Hydroxyurea Segal, J.B., Et Al., (2008), Hydroxyurea

¶ … Hydroxyurea

Segal, J.B., et al., (2008), Hydroxyurea for the Treatment of Sickle Cell Disease,

Baltimore, MD: AHRQ

Variables/Key Concepts

To determine efficacy, effectiveness, harms, and barriers of the use of hydroxyurea treatment of sickle cell disease for determining solutions and further research need.

Sampling

Research studies based on variables.

Conducted by experts in sickle cell disease management, clinical trial methodology, systematic review, epidemiological studies, and ethics and adherence research.

Instruments/Data Collection

Instruments were quality assessment, data extraction, and evidence grading. Literature inclusion tailored toward research questions. Studies on children and adults evaluated separately. Included randomized trials, non-randomized trials, cohort studies with control groups, and pre/post studies. Evaluations of data was based on variable-based research questions.

Results

Hydroxyurea lowered the rate of hospitizations among children with sickle cell disease and raised HbF cell percentages.

Strengths/Limitations

Limited evidence for toxicity, barriers, and guide dosing. Insufficient evidence for efficacy and safety.

Critique

Even with the use of experts, there was no appropriate design method mentioned. Identifies research needs for toxicity, guide dosing, efficacy, and safety.

Strouse, J.J., et al., (2008), Hydroxyurea for Sickle Cell Disease: A Systematic Review for Efficacy and Toxicity in Children, Pediatrics, 122:6. Doi:10.1542/peds.2008-0441,

Retrieved from http://www.pediactricsdigest.mobi/content/122/6/1332.full

Variables/Key Concepts

A synthesized literature review on the efficacy, effectiveness, and toxicity of hydroxyurea in children.

Sampling

Selected randomized trials, observational studies, and case reports that evaluated the efficacy, and toxicity of hydroxyurea in children with sickle cell disease.

Design/Method

Two reviewers abstracted data sequentially in study design, patient characteristics, and outcomes. Assessed study quality independently.

Instruments/Data Collection

Instruments were databases, quality assessment, data synthesis with detailed evidence tables, and evidence grading. Used studies of randomized controlled trials, cohort studies with control groups, and pre/post studies.

Results

Strong evidence of increasing HbF and decreasing hospitalization. Moderate evidence of decreasing pain crisis and support of increased risk of reversible cytopenias, and rash. Weak evidence for decreasing neurologic events. Distance to referral center and need of frequent monitoring may be barriers to use.

Strengths/Limitations

Limited predictors to response.

Critique

No defined methodology. Good to evaluate for barriers to use of hydroxyurea as well as suggestions for further research on neurologic events and more evidence on reducing pain measures.

Kavanagh, P.L., et al., (2011), Management of Children with Sickle Cell Disease: A

Comprehensive Review of Literature, Pediatrics, 128:6. Doi:10.1541/peds.2010-3686,

pp e1552-e1574, retrieved from http://pediatrics.aappublications.org/content/1128/6/e1552.full.pdf+hml

Variables/Key Concepts

Review the quality of literature for preventative interventions and treatment of complications to facilitate the use of evidence-based medicine and need of further research.

Sampling

Randomized cohort cases, controlled studies, multiple time series studies, and uncontrolled studies.

Design/Method

Identified data based on relevant topics dated from January 1995 to April 2010. Data was independently reviewed by two experts.

Instruments/Data Collection

Instruments were common form, U.S. Preventative Service Task Force ratings, RCTs, and meta-analysis categorized. Isolated studies based on relevancy of 28 topics concerning National Institute of Health guidelines for the care of children with sickle cell disease under three categories.

Results

Lack of quality research for pediatric sickle cell disease related topics, many areas lack RCTs, and more research is needed for health and well-being of children with sickle cell disease.

Strengths/Limitations

Possible missed studies due to refined strategies, more than one third of studies included adults and children, and limited language to English, eliminating foreign studies.

Critique

This study is good for further research needs in identifying quality of care improvements for children with sickle cell anemia.

Wang, C.J., et al., (2011), Quality of Care Indicators for Children with Sickle Cell

Disease, Pediatrics, 128:3. Doi:10:1542/peds.2010-1791, pp 484-493, retrieved from http://pediatrics.aappublications.org/content/128/3/484.full.pdf+html

Variables/Key Concepts

To develop quality of care indicators for children with sickle cell disease by addressing the complications of the illness.

Sampling

Non-randomized controlled trials, cohort or case-controlled studies, multiple time series, descriptive studies, and expert opinion.

Design/Methods

A qualitative study using the Rand/University of California Los Angeles appropriations method, comprehensive literature review with evidence ratings, and two rounds of anonymous expert panel ratings.

Instruments/Data Collection

OVID Medline and Cochran databases, collected articles dated January 1995 to April 2010 based on 29 topics relevant to children

Results

Identified 41 indicators according to type of care.

Strengths/Limitations

List of indicators did not address all of the original list due to lack of concrete evidence to link program measures.

Critique

This study would be good to use to address indicators for quality improvement measures in standards of care.

Hankins, J.S., et al., (2005), Long-term hydroxyurea therapy for infants with sickle cell anemia: The HUSOFT extension study, Journal of the American Society of Hematology, 106:7. Doi:10.1182/blood-2004-12-4973, pp 2269-2275, retrieved from http://bloodjournal.hematologylibrary.org/content/106/7/2269.full