Protocol
Creators: name: Pakhale, Smita
Publisher: name: OSF Registries
Year: 2022
Subjects: health policy
poverty
sickle cell
social determinants of health
systematic review
vulnerable populations

description: Introduction
Prevalence, morbidity, and mortality for many diseases have decreased over the years due to advancement in medical knowledge, health awareness and policies. However, diseases affecting racialized minorities such as sickle cell disease (SCD) continue to impose significant medical, financial, and emotional burden with higher morbidity and mortality. SCD is a hemoglobinopathy that distorts the shape of the red blood cell leading to many complications including anemia, pain crisis and infections.(1) Despite progress in treatment, the life expectancy of individuals with SCD remains 2 to 3 decades shorter than the general population.(2) As a result, SCD has been declared a public health priority by the World Health Organization in 2006.(3)
Approximately 30 million people globally have SCD with 1000 children being born with SCD daily.(3) SCD is especially prevalent in many low-income racialized minority populations. Globally, people belonging to low-income racialized minority populations suffer poorer health than their counterparts due to bio-psycho-socio-economic-structural, political, and demographic factors. s.(4, 5) People living with SCD suffer disproportionately high premature deaths, repeated emergency room visits and hospitalizations due to acute complications, and significant multi-organ life-limiting long-term complications. Comprehensive clinical care, and research are sorely lacking globally, unlike another hereditary disease, Cystic Fibrosis (CF), affecting Caucasian populations. 
We aim to describe socio-demographics of SCD populations globally, highlight policy limitations and urgently needed changes for achieving comprehensive, equitable SCD clinical care and to promote research.

Methods
This study will adhere to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines and an a priori protocol will be published on Open Science Framework.(6)

Searches
A comprehensive search of OVIDMedline and other databases will be done.

Search Strategy
Search will be performed using the keywords “Sickle Cell Anemia,” “Social Determinants of Health,” “Vulnerable Populations,” “Homeless,” “Poverty,” “Health Policy,” “Health care reform,” “sociodemographic,” and any other pertinent keywords.


Inclusion criteria
Articles pertaining to: SCD prevalence; the population affected; availability of newborn screening (systematic or sporadic); nature of available care available (multi-disciplinary, comprehensive, organized/not-organized, fragmented, community-based, etc.); and availability of dedicated research funding will be included.

Exclusion criteria
Any irrelevant articles that do not meet the inclusion criteria mentioned above will be excluded.
Data extraction
Two independent reviewers will be simultaneously extracting data and information from the selected articles. A third reviewer will act as an adjudicator in case of any discrepancies and conflicts in decision making for inclusion and exclusion of the articles.


References

1. US Department of Health Human Services. National Heart, Lung and Blood Institute. Evidence-based Management of Sickle Cell Disease—Expert Panel Report, 2014. 2018.
2. Lanzkron S, Carroll CP, Haywood Jr C. Mortality rates and age at death from sickle cell disease: US, 1979–2005. Public health reports. 2013;128(2):110-6.
3. WHO. Sickle-cell anaemia: Report by the Secretariat FIFTY-NINTH WORLD HEALTH ASSEMBLY: Provisional agenda item 11.4 Geneva: World Health Organization; 2006.
4. Williams H, Silva S, Cline D, Freiermuth C, Tanabe P. Social and behavioral factors in sickle cell disease: employment predicts decreased health care utilization. Journal of health care for the poor and underserved. 2018;29(2):814.
5. Linton EA, Goodin DA, Hankins JS, Kanter J, Preiss L, Simon J, et al. A survey-based needs assessment of barriers to optimal sickle cell disease care in the emergency department. Annals of emergency medicine. 2020;76(3):S64-S72.
6. Moher D, Liberati A, Tetzlaff J, Altman DG. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. BMJ. 2009;339:b2535.