNF-alpha concentrations were higher in children who had an infection than in those who did not. No significant effect of pain crisis or blood transfusion was observed. Tumor necrosis factor alpha concentrations were above normal (> 40 pg/mL) in 15% of controls, 34% of children with SCD, and 52% of children with SCD who had an infection and 33% of those who did not. A higher percentage of children who had elevated TNF-alpha levels had weight (46% versus 31%) or height (50% versus 28.6%) deficits than children who had normal TNF-alpha levels. These results indicate that most children with SCD in stable condition have normal TNF-alpha concentrations and that those with high TNF-alpha levels are more likely to have growth deficits.     

Brief report: Assessment of children's gastrointestinal symptoms for clinical trials

OBJECTIVE: To conduct a pilot study evaluating a procedure for assessment of daily symptoms and functioning in pediatric patients. METHOD: Participants included 11 parent-child dyads referred to a tertiary care center for evaluation of constipation and abdominal pain. Each family was provided a hand-held computer and modem. For 7 consecutive days, parents and children (ages 6-10 years) responded as a team to questions regarding the level of children's gastrointestinal symptoms and the extent to which symptoms interfered with the day's activities. Parents responded to a telephone interview evaluating the procedure. RESULTS: Parents reported that children understood most questions and that responses entered into the computer were accurate. Parents and children were enthusiastic about the data collection method. Some technical problems arose in use of the computers. CONCLUSIONS: Within the limitations of a small sample, this data collection procedure appears to have promise for evaluating pediatric symptom outcomes.     

Pain in children and adolescents with sickle cell disease: a descriptive study

In sickle cell disease, vaso-occlusion in the small blood vessels leads to bone or joint pain which is variable in intensity and duration. An essential first step toward the development of specific treatment guidelines for such painful episodes in children and adolescents is the accurate evaluation of pain. The systematic assessment of vaso-occlusive pain is addressed through two separate studies. In the first, 35 pediatric sickle cell disease patients between 5 and 16 years of age were evaluated in an outpatient clinic with the Varni/Thompson Pediatric Pain Questionnaire. In the second, data were gathered over the course of hospitalizations for uncomplicated vaso-occlusive episodes in 17 adolescent patients. Results showed that this pain experience can be quantified, that vaso-occlusive pain spans a broad range of intensity levels, and that there are a number of socioemotional factors associated with the pain experience. Further research to systematically assess the psychometric properties of pain assessment instrument is recommended.     

The interplay of sleep disturbance, anxiety, and depression in children

OBJECTIVE: To review and critically evaluate the association between sleep, anxiety, and depression in children and provide recommendations for future research. METHODS: A literature search was conducted using MEDLINE and PsychINFO computerized databases and bibliographies of relevant articles. RESULTS: A surprisingly small but growing research base exists on the relation between sleep disturbance, anxiety, and depression in pediatric populations. Existing research indicates a significant symptom overlap between anxiety, depression, and sleep. This overlap may complicate proper assessment and treatment of children with these disorders. CONCLUSIONS: Future research should ensure adequate assessment for symptoms of anxiety and depression when examining sleep disturbance in children. Likewise, research on anxiety and depression should include assessment for symptoms of disturbed sleep. Bridging the gap between these literatures should provide further insights into the etiologies of these disorders, increase symptom detection, and improve the clinical care of children and their families.     

Cognitive coping skills training in children with sickle cell disease pain

This study was designed to examine whether brief training in cognitive coping skills would enhance pain coping strategies and alter pain perception in children and adolescents with sickle cell disease (SCD). Forty-nine participants with SCD were randomly assigned to either a cognitive coping skills condition or a standard care control condition. At pre- and posttesting, coping strategies and pain sensitivity using laboratory pain stimulation were measured. Results indicated that in comparison to the randomly assigned control condition, brief training in cognitive coping skills resulted in decreased negative thinking and lower pain ratings during low intensity laboratory pain stimulation.     

Daily coping practice predicts treatment effects in children with sickle cell disease

OBJECTIVE: To examine the 1-month effects of a pain coping skills intervention in children with sickle cell disease (SCD). METHODS: Forty-six African American children (8-17 years old) were randomly assigned to either a coping skills condition or a standard care control condition. Children were asked to practice daily with audiotaped instructions of skills (e.g., relaxation, imagery). RESULTS: Multivariate analyses of summary measures indicated that children in the coping intervention (versus control group) reported a significantly more active approach to managing pain. Multilevel random effects models applied to daily diary data indicated that on pain days when children practiced their strategies, they had fewer health care contacts, fewer school absences, and less interference with household activities than on days when they did not practice. CONCLUSIONS: Brief training in coping skills followed by minimal therapist contact may lead to a range of benefits when children practice with their skills on a consistent basis.