Systematic Review: Pain and Emotional Functioning in Pediatric Sickle Cell Disease

Abstract

The objective of this systematic review was to assess the relationship between pain (frequency/intensity/duration, impairment, coping) and emotional functioning in pediatric Sickle Cell Disease, and evaluate the state of the literature. Studies were included if they met each of the following criteria: (a) primarily pediatric sample of youth or young adults up to age 21 years with SCD, (b) examined emotional functioning including anxiety and/or depressive and/or internalizing symptoms, and/or affect, (c) examined pain intensity/frequency/duration and/or pain-related impairment and/or pain coping as it relates to emotional functioning, as defined above. Using the established guidelines for systematic reviews, we searched PsycINFO, PubMED, and CINAHL databases for studies published through June 2018. Screening resulted in 33 studies meeting inclusion criteria. Study data were extracted and evaluated for scientific merit, resulting in four studies being removed. 29 studies were included in the final synthesis. Studies provide strongest evidence of a relationship between increased pain frequency and higher depressive and anxiety symptoms. There are moderate-to-strong associations between pain-related impairment and depressive symptoms, and small-to-strong associations between pain-related impairment and anxiety. When examining pain-coping strategies, maladaptive cognitive strategies show the strongest association with emotional functioning. There is a need for more adequately powered, prospective studies based on theoretical frameworks in order to advance our understanding of the relationship between pain and emotional functioning in pediatric SCD.

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Fig. 1

From Moher et al. (2009)

References

  1. Alderfer, M. A., Long, K. A., Lown, E. A., Marsland, A. L., Ostrowski, N. L., Hock, J. M., & Ewing, L. J. (2010). Psychosocial adjustment of siblings of children with cancer: A systematic review. Psycho-oncology,19, 789–805. https://doi.org/10.1002/pon.1638.

    Article  PubMed  Google Scholar 

  2. Bakshi, N., Lukombo, I., Belfer, I., & Krishnamurti, L. (2018). Pain catastrophizing is associated with poorer health-related quality of life in pediatric patients with sickle cell disease. Journal of Pain Research,11, 947–953.

    Article  Google Scholar 

  3. Bakshi, N., Lukombo, I., Shnol, H., Belfer, I., & Krishnamurti, L. (2017). Psychological characteristics and pain frequency are associated with experimental pain sensitivity in pediatric patients with sickle cell disease. The Journal of Pain,18, 1216–1228.

    Article  Google Scholar 

  4. Barakat, L. P., Patterson, C. A., Daniel, L. C., & Dampier, C. (2008). Quality of life among adolescents with sickle cell disease: Mediation of pain by internalizing symptoms and parenting stress. Health and Quality of Life Outcomes,6, 1–9.

    Article  Google Scholar 

  5. Barakat, L. P., Schwartz, L. A., Simon, K., & Radcliffe, J. (2007). Negative thinking as a coping strategy mediator of pain and internalizing symptoms in adolescents with sickle cell disease. Journal of Behavioral Medicine,30, 199–208. https://doi.org/10.1007/s10865-007-9103-x.

    Article  PubMed  Google Scholar 

  6. Bennett, D. S. (1994). Depression among children with chronic medical problems: A meta-analysis. Journal of Pediatric Psychology,19, 149–169.

    CAS  Article  Google Scholar 

  7. Benton, T. D., Ifeagwu, J. A., & Smith-Whitley, K. (2007). Anxiety and depression in children and adolescents with sickle cell disease. Current Psychiatry Reports,9, 114–121.

    Article  Google Scholar 

  8. Burlew, K., Telfair, J., Colangelo, L., & Wright, E. C. (2000). Factors that influence adolescent adaptation to sickle cell disease. Journal of Pediatric Psychology,25, 287–299.

    CAS  Article  Google Scholar 

  9. Cartwright-Hatton, S., McNicol, K., & Doubleday, E. (2006). Anxiety in a neglected population: Prevalence of anxiety disorders in pre-adolescent children. Clinical Psychology Review,26, 817–833.

    Article  Google Scholar 

  10. 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.

    Article  Google Scholar 

  11. Cohen, J. (1988). Statistical power analysis for the behavioral sciences (2nd ed.). Hillsdale, NJ: Lawrence Erlbaum Associates.

    Google Scholar 

  12. Cozzi, L., & Tryon, W. W. (1987). The effectiveness of biofeedback-assisted relaxation in modifying sickle cell disease. Biofeedback and Self-Regulation,12, 51–61. https://doi.org/10.1007/BF01000078.

    CAS  Article  PubMed  Google Scholar 

  13. Dampier, C., Barry, V., Gross, H. E., Lui, Y., Thornburg, C. D., DeWalt, D. A., & Reeve, B. B. (2016). Initial evaluation of the pediatric PROMIS® health domains in children and adolescents with sickle cell disease. Pediatric Blood Cancer,63, 1031–1037. https://doi.org/10.1002/pbc.25944.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Eiser, C. (1990). Psychological effects of chronic disease. Journal of Child Psychology and Psychiatry,31, 85–98.

    CAS  Article  Google Scholar 

  15. Fisher, K., Laikin, A. M., Howard Sharp, K. M., Criddle, C. A., Palermo, T. M., & Karlson, C. W. (2018). Temporal relationship between daily pain and actigraphy sleep patterns in pediatric sickle cell disease. Journal of Behavioral Medicine,41, 416–422. https://doi.org/10.1007/s10865-018-9918-7.

    Article  PubMed  Google Scholar 

  16. Gil, K. M., Anthony, K. K., Carson, J. W., Redding-Lallinger, R., Daeschner, C. W., & Ware, R. E. (2001). Daily coping practice predicts treatment effects in children with sickle cell disease. Journal of Pediatric Psychology,26, 163–173. https://doi.org/10.1093/jpepsy/26.3.163.

    CAS  Article  PubMed  Google Scholar 

  17. Gil, K. M., Carson, J. W., Porter, L. S., Ready, J., Valrie, C., Redding-Lallinger, R., & Daeschner, C. (2003). Daily stress and mood and their association with pain, health-care use, and school activity in adolescents with sickle cell disease. Journal of Pediatric Psychology,28, 363–373. https://doi.org/10.1093/jpepsy/jsg026.

    Article  PubMed  Google Scholar 

  18. Gil, K. M., Edens, J. L., Wilson, J. J., Raezer, L. B., Kinney, T. R., Schultz, W. H., & Daeschner, C. (1997). Coping strategies and laboratory pain in children with sickle cell disease. Annals of Behavioral Medicine,19, 22–29.

    CAS  Article  Google Scholar 

  19. Gil, 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.

    CAS  Article  Google Scholar 

  20. Goldstein-Leever, A., Cohen, L. L., Dampier, C., & Sil, S. (2018). Parent pain catastrophizing predicts child depressive symptoms in youth with sickle cell disease. Pediatric Blood & Cancer,65, e27027. https://doi.org/10.1002/pbc.27027.

    Article  Google Scholar 

  21. Graumlich, S. E., Powers, S. W., Byars, K. C., Schwarber, L. A., Mitchell, M. J., & Kalinyak, K. A. (2001). Multidimensional assessment of pain in pediatric sickle cell disease. Journal of Pediatric Psychology,26, 203–214.

    CAS  Article  Google Scholar 

  22. Graves, J. K., Hodge, C., & Jacob, E. (2016). Depression, anxiety, and quality of life in children and adolescents with sickle cell disease. Pediatric Nursing,42, 113.

    PubMed  Google Scholar 

  23. Hassell, K. L. (2010). Population estimates of sickle cell disease in the U.S. American Journal of Preventative Medicine,38, S512–S521.

    Article  Google Scholar 

  24. Hoff, A. L., Palermo, T. M., Schluchter, M., Zebracki, K., & Drotar, D. (2006). Longitudinal relationships of depressive symptoms to pain intensity and functional disability among children with disease-related pain. Journal of Pediatric Psychology,31, 1046–1056. https://doi.org/10.1093/jpepsy/jsj076.

    Article  PubMed  Google Scholar 

  25. Jerrell, J. M., Tripathi, A., & McIntyre, R. S. (2011). Prevalence and treatment of depression in children and adolescents with sickle cell disease: A retrospective cohort study. The Primary Care Companion for CNS Disorders. https://doi.org/10.4088/pcc.10m01063.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Karlson, C. W., Baker, A. M., Bromberg, M. H., Elkin, T. D., Majumdar, S., & Palermo, T. M. (2017). Daily pain, physical activity, and home fluid intake in pediatric sickle cell disease. Journal of Pediatric Psychology,42, 335–344. https://doi.org/10.1093/jpepsy/jsw061.

    Article  PubMed  Google Scholar 

  27. Lemanek, K. L., Hahn, A., & McNaull, M. (2017). Sickle cell disease. In M. C. Roberts & R. G. Steele (Eds.), Handbook of pediatric psychology (pp. 269–283). New York: Guilford Press.

    Google Scholar 

  28. Lemanek, K. L., Ranalli, M., & Lukens, C. (2009). A randomized controlled trial of massage therapy in children with sickle cell disease. Journal of Pediatric Psychology,34, 1091–1096. https://doi.org/10.1093/jpepsy/jsp015.

    Article  PubMed  Google Scholar 

  29. Merikangas, K.R., He, J., Burstein, M., Swanson, S.A., Avenevoli, S., Cui, L., … Swendsen, J. (2010). Lifetime prevalence of mental disorders in U.S. adolescents: Results from the National Comorbidity Survey Replication—Adolescent Supplement. Journal of the American Academy of Child and Adolescent Psychiatry, 49, 980–989.

  30. Moher, D., Liberati, A., Tetzlaff, J., Altman, D. G., & The PRISMA Group. (2009). Preferred reporting items for systematic reviews and meta-analyses: The PRISMA statement. PLoS Med,6, e1000097. https://doi.org/10.1371/journal.pmed1000097.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Mojtabai, R., Olfson, M., & Han, B. (2016). National trends in the prevalence and treatment of depression in adolescents and young adults. Pediatrics,138, e20161878. https://doi.org/10.1542/peds.2016-1878.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Moody, K., Abrahams, B., Baker, R., Santizo, R., Manwani, D., Carullo, V., … Carroll, A. (2017). A randomized trial of yoga for children hospitalized with sickle cell vaso-occlusive crisis. Journal of Pain and Symptom Management, 53, 1026–1034. https://doi.org/10.1016/j.jpainsymman.2016.12.351.

  33. Morgan, G. A., Gliner, J. A., & Harmon, R. J. (1999). Quantitative research approaches. Journal of the American Academy of Child and Adolescent Psychiatry,38, 1595–1597.

    CAS  Article  Google Scholar 

  34. Neale, J., & Liebert, R. (1986). Science and behavior: An introduction to methods of research. Englewood Cliffs, NJ: Prentice-Hall.

    Google Scholar 

  35. Noll, R. B., Vannatta, K., Koontz, K., Kalinyak, K., Bukowski, W. M., & Davis, W. H. (1996). Peer relationships and emotional well-being of youngsters with sickle cell disease. Child Development,67, 423–436.

    CAS  PubMed  Google Scholar 

  36. Palermo, T. M. (2013). New guidelines for publishing review articles in JPP: Systematic reviews and topical reviews. Journal of Pediatric Psychology,38, 5–9.

    Article  Google Scholar 

  37. Palermo, T. M., Platt-Houston, C., Kiska, R. E., & Berman, B. (2005). Headache symptoms in pediatric sickle cell patients. Journal of Pediatric Hemotology/Oncology,27, 420–424. https://doi.org/10.1097/01.mph.0000175408.27180.8e.

    Article  Google Scholar 

  38. Palermo, T. M., Riley, C. A., & Mitchell, B. A. (2008). Daily functioning and quality of life in children with sickle cell disease pain: Relationship with family and neighborhood socioeconomic distress. The Journal of Pain,9, 833–840. https://doi.org/10.1016/j.jpain.2008.04.002.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Palermo, T. M., Valrie, C. R., & Karlson, C. W. (2014). Family and parent influences on pediatric chronic pain: A developmental perspective. American Psychologist,69, 142–152.

    Article  Google Scholar 

  40. Panepinto, J. A., & Bonner, M. (2012). Health-related quality of life in Sickle Cell Disease: Past, present, and future. Pediatric Blood & Cancer,59, 377–385.

    Article  Google Scholar 

  41. Peterson, C. C., Palermo, T. M., Swift, E., Beebe, A., & Drotar, D. (2005). Assessment of psychoeducational needs in a clinical sample of children with sickle cell disease. Children’s Health Care,34, 133–148.

    Article  Google Scholar 

  42. Pillai Riddell, R., Racine, N., Craig, K. D., & Campbell, L. (2013). Psychological theories and biopsychosocial models in paediatric pain. In P. J. McGrath, et al. (Eds.), Oxford textbook of paediatric pain (pp. 85–94). Oxford: Oxford University Press.

    Chapter  Google Scholar 

  43. Pinquart, M., & Shen, Y. (2010). Depressive symptoms in children and adolescents with chronic physical illness: An updated meta-analysis. Journal of Pediatric Psychology,36, 375–384. https://doi.org/10.1093/jpepsy/jsq104.

    Article  PubMed  Google Scholar 

  44. Schlenz, A. M., Schatz, J., & Roberts, C. W. (2016). Examining biopsychosocial factors in relation to multiple pain features in pediatric sickle cell disease. Journal of Pediatric Psychology,41, 930–940. https://doi.org/10.1093/jpepsy/jsw003.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Sil, S., Cohen, L. L., & Dampier, C. (2016). Psychosocial and functional outcomes in youth with chronic sickle cell pain. Clinical Journal of Pain,32, 527–533. https://doi.org/10.1097/AJP.0000000000000289.

    Article  PubMed  Google Scholar 

  46. Thompson, R. J., Gil, K. M., Burbach, D. J., Keith, B. R., & Kinney, T. R. (1993). Role of child and maternal processes in the psychological adjustment of children with sickle cell disease. Journal of Consulting and Clinical Psychology,61, 468–474.

    Article  Google Scholar 

  47. Thompson, R. J., Gil, K. M., Keith, B. R., Gustafson, K. E., George, L. K., & Kinney, T. R. (1994). Psychological adjustment of children with sickle cell disease: Stability and change over a 10-month period. Journal of Consulting and Clinical Psychology,62, 856–860.

    Article  Google Scholar 

  48. Tsao, J. C., Jacob, E., Seidman, L. C., Lewis, M. A., & Zeltzer, L. K. (2014). Psychological aspects and hospitalization for pain crises in youth with sickle-cell disease. Journal of Health Psychology,19, 407–416. https://doi.org/10.1177/1359105312471570.

    Article  PubMed  Google Scholar 

  49. Unal, S., Toros, F., Kutuk, M. O., & Uyaniker, M. G. (2011). Evaluation of the psychological problems of children with sickle cell anemia and their families. Pediatric Hematology and Oncology,28, 321–328.

    Article  Google Scholar 

  50. Valrie, C. R., Gil, K. M., Redding-Iallinger, R., & Daeschner, C. (2008). Brief report: Daily mood as a mediator or moderator of the pain-sleep relationship in children with sickle cell disease. Journal of Pediatric Psychology,33, 317–322. https://doi.org/10.1093/jpepsy/jsm058.

    Article  PubMed  Google Scholar 

  51. Wagner, J. L., Connelly, M., Brown, R. T., Taylor, L. C., Rittle, C., & Wall-Cloues, B. (2004). Predictors of social anxiety in children and adolescents with sickle cell disease. Journal of Clinical Psychology in Medical Setting,11, 243–252.

    Article  Google Scholar 

  52. Wakefield, E. O., Popp, J. M., Dale, L. P., Santanelli, J. P., Pantaleao, A., & Zempsky, W. T. (2017). Perceived racial bias and health-related stigma among youth with sickle cell disease. Journal of Developmental and Behavioral Pediatrics,38, 129–134. https://doi.org/10.1097/DBP.0000000000000381.

    Article  PubMed  Google Scholar 

  53. Walco, G. A., & Dampier, C. D. (1990). Pain in children and adolescents with sickle cell disease: A descriptive study. Journal of Pediatric Psychology,15, 643–658.

    CAS  Article  Google Scholar 

  54. Wallander, J. L., & Varni, J. W. (1992). Adjustment in children with chronic physical disorders: Programmatic research on a disability-stress-coping model. In A. M. La Greca, L. J. Siegel, J. L. Wallander, & C. E. Walker (Eds.), Advances in pediatric psychology. Stress and coping in child health (pp. 279–298). New York: Guilford Press.

    Google Scholar 

  55. Wallander, J., & Varni, J. W. (1998). Effects of pediatric chronic physical disorders on child and family adjustment. Journal of Child Psychology and Psychiatry,39, 29–46.

    CAS  Article  Google Scholar 

  56. Zempsky, W. T., Palermo, T. M., Corsi, J. M., Lewandowski, A. S., Zhou, C., & Casella, J. F. (2013). Daily changes in pain, mood and physical function in youth hospitalized for sickle cell disease pain. Pain Research & Management,18, 33–38.

    Article  Google Scholar 

  57. Zempsky, W. T., Wakefield, E. O., Santanelli, J. P., New, T., Smith-Whitley, K., Casella, J. F., & Palermo, T. M. (2017). Widespread pain among youth with sickle cell disease hospitalized with vaso-occlusive pain. Clinical Journal of Pain,33, 335–339. https://doi.org/10.1097/AJP.0000000000000403.

    Article  PubMed  Google Scholar 

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Acknowledgements

This work was supported by a grant from the National Institute of General Medical Sciences of the National Institutes of Health (Grant No. P20GM109021) and the Nemours Center for Healthcare Delivery Science of the Nemours Children’s Health System. The authors thank Dr. Melissa Alderfer for her consultation on this systematic review.

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Steven K. Reader, Laura M. Rockman, Katherine M. Okonak, Nicole M. Ruppe, Colleen N. Keeler, and Anne E. Kazak declare that they have no conflicts of interest.

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Reader, S.K., Rockman, L.M., Okonak, K.M. et al. Systematic Review: Pain and Emotional Functioning in Pediatric Sickle Cell Disease. J Clin Psychol Med Settings 27, 343–365 (2020). https://doi.org/10.1007/s10880-019-09647-x

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Keywords

  • Systematic review
  • Sickle cell disease
  • Pain
  • Depression
  • Anxiety