Individuals with sickle cell disease (SCD) face unique challenges and barriers to care as they age and transition from pediatric to adult care settings.
Objective:
The purpose of this scoping review was to explore the factors that influence the health behaviors of individuals with SCD after they have transitioned to adult care settings.
Methods:
This scoping review followed the Joanna Briggs Institute methodology. PubMed, Web of Science, and CINAHL were searched in March 2024 to identify articles meeting the inclusion criteria. A total of 162 articles were identified and imported into Covidence and screened for eligibility.
Results:
A total of 19 articles were included in this review. The content analysis revealed 2 main themes with underlying subthemes. Theme 1: interpersonal factors with subthemes (a) the need for independence and (b) identity conflict, and Theme 2: environmental factors with subthemes (a) lack of knowledge and resulting stigma and (b) access to care.
Discussion:
The results of this scoping review demonstrate that there is a complex interaction of interpersonal and environmental factors that influence the health behaviors of individuals with SCD after they transition to adult care settings. Further research should explore the social environment and individual psychosocial challenges that patients with SCD face. Results can help inform interventions to prepare adolescents and young adults, as well as their families, for the transition to adult care settings.
KanterJSmithWRDesaiPC, et al. Building access to care in adult sickle cell disease defining models of care, essential components, and economic aspects. Blood Adv. 2020;4(16):3804-3813. doi:10.1182/bloodadvances.2020001743
PorterJSWesleyKMRupffRJHankinsJSZhaoMS.Pediatric to adult care transition: perspectives of young adults with sickle cell disease. J Pediatr Psychol. 2017;42(9):1016-1027. doi:10.1093/jpepsy/jsx088
8.
RenedoAMilesSChakravortyS, et al. Not being heard: barriers to high quality unplanned hospital care during young people’s transition to adult services—evidence from “this sickle cell life” research. BMC Health Serv Res. 2019;19(1):876. doi:10.1186/s12913-019-4726-5
9.
EssienEAWinter-EtengBFOnukoguCUNkanghaDDDanielFM.Psychosocial challenges of persons with sickle cell anemia: a narrative review. Medicine. 2023;102(47):e36147. doi:10.1097/MD.0000000000036147
10.
NjokuFPughNBrambillaD, et al. Mortality in adults with sickle cell disease: results from the Sickle Cell Disease Implementation Consortium (SCDIC) registry. Am J Hematol. 2024;99(5):900-909. doi:10.1002/ajh.27279
11.
KarkoskaKMcGannPT.Changing trends in sickle cell disease-related mortality in the United States over four decades. Blood. 2023;142(suppl 1):925. doi:10.1182/blood-2023-177988
12.
LubeckDAgodoaIBhaktaN, et al. Estimated life expectancy and income of patients with sickle cell disease compared with those without sickle cell disease. JAMA Netw Open. 2019; 2(11):e1915374. doi:10.1001/jamanetworkopen.2019.15374
13.
WhitePSchmidtAShorrJIlangoSBeckDMcManusM. Six Core Elements of Health Care Transition 3.0. Got Transition, The National Alliance to Advance Adolescent Health; 2020.
14.
SaulsberryACPorterJSHankinsJS.A program of transition to adult care for sickle cell disease. Hematology. 2019; 2019(1):496-504. doi:10.1182/hematology.2019000054
15.
ArjunanAMossDDe CastroLM.Healthcare utilization patterns and health quality indicators in sickle cell disease patients transitioning from pediatric to adulthood. Blood. 2018;132(suppl 1):3513. doi:10.1182/blood-2018-99-117558
16.
BlinderMADuhMSSasaneMTraheyAPaleyCVekemanF.Age-related emergency department reliance in patients with sickle cell disease. J Emerg Med. 2015;49(4):513-522. doi:10.1016/j.jemermed.2014.12.080
17.
Clayton-JonesDMatthieNTreadwellM, et al. Social and psychological factors associated with health care transition for young adults living with sickle cell disease. J Transcult Nurs. 2021;32(1):21-29. doi:10.1177/1043659619896837
18.
KayleMDochertySLSloaneR, et al. Transition to adult care in sickle cell disease: a longitudinal study of clinical characteristics and disease severity. Pediatr Blood Cancer. 2019;66(1):e27463. doi:10.1002/pbc.27463
GuarinoSHJainAMadisettiM, et al. Establishing a definition of transition of care in sickle cell disease: National Alliance of Sickle Cell Disease Centers consensus. Blood. 2024;144(suppl 1):3622. doi:10.1182/blood-2024-207698
21.
AuroraTNascimentoEMouraP, et al. Variability in health outcomes according to pediatric to adult transfer-of-care status among young adults with sickle cell disease. J Adolesc Health. 2025;76(6):1001-1007. doi:10.1016/j.jadohealth.2025.01.023
22.
GrismoreCRobertsLRListerZD, et al. Barriers to care for adults with sickle cell disease: a qualitative descriptive study. Health Expect. 2025;28(3):e70310. doi:10.1111/hex.70310
23.
PorterJS.Advancing health care transition for sickle cell disease—a path forward through standardization. JAMA Netw Open. 2025;8(4):e254974. doi:10.1001/jamanetworkopen.2025.4974
24.
ViolaAPorterJShipmanJBrooksEValrieC.A scoping review of transition interventions for young adults with sickle cell disease. Pediatr Blood Cancer. 2021;68(9):e29135. doi:10.1002/pbc.29135
25.
CaldwellEPRosonetLE.The influence of health-seeking behaviors on the health literacy of adolescents with sickle cell disease. J Pediatr Oncol Nurs. 2021;38(5):307-312. doi:10.1177/10434542211011045
26.
CrearySEModiACStanekJR, et al. Allocation of treatment responsibility and adherence to hydroxyurea among adolescents with sickle cell disease. J Pediatr Psychol. 2019;44(10):1196-1204. doi:10.1093/jpepsy/jsz061
27.
Goldstein-LeeverAPeughJLQuinnCTCrosbyLE.Disease self-efficacy and health-related quality of life in adolescents with sickle cell disease. J Pediatr Hematol Oncol. 2020;42(2): 141-144. doi:10.1097/mph.0000000000001363
28.
HowellKEHeitzerAMLongoriaJN, et al. Assessment of transition readiness to predict health care utilization during transition to adult care in sickle cell disease. Expert Rev Hematol. 2022;15(12):1063-1072. doi:10.1080/17474086.2022.2144216
29.
JonesKENymanTMDalyBPJacobsonLATaraziRA.Executive functioning predicts adaptive functioning and self-care independence in pediatric sickle cell disease. J Pediatr Psychol. 2022;47(2):206-214. doi:10.1093/jpepsy/jsab094
30.
Kwarteng-SiawMPaintsilVTobohCKOwusu-AnsahAGreenNS.Assessment of transition readiness in adolescents with sickle cell disease and their caretakers, a single institution experience. Int J Hematol Res. 2017;3(1):171-179. doi:10.17554/j.issn.2409-3548.2017.03.47
31.
ManwaniDDoyleMHDavidsonL, et al. Transition navigator intervention improves transition readiness to adult care for youth with sickle cell disease. Acad Pediatr. 2022;22(3):422-430. doi:10.1016/j.acap.2021.08.005
32.
Speller-BrownBVartyMThanielLJacobsMB.Assessing disease knowledge and self-management in youth with sickle cell disease prior to transition. J Pediatr Oncol Nurs. 2018;2018:1043454218819447. doi:10.1177/1043454218819447
33.
TreadwellMJohnsonSSislerI, et al. Self-efficacy and readiness for transition from pediatric to adult care in sickle cell disease. Int J Adolesc Med Health. 2016;28(4):381-388. doi:10.1515/ijamh-2015-0014
34.
VartyMSpeller-BrownBWakefieldBJRavertRDKellyKPPopejoyLL.Decision-making involvement, self-efficacy, and transition readiness in youth with sickle cell disease. Nurs Res. 2022;71(1):12-20. doi:10.1097/NNR.0000000000000550
PollockDPetersMDJKhalilH, et al. Recommendations for the extraction, analysis, and presentation of results in scoping reviews. JBI Evid Synth. 2023;21(3):520-532. doi:10.11124/JBIES-22-00123
37.
Bemrich-StolzCJHalanychJHHowardTHHilliardLMLebensburgerJD.Exploring adult care experiences and barriers to transition in adult patients with sickle cell disease. Int J Hematol Ther. 2015;1(1):10.15436/2381-1404.15.003. doi:10.15436/2381-1404.15.003
38.
LaboreNMawnBDixonJAndemariamB.Exploring transition to self-management within the culture of sickle cell disease. J Transcult Nurs. 2017;28(1):70-78. doi:10.1177/1043659615609404
39.
MatthieNHamiltonJWellsDJeneretteC.Perceptions of young adults with sickle cell disease concerning their disease experience. J Adv Nurs. 2016;72(6):1441-1451. doi:10.1111/jan.12760
40.
RenedoAMilesSMarstonC.Transitions to adulthood: self-governance and disciplining in the making of patient citizens. Sociol Health Illn. 2020;42(3):481-495. doi:10.1111/1467-9566.13019
41.
SobotaAEUmehEMackJW.Young adult perspectives on a successful transition from pediatric to adult care in sickle cell disease. J Hematol Res. 2015;2(1):17-24. doi:10.12974/2312-5411.2015.02.01.3
42.
ViolaASLevonyan-RadloffKDrachtmanR, et al. Understanding barriers to transition from pediatric to adult care among young adults with sickle cell disease to develop a transition mentor program. Clin Pract Pediatr Psychol. 2021;9(1):68-81. doi:10.1037/cpp0000372
43.
MatthieNRossDSinhaCKhemaniKBakshiNKrishnamurtiL.A qualitative study of chronic pain and self-management in adults with sickle cell disease. J Natl Med Assoc. 2019;111(2):158-168. doi:10.1016/j.jnma.2018.08.001
44.
McClishDKSmithWRLevensonJL, et al. Comorbidity, pain, utilization, and psychosocial outcomes in older versus younger sickle cell adults: the PiSCES project. Biomed Res Int. 2017;2017:4070547. doi:10.1155/2017/4070547
45.
SinghAYanKBrandowAMPanepintoJA.Longitudinal trend in emergency department reliance for pain among sickle cell disease patients in Wisconsin. J Pediatr Hematol Oncol. 2019;41(7):e438-e442. doi:10.1097/mph.0000000000001533
46.
GallagherMEChawlaABradyBLBadawySM.Heterogeneity of the long-term economic burden of severe sickle cell disease: a 5-year longitudinal analysis. J Med Econ. 2022; 25(1):1140-1148. doi:10.1080/13696998.2022.2133824
47.
KayleMValleJPaulukonisS, et al. Impact of Medicaid expansion on access and healthcare among individuals with sickle cell disease. Pediatr Blood Cancer. 2020;67(5):e28152. doi:10.1002/pbc.28152
48.
MainousAGRooksBTannerRJCarekPJBlackVCoatesTD.Shared care for adults with sickle cell disease: an analysis of care from eight health systems. J Clin Med. 2019;8(8):1154. doi:10.3390/jcm8081154
49.
NworaCPrinceEJPughLWeaverMSPeckerLH.How young adults with sickle cell disease define “being a good patient” in the adult healthcare system. Pediatr Blood Cancer. 2024;71(2):e30786. doi:10.1002/pbc.30786
50.
SezginEWeilerMWeilerALinSHartL.It is a life journey: a roadmap of teens with chronic diseases in transitioning to independence. J Pediatr Health Care. 2020;34(4):346-355. doi:10.1016/j.pedhc.2020.02.001
51.
MartireLMHelgesonVS.Close relationships and the management of chronic illness: associations and interventions. Am Psychol. 2017;72(6):601-612. doi:10.1037/amp0000066
WhittemoreRDixonJ.Chronic illness: the process of integration. J Clin Nurs. 2008;17(7b):177-187. doi:10.1111/j.1365-2702.2007.02244.x
54.
BulginDTanabePJeneretteC.Stigma of sickle cell disease: a systematic review. Issues Ment Health Nurs. 2018;39(8):675-686. doi:10.1080/01612840.2018.1443530
55.
FarooqFMogayzelPJLanzkronSHaywoodCStrouseJJ.Comparison of US federal and foundation funding of research for sickle cell disease and cystic fibrosis and factors associated with research productivity. JAMA Netw Open. 2020;3(3): e201737. doi:10.1001/jamanetworkopen.2020.1737
56.
StrouseJJLobnerKLanzkronSMHaywoodC.NIH and national foundation expenditures for sickle cell disease and cystic fibrosis are associated with PubMed publications and FDA approvals. Blood. 2013;122(21):1739. doi:10.1182/blood.V122.21.1739.1739
57.
BahrNCSongJ.The effect of structural violence on patients with sickle cell disease. J Health Care Poor Underserved. 2015;26(3):648-661. doi:10.1353/hpu.2015.0094
58.
SmithWRValrieCSislerI.Structural racism and impact on sickle cell disease: sickle cell lives matter. Hematol Oncol Clin North Am. 2022;36(6):1063-1076. doi:10.1016/j.hoc.2022.08.008
59.
Power-HaysAMcGannPT.When actions speak louder than words—racism and sickle cell disease. N Engl J Med. 2020;383(20):1902-1903. doi:10.1056/NEJMp2022125
ReichJCantrellMASmeltzerSC.An integrative review: the evolution of provider knowledge, attitudes, perceptions and perceived barriers to caring for patients with sickle cell disease 1970-now. J Pediatr Hematol Oncol Nurs. 2023;40(1):43-64. doi:10.1177/27527530221090179
63.
BrownTBrownSDelerCRivlinK.Insights from a sickle cell disease community-based organization stigma intervention. J Sickle Cell Dis. 2024;1(suppl 1):53. doi:10.1093/jscdis/yoae002.033
64.
JeneretteCMBrewerCAAtagaKI.Care seeking for pain in young adults with sickle cell disease. Pain Manag Nurs. 2014;15(1):324-330. doi:10.1016/j.pmn.2012.10.007
65.
MulchanSSValenzuelaJMCrosbyLEDiaz Pow SangC.Applicability of the SMART model of transition readiness for sickle-cell disease. J Pediatr Psychol. 2016;41(5):543-554. doi:10.1093/jpepsy/jsv120
66.
FenchelLJacksonFWalkerB, et al. Improving transition of emerging adults with sickle cell disease to adult care through a multidisciplinary process: the development of a transition clinic to support transition success. Blood. 2023;142(suppl 1): 5055. doi:10.1182/blood-2023-177747
ThomsonAMMcHughTAOronAP, et al. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021. Lancet Haematol. 2023;10(8): e585-e599. doi:10.1016/S2352-3026(23)00118-7
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