Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.

IMPORTANCE: Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. OBJECTIVE: To support and expand the number of health professionals able and willing to provide care for persons with SCD. EVIDENCE REVIEW: Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1, 2014, addressed key questions developed by the expert panel members and methodologists. FINDINGS: Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (≥200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria in adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated. CONCLUSIONS AND RELEVANCE: Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD.

NHLBI step-by-step approach to adapting cardiovascular training and education curricula for diverse audiences.

Racial and ethnic minority communities need to be involved in developing health information to ensure its cultural appropriateness, improve its acceptability, and stimulate adoption of healthy behaviors. The National Heart, Lung, and Blood Institute at the National Institutes of Health adapted a heart-health curriculum for Latinos into culturally appropriate curricula for American Indians/Alaska Natives, African Americans, and Filipinos. Lessons learned from this process can assist public health practitioners interested in adapting science-based heart-health information into practical health education messages that meet the cultural and contextual needs of diverse groups.

Role of community programs in controlling blood pressure.

Despite more than 30 years of intense activity to improve control--and more recently prevention--high blood pressure continues to be a major public health problem. Evidence-based reviews have identified best practices and quality improvement strategies to address prevention and control. Since the 1970s, community-based programs have been instrumental in raising awareness, increasing knowledge, and promoting changes in health behavior to improve blood pressure control. Most of these programs have emphasized the use of partnerships and involvement of community residents in conducting screening and referral activities, implementing clinical practice guidelines, and increasing healthy eating and physical activity. Many also have used health care team approaches, including the use of trained community health workers to deliver targeted, culturally sensitive heart health education, particularly related to the prevention of cardiovascular disease risk factors in general and high blood pressure in particular. Increased focus on implementation of evidence-based lifestyle and clinical management strategies coupled with community-based approaches may help increase blood pressure control rates within communities.