Risk factors for mortality in patients with sickle cell disease: an integrative review

ABSTRACT

Objective

To summarize the risk factors and mortality indicators in sickle cell disease patients.

Method

Integrative review searched publications in journals in CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO, and Web of Science databases. The guiding question was elaborated through the Population, Variable, Outcome (PVO) strategy and the search was from October-to-November 2018, at the Coordination of Higher Level Personnel Improvement Periodicals’ Gate. The keywords anemia, sickle cell “and” mortality “and” survival and their synonyms were used.

Results

From 18/19 articles were cohort and one randomized controlled trial. The sample consisted mostly of females and HbSS genotype. The cumulative mortality rate and the overall mortality curve were the most repeated. Seven studies identified risk factors with a statistically significant association with death. The most frequent were low hemoglobin level, liver variables (alkaline phosphatase and oxalacetic glutamic transaminase enzymes) and cardiovascular variables (tricuspid valve regurgitation speed ≥ 2.5m/s).

Conclusion and implications for practice

Mortality indicators are tools for better management of sickle cell disease’s patient, prevention of risks and complications. There is a need for further studies on the factors related to mortality of these patients. Preventing the causes that lead to death will certainly improve the quality of life and survival of this population.

Keywords:
Sickle Cell Disease; Causes of death; Rates, rations and proportions