**5. Future of SCD pain treatment**

Until SCD can be fully cured (possibly with gene therapy), new and improved treatment modalities are necessary. The information currently available about SCD is that rapid and aggressive therapy at the first sign of a VOE help reduce the length of the event and even abort it. Currently, patients with VOE receive treatment 2–3 days after the onset of the prodromal signs [16]. Measurement should be made in the future to offer patients with SCD methods of treatment available at home such as opioids (IV or IM), supplemental oxygen, and rapid hydration methods. Anti-inflammatory agents and oral opioids represent the current home treatment modalities. Vasodilators also represent a fundamental therapeutic weapon for VOE, especially when used early. Administration of nitric oxide (NO) in the emergency department has shown to abort the crisis in some patients; however, no benefice was found when NO was used during the hospital stay [59–61]. Perhaps NO could be offer as a home medication and its use would be even earlier than it would be in the ED. Recently, reports have emerged of small doses of opioid antagonist in combination with an agonist enhanced the analgesic effect and delayed the development of tolerance [62]. Recent trials targeting the inhibition of the capsaicin receptor transient receptor potential vanillin 1 (TRPV1) showed promising results in relieving pain in patients with SCD [63].

**References**

10665/1682

gov/books/NBK1377/

Mar;**9**(2):111-116

jama/fullarticle/357392

630269.2011.610478

cdc.gov/ncbddd/sicklecell/data.html

Opinion in Hematology. 2002 Mar;**9**(2):101-106

brary.wiley.com/doi/abs/10.1002/ajh.20336

Society of Hematology. Education Program. 2007;**1**:97-105

[1] Regional Committee for Africa 60. S-Cell Disease: A strategy for the WHO African Region. 2011 May 26 [cited 2018 Apr 28]. Available from: http://apps.who.int/iris/handle/

Pain Management for the Sickle Cell Patient http://dx.doi.org/10.5772/intechopen.79495 29

[2] Bender MA. Sickle Cell Disease. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJ, Stephens K, et al, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993 [cited 2018 Apr 28]. Available from: http://www.ncbi.nlm.nih.

[3] Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: An increasing global health

[4] CDC. Data and Statistics | Sickle Cell Disease | NCBDDD | CDC [Internet]. Centers for Disease Control and Prevention. 2016 [cited 2018 Apr 30]. Available from: https://www.

[5] Diallo D, Tchernia G. Sickle cell disease in Africa. Current Opinion in Hematology. 2002

[6] Scott RB. Health care priority and sickle cell anemia. JAMA [Internet]. 1970 Oct 26 [cited 2018 Apr 7];**214**(4):731-734. Available from: https://jamanetwork.com/journals/

[7] Ballas SK. Update on pain management in sickle cell disease. Hemoglobin [Internet]. 2011 Oct 1 [cited 2018 Apr 5];**35**(5-6):520-529. Available from: https://doi.org/10.3109/03

[8] Frenette PS. Sickle cell vaso-occlusion: Multistep and multicellular paradigm. Current

[9] Ballas SK. Current issues in sickle cell pain and its management. Hematology. American

[10] Plankton S, Haywood C, Segal JB, Dover GJ. Hospitalization rates and costs of care of patients with sickle-cell anaemia in the state of Maryland in the era of Hydroxyurea. American Journal of Haematology [Internet]. 2006 Dec 1 [cited 2018 Apr 4];**81**(12): 927-932. Available from: https://onlinelibrary.wiley.com/doi/abs/10.1002/ajh.20703 [11] Ballas SK, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. American Journal of Hematology [Internet]. 2005 May 1 [cited 2018 May 23];**79**(1):17-25. Available from: https://onlineli-

[12] AlJuburi G, Laverty AA, Green SA, Phekoo KJ, Bell D, Majeed A. Socio-economic deprivation and risk of emergency readmission and inpatient mortality in people with sickle cell disease in England: observational study. Journal of Public Health (Oxford, England)

problem. Bulletin of the World Health Organization. 2001;**79**(8):704-712
