SICKLE CELL DISEASE (SCD)
IS A GROUP OF INHERITED
HEMATOLOGIC DISORDERS THAT
CAN LEAD TO WIDESPREAD DAMAGE1
US statistics regarding SCD2-4
Americans living
with SCD2
Lower mean life expectancy in SCD
African Americans
with SCD
African Americans
national average
Black or African Americans
born with SCD2
How does this compare to
other serious conditions?5
NEWLY DIAGNOSED
THE SCD COMMUNITY IS
MARKEDLY UNDERSERVED
Although more people in the US are living with SCD than with other genetic conditions, such as cystic fibrosis (~30,000 people) or hemophilia (~20,000 people), they have considerably less access to comprehensive coordinated care.6-8
While there are extensive networks of multidisciplinary specialty centers for both cystic fibrosis and hemophilia, there is no comparable national network of treatment centers for SCD.8
of people with SCD usually receive their care in the emergency room setting, which can lead to inefficient and suboptimal management of their chronic condition.9,10
Sickle cell anemia is the most common and typically most severe form of SCD in the US11
GENERAL CLINICAL MANIFESTATIONS IN THE Walk-PHaSST STUDY (N=674)12
| Genotype | Prevalence | >3 pain episodes | Leg ulcers | Hb g/dL | LDH U/L |
|---|---|---|---|---|---|
| HbSS (sickle cell anemia) | ~75% | 31% | 22% | 8.6 | 437 |
| HbSC | ~18% | 28% | 9% | 11.6 | 245 |
| HbSβ+-thalassemia | ~4% | 26% | 11% | 11.1 | 245 |
HbSS = hemoglobin SS; HbSC = hemoglobin SC; HbSβ+-thalassemia = hemoglobin Sβ+-thalassemia; Hb = hemoglobin; LDH = lactate dehydrogenase
Sickle Cell Disease Resources
The National Heart, Lung, and Blood Institute
The National Heart, Lung, and Blood Institute has developed comprehensive, evidence-based guidelines for the diagnosis and management of sickle cell disease
American Society of Hematology
American Society of Hematology developed clinical practice guidelines on the management of sickle cell disease
Sickle Cell Disease Coalition
A library of useful links to sickle cell disease resources for providers
Sickle Cell Disease National Resource Directory
From the Centers for Disease Control and Prevention (CDC), this resource directory lists sickle cell disease national agencies, specialty care centers, and community-based organizations
Sickle Cell Information Center
Sponsored by the Emory School of Medicine, this online center highlights the latest clinical updates in sickle cell disease and webinars, as well as personal narratives from people impacted by SCD
See the impact of HbS polymerization
References: 1. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323. 2. Centers for Disease Control and Prevention. Data & Statistics on Sickle Cell Disease. https://www.cdc.gov/ncbddd/sicklecell/data.html. Updated October 21, 2019. Accessed November 12, 2019. 3. Lanzkron S, Carroll CP, Haywood C Jr. Mortality rates and age at death from sickle cell disease: US, 1979–2005. Public Health Rep. 2013;128(2):110-116. 4. Xu J, Murphy SL, Kochanek KD, Bastian B, Arias E. Deaths: final data for 2016. Natl Vital Stat Rep. 2018;67(5):1-76. 5. Centers for Disease Control and Prevention. Leading cancer cases and deaths, male and female, 2015. United States cancer statistics: data visualizations. https://gis.cdc.gov/Cancer/USCS/DataViz.html. Updated June 2018. Accessed April 30, 2019. 6. Cystic Fibrosis Foundation. About cystic fibrosis. What is CF? Cystic Fibrosis Foundation website. https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/. Accessed May 3, 2019. 7. National Hemophilia Foundation. Fast facts. National Hemophilia Foundation website. https://www.hemophilia.org/About-Us/Fast-Facts. Accessed May 6, 2019. 8. Grosse SD, Schechter MS, Kulkarni R, Lloyd-Puryear MA, Strickland B, Trevathan E. Models of comprehensive multidisciplinary care for individuals in the United States with genetic disorders. Pediatrics. 2009;123(1):407-412. 9. Commercial Overview. August 2018. Data on file, Global Blood Therapeutics. 10. Hemker BG, Brousseau DC, Yan K, Hoffmann RG, Panepinto JA. When children with sickle‐cell disease become adults: lack of outpatient care leads to increased use of the emergency department. Am J Hematol. 2011;86(10):863‐865. 11. National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report, 2014. https://www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease. Published September 2014. Accessed April 8, 2019. 12. Saraf SL, Molokie RE, Nouraie M, et al. Differences in the clinical and genotypic presentation of sickle cell disease around the world. Paediatr Respir Rev. 2014;15(1):4-12.
