Every year on June 19, the global sickle cell community unites to celebrate World Sickle Cell Day. For the sickle cell community, this day holds much significance. Also known as World Sickle Cell Awareness Day, this day is observed internationally to increase much-needed education and awareness of a disease that affects millions of people around the globe.
In 2006, the World Health Organization (WHO) recognized sickle cell disease (SCD) as a global health problem. Just one year earlier, the African Union and the United Nations Educational, Scientific, and Cultural Organization (UNESCO) had also recognized the disease as a global health problem, thanks to the advocacy of the Sickle Cell Disease International Organization (SCDIO), the support of the Republic of Congo and the Republic of Senegal, and the commitment in the scientific world. Their efforts brought to light how SCD was and is a heritable blood disorder with devastating effects.
In response, in 2008, the United Nations (UN) General Assembly created a resolution that formally recognized SCD as a public health problem. They declared SCD as “one of the most lethal genetic disorders” and “one of the world’s foremost genetic diseases.” The UN’s resolution served to increase the public’s awareness of SCD and its cure at a national and international level. The resolution was also created to improve the quality of life for patients with SCD worldwide and to observe June 19 as World Sickle Cell Day to continue raising awareness.
June 19, 2009 was the first date that World Sickle Cell Day was celebrated.
In 2001, a Parisian woman originally from the Democratic Republic of Congo motivated and gathered an inspired group of sickle cell patients and researchers in France and in Francophone Africa. Her name: Edwidge Ebakisse Badassou, the mother of a child with sickle cell disease who was determined, persuasive, committed, and bold and became known to this day as the “First Lady” of the Francophone sickle cell advocacy world. She desired to change the political future of sickle cell disease and had a specific vision for development politics. She created the framework by which researchers, physicians, patients, and patient advocate groups, as well as local government bodies and development agencies from multiple francophone countries would collaborate to provide resources and raise attention on sickle cell disease. To unite them and increase their interests around sickle cell, she created the RFLD organization (Francophone Network for the Fight Against SCD) in 2001; RFLD later became OILD (the International Organization for the Fight Against Sickle Cell) in 2003 to gain a broader, more international reach. Ebakisse Badassou soon organized the first International Congress of the International Organization to Combat Sickle Cell Anemia, which convened at the United Nations Educational, Scientific, and Cultural Organization (UNESCO) Headquarters in Paris in January 2002. During the 2002 UNESCO meeting, Ebakisse Badassou explained that her “goal was to make SCD a global public health priority so that the disparities she witnessed traveling between Paris care sites and those in Africa might be addressed.” She wanted to find a way to insert sickle cell into the growing string of illnesses, like tuberculosis, HIV/AIDS, and at the time, malaria, that the United Nations and the WHO had made health priorities for global development.
Ebakisse Badassou invited several African First Ladies to attend the 2002 UNESCO meeting as representatives of “member states” that were affected by SCD. Madame Antoinette Sassou Nguesso, First Lady of the Republic of Congo, and Madame Viviane Wade, First Lady of Senegal, joined forces to help establish a centre in Brazzaville, the Congolese capital, to study the disease, raise awareness, and bring scientists and medical professionals together to discuss prevention, care, and treatment. Through Congo Assistance, Madame Sassou Nguesso’s non-profit foundation, she brought sickle cell disease to the attention of the government and mobilized resources for the National Reference Centre for Sickle Cell Disease (CNRD). In 2016, Madame Sassou Nguesso led a health delegation visit to Howard University. During the visit, Howard University President Wayne A. I. Frederick pledged to develop and strengthen collaborations between the Howard Center for Sickle Cell Disease and the newly established National Sickle Cell Disease Center of Brazzaville, Congo. Today, Madame Sassou N’Guesso and Madame Wade remain OILD’s two official sponsors.
Madame Sassou Nguesso’s endeavors with UNESCO, as President of the Congo Assistance Foundation, and as a sponsor of SCDIO in advocacy of raising the awareness of SCD in the Congo and globally have continued to advance the collective work of OILD. She received the WHO Director-General’s Health Leaders Recognition to celebrate her contribution to the control of sickle cell anemia globally at the 72nd session of the World Health Assembly in Geneva.
As a heritable blood disorder, sickle cell disease and related disorders impact nearly all organs of the body. Early research was conducted to understand the impacts of the disease. Most importantly, researchers found that those with the disease had higher mortality rates compared to the general population. Early mortality came from sickle-related complications that affect multiple organs due to years of ongoing inflammation and vasculopathy (damage of the blood vessels due to a disease). Early research helped scientists, organizations, and nations to understand that those with sickle cell disorders experienced damage to organs including the liver, kidney, lungs, heart, and spleen that could lead to death and were at increased risks for stroke, acute chest syndrome, blindness, bone damage, and priapism. Although survival estimates have improved, better survival rates are most possible in high-income countries. Nonetheless, whether in a low- or high-income country, the life expectancy of individuals living with SCD is still significantly shortened compared to the general population. Further, research remains limited on the effects of SCD on reproductive and mental health.
SCD still needs high-quality and enhanced research, evaluation, and monitoring solutions to manage and treat chronic pain, acute pain and events, priapism, liver dysfunction, multi-organ failure, delayed reactions to transfusions, and many other complications more effectively.
Sickle Cell Disease affects many people in various regions around the world. The disease originated in sub-Saharan Africa, the Indian subcontinent, and the Arabian Peninsula, including the Middle East and the Mediterranean region. SCD is widespread in these regions, and has become abundant in new areas, like the US and western and northern Europe due to involuntary (through the African slave trade) and voluntary population migration.
Epidemiologists estimate that more than 300,000 children are born each year with SCD, with about two thirds of whom are born in African countries. Nigeria, India, and the Democratic Republic of Congo bear half the global burden of SCD. Epidemiologists also predict that by 2050, there will be about 400,000 babies born with SCD annually.
The increasing number of people affected by the disease makes prevention, early diagnosis, and early management a necessary point of focus. Universal newborn screening programs were implemented more than a decade ago in the UK and in the US. Early screening combined with prophylactic vaccination and antibiotics led to a significant decrease in early mortality in these and other high-income countries. However, these impactful national programs have not been possible in many African countries, nor in India, where they would have had the most impact and would be of great value to low- and middle-income countries.
The limited access to newborn screening programs is one example of health inequities that exist and affect those with SCD. Regarding treatment, research, and funding, inequities in these areas exist not just between high- and low-income countries, but even within countries that have the available resources, like the US. Often, patients with SCD experience bias, stigmatization under care, and malpractice.
As previously mentioned, June 19 is a day that is dedicated to raising awareness of sickle cell disorders globally and celebrating the achievements of those living with sickle cell disease. It is recognized around the world as a day to also bring advocates together and continue highlighting the need for enhanced research and funding towards better treatments.
Public awareness is important to increase enthusiasm and support, mobilize ourselves and others to take action, and mobilize local knowledge and resources. Raising awareness of World Sickle Cell Day will help to inform and educate people about SCD to influence their attitude, behaviors, and beliefs about the disease. It creates accountability and promotes participation in ways that support the advancement of patient care, treatments, research, and overall understanding.
What are ways that you can help raise awareness on World Sickle Cell Day?
Today, World Sickle Cell is observed and celebrated in several ways. Nonprofit organizations disseminate facts, resources, and information for those with SCD and their caregivers, along with information that help other audiences learn and understand SCD. These organizations provide a community and “gathering place” for those affected by SCD to share their stories and experiences to support each other and amplify their voices around the world.
Companies and pharmaceutical companies may partner with these nonprofit organizations to increase awareness through events, programs, fundraisers, scholarships, contests, and talent shows to recognize and celebrate those with SCD. These partnerships may also support collaboration towards creating new treatments.
Healthcare centers or organizations also observe World Sickle Cell Day by holding local events that combine family-friendly activities with providing information on available resources at local healthcare centers and programs. They may also share information on new ways of managing the disease through lifestyle behaviors, over-the-counter medicines, and new clinical treatments.
Finally, advocates continue their efforts on World Sickle Cell Day to promote funding for research, policies, and best practices in healthcare settings that reduce bias and stigmas related to SCD. Advocates also lobby congressional leaders for national policies that benefit the lives of those affected by SCD.