Skip to main content
Log in

The management of sickle cell pain

  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Treatment of pain in the setting of sickle cell disease remains unsatisfactory. The approach remains to treat the pain symptomatically with escalating doses of non-opioid and opioid medications while any underlying inciting process is investigated. For the majority of patients with sickle cell disease, pain will always be part of their lives. Advances in the treatment of sickle cell pain will depend on multiple approaches, including both pharmacologic and nonpharmacologic.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Shapiro BS, Benjamin LJ, Payne R, Heidrich G: Sickle cell-related pain: perceptions of medical practitioners. J Pain Symptom Manage 1997, 14:168–174.

    Article  PubMed  CAS  Google Scholar 

  2. Wright K, Adeosum O: Barriers to effective pain management in sickle cell disease. Br J Nurs 2009, 18:158–161.

    PubMed  Google Scholar 

  3. Platt OS: Hydroxyurea for the treatment of sickle cell anemia. N Engl J Med 2008, 358:1362–1369.

    Article  PubMed  CAS  Google Scholar 

  4. Platt OS, Thorington BD, Brambilla DJ, et al.: Pain in sickle cell disease. Rates and risk factors. N Engl J Med 1991, 325:11–16.

    PubMed  CAS  Google Scholar 

  5. Alexander N, Higgs D, Dover G, Serjeant GR: Are there clinical phenotypes of homozygous sickle cell disease? Br J Haematol 2004, 126:606–611.

    Article  PubMed  Google Scholar 

  6. Smith WR, Penberthy LT, Bovbjerg VE, et al.: Daily assessment of pain in adults with sickle cell disease. Ann Intern Med 2008, 148:94–101.

    PubMed  Google Scholar 

  7. Bunn HF: Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997, 337:762–769.

    Article  PubMed  CAS  Google Scholar 

  8. Turhan A, Weiss LA, Mohandas N, et al.: Primary role for adherent leukocytes in sickle cell vascular occlusion: a new paradigm. Proc Natl Acad Sci U S A 2002, 99:3047–3051.

    Article  PubMed  CAS  Google Scholar 

  9. Kato GJ, Gladwin MT, Steinberg MH: Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 2007, 21:37–47.

    Article  PubMed  Google Scholar 

  10. Kato GJ, Gladwin MT: Evolution of novel small-molecule therapeutics targeting sickle cell vasculopathy. JAMA 2008, 300:2638–2646.

    Article  PubMed  CAS  Google Scholar 

  11. Morrissey LK, Shea JO, Kalish LA, et al.: Clinical practice guideline improves the treatment of sickle cell disease vasoocclusive pain. Pediatr Blood Cancer 2009, 52:369–372.

    Article  PubMed  Google Scholar 

  12. Melzer-Lange MD, Walsh-Kelly CM, Lea G, et al.: Patient-controlled analgesia for sickle cell pain crisis in a pediatric emergency department. Pediatr Emerg Care 2004, 20:2–4.

    Article  PubMed  Google Scholar 

  13. van Beers EJ, van Tuijn CF, Nieuwkerk PT, et al.: Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol 2007, 82:955–960.

    Article  PubMed  Google Scholar 

  14. Setty BN, Rao AK, Stuart MJ: Thrombophilia in sickle cell disease: the red cell connection. Blood 2001, 98:3228–3233.

    Article  PubMed  CAS  Google Scholar 

  15. Austin H, Key NS, Benson JM, et al.: Sickle cell trait and the risk of venous thromboembolism among blacks. Blood 2007, 110:908–912.

    Article  PubMed  CAS  Google Scholar 

  16. Charache S, Terrin ML, Moore RD, et al.: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995, 332:1317–1322.

    Article  PubMed  CAS  Google Scholar 

  17. Steinberg MH, Barton F, Castro O, et al.: Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA 2003, 289:1645–1651.

    Article  PubMed  CAS  Google Scholar 

  18. Kinney TR, Helms RW, O’Branski EE, et al.: Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood 1999, 94:1550–1554.

    PubMed  CAS  Google Scholar 

  19. Zimmerman SA, Schultz WH, Burgett S, et al.: Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia. Blood 2007, 110:1043–1047.

    Article  PubMed  CAS  Google Scholar 

  20. Brawley OW, Cornelius LJ, Edwards LR, et al.: National Institutes of Health Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med 2008, 148:932–938.

    PubMed  Google Scholar 

  21. Vichinsky EP, Neumayr LD, Earles AN, et al.: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000, 342:1855–1865.

    Article  PubMed  CAS  Google Scholar 

  22. Barakat LP, Patterson CA, Daniel LC, Dampier C: Quality of life among adolescents with sickle cell disease: mediation of pain by internalizing symptoms and parenting stress. Health Qual Life Outcomes 2008, 6:60.

    Article  PubMed  Google Scholar 

  23. Pence L, Valrie CR, Gil KM, et al.: Optimism predicting daily pain medication use in adolescents with sickle cell disease. J Pain Symptom Manage 2007, 33:302–309.

    Article  PubMed  Google Scholar 

  24. Angst MS, Clark JD: Opioid-induced hyperalgesia: a qualitative systematic review. Anesthesiology 2006, 104:570–587.

    Article  PubMed  CAS  Google Scholar 

  25. Benjamin L: Pain management in sickle cell disease: palliative care begins at birth? Hematology Am Soc Hematol Educ Program 2008, 2008:466–474.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Robert E. Richard.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Richard, R.E. The management of sickle cell pain. Current Science Inc 13, 295–297 (2009). https://doi.org/10.1007/s11916-009-0047-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11916-009-0047-4

Keywords

Navigation