SPECIAL REPORT: Sickle Cell Awareness- A Public Health Imperative for Nigeria
By Hadiza Yusuf Ahmad
Sickle Cell Disease (SCD) is a genetic condition that affects the blood. In people with SCD, the red blood cells, which normally look like round disks, become shaped like a crescent or a sickle.
This change in shape makes it difficult for the cells to move through blood vessels and carry oxygen to the body’s organs. SCD is mostly inherited from parents who carry the sickle cell gene.
According to the 2022 research by PubMed Central, Nigeria has the highest global burden of sickle cell disease, with an estimated 2-3% of the population affected which translates to approximately 150,000 newborns diagnosed annually.
These statistics were further consolidated in 2024 by the Nigeria Policy Impact which states that Nigeria is the epicenter of sickle cell, accounting for 4-6 million of the estimated 50 million global cases.
These figures, sourced from reputable organizations, Highlight the essential need to address the challenges faced by individuals living with sickle cell in Nigeria, particularly during the harmattan season.
Normally, the harmattan season brings a chill to the air for many, but for Aisha, who is suffering from Sickle Cell Disease (SCD), it brings a life-threatening struggle with agonizing pain. Since the onset of the dry season, she has endured multiple hospital visits, each a distressing reminder of the constant battle against sickle cell anemia.
For her, relapses are frequent, often occurring immediately after treatment. To manage the Unbearable pain, Aisha undergoes regular blood transfusions, a procedure she faces every two months. Yet, even these transfusions offer only temporary relief, with Aisha sometimes experiencing a relapse before even leaving the hospital.
Also, Ayuba Ibrahim (not his real name), a sickle cell patient who was groaning in pain in the hospital but whenever his parents tried to comfort him, he became furious and told them to leave him alone. He blamed their decision to marry despite warnings about genetic incompatibility for his current condition, battling life-threatening crises almost every day.
Maryam, another patient, shared her despair, stating, “I have given up on life because this illness feels endless. Every day brings a new challenge. I can wake up feeling strong, but within hours, I’m struck by a crisis that leaves me hospitalized for days. I urge couples to take genotype testing seriously. Ignoring genetic compatibility can have devastating consequences for future generations.” She remarked sadly.
Maryam’s mother recounted her experience saying “I got married in a time with little awareness about genotypes. I didn’t even know what genotype was, and as a child, Maryam rarely showed signs of illness. She used to fall sick once a year, recover fully, and continue with her activities. But in recent years, she has suffered severe and frequent crises. Sometimes, we doubt if she’ll make it through.
“If I had known about genotype testing and the impact of genetic incompatibility, I would never have gone through with the marriage. Now, I encourage younger generations to make informed decisions and avoid the pain we are experiencing,” she said.
The pitiful mother recounts how financial constraints forced them to integrate traditional medicine into the treatment of Maryam’s sickness.
“Financial challenges have been overwhelming. We barely manage to get by. At one point, we turned to traditional herbal medicine, believing it could cure her. Unfortunately, it only made things worse. Dehydration, which triggers crises, was exacerbated when the herbal practitioner prohibited her from drinking water, insisting she only take water infused with herbs. It turned out he was a quack. Now I know better and caution others against such practices.” She added.
A medical health expert, Dr. Aliyu Shehu, who is a senior registrar in the Hematology Department at Aminu Kano Teaching Hospital (AKTH), has shed more light on the complexities of sickle cell disease (SCD), emphasizing its genetic origins, associated complications, and strategies for prevention and management.
Dr. Shehu explained that sickle cell disease is a hereditary blood disorder caused by abnormal hemoglobin production. This condition, he noted, leads to the production of defective red blood cells prone to “sickling” when exposed to stressors such as low oxygen levels, extreme weather conditions, infections, or other illnesses.
“When sickling occurs, it can block blood vessels and lead to various crises, including vaso-occlusive, hemolytic, and aplastic crises,” Dr. Shehu said. He stressed that SCD can affect every part of the body, from the brain and liver to the kidneys and even the skin, often resulting in complications such as leg ulcers that are resistant to treatment.
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Furthermore, Dr. Shehu categorized the disease into three main types: sickle cell anemia, sickle cell disease, and sickle cell trait. He explained that sickle cell anemia also referred to as hemoglobin SS, is the most severe form, as both inherited hemoglobin genes are abnormal.
According to him, prevention begins with genetic screening before marriage. “Parents must be screened to identify their carrier status because a child inherits the disease when they receive the abnormal gene (S) from both parents,” he advised.
In managing crises, Dr. highlighted the role of fetal hemoglobin (hemoglobin F) in mitigating symptoms. “Fetal hemoglobin, which is present at birth, prevents sickling. However, as children grow, it is replaced by adult hemoglobin (hemoglobin A), which contributes to the onset of sickle cell crises. In some cases, individuals retain higher levels of hemoglobin F into adulthood, leading to milder symptoms,” he explained.
He also touched on the impact of coexisting genetic disorders, such as thalassemia, in reducing the severity of sickle cell disease. “While thalassemia is generally harmful, its coexistence with sickle cell anemia can lessen the frequency of crises by modifying the structure of the hemoglobin chains,” he noted.
He also said sometimes to manage sickle cell, the patient has to undergo blood transfusion which involves transferring healthy red blood cells from a donor to the patient. This helps increase oxygen delivery in the body, reduce the number of sickled cells, and manage complications like severe anemia or organ damage. It can alleviate symptoms, prevent strokes, and improve overall health, but it requires careful matching and monitoring to avoid risks like iron overload or reactions. He said.
He added that the best way to manage a medical crisis is to visit a hospital immediately when it occurs to receive proper treatment and medication.
Dr. Shehu highlighted a significant observation regarding the occurrence of sickle cell crises, emphasizing that the condition is often aggravated during the heating season. He explained that dehydration is a major contributing factor, as even healthy individuals are prone to dehydration during this period.
However, for individuals with sickle cell anemia, dehydration exacerbates the risk of crises, contrary to the common belief that crises are more frequent during harmattan. He noted that crises are minimal during harmattan compared to the heat season.
According to Dr. Shehu, the most effective approach to combating sickle cell anemia is prevention. He stressed the importance of couples knowing their genotypes before marriage. Governments, he suggested, should implement and enforce policies discouraging marriages between individuals with incompatible genotypes, such as AS and AS or AS and SS, to reduce the prevalence of the disease in future generations.
Dr. Shehu dismissed claims by traditional medicine practitioners that they can cure sickle cell anemia. “There has never been any herbal medicine proven to cure sickle cell anemia or change an individual’s genotype,” he stated. He shared cases of patients who were misled into relying on herbal treatments, only to suffer more frequent crises and return to conventional medical care with worsened conditions.
The medical expert also noted that while bone marrow transplants remain the only known cure for sickle cell anemia, He cautioned about the risks involved. Many patients face rejection after a successful transplant, as the body perceives the foreign cells as threats and launches an immune response. This rejection can lead to complications and, in some cases, death. Although some patients have had successful transplants, the procedure remains risky and inaccessible to many.
Thus, Dr. Shehu underscored the need for government-led awareness campaigns to educate the public about sickle cell anemia. Increased awareness of the disease’s impact and prevention strategies could significantly reduce its prevalence and ensure a healthier future generation.
He added that the financial burden of managing sickle cell anemia is overwhelming for many families. Medications, some of which must be taken hourly, are often prohibitively expensive. While patients covered by the National Health Insurance Scheme (NHIS) pay less, many still struggle to afford treatment. Dr. Shehu emphasized that the high cost of care leaves numerous patients untreated even after enduring severe crises.
Dr. Shehu called for increased education, and policy implementation, to alleviate the challenges faced by individuals living with sickle cell anemia and their families, by emphasizing the importance of public awareness, early diagnosis, and lifestyle adjustments to reduce the frequency and severity of crises. He encouraged individuals and families affected by the condition to seek medical advice and support to manage its impacts effectively. He added that sickle cell disease is worth avoiding as it can be preventable
Sickle cell disease remains a significant public health concern, and experts like Dr. Shehu continue to advocate for preventive measures and better care for those living with the condition.
Hadiza Yusuf Ahmad is a PRNigeria Fellow 2024, she writes from Kano and can be reached via [email protected]