The 19th of June is World Sickle Cell day- a United Nations recognized day for creating awareness of the disease at both national and international level.

Sickle Cell Disease (SCD) is a group of inheritable disorders which affect the red blood cell (RBC). The genetic nature of the disease means that it is passed on to children from their parents; one cannot be infected by others with SCD.

SCD although discovered in 1910 by Dr. James Herrick in a patient from the Island of Grenada can be said to have been known in Africa for over five thousand years ago as evident by its many names in various dialects eg ahututuo, nududui, gburigburihi, tswetswetswe, amosani etc. The SCD though predominant in people of African, Caribbean and Mediterranean origin is now seen all over the world due to migration and inter-marriages and equally affects males and females.

The disease affects millions worldwide with 80% of those affected located in Sub-Saharan Africa. In Ghana 25% of the populace are sickle cell carriers (they have the potential to pass the disease to their children but have no disease themselves) and 15,000 of our newborns are affected annually.

SCD cause the generally round doughnut shaped RBC to transform to a sickle or banana shaped RBC which is less flexible and sticky. These sticky RBC only last for 10-20 days unlike the round flexible RBC found in people without SCD which lasts for 120 days before it is recycled leading to low blood and a recurrent need for blood transfusion. Also, the sickled cells also stick to the inner surface of the tubes that blood flows through and blocks movement of blood with resultant painful episodes which requires pain medications and leads to missed days from work and school.

Patients with SCD also suffer complications like stroke, recurrent infections, bone disease, heart problems, blindness and even sustained painful unprovoked erections (priapism) which can lead to infertility.

Over the years, the mystery surrounding the disease has slowly unfolded through basic and applied research. Patients are now offered comprehensive care right from birth through Newborn screening programs (NSP) with the ultimate aim of enrolling affected children at sickle cell treatment centers.

Early enrolment ensures access to quality comprehensive care including education, infection prevention and control, management of acute and chronic complication as well as disease modifying therapy like hydroxyurea. These interventions do not only improve the life expectancy of people living with SCD but it also improves their quality of life.

Hydroxyurea was the first disease modifying therapy approved in the United States by the FDA for SCD and in October 2018, it was approved by the Food and Drugs Authority in Ghana for use in children and adults with SCD. Hydroxyurea, an anti-cancer drug has been shown to prevent the RBC from transforming into the sickled or banana-shaped rigid form which is responsible for the painful episodes and low blood levels seen in patient with SCD.

It is an oral medication taken once daily and it prevents complications like the painful episodes, stroke, chest problems, bone damage and recurrent hospitalization which characterize the disease. This medication inadvertently reduces the number of missed days from school and work and ultimately improves the quality of life of patients on it. Unlike the newer disease modifying drugs, hydroxyurea is available and accessible. It is also less expensive comparatively and the fact that it is taken by mouth gives it an advantage over its counterpacts.

On the 22nd of December 2008, the United Nations (UN) General Assembly adopted a resolution recognizing SCD as a public health problem. The assembly urged UN agencies, funds, programs, international institutions, development partners and member states especially states in which SCD is a public health problem to support every effort being made to combat the disease. They were to encourage the health systems in managing the disease by establishing specialized centers and national programs as well as supporting research to help improve quality of lives of people living with SCD.

In line with this resolution, Ghana in partnership with various institutions extended its Newborn screening program (NSP) to include about four more health facilities.

Again, on the 6th November 2019, another formidable Public-Private initiative was signed between the Ghana Health Service and Ministry of Health on one side and global medicine company Novartis on the other hand. Under the partnership, NOVARTIS among other things was to provide the critical drug, Hydroxyurea, which will initially be provided free of charge to patients and will subsequently be subsidized and provided by the National Health Insurance Scheme(NHIS).

The theme for this Public-Private Partnership is “To Ease the Pain and Improve the Lives of People with Sickle Cell Disease in Ghana” and indeed over the last 18 months the free Hydroxyurea which Novartis gave to SCD patients in Ghana did just that. During the launch of this partnership in November 2019, the presence of His Excellency Dr. Mahamadu Bawumia put a smile on my face as I thought in my heart, finally SCD will get the recognition it deserves.

His Excellency, please we have come to the end of the free hydroxyurea by Novartis and the medication is still not on the NHIS. On this Sickle Cell Awareness day, we the health care providers lend our voice to the hundreds of grateful but worried patients with SCD who have benefited from this medication to send you a gentle reminder to help put it on the NHIS.

Over the last 18 months, my patients like many across Ghana have had a taste of how it feels like to live a life without pain, to live without missing out on school or work. They and their families have seen some semblance of normalcy in their lives and have even begun to dream big. Please, let us not crush their dreams even before it has a chance to bud as a considerable number of patients may not be able to afford being on it.

So, on this day, 19th of June 2021, let us shine the light on Sickle Cell Disease and lend not only our voices but put in efforts that will help improve the physical, emotional and economic well-being of people living with SCD in Ghana.