By Michael Gwarisa
Globally, about 8 Million people live with Sickle Cell Disease (SCD), according to the Lancet 2023 data. The disease is largely prevalent in populations with ancestral ties to Sub-Saharan Africa, regions in the Western Hemisphere namely, South America, the Caribbean, and Central America), Saudi Arabia, India and Mediterranean countries such as Turkey, Greece, and Italy.
Sickle Cell Disease is a lifelong chronic disease and most of the children born with the disease in Sub-Saharan Africa struggle to access services due to lack of diagnostic tools, and proven therapies to manage the diseases complications, amongst a host of other factors.
Eunice Achieng Owino, a Kenyan woman living with Sickle Cell Disease is one of the lucky ones who has lived into her adulthood with the disease despite the early death sentence that is usually associated with Sickle Cell Disease. However, she says growing up with Sickle Cell Disease has not been easy.
As the fifth child in a family of six children, I was diagnosed with Sickle Cell Disease at the age of eight (8) months which made growing up difficult as I spent half of my childhood life in and out of the hospital,” said Eunice during an interview with HealthTimes at the inaugural International Conference on PEN-Plus in Africa, in Tanzania.
The question of “Why me?” tormented her as she grew up. However, when she heard about her sister who passed before she was born, it made her reflect more and start taking care of herself.
“When I turned thirteen (13) years I began to understand the condition that is, sickle cell disease. I also began being able to take care of myself and manage the disease by drinking warm during cold seasons, drinking lemon water, eating vegetables, and fruits, taking medication at the right time and going for my regular check-ups every month. This is how I have lived with Sickle Cell till today.”
Even though Eunice is not married, her supportive family has helped her navigate some of the challenges and obstacles of the disease. Some of the complications associated with Sickle Cell disease include pain episodes, organ damage, hand and foot syndrome, ocular complications, acute chest syndromes, stroke and early death. Data shows that individuals with sickle cell disease have a short lifespan varying from 20 to more than 40 years less than the general population depending on where they live.
However, currently there is no Universal treatment options of Sickle Cell Disease and options vary depending on individual symptoms. Treatment processes may include blood transfusions, high fluid intake, intravenous therapy and pain medications. As for the accessibility and affordability of sickle cell medications, it can vary depending on factors such as Geographic Location, Healthcare Infrastructure, and Health Insurance Coverage. In some regions, access to specialized care and medications for Sickle Cell Disease may be limited, leading to challenges in Affordability and Availability.
There are more children being born with the diseases on the African continent and unlike Eunice, most of them do not live to celebrate their fifth birthday. Lancet data shows that 75 percent of the more than 500,000 annual global children born with Sickle Cell Disease occur in Sub-Saharan Africa.
Eunice believes governments in Sub-Saharan Africa should scale up screening for sickle cell disease at birth as it can significantly increase the survival chances of children born with the condition.
“Newborn screening programs for sickle cell disease involve testing newborns shortly after birth to identify those who have the condition or sickle cell trait. Early detection through newborn screening allows for prompt intervention and medical management, which can improve health outcomes and reduce the risk of complications associated with sickle cell disease.”
She adds that for individuals planning to get married but do not know their sickle cell status, it is important to prioritize their health and well-being, as well as the potential health of their future children.
“Both partners should undergo genetic testing for sickle cell trait or disease before getting married or starting a family. Genetic testing can provide valuable information about each partner’s haemoglobin genotype and the risk of passing on sickle cell traits or diseases to their children. Consider seeking genetic counseling from a qualified healthcare provider or genetic counsellor. Genetic counselling can help couples understand the implications of their test results, assess their risk of having children with sickle cell disease, and explore options for family planning.
Speaking during a Side Event on addressing Sickle Cell Disease (SCD) through the Public Health Lens at the ICCPA Conference in Tanzanai, Dr Jane Hankins, the Director St. Judes Global Haematology Program said very few regions in the world have newborn screening for Sickle Cell diseases at the national level and Africa is not one of them
“This is what I think we should be doing in terms of addressing Sickle Cell Diseases. We are not counting and diagnosing the diseases earlier on. No country in Africa has Universal Newborn screening. Currently, there are only pilot studies happening in Africa and some –parts of the world on newborn screening,” said Dr Hankins.
She added that the world was spending most time on studies that are constrained at University teaching hospitals and less effort on scaling up studies to children outside urban areas who are not receiving care in University and tertiary hospitals.
“We need a holistic plan that is really an end and a solution to SCD. However, we are not identifying, we are not diagnosing and we are not treating the diseases. There are multiple reasons why we are not following and liking cases to care. We cannot look at one bucket in isolation, we have to look holistically at the end-to-end or entire spectrum solutions to sickle cell disease. This means we have to diagnose at birth, link to care, implement of evidence based interventions and build a sustainable global haematology community.”
Ghana is one of the few African countries that have improved early diagnosis of sickle cell Diseases through their point of care screening pilot project at Tamale University.
Dr Alhassan Abdul-Mumin, the Associate Professor and Director of Medical Affairs at the Ghana Tamale Teaching Hospital and University for Development Studies said scaling up newborn sickle cell disease screening could turn around treatment outcomes for children in Africa
“In my work, I would notice that the majority of the patients that we would receive were diagnosed late and they would come in with some serious complications. So when I got the opportunity to work with St. Judes, there was a chance for us to work on a project, so I decided to look at how we can enhance early diagnosis and link to care for children with Sickle Cell Diseases. I picked newborn screening for sickle disease which we started in the Tamale Teaching Hospital,” said Dr Abdul-Mumin.
Through the project, they have also started training healthcare workers who work with children with Sickle Cell disease in the Tamale area. They are also training midwives and nurses who work at the Tamale facility in the post-natal wards and immunisation clinic. They have also introduced point-of-care screening for new-born since September 2023. Ghana also has a combined adult and pediatric sickle cell disease clinic. Most children have been linked to care early following this development.
Meanwhile, there have been advancements in gene therapy for sickle cell diseases and these according to experts, offer promising alternatives to traditional treatments. There are also new sickle cell disease drugs that have been recently approved and these include crizalizumab, voxelotor, and L-glutamine. The drugs have been noted to decrease acute pain events and improve anemia. The therapies aim to address the underlying genetic cause of sickle cell disease, potentially providing a curative approach for some individuals.
