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Sickle Cell Disease

Sickle cell disease (SCD) is an inherited red blood cell disorder that affects around 100,000 people in the United States, largely individuals of African ancestry, but increasingly in Latino and other populations as well. Individuals with SCD experience painful episodes when their red blood cells morph into a crescent (“sickle”) shape and get stuck in small blood vessels. This blockage inhibits blood flow, which deprives tissues of oxygen and causes severe pain and tissue damage.

SCD is characterized by:

• Chronic anemia
• Unpredictable episodes of pain and end-organ damage
• Poor health-related quality of life
• Increased healthcare use
• Early mortality

Image of Sickle Cells in Blood Sample http://en.wikipedia.org/wiki/File:Sickle_cells.jpg

Only a few decades ago, a child with sickle cell disease had a life expectancy of 14 years. Today, those with SCD can live much longer. Early identification and multi-disciplinary, coordinated treatment is critical to enable SCD patients to effectively manage their disease.

In the US, around two million people carry the sickle cell trait but show no symptoms. If two carriers have children together, their chance of having a child with SCD is one in four.
 

What NICHQ Is Doing About It

NICHQ is leading three initiatives to improve care for those living with sickle cell disease and SCD trait.

Improving Sickle Cell Transitions of Care through Health Information Technology is a project that seeks to understand if and how a patient-centered, technology-based tool can improve the health of individuals with sickle cell disease (SCD) during care transitions. The project is funded by the Agency for Healthcare Research and Quality (AHRQ). Partners for the project include healthcare consulting firm The Lewin Group, Children’s National Medical Center, Cincinnati Children’s Hospital Medical Center and Nemours.

WISCH (Working to Improve Sickle Cell Healthcare) is a national initiative using collaboration, innovation, and quality improvement techniques to help improve the quality of life for individuals living with SCD and trait across the lifespan. WISCH consists of two quality-improvement projects, both funded by the US Department of Health and Health Resources and Services Administration (HRSA), to help improve screening and care for sickle cell patients: the Sickle Cell Disease Treatment Demonstration Program and the Sickle Cell Disease Newborn Screening Program.

NICHQ is working with 15 grantee sites through these two HRSA-funded projects to improve systems of detection and care for people living with SCD. Teams made up of healthcare providers, patients, hematologists, nurses and others are using quality improvement techniques to implement:

• Timely acute care management
• Better coordination of care
• Better transition from pediatric to adult care
• Improved screening, counseling, and education for individuals with SCD and trait
• Enhanced education for providers on treating, assessing and monitoring SCD 

Additional Resources

• Read a fact sheet from the CDC: What You Should Know About Sickle Cell Disease [PDF]
• Peruse stories and videos of patients’ experiences published by the New York Times: Patient Voices: Sickle Cell Anemia
• Listen to NICHQ's Dr. Suzette Oyeku talk about the experience of people living with sickle cell disease on a WNYC Radio talk show.
• Read a paper on managing children with sickle cell disease, with contributions from NICHQ Faculty Patricia Kavanagh and C. Jason Wang: Management of Children with Sickle Cell Disease: A Comprehensive Review of the Literature [PDF]

Visit these sites for more resources:

• Sickle Cell Disease Association of America
• Sickle Cell Information Center
• National Heart Lung and Blood Institute