About Sickle Cell Disease

Sickle cell disease (SCD) is a serious, complex, lifelong red blood cell (RBC) disorder1

SCD is characterized by hemolytic anemia and by the production of abnormal hemoglobin (Hb).1,2

It affects about 100,000 people in the US, primarily those of non-Hispanic Black or African-American (more than 90%) and Hispanic or Latino (3%-9%) descent. Other groups affected include those of Mediterranean, Middle Eastern, Caribbean, South American, and Indian descent.3

SCD genotypes4,5

HbSS
~75% of patients with SCD
HbSC
~18% of patients with SCD
HbSβ+-thalassemia
4.0% of patients with SCD
HbSβ0-thalassemia
1.6% of patients with SCD

Regardless of genotype, SCD can result in both acute and chronic complications, leading to significantly reduced life expectancy:
~30 years earlier than that of the general population.1,6,7

Design

A large, multicenter cohort (N=674) published in 2011 was used to determine the breakdown of genotypes among SCD patients of predominantly African origin in the Americas and the UK.5

HbSC=hemoglobin SC; HbSS=hemoglobin SS disease; HbSβ+=hemoglobin S β-plus; HbSβ0=hemoglobin S β-zero.

The impacts of SCD are systemic and result in both acute and chronic complications, including organ damage1,8

Some key acute and chronic complications of SCD include:

Acute Complications8,9

  • Brain

    Stroke

  • Eye

    Retinal detachment, retinal artery occlusion, vision loss

  • Lungs

    Acute chest syndrome, pulmonary embolism, pneumonia

  • Genitourinary

    Priapism

  • Pain

    Pain crisis and dactylitis

  • Other

    Infections, mobility issues

Diagram of a body with acute complications of sickle cell disease affecting the brain, eyes, lungs, and more

Chronic Complications8,9

  • Brain

    Silent cerebral infarction, cognitive impairment

  • Heart

    Pulmonary hypertension, cardiomyopathy

  • Liver

    Liver disease, cholelithiasis

  • Kidney

    Chronic kidney disease, proteinuria, end-stage renal disease

  • Genitourinary

    Erectile dysfunction

  • Bone-skin

    Osteonecrosis, leg ulcers

  • Other

    Jaundice, fatigue, mobility issues

Diagram of a body with chronic complications of sickle cell disease affecting the brain, heart, liver, kidney, and more

SCD is progressive, and serious complications accumulate over time1,10,11

SCD complications by age12

Line graph showing mean number of days with sickle cell disease complications increases over time with ageLine graph showing mean number of days with sickle cell disease complications increases over time with age

Figure adapted from Blinder et al.

Complications include: chronic obstructive pulmonary disease, pneumonia, asthma, pulmonary hypertension, acute chest syndrome, congestive heart failure, cardiomegaly, arrhythmia, cardiomyopathy, leg ulcers, and avascular necrosis.12

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References:

1. Kavanagh PL, Fasipe TA, Wun T. Sickle cell disease: a review. JAMA. 2022;328(1):57-68. doi:10.1001/jama.2022.10233 2. Xu JZ, Thein SL. Revisiting anemia in sickle cell disease and finding the balance with therapeutic approaches. Blood. 2022;139(20):3030-3039. doi:10.1182/blood.2021013873 3. Data and statistics on sickle cell disease. Centers for Disease Control and Prevention. Updated May 15, 2024. Accessed June 25, 2026. https://www.cdc.gov/sickle-cell/data/index.html 4. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031. doi:10.1016/S0140-6736(10)61029-X 5. 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. doi:10.1016/j.prrv.2013.11.003 6. Telen MJ, Malik P, Vercellotti GM. Therapeutic strategies for sickle cell disease: towards a multi-agent approach. Nat Rev Drug Discov. 2019;18(2):139-158. doi:10.1038/s41573-018-0003-2 7. Kato GJ, Piel FB, Reid CD, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010. doi:10.1038/nrdp.2018.10 8. Colombatti R, Jastaniah W, Makani J, Andemariam B. Sickle cell disease. Lancet. 2026;407(10533):1095-1111. doi:10.1016/S0140-6736(25)02278-0 9. Osunkwo I, Andemariam B, Minniti CP, et al. Impact of sickle cell disease on patients’ daily lives, symptoms reported, and disease management strategies: results from the international Sickle Cell World Assessment Survey (SWAY). Am J Hematol. 2021;96(4):404-417. doi:10.1002/ajh.26063 10. Gladwin MT. Cardiovascular complications and risk of death in sickle-cell disease. Lancet. 2016;387(10037):2565-2574. doi:10.1016/S0140-6736(16)00647-4 11. Ballas SK, Kuypers FA, Gordeuk VR, Hankins JS, Thompson AA, Vichinsky E. Time to rethink haemoglobin threshold guidelines in sickle cell disease. Br J Haematol. 2021;195(4):518-522. doi:10.1111/bjh.17578 12. Blinder MA, Vekeman F, Sasane M, Trahey A, Paley C, Duh MS. Age-related treatment patterns in sickle cell disease patients and the associated sickle cell complications and healthcare costs. Pediatr Blood Cancer. 2013;60(5):828-835. doi:10.1002/pbc.24459