Sickle Cell Disease

Numerous organizations including the World Health Organization (WHO) and the United Nations (UN) recognize sickle cell disease (SCD) as a global health issue. Caused by a genetic hemoglobin mutation, SCD has a profound impact on people living with the disease, often limiting their education, career opportunities, and quality of life.

Debilitating lifelong complications include anemia, infections, stroke, tissue damage, organ failure, episodes of intense pain, and premature death. Pain, the most common clinical manifestation, causes tremendous suffering. About 10% of children with SCD have a symptomatic stroke which can result in learning and other lifelong disabilities. In the US, total lifetime health care costs are estimated to be nearly $1M for an average person with SCD reaching age 45. Annual costs are estimated at over $10K for children and over $30K for adults.

Despite significant advances in the understanding and treatment of SCD over the past century, many basic scientific processes are still not fully understood. There is currently only one FDA-approved drug (hydroxyurea) for adults with SCD which is also used off-label in children. Bone marrow transplantation can be curative but is limited primarily to SCD patients in developed areas of the world, while most of the SCD population lives in under-resourced countries. Enormous opportunity exists to increase access to diagnostic and therapeutic interventions and provide comprehensive care and support services to people living with SCD around the world. Awareness and screening are crucial for both prevention through genetic counseling and diagnosis to activate early treatment.

Data and Statistics

SCD is most prevalent in malaria endemic parts of the world, primarily Africa, the Middle East, and South Asia.1
  • Globally, SCD occurs in approximately 300,000 births annually1, with 46% of global SCD newborns from Nigeria, Congo, and Tanzania.2
  • By 2050, the number of people with SCD is expected to increase by about 30% globally.2
  • In Africa, more than 50% of children born with SCD will die before reaching age five.3 In India, 20% of children with SCD die by age two.4
  • SCD affects approximately 100,000 Americans and 1 out of every 365 African-American births.1
  • One study in the US found that despite universal newborn screening, only 31% of SCD cases receive long-term follow-up after diagnosis.5
  • In Africa, over 90% of children with SCD do not survive to adulthood (compared to 6% in the US).6
  • Full newborn screening and vaccinations could save 5 million lives globally by the year 2050.2
Sickle Cell Trait carriers (HbA + HbS) do not always exhibit symptoms, and unaware carriers are highly likely to pass on the gene to their children.2

1. Centers for Disease Control and Prevention; World Health Organization; Piel et al. 2013. Lancet 381: 142-51.

2. Frederic B. Piel, Simon I. Hay, Sunetra Gupta, David J. Weatherall and Thomas N. Williams, “Global Burden of Sickle Cell Anaemia in Children under Five, 2010-2050: Modelling Based on Demographics, Excess Mortality, and Interventions,” PLOS 10, no. 7 (July 2013): e1001484

3. Scott D. Grosse, Isaac Odame, Hani K. Atrash, Djesika D. Amendah, Frederic B. Piel and Thomas N. Williams, “Sickle Cell Disease in Africa: A Neglected Cause of Early Childhood Mortality,” American Journal of Preventive Medicine 41, no. S4 (December 2011): S398-405.

4. Sanjay Tewari and David Rees, “Morbidity pattern of sickle cell disease in India: A single centre perspective,” Indian Journal of Medical Research 138, no. 3 (September 2013): 288-290

5. Cynthia S. Minkovitz, Holly Grason, Marjory Ruderman and James F. Casella, “Newborn Screening Programs and Sickle Cell Disease,” American Journal of Preventive Medicine 51, no. S1 (June 2016): S39-47

6. The National Institutes of Health, www.fic.nih.gov/News/GlobalHealthMatters/november- december-2014/Pages/sickle-cell-disease.aspx

Knowledge Base

Articles in our Knowledge Base include SickleSCAN validation studies and recommendations for advancing screening and diagnosis in low-resource countries. A link to a comprehensive list of SCD articles in the American Society of Hematology’s (ASH) journal is also provided.

  • Sickle-cell anemia: a rapid screening test on the continent
  • Validation of a novel point of care testing device for sickle cell disease
  • An accurate and affordable test for the rapid diagnosis of sickle cell disease could revolutionize the outlook for affected children born in resource-limited setting
  • Characteristics of a rapid, point-of-care lateral flow immunoassay for the diagnosis of sickle cell disease
  • Sickle cell anemia in sub-Saharan Africa: advancing the clinical paradigm through partnerships and research
  • Additional SCD articles in Blood, the official journal of ASH

Other Resources

Excellent resources can be found at the websites of SCD advocacy organizations. We hope you find the following links useful:

Ask Our Team

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