Abstract
Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. The disease is characterized by chronic hemolytic anemia, as well as acute and chronic complications. One of the most intractable problems encountered by children with SCD is the painful episode that results from tissue ischemia due to vaso-occlusion. Pain related to SCD is unique among pain syndromes due to the unpredictable, recurrent, and often persistent nature of the disease, as well as the recurring and essential need for the use of opioids. Painful vaso-occlusive episodes (VOE) are a principal cause of morbidity and account for a significant number of emergency department and hospital admissions. When untreated or inadequately managed, the pain of VOE may cause both short- and long-term consequences. Despite the fact that pain is an almost universal feature of the disease, children with SCD may form one of the most undertreated and understudied populations. One of the factors contributing to poor pain management is conflicting perceptions between patients, their families, and healthcare professionals about pain that is reported and analgesia that is required. Pain management guidelines have recently been published in an effort to overcome barriers in the assessment and management of pain related to SCD.
Although there is considerable variability in the way SCD pain is managed, the standard treatment protocol for painful episodes has been rest, rehydration, and analgesia. However, pain control for children with SCD is often a difficult and complex process, and one that requires frequent systematic pain assessments and continuous adjustment of comfort measures, especially analgesics. There are a variety of analgesic agents to choose from, such as acetaminophen (paracetamol), oral or parenteral nonsteroidal anti-inflammatory drugs, and oral or parenteral opioids. Each of these options has advantages and disadvantages to their use. Continuous infusions of analgesics and patient controlled analgesia have been shown to be effective and widely used in hospital settings to manage severe pain. However, the opioid dose required to achieve pain relief varies considerably within each painful episode, from one episode to another, and between individual patients.
Although not yet curable in humans, pain related to SCD can be effectively managed in most patients by using a comprehensive approach that incorporates pharmacologic, psychologic, behavioral, and physical pain management strategies.
Similar content being viewed by others
References
Wang WC, Lukens JN. Sickle cell anemia and other sickling syndromes. In: Lee GR, Forester J, Lukens J, et al., editors. Wintrobe’s clinical hematology. 10th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 1999: 1346–82
Friedman J, Jarvis A, Kirby MA, et al. Guidelines for inpatient management of children with sickle cell disease. Toronto (ON): The Hospital for Sick Children, 2000
Kaul DK, Fabry ME, Nagel RL. The pathophysiology of vascular obstruction in the sickle syndromes. Blood Rev 1996; 10: 29–44
Shapiro BS. Management of painful episodes in sickle cell disease. In: Schechter NL, Berde CB, Yaster M, editors. Pain in infants, children, and adolescents. Baltimore (MD): Williams & Wilkins, 1993: 385–409
Franck LS, Treadwell M, Jacob E, et al. Assessment of sickle cell pain in children and young adults using the adolescent pediatric pain tool. J Pain Symptom Manage 2002; 23: 114–20
Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood 1994; 84: 643–9
Vichinsky EP, Styles LA, Colangelo LH, et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. The Cooperative Study of Sickle Cell Disease. Blood 1997; 89: 1787–92
Yaster M, Kost-Byerly S, Maxwell LG. The management of pain in sickle cell disease. Pediatr Clin North Am 2000; 47: 699–710
Ballas SK. Sickle cell pain: progress in pain research and management. Vol 11. Seattle (WA): IASP Press, 1998
Shapiro BS, Dinges DF, Orne ED, et al. Home management of sickle-cell-related pain in children and adolescents: Natural history and impact on school attendance. Pain 1995; 61: 139–44
Beyer JE, Simmons LE, Woods GM, et al. A chronology of pain and comfort in children with sickle cell disease. Arch Pediatr Adolesc Med 1999; 153(9): 913–20
Conner-Warren RL. Pain intensity and home pain management of children with sickle cell disease. Iss Compr Pediatr Nurs 1996, 195
Barbarin OA, Whitten CF, Bonds SM. Estimating rates of psychosocial problems in urban and poor children with sickle cell anemia. Health Soc Work 1994; 19: 112–9
Eaton ML, Haye JS, Armstrong FD, et al. Hospitalizations for painful episodes: association with school absenteeism and academic performance in children and adolescents with sickle cell anemia. Issues Compr Pediatr Nurs 1995; 18(1): 1–9
Fuggle P, Shand PAX, Gill LJ, et al. Pain, quality of life, and coping in sickle cell disease. Arch Dis Child 1996; 75: 199–203
Graumlich SE, Powers SW, Byars KC, et al. Multidimensional assessment of pain in pediatric sickle cell disease. J Pediatr Psychol 2001; 26: 203–14
Gil KM, Anthony KK, Carson JW, et al. Daily coping practice predicts treatment effects in children with sickle cell disease. J Pediatr Psychol 2001; 26: 163–73
Burlew K, Telfair J, Colangelo L, et al. Factors that influence adolescent adaptation to sickle cell disease. J Pediatr Psychol 2000; 25: 287–99
Benjamin LJ, Dampier CD, Jacox AK, et al. Guidelines for the management of acute and chronic pain in sickle cell disease. APS clinical practice guidelines. No. 1. Glenview (IL): American Pain Society, 1999: 12–3
Beyer JE. Judging the effectiveness of analgesia for children and adolescents during vaso-occlusive events of sickle cell Disease. J Pain Symptom Manage 2000; 19: 63–72
Schechter NL. The management of pain in sickle cell disease. In: McGrath PJ, Finley GA, editors. Chronic and recurrent pain in childhood: progress in pain research. Vol. 13. Seattle (WA): IASP Press, 1999: 99–114
Elander J, Midence K. A review of evidence about factors affecting quality of pain management in sickle cell disease. Clin J Pain 1996; 12: 180–93
Fertleman CR, Gallagher A, Rossiter MA. Evaluation of a fast track admission policy for children with sickle cell crises: questionnaire survey of parents’ preferences. BMJ 1997; 315: 650
Sutton M, Atweh GF, Cashman TD, et al. Resolving conflicts: misconceptions and myths in the care of the patient with sickle cell disease. Mt Sinai J Med 1999; 66: 282–5
Shapiro BS, Benjamin LJ, Payne R, et al. Sickle cell-related pain: perceptions of medical practitioners. J Pain Symptom Manage 1997; 14: 168–74
Weissman DE, Haddox JD. Opioid pseudoaddiction: an iatrogenic syndrome. Pain 1989; 36: 363–6
Ballas SK. Sickle cell disease: current clinical management. Semin Hematol 2001; 38: 307–14
Franck LS, Greenberg CS, Stevens B. Pain assessment in infants and children. Pediatr Clin North Am 2000; 47: 487–512
Beyer JE, Platt AF, Kinney TR, et al. Practical guidelines for the assessment of children with sickle cell disease. J Soc Pediatr Nurs 1999; 4: 61–73
Merkel S, Voepel-Lewis T, Shayevitz J, et al. The FLACC: a behavioral scale for scoring postoperative pain in young children. Pediatr Nurs 1997; 23: 293–7
McGrath PJ, Johnson G, Goodman JT, et al. The CHEOPS: a behavioral scale to measure postoperative pain in children. In: Fields HL, Dubner R, Cervero F, editors. Advances in pain research and therapy. Vol. 9. New York: Raven Press, 1985: 395–402
Gil KM, Phillips G, Edens J, et al. Observation of pain behaviors during episodes of sickle cell disease pain. Clin J Pain 1994; 10: 128–32
Beyer JE, Knott C. Construct validity estimation for the African-American and Hispanic Oucher scale. J Pediatr Nurs 1998; 13: 20–31
Wong DL, Baker CM. Pain in children: comparison of assessment scales. Pediatr Nurs 1988; 14: 9–17
Savedra MC, Tesler MD, Holzemer WL, et al. Testing a tool to assess postoperative pediatric and adolescent pain. In: Tyler DC, Krane RJ, editors. Advances in pain research and therapy. Vol. 15. New York: Raven Press, 1990: 85–94
Varni JW, Thompson KL, Janson V. The Varni/Thompson pediatric pain questionnaire (I): chronic musculoskeletal pain in juvenile rheumatoid arthritis. Pain 1987; 28: 27–38
World Health Organization. Cancer relief 1986. Geneva: Office of Publications, World Health Organization, 1986
Tobias J. Weak analgesics and nonsteroidal antiinflammatory agents in the management of children with acute pain. Pediatr Clin North Am 2000; 47(3): 527–43
Hardwick WE, Givens TG, Monroe KW, et al. Effect of ketorolac in pediatric sickle cell vaso-occlusive pain crisis. Pediatr Emerg Care 1999; 15: 179–82
Houck CS, Wilder RT, McDermott JS, et al. Safety of intravenous ketorolac therapy in children and cost savings with a unit dosing system. J Pediatr 1996; 129: 292–6
Grisham JE, Vichinshy EP. Ketorolac versus meperidine in vaso-occlusive crisis: a study of safety and efficacy. Int J Pediatr Hematol Oncol 1996; 3: 239–47
Griffin TC, McIntire D, Buchanan GR. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. N Engl J Med 1994; 330: 733–7
Woods GM, Parson PM, Strickland DK. Efficacy of nalbuphine as a parenteral anaglesic for the treatment of painful episodes in children with sickle cell disease. J Assoc Acad Minor Phys 1992; 3: 90–2
Nadvi SZ, Sarnaik S, Ravindranath Y. Low frequency of meperidine-associated seizures in sickle cell disease. Clin Pediatr 1999, 462
Jacobson S, Kopecky EA, Joshi P, et al. Randomized trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet 1997; 350(9088): 1358–61
Pegelow CH. Survey of pain management therapy provided for children with sickle-cell disease. Clin Pediatr 1992; 31: 211–4
Turner E, Shapiro B. Pharmacological management of pain. In: Shapiro B, Schechter N, Ohene-Frempong K, editors. The genetic resource, sickle cell disease-related pain: assessment and management. Mt Desert (ME): New England Regional Genetics Group, 1994: 27–38
Holbrook CT. Patient-controlled analgesia pain management for children with sickle cell disease. J Assoc Acad Minor Phys 1990; 1: 93–6
Shapiro BS, Cohen DE, Howe CJ. Patient-controlled analgesia for sickle cell-related pain. J Pain Symptom Manage 1993; 8: 22–8
Schechter NL, Berrien FB, Katz SM. The use of patient-controlled analgesia in adolescents with sickle-cell pain crisis: a preliminary report. J Pain Symptom Manage 1988; 3: 109–13
Stinson J, Naser B. Analgesie autocontrolee chez les enfants atteints d’anemie falciforme: L’experience d’un centre canadien. Doul et Analg 2000; 1: 21–6
Trentadue NO, Kachoyeanos MK, Lea G. A comparison of two regimens of patient-controlled analgesia for sickle cell disease. J Pediatr Nurs 1998; 13: 15–9
Robieux IC, Kellner JD, Coppes MJ, et al. Analgesia in children with sickle cell crisis: comparison of intermittent opioids vs continuous intravenous infusion of morphine and placebo-controlled study of oxygen inhalation. Pediatr Hematol Oncol 1992; 9: 317–26
Cole TB, Sprinkle RH, Smith SJ, et al. Intravenous narcotic therapy for children with severe sickle cell crisis. Am J Dis Child 1986; 140: 1255–9
Palozzi L, Stinson J. Patient controlled analgesia: nursing self-learning module. Toronto (ON): The Hospital for Sick Children, 2000
Monitto CL, Greenberg RS, Kost-Byerly S, et al. The safety and efficacy of parent-/nurse-controlled analgesia in patients less than six years of age. Anesth Analg 2000; 91: 573–9
Yaster M, Tobias JR, Billette C, et al. Epidural analgesia in the management of severe vaso-occlusive sickle cell crisis. Pediatrics 1994; 93: 310–5
Tobias JD. Indications and application of epidural anesthesia in a pediatric population outside the perioperative period. Clin Pediatr 1993; 32(2): 81–5
Christensen ML, Wang WC, Harris S, et al. Transdermal fentanyl administration in children and adolescents with sickle cell disease. J Pediatr Hematol Oncol 1996; 18: 372–6
Dampier CD, Setty BN, Logan J, et al. Intravenous morphine pharmacokinetics in pediatric patients with sickle-cell disease. J Pediatr 1995; 126: 461–7
Koumoukelis H. Paediatric pain management dosing guidelines. Toronto (ON): Department of Anaesthesia, The Hospital for Sick Children, 2002
Walco GA, Erickson CJ, Eckman J. Non-pharmacologic management of pain. In: Shapiro BS, Schechter NL, Ohene-Frempong K, editors. The genetic resource, sickle cell disease-related pain: assessment and management. Mt Desert (ME): New England Regional Genetics Group, 1994: 39–42
Anie KA, Green J. Psychological therapies for sickle cell disease and pain (Cochrane Review). Available in The Cochrane Library [database on disk and CD ROM]. Updated quarterly. The Cochrane Collaboration; issue 3. Oxford: Oxford Update Software, 2001
Dinges DF, Whitehouse WG, Orne EC, et al. Self-hypnosis training as an adjunctive treatment in the management of pain associated with sickle cell disease. Int J Clin Exp Hypn 1997; 45(4): 417–32
Thomas VN, Wison-Barnett J, Goodhart F. The role of cognitive-behavioral therapy in the management of pain in patients with sickle cell disease. J Advan Nurs 1998; 27: 1002–9
Cozzi L, Tyron WW, Sedlacek K. The effectiveness of biofeedback-assisted relaxation in modifying sickle cell crisis. Biofeedback Self Regul 1987; 12: 51–61
National Institutes of Health. Acupuncture: NIH consensus statement 1998. Bethesda (MD): National Institutes of Health, 1998
National Institutes of Health Technology Assessment Panel. Integration of behavioral and relaxation approaches into the treatment of chronic pain and insomnia. JAMA 1996; 276: 313–8
Wang WC, George SL, Willimas JA. Transcutaneous electrical nerve stimulation treatment of sickle-cell pain crisis. Acta Haematol 1988; 80: 99–102
Zeltzer L, Dash J, Holland JP. Hypnotically induced pain control in sickle cell anemia. Pediatrics 1979; 64: 533–6
Acknowledgements
The authors would like to acknowledge Dr J. Friedman, Department of Paediatric Medicine, and Lori Palozzi, RN, MScN, ACNP, Department of Anaesthesia, The Hospital for Sick Children, for their thoughtful feedback in the preparation of this manuscript.
Jennifer Stinson is funded by the Canadian Nurses Foundation/Hospital for Sick Children’s Foundation/Canadian Institute of Health Research, and the Hospital for Sick Children Clinician Scientist Fellowship. The author has no conflicts of interest that are directly relevant to the content of this manuscript.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Stinson, J., Naser, B. Pain Management in Children with Sickle Cell Disease. Pediatr-Drugs 5, 229–241 (2003). https://doi.org/10.2165/00128072-200305040-00003
Published:
Issue Date:
DOI: https://doi.org/10.2165/00128072-200305040-00003