A MILESTONE IN SICKLE CELL TREATMENT
A potential cure for people living with sickle cell disorder has been approved in England, writes SONNY IROCHE
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Sickle cell anemia is a genetic blood disorder characterized by the production of abnormal hemoglobin, known as hemoglobin S (HbS). Sickle cell disease changes the shape of blood cells into crescent (or shape of sickle), hindering blood flow. This condition leads to the distortion of red blood cells into a sickle or crescent shape, which can cause various health complications. This blood disorder affects individuals of African descent, but it is also found in people from other ethnic backgrounds.
Apart from afflicting individuals of African descent, the disorder also affects people of Mediterranean – countries around the Mediterranean Sea, such as Greece, Italy, and Turkey. People of Middle Eastern descent, including parts of Saudi Arabia, and other Gulf countries, are also at risk.
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Sickle Cell anemia also afflicts people from the Caribbean, particularly those with African ancestry. The disease is caused by a mutation in the HBB gene, which provides instructions for making the beta-globin subunit of hemoglobin. The disorder follows an autosomal recessive inheritance pattern, meaning that a child must inherit two copies of the mutated gene (one from each parent) to develop the disease. If a person inherits only one mutated gene, they are considered a carrier (sickle cell trait) but typically do not exhibit symptoms.
Symptoms of sickle cell anemia can vary in severity and may include: Anemia: Due to the rapid destruction of sickle-shaped red blood cells, individuals often experience fatigue, weakness, and pallor.
Pain Crises: Sudden episodes of severe pain, known as vaso-occlusive crises, occur when sickle-shaped cells block blood flow in small blood vessels. These episodes can affect various parts of the body, including the chest, abdomen, and joints.
Swelling: Painful swelling in the hands and feet, known as dactylitis, can occur in infants and young children.
Frequent infections: Sickle cells can damage the spleen, increasing susceptibility to infections, particularly from encapsulated bacteria.
Delayed Growth: Children with sickle cell anemia may experience delayed growth and puberty due to chronic anemia.
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Vision Problems: Blockages in the blood vessels of the eyes can lead to vision issues.
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Sickle cell anemia can lead to several serious complications, including: Acute Chest Syndrome: A potentially life-threatening condition characterized by chest pain, fever, and difficulty in breathing caused by lung infections or blocked blood vessels in the lungs.
Stroke: Blockages in blood flow to the brain can result in stroke, particularly in children and adolescents with the disease.
Organ Damage: Chronic reduced blood flow can lead to damage in organs such as the kidneys, liver, and spleen.
Leg Ulcers: Poor circulation can result in painful ulcers on the legs.
Sickle cell anemia is typically diagnosed through blood tests, including:
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– Hemoglobin Electrophoresis: This test identifies different types of hemoglobin and can confirm the presence of hemoglobin S.
Newborn Screening: Many countries routinely screen newborns for sickle cell disease as part of standard health checks.
Pain Management: Over-the-counter pain relievers or prescription medications may be used to manage pain crises.
Hydroxyurea: This medication can help reduce the frequency of pain crises and acute chest syndrome by increasing fetal hemoglobin levels, which helps prevent sickling.
Blood Transfusions: Regular blood transfusions can help reduce anemia and the risk of stroke, especially in children.
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– Folic Acid Supplements: These can help support red blood cell production.
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– Bone Marrow or Stem Cell Transplant: In some cases, a transplant may offer a potential cure, especially if performed in childhood.
Preventive Measures: Vaccinations and antibiotics are often recommended to reduce the risk of infections.
Individuals with sickle cell anemia can benefit from lifestyle changes, including staying hydrated, avoiding extreme temperatures, and managing stress. Regular medical check-ups and monitoring are essential to address complications early.
This break through in the treatment of the disease, comes as a great relief to many sufferers of the ailment, especially, when a lot of resources have been deployed in the past in drug discovery. Clinical trials find a one-time gene therapy ECA-CEO offers functional cure in 95% of patients.
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According to the publication in The Guardian UK newspaper, of January 31, 2025:
“A groundbreaking £1.65 million treatment offering a potential cure for people in England living with sickle cell disease has been approved for use on the NHS, the medicine watchdog has announced.
Campaigners welcomed news of the approval of the one-time gene therapy, known as exagamglogene autotemcel, or exa-cel, which edits the faulty gene in patient’s own stem cells.
The NHS set that 50 patients, older children and adults with severe form of the disease, who are suitable for a stem cell transplant but without a matched donor, will each year receive the cutting-edge treatment.
Clinical trials for exa-cel have indicated that the therapy can stop painful sickle cell crises, which occur when blood vessels become blocked, with researchers finding that there was a “functional cure” in 96.6% of participants who received the treatment”.
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According to the newspaper, “Toby Bakare, 35, from South London, who has sickle cell disease but received a stem cell transplant, said: “it’s a great day, so many people living with sickle cell disease will now have access to treatment that gets rid of pain [that] is a blight on your life. I’m lucky I had a sister who was a match.
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Prof Bola Owolabi, the director of the National Healthcare Inequalities Improvement Programme at NHS England, said: “This represents a monumental step forward in the treatment of people with sickle cell disorder, which is a condition that mostly affects people of Black African and Black Caribbean heritage. This groundbreaking therapy, available on the NHS, represents a very real prospect of a cure for this devastating disorder.
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While John James OBE, the chief executive of the Sickle Cell Society said: “We are absolutely thrilled to see this groundbreaking gene therapy treatment available on the NHS from today. The significance of this milestone for the sickle cell community cannot be understated”
Now coming back to how Sickle Cell disorder could be tackled in Nigeria, it is essential to acknowledge the unwavering dedication and perseverance of Prof. Olu Akinyanju, a globally recognized hematologist and the founder and chairman of the Sickle Cell Foundation of Nigeria. In a recent article in Pharmanews, it was noted that “any discussion about the management of sickle cell disease in Nigeria and Africa would be incomplete without mentioning Professor Akinyanju.” He played a pivotal role in establishing the National Sickle Cell Centre in Lagos.
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With advancements in the treatment of sickle cell disorder emerging from England, it is crucial for both the public and private sectors in Nigeria—the country with the largest population of Black individuals—to enhance their collaborative efforts in supporting the National Sickle Cell Centre, led by Prof. Akinyanju. This partnership is vital for securing access to some of the medications already available through the NHS in England, aimed at reducing the pain and suffering of those affected by this condition.
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Iroche is a Senior Academic Visitor at the African Studies Centre of the University of Oxford 2022-23
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Comment2
· TURNING THE TIDE IN EDO
JOHN MAYAKI reckons that Okpebholo is renewing the hope of Edo people
President Bola Tinubu may have run on Renewed Hope, but the slogan is finding fine expression in Edo State, where Senator Monday Okpebholo is restoring power to the people following an eight-year misadventure of the last administration. Okpebholo emerged victorious from an election that tested democracy’s true essence in the state as it challenged the people to answer the question of whether government exists to serve their real interests or those of errant salesmen in suits with forked tongues.
Okpebholo’s pitch was simple but effective. He called on the people: “Come, let us build a government in our own image and for our interests.” They heeded his call and placed their trust in him. 100 days later, they are counting the gains. One event is particularly illustrative – just weeks into his administration, Governor Okpebholo terminated what was effectively the slave contract of 360 cleaners and absorbed them into the state civil service, where they now earn the minimum wage along with other social protections.
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He rescued these workers, mostly women, from the grip of foreign contractors who paid them a pittance while billing the government exorbitantly under an outsourcing arrangement. This exploitative and extortionate model, widely adopted by the previous administration, impoverished Edo sons and daughters while enriching outsiders. It funneled money out of the state, depriving its people of real opportunities. Reports even emerged of jollof rice being flown into the state because the government apparently couldn’t trust the culinary skills of Edo indigenes.
Over the past 100 days, Okpebholo has been undoing such anomalies, restoring a system that dignifies, empowers, and invests in the potential of Edo people. The consultants in suits – who offered nothing but glossy reports and graphs that never left the computer screens – have been replaced with citizens of the state who have a deep understanding of local issues and the intelligence to address them. It is no longer a government of consultants, by consultants, and for consultants.
Some say the positive change is felt even in the weather. Perhaps the gods are pleased with the emergence of a governor that truly respects traditions and Kings, and doesn’t covet heirlooms and artefacts that are not his to claim.
Okpebholo has made bold moves to revive the state’s economy and stimulate growth. On infrastructure, he has resumed action on abandoned road projects crucial to economic activity and access to trade centers. The Benin-Auchi road, neglected for years despite its importance, is finally receiving attention. The long-forgotten Upper Ekenwan road is now nearing completion, bringing relief to thousands of commuters. The Aduwawa-Upper Mission junction, a vital artery for trade and transport, is back under construction, undoing the damage of years of neglect. In rural areas, long-overdue repairs are opening up communities once cut off from development, reconnecting them to the state’s economic lifelines.
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But roads alone do not bring prosperity – safety does. Under Okpebholo, security has taken center stage. He inherited a state where crime festered under the previous administration’s lack of decisive action. Cult-related violence and highway robberies had become the norm, leaving businesses and residents in fear. That tide is turning. Over 70 new Hilux patrol vehicles have been deployed to security agencies, dramatically improving their capacity to respond to crime. Intelligence-sharing among security forces has been strengthened, and collaboration with local vigilantes has brought community-led solutions into the fold.
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Beyond restoring order, Okpebholo is restoring dignity. His administration has put an end to the exploitative grip of market unions that thrived on extortion, inflating prices and making daily survival a struggle for ordinary Edo citizens. By suspending these oppressive groups, he has stabilized market prices, bringing much-needed relief to traders and consumers alike. Recognizing that economic empowerment is key to lasting change, he has also introduced interest-free loans for small businesses, ensuring that Edo’s traders, artisans, and entrepreneurs can grow without being crushed under the weight of exorbitant borrowing costs.
In healthcare, he has moved swiftly to correct the negligence of his predecessor. A modern health center is under construction in Udomi, Irua, expanding access to quality medical services. His N63.9 billion allocation for healthcare in the 2025 budget underscores a firm commitment to reversing years of underinvestment in the sector. Additionally, a N1.8 billion health insurance scheme has been launched, ensuring that the people of Edo no longer have to choose between their health and financial ruin.
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Education, too, is getting the attention it deserves. The subvention to Ambrose Alli University has been significantly increased to stabilize the institution, which had suffered from chronic underfunding. Decisive action has been taken to resolve the long-standing plight of medical students from the 2015 set, who had been left in academic limbo by the last administration’s failures. Even the controversial renaming of Edo State University has been reversed, restoring its original identity in alignment with the will of the people.
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The past 100 days have marked the beginning of a government that listens, acts, and delivers. Okpebholo is proving that governance is not a private business venture or a playground for consultants – it is about real people, real progress, and a renewed hope that Edo State is once again on the path to greatness.
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Mayaki is a journalist, farmer and an Oxford University, and Cambridge University-trained entrepreneurship, leadership and sustainability expert
Letter2
Igalla Maidens as Objects of Ritual Killing
Igalla ladies, particularly the Christian women, are the trusting type. That’s what the native church at Igalla land preaches, one formerly known as the Qua Iboe Mission and there is the other known as something like Fellowship of Brethren. What is becoming of this trust? Young Igalla university graduates in Abuja are lured into relationships by unscrupulous pagan grooming gangs from Nigeria’s Midwest and West and then these girls are offered as human sacrifices in pagan juju rituals. No, the damned pagans don’t offer up their own, especially the Bini. They go for vulnerable, well-bred maidens. Yet the Nigerian state argue that a return to “native culture” is cool. The children of those who advocate a return to “culture” are safe in the US and elsewhere.
Sunday Adole Jonah,
Department of Physics, Federal University of Technology, Minna, Niger State
Letter3
Swear to Obey
Doctors swear to follow the ‘Hippocratic Oath’ which requires in simple terms that the doctor does the best they can for their patient and doesn’t cause harm.
This is a simple standard that doctors seem to have no problem following but what of the US doctors’ proposed boss, Robert Kennedy Jr? He has expressed a range of contradictory views on medical issues including vaccines, abortions and even the food industry. It is not safe or sensible to allow him to take the position.
Health is far more important than political favours.
Dennis Fitzgerald,
Melbourne, Australia
Letter1
Satan, Witchcraft and Misinterpretation of Dreams
The Advocacy for Alleged Witches (AfAW) draws the attention of the public to a Facebook post by Helen Ukpabio of the Liberty Gospel Church linking dreams to Satan and Witchcraft. In the post, Ukpabio stated: One of the greatest means of Satan attacking humanity is through dreams and incidentally, the dream is so misconstrued. Whereas dream is one of the greatest weapons in(sic) Satan distributing problems to man. He allows you to be the one carrying your load. He allows you to be the one carrying your death sentence in your hand. He shows you what he wants to do to you through dreams, whereas many (sic) when they have these dreams they said: “The Lord showed me”.
Ukpabio had no idea of what she was talking about. She lacked basic knowledge of psychology and a basic understanding of dreams. Dreams are natural, not supernatural occurrences. They have nothing to do with Satan, the devil, or demons as she proposed. According to the online source, Wikipedia: “A dream is a succession of images, ideas, emotions, and sensations that usually occur involuntarily in the mind during certain stages of sleep”. These images, which could make you feel happy, sad, or fearful, represent unconscious desires and wishes based on information and experiences gathered during the day when one is awake. Dream experiences are not satanic attacks on human beings.
As expected, Ukpabio linked dream experiences to witchcraft. She stated: “Now since these dreams came from the realm of satanic affliction, Beelzebub which is called Ba’alzebub takes the glory because it is the spirit, the servient spirit of leviathan in charge of witchcraft. And one of the greatest ways in which witchcraft, ancient altar, and altars, can distribute problems to mankind is through dreams. Once you lie down to sleep, the bible says you shall sleep and your sleep shall be sweet, now instead of you to sleep and sleep sweetly you sleep into activities, this is not of God”. Ukpabio is mistaken. Dreams have no connection with witchcraft, Satan, or Beelzebub. Her interpretation of dreams is based on ignorance, fear, and twisted Christian superstitions.
The Advocacy for Alleged Witches urges the public to ignore the falsehoods, mistaken ideas, and witch-hunting teachings of Helen Ukpabio and her so-called Liberty Gospel Church. Their misinterpretations and witch propaganda have motivated people to commit atrocities and horrific abuses. The public should disregard Ukpabio’s misinformation and disinformation about dreams. Her teachings are misleading and have no basis in science, reason, or 21st-century Christianity.
AfAW employs the “informaction” theory of change in combating witch hunts in the region. It dispels misinformation and combines information dissemination with actionable steps.
Leo Igwe directs the Advocacy for Alleged Witches
