Sickle Cell Disease Guidelines
What the NHLBI Has Done Up to Now

The NHLBI has coordinated the development of a series of activities related to the management of SCD since 1984.

2002 guidelines

The most recent published guidelines are the 2002 Fourth Edition on the Management of Sickle Cell Disease. Since this publication, the NHLBI has provided continued leadership and direction in promoting state-of-the-art science in sickle cell treatment.

2008 conference on hydroxyurea

In February 2008, the NHLBI (with other NIH and HHS groups) held a conference focused on hydroxyurea treatment - The NIH Hydroxyurea Treatment for SCD Consensus Conference. The conference panel assessed the available scientific evidence. They concluded that hydroxyurea treatment for patients who have sickle cell anemia is underutilized and more adolescents and adults should be treated with hydroxyurea.

Research has shown that patients who have SCD and are taking hydroxyurea experience fewer pain crises and hospital admissions. The conference panel advocated increased use of the drug with appropriate monitoring and continuing follow-up of children in ongoing clinical trials.

2008 new structure and innovations in SCD program

In March 2008, after a rigorous program assessment, extensive public input, and advice from the National Heart Lung and Blood Advisory Committee, the NHLBI announced a comprehensive and innovative restructuring of its research SCD program. As a result, the NHLBI is moving forward with the following innovations to its SCD portfolio:

• Basic science: Support for basic research will be expanded through funding of investigator-initiated grant applications and through NHLBI-initiated RFAs focused on the pathophysiology of SCD, the biology of pain in SCD, fetal hemoglobin switching, and genetic modifiers of disease expression and progression.
• Translational and clinical research: The NHLBI reconfigured the Comprehensive Sickle Cell Center (CSCC) program into a Basic and Translational Research Program (BTRP) with dedicated training components.
• Participation in clinical research: The scope of clinical research trials will be broadened to allow a greater number of people with SCD to participate in NIH-sponsored clinical research trials.
• Community outreach: In partnership with the Sickle Cell Disease Association of America and other patient advocacy groups and professional organizations, the NHLBI will develop evidence-based guidelines for the care of people with SCD across the lifespan that can be used by health care practitioners throughout the world.
• New resources for medical care: The NHLBI is in the process of creating resources that will improve the medical care that is provided to individuals living with sickle cell disease.

2009 workshop on awareness and educational needs of SCD community

In September 2009, the NHLBI held a workshop to help inform NHLBI program staff of the awareness and educational needs that the sickle cell community would like to see addressed on a national scale - the SCD Awareness and Education Campaign Strategy Development Workshop. This workshop brought together individuals with sickle cell disease, community based organizations that serve the sickle cell population and representatives from the health care community. The goals were to determine where there are gaps in sickle cell awareness and education, what approaches could be taken to help close these gaps, and what partners can help the NHLBI address the health communication needs of the sickle cell community.

2009 - present

The result of all these deliberations is the current major effort at NHLBI in leading the development of a set of evidence-based guidelines for the management of sickle cell disease. The guidelines will be principally aimed at primary care practitioners and their patients to help manage the ongoing processes of the disease. The guidelines will also be valuable to hematology specialists and their patients. The process also will identify areas where additional research is needed to obtain evidence that can inform practice