Research to Advance Health and Healthcare for Individuals with Sickle Cell Disease: A Drop in the Bucket of Needed Research

This issue is focused on research engaging individuals with sickle cell disease (SCD), the world’s most common inherited lethal blood condition. Over a century ago, Herrick first described SCD in a young man from Africa who was attending college in Chicago, Illinois, United States (U.S.) (Herrick 1910). SCD affects individuals from Sub-Saharan Africa, Saudi Arabia, India, Mediterranean, South America, Central America, and Cuba. Incidence SCD varies around the globe ranging from 150,000 babies born annually with SCD in Nigeria ((SCAF) 2016) to 2,000 such babies born annually in the USA ((NNSGRS) 2014), where SCD is considered a rare disease. The prevalence of SCD in the U.S is 100,000 from a population of 330,000,000 ((CDC) 2015), and the annual healthcare costs for the Americans with SCD totals $2.4 billion U.S. dollars (Lanzkron et al. 2010). The research presented in this issue focuses on samples of children and adults with SCD recruited from across the U.S.

Research focused on SCD has been multifaceted over the past century. Recent research has focused on stem cell transplant as a treatment that cures SCD but does not reduce the risk of the cured individual’s biological children inheriting the disease. Reproductive health issues such as inheritance of SCD is an area needing intervention research built on solid evidence from quantitative and qualitative approaches. Such research is important for its potential to advance primary prevention of SCD, which would reduce the burden of SCD on individuals, families, communities, and healthcare systems around the world. Potential for such an impact begins with research that critically and systematically evaluates interventions such as the CHOICES reproductive health intervention reported in this issue (Hershberger et al. 2015).

The burden of SCD on the individual has been a frequent focus of nurse and clinical scientists’ research. Cognitive function and pain are two burdensome issues for individuals with SCD and others. Cognitive function measurement is increasingly recognized as needing additional research and Crawford et al. (2015) share insightful findings from adults with SCD. Pain and SCD are so intimately intertwined, that African tribal words for the disease, are onomatopoeic for pain and were spoken hundreds of years before western medicine recognized the disease. Clarification of measurement issues and testing interventions for pain of SCD are the foci of studies reported in this issue (Ezenwa et al. 2015, Ezenwa et al. 2016).

Optimizing healthcare delivery for children and young adults with SCD remains an important focus for nursing research. In this issue, Jacob et al. (2015) profiles barriers to primary care for children with SCD. Matthie et al. (2015) present results relevant to young adults with SCD transitioning from pediatric care to adult care. Together, these two studies provide current evidence that can be used to improve care for children and adults with SCD.

The six studies appearing in this special issue focused on SCD were funded by extramural and intramural sources within the U.S. Three of the six studies were funded by the U.S. National Institutes of Health (NIH), one by NIH and the Agency for Healthcare Research and Quality, one by NIH and an intramural source, and one by intramural sources. This research funding support is encouraging and welcomed, especially since this area of science has been funded at lower levels in the USA than other genetic conditions, such as cystic fibrosis, which affects only 30,000 individuals in the U.S. (Smith et al. 2006). The focus on experiences of individuals with SCD provides important evidence to support nursing care in the U.S, but it is only a drop in the bucket of research needs. Additional research is needed to extend these findings globally and fully represent the needs of individuals, families, and communities affected by SCD.