Abstract
Sickle cell disease is an illness that predominantly affects African Americans in the United States, where it is an important and under-recognized problem. In this country, research funding for sickle cell disease is substantially lower than that for other monogenic diseases such as cystic fibrosis. Such disparities in research funding lead to limited knowledge translation or therapeutic innovation, ultimately affecting the care of patients with sickle cell disease. Moreover, disparities in access to and quality of healthcare affect outcomes in sickle cell disease, including those for respiratory health. Several steps, including increased research funding from private foundations and government agencies, standardization and accreditation for the delivery of care, and other measures to reduce healthcare disparities (e.g., improved management of pain and co-existing asthma), should have substantial benefits for subjects with SCD, including prolonging and improving life while reducing healthcare costs.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Scott RB. Sickle-cell anemia—high prevalence and low priority. N Engl J Med. 1970;282(3):164–5. doi:10.1056/NEJM197001152820312.
McClish DK, Smith WR, Dahman BA, Levenson JL, Roberts JD, Penberthy LT, et al. Pain site frequency and location in sickle cell disease: the PiSCES project. Pain. 2009;145(1–2):246–51. doi:10.1016/j.pain.2009.06.029.
Hassell K, Pace B, Wang W, Kulkarni R, Luban N, Johnson CS, et al. Sickle cell disease summit: from clinical and research disparity to action. Am J Hematol. 2009;84(1):39–45. doi:10.1002/ajh.21315.
Strouse JJ, Lobner K, Lanzkron S, Haywood C. 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.
Smith LA, Oyeku SO, Homer C, Zuckerman B. Sickle cell disease: a question of equity and quality. Pediatrics. 2006;117(5):1763–70. doi:10.1542/peds.2005-1611.
Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115(17):3447–52. doi:10.1182/blood-2009-07-233700.
Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 2004;103(11):4023–7. doi:10.1182/blood-2003-11-3758.
Serjeant GR. Treatment of sickle cell disease in early childhood in Jamaica. Am J Pediatr Hematol Oncol. 1985;7(3):235–9.
Woods K, Karrison T, Koshy M, Patel A, Friedmann P, Cassel C. Hospital utilization patterns and costs for adult sickle cell patients in Illinois. Public Health Rep. 1997;112(1):44–51.
Raphael JL, Dietrich CL, Whitmire D, Mahoney DH, Mueller BU, Giardino AP. Healthcare utilization and expenditures for low income children with sickle cell disease. Pediatr Blood Cancer. 2009;52(2):263–7. doi:10.1002/pbc.21781.
Carroll CP, Haywood Jr C, Fagan P, Lanzkron S. The course and correlates of high hospital utilization in sickle cell disease: evidence from a large, urban Medicaid managed care organization. Am J Hematol. 2009;84(10):666–70. doi:10.1002/ajh.21515.
Lanzkron S, Carroll CP, Haywood Jr C. The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database. Am J Hematol. 2010;85(10):797–9. doi:10.1002/ajh.21807.
Laurence B, Haywood Jr C, Lanzkron S. Dental infections increase the likelihood of hospital admissions among adult patients with sickle cell disease. Community Dent Health. 2013;30(3):168–72.
Aisiku IP, Penberthy LT, Smith WR, Bovbjerg VE, McClish DK, Levenson JL, et al. Patient satisfaction in specialized versus nonspecialized adult sickle cell care centers: the PiSCES study. J Natl Med Assoc. 2007;99(8):886–90.
Porter J, Feinglass J, Artz N, Hafner J, Tanabe P. Sickle cell disease patients’ perceptions of emergency department pain management. J Natl Med Assoc. 2012;104(9–10):449–54.
Haywood Jr C, Lanzkron S, Ratanawongsa N, Bediako SM, Lattimer-Nelson L, Beach MC. Hospital self-discharge among adults with sickle-cell disease (SCD): associations with trust and interpersonal experiences with care. J Hosp Med. 2010;5(5):289–94. doi:10.1002/jhm.643.
Haywood Jr C, Lanzkron S, Ratanawongsa N, Bediako SM, Lattimer L, Powe NR, et al. The association of provider communication with trust among adults with sickle cell disease. J Gen Intern Med. 2010;25(6):543–8. doi:10.1007/s11606-009-1247-7.
Elander J, Beach MC, Haywood Jr C. Respect, trust, and the management of sickle cell disease pain in hospital: comparative analysis of concern-raising behaviors, preliminary model, and agenda for international collaborative research to inform practice. Ethn Health. 2011;16(4–5):405–21. doi:10.1080/13557858.2011.555520.
Bediako SM, Lanzkron S, Diener-West M, Onojobi G, Beach MC, Haywood Jr C. The measure of sickle cell stigma: initial findings from the improving patient outcomes through respect and trust study. J Health Psychol. 2014;21:808–20. doi:10.1177/1359105314539530.
Haywood Jr C, Williams-Reade J, Rushton C, Beach MC, Geller G. Improving clinician attitudes of respect and trust for persons with sickle cell disease. Hosp Pediatr. 2015;5(7):377–84. doi:10.1542/hpeds.2014-0171.
Lazio MP, Costello HH, Courtney DM, Martinovich Z, Myers R, Zosel A, et al. A comparison of analgesic management for emergency department patients with sickle cell disease and renal colic. Clin J Pain. 2010;26(3):199–205. doi:10.1097/AJP.0b013e3181bed10c.
Pritchard KA, Feroah TR, Nandedkar SD, Holzhauer SL, Hutchins W, Schulte ML, et al. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol. 2011;46:389–96. doi:10.1165/rcmb.2011-0097OC.
Leong MA, Dampier C, Varlotta L, Allen JL. Airway hyperreactivity in children with sickle cell disease. J Pediatr. 1997;131(2):278–83.
Koumbourlis AC, Zar HJ, Hurlet-Jensen A, Goldberg MR. Prevalence and reversibility of lower airway obstruction in children with sickle cell disease. J Pediatr. 2001;138(2):188–92. doi:10.1067/mpd.2001.111824.
Vendramini EC, Vianna EO, De Lucena AI, De Castro FB, Martinez JA, Terra-Filho J. Lung function and airway hyperresponsiveness in adult patients with sickle cell disease. Am J Med Sci. 2006;332(2):68–72.
Sylvester KP, Patey RA, Rafferty GF, Rees D, Thein SL, Greenough A. Airway hyperresponsiveness and acute chest syndrome in children with sickle cell anemia. Pediatr Pulmonol. 2007;42(3):272–6. doi:10.1002/ppul.20571.
Ozbek OY, Malbora B, Sen N, Yazici AC, Ozyurek E, Ozbek N. Airway hyperreactivity detected by methacholine challenge in children with sickle cell disease. Pediatr Pulmonol. 2007;42(12):1187–92. doi:10.1002/ppul.20716.
Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108(9):2923–7. doi:10.1182/blood-2006-01-011072.
Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with increased mortality in individuals with sickle cell anemia. Haematologica. 2007;92(8):1115–8.
Glassberg J, Spivey JF, Strunk R, Boslaugh S, DeBaun MR. Painful episodes in children with sickle cell disease and asthma are temporally associated with respiratory symptoms. J Pediatr Hematol Oncol. 2006;28(8):481–5. doi:10.1097/01.mph.0000212968.98501.2b.
Glassberg JA, Chow A, Wisnivesky J, Hoffman R, Debaun MR, Richardson LD. Wheezing and asthma are independent risk factors for increased sickle cell disease morbidity. Br J Haematol. 2012;159:472–9. doi:10.1111/bjh.12049.
Glassberg JA, Wang J, Cohen R, Richardson LD, DeBaun MR. Risk factors for increased ED utilization in a multinational cohort of children with sickle cell disease. Acad Emerg Med. 2012;19(6):664–72. doi:10.1111/j.1553-2712.2012.01364.x.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Glassberg, J., DeBaun, M.R. (2017). Sickle Cell Disease. In: Celedón, J. (eds) Achieving Respiratory Health Equality. Respiratory Medicine. Humana Press, Cham. https://doi.org/10.1007/978-3-319-43447-6_11
Download citation
DOI: https://doi.org/10.1007/978-3-319-43447-6_11
Published:
Publisher Name: Humana Press, Cham
Print ISBN: 978-3-319-43445-2
Online ISBN: 978-3-319-43447-6
eBook Packages: MedicineMedicine (R0)