About Sickle Cell Disease
Sickle cell disease (SCD) is an inherited, lifelong blood disorder that causes individuals to produce abnormal hemoglobin, causing their red blood cells to become rigid and sickle-shaped. These sickled cells have a shortened lifespan, resulting in a constant shortage of red blood cells. When these sickled cells travel through the blood, they often get stuck in the smaller blood vessels, blocking other oxygen-rich red blood cells from freely flowing throughout the body. This leads to complications ranging in severity, including severe pain, acute chest syndrome (a condition that lowers the level of oxygen in the blood), stroke, organ damage, and even premature death.
Understanding the Impact of Sickle Cell Disease
This infographic can help you better understand what SCD is and how it impacts individuals across the globe.
Globally, it is estimated that 300,000 infants are born annually with sickle cell disease. The majority of these births occur in low-resource countries, especially in sub-Saharan Africa where the World Health Organization estimates 70 percent of the world’s population of children with SCD live. The majority of children suffer from chronic pain and numerous infections; ultimately, many die before their fifth birthday.
In the United States, nearly 100,000 individuals have SCD. The Centers for Disease Control and Prevention estimates that SCD affects 1 out of every 365 black or African-American births, and 1 out of every 16,300 Hispanic-American births.
Signs & Symptoms
Symptoms of SCD can range from mild to severe enough to require frequent hospitalizations.
Possible signs of SCD include:
- Anemia (looking pale)
- Dark urine
- Yellow eyes
- Painful swelling of the hands and feet
- Frequent pain episodes
- Stunted growth
Treatment & Quality Care
Hydroxyurea, an FDA-approved drug for adults with SCD (often used off-label in children), improves the course of SCD and might lead to significant health care cost reductions. In a two year pediatric study, overall health care costs for children on hydroxyurea were $1.8 million, compared with $2.5 million for those who did not receive this treatment. Unfortunately, hydroxyurea is not regularly prescribed and adherence to the therapy is poor. In addition to hydroxyurea, blood transfusions can help to manage SCD, but they can lead to abnormally high levels of iron in the blood, which can cause long-term organ damage and reactions due to a mismatch between the donors and recipients.
In addition to hydroxyurea, there has been early success in curing SCD through stem cell transplant, but this process is costly, dangerous, and is not widely available. Additional treatments and cures lie just over the horizon - emerging technologies like genome editing may hold the key to improving outcomes for individuals with SCD, but further research must be undertaken to speed the discovery and deployment of these options.
The status quo is unacceptable, and we are setting out to change it
People with sickle cell disease (SCD) are afflicted on two fronts — one by having a serious, chronic condition that inflicts pain and other complications — the other by a fragmented system of care.
Today, there are opportunities to transform this disease and the way we care for people with SCD. We are launching an international call to action on SCD by bringing together researchers, clinicians, individuals with sickle cell disease and their families, policymakers, and the private sector to focus our collective efforts and change the state of SCD around the world.
The Sickle Cell Disease Coalition is composed of public health, research, and provider organizations, patient groups, faith-based organizations, federal agencies, industry representatives, and foundations with an interest in Sickle Cell Disease.
- ASH Research Collaborative
- Africa Sickle Cell Alliance
- America's Blood Centers
- American Academy of Emergency Medicine
- American Academy of Pediatrics*
- American Psychological Association (APA)
- American Public Health Association*
- American Red Cross
- American Society for Transplantation and Cellular Therapy (ASTCT)
- American Society for Apheresis
- American Society for Clinical Pathology
- American Society of Gene & Cell Therapy
- American Society of Hematology (ASH)*
- American Society of Nephrology
- American Society of Pediatric Hematology/Oncology (ASPHO)
- American Thoracic Society
- Association Suisse Drépano
- Association of Maternal & Child Health Programs (AMCHP)
- Association of Pediatric Hematology/Oncology Nurses
- Association of Public Health Laboratories*
- Australian Sickle Cell Advocacy Inc.
- Axis Advocacy
- Bluebird Bio
- CSL Behring
- Caribbean Institute for Health Research (CAIHR)
- Cayenne Wellness Center
- Centers for Disease Control and Prevention (CDC)
- Centers for Medicare & Medicaid Services (CMS)
- Cerus Corporation
- Chiesi USA
- College of American Pathologists
- Crispr Therapeutics
- Doris Duke Charitable Foundation
- Emergency Department Sickle Cell Care Collaborative*
- Emmaus Life Sciences, inc.
- European Sickle Cell Federation (ESCF)
- FMDT SOS GLOBI
- Food and Drug Administration (FDA)
- Forma Therapeutics
- Foundation for Sickle Cell Disease Research
- Foundation for Women and Girls With Blood Disorders
- Functional Fluidics
- Global Alliance of Sickle Cell Disease Organizations (GASCDO)
- Global Blood Therapeutics
- Global Sickle Cell Disease Network
- Hematology and Oncology Society of Africa*
- Hemex Health
- International Association of Sickle Cell Nurses and Professional Associates (IASCNAPA)
- Martin Center Sickle Cell Initiative
- Medunik USA
- Mellamia Global Foundation
- Muhimbilii University of Health and Allied Sciences (MUHAS) Sickle Cell Programme
- National Black Nurses Association (NBNA)
- National Heart, Lung, and Blood Institute (NHLBI)
- National Institute for Children's Health Quality (NICHQ)
- National Marrow Donor Program
- National Medical Association (NMA)
- National Minority Quality Forum
- Our Sickle Cell Educare Warriors Initiative (OSCEWI)
- Pathways to Trust
- Patient Advocate Foundation
- Prolong Pharmaceuticals
- Reach a Child Initiative Zambia (RACIZ)
- Sanofi Genzyme
- Sick Cells
- Sickle Cell 101
- Sickle Cell Adult Provider Network (SCAPN)
- Sickle Cell Aid Foundation
- Sickle Cell Association of Texas Marc Thomas Foundation
- Sickle Cell Awareness Group of Ontario (SCAGO)
- Sickle Cell Community Consortium (SCCC)
- Sickle Cell Disease Association of America (SCDAA)*
- Sickle Cell Disease Association of America, Michigan
- Sickle Cell Disease Foundation of California
- Sickle Cell Foundation of Georgia, inc.
- Sickle Cell Research Centre - Uganda
- Sickle Cell Society UK
- Sickle Cell Transplant and Advocacy Research Alliance (STAR)
- Sickle Cell Warriors
- Sickle Cell and Thalassemia Ireland
- Smile Sickle Cell Foundation
- Tanzania Sickle Cell Warriors Organization
- Terumo BCT
- Thalassaemia International Federation
- The Emmes Corporation
- The Shikuri Project Charitable Trust
- Vertex Pharmaceuticals
*Member belongs to the Steering Committee.