Skip to main content Accessibility help
×
Hostname: page-component-848d4c4894-pftt2 Total loading time: 0 Render date: 2024-05-17T19:14:19.527Z Has data issue: false hasContentIssue false

Chapter 28 - Sickle Cell Disease

from Part VI - Misc

Published online by Cambridge University Press:  01 December 2023

Omar Viswanath
Affiliation:
Creighton University, Omaha
Ivan Urits
Affiliation:
Southcoast Brain & Spine Center, Wareham
Get access

Summary

Sickle cell crisis is a term used to capture myriad acute manifestations of sickle cell disease (SCD). Underlying pathophysiology is due to polymerization of HbS, sickling of erythrocytes, and microvascular occlusion and injury. Sickle cell disease and related pain is common in the US, especially among black Americans. Routine newborn screening picks up the majority of cases and helps guide prevention and treatment of acute pain crises early on. The mainstay of treating vaso-occlusive crises is analgesia and is often achieved with NSAIDs, opioids, or combination therapy.

Type
Chapter
Information
Publisher: Cambridge University Press
Print publication year: 2023

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Borhade, MB, Kondamudi, NP. Sickle cell crisis. In StatPearls. 2022. pp. 365379. Treasure Island (FL): StatPearls Publishing.Google Scholar
Sundd, P, Gladwin, MT, Novelli, EM. Pathophysiology of sickle cell disease. Annu Rev Pathol Mech Dis. 2019;14(1):421058352. doi: 10.1146/annurev-pathmechdis-012418-012838.CrossRefGoogle ScholarPubMed
Dampier, C, Palermo, TM, Darbari, DS et al. AAPT diagnostic criteria for chronic sickle cell disease pain. J Pain. 2017;18(5):490498.CrossRefGoogle ScholarPubMed
Brandow, AM, DeBaun, MR. Key components of pain management for children and adults with sickle cell disease. Hematol Oncol Clin North Am. 2018;32(3):535550.CrossRefGoogle ScholarPubMed
Strouse, J. Sickle cell disease. Handb Clin Neurol. 2016;138:311324.CrossRefGoogle ScholarPubMed
Azar, S, Wong, TE. Sickle cell disease: A brief update. Med Clin North Am. 2017;101(2):375393.CrossRefGoogle ScholarPubMed
Cacciotti, C, Vaiselbuh, S, Romanos-Sirakis, E. Pain management for sickle cell disease in the pediatric emergency department: Medications and hospitalization trends. Clin Pediatr. 2017;56:11091114.CrossRefGoogle ScholarPubMed
Tran, H, Gupta, M, Gupta, K. Targeting novel mechanisms of pain in sickle cell disease. Blood. 2017;130(22):23772385.CrossRefGoogle ScholarPubMed
Nobrega, R, Sheehy, KA, Lippold, C et al. Patient characteristics affect the response to ketamine and opioids during the treatment of vaso-occlusive episode-related pain in sickle cell disease. Pediatr Res. 2018;83(2):445454.CrossRefGoogle ScholarPubMed
Riley, TR, Boss, A, McClain, D, Riley, TT. Review of medication therapy for the prevention of sickle cell crisis. Pharm Ther. 2018;43(7):417437.Google ScholarPubMed
Lubega, FA, DeSilva, MS, Munube, D et al. Low dose ketamine versus morphine for acute severe vaso occlusive pain in children: A randomized controlled trial. Scand J Pain. 2018;18(1):1927.CrossRefGoogle ScholarPubMed
Palm, N, Floroff, C, Hassig, TB, Boylan, A, Kanter, J. Low-dose ketamine infusion for adjunct management during vaso-occlusive episodes in adults with sickle cell disease: A case series. J Pain Palliat Care Pharmacother. 2018;32(1):2026.CrossRefGoogle ScholarPubMed
Young, JR, Sawe, HR, Mfinanga, JA et al. Subdissociative intranasal ketamine plus standard pain therapy versus standard pain therapy in the treatment of paediatric sickle cell disease vaso-occlusive crises in resource-limited settings: Study protocol for a randomised controlled trial. BMJ Open. 2017;7(7):e017190.CrossRefGoogle ScholarPubMed
Kelly, GS, Stewart, RW, Strouse, JJ, Anders, JF. Intranasal fentanyl improves time to analgesic delivery in sickle cell pain crises. Am J Emerg Med. 2018;36(7):13051307. doi: 10.1016/j.ajem.2017.11.015.CrossRefGoogle ScholarPubMed
Rousseau, V, Morelle, M, Arriuberge, C et al. Efficacy and tolerance of lidocaine 5% patches in neuropathic pain and pain related to vaso-occlusive sickle cell crises in children: A prospective multicenter clinical study. Pain Pract. 2018;18(6):788797.CrossRefGoogle ScholarPubMed
Brittain, JE, Anea, C, Desai, P et al. Effect of eptifibatide on inflammation during acute pain episodes in sickle cell disease. Am J of Hematol. 2018;93:E99E101.CrossRefGoogle ScholarPubMed
Bhatt, RR, Martin, SR, Evans, S et al. The effect of hypnosis on pain and peripheral blood flow in sickle-cell disease: A pilot study. J Pain Res. 2017;10:16351644.CrossRefGoogle ScholarPubMed
Agrawal, AK, Robertson, S, Litwin, L et al. Virtual reality as complementary pain therapy in hospitalized patients with sickle cell disease. Pediatr Blood Cancer. 2019;66(2):e27525. doi: 10.1002/pbc.27525.CrossRefGoogle ScholarPubMed
Palermo, TM, Zempsky, WT, Dampier, CD et al. iCanCope with sickle cell pain: Design of a randomized controlled trial of a smartphone and web-based pain self-management program for youth with sickle cell disease. Contemp Clin Trials. 2018;74:8896.CrossRefGoogle ScholarPubMed
Palermo, TM, Dudeney, J, Santanelli, JP, Carletti, A, Zempsky, WT. Feasibility and acceptability of internet-delivered cognitive behavioral therapy for chronic pain in adolescents with sickle cell disease and their parents. J Pediatr Hematol Oncol. 2018;40(2):122127.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×