With an estimated 150,000 annual births of babies with sickle cell anaemia -regarded as the most common form of sickle cell disease, Nigeria takes almost half of the share of more than 300,000 babies with severe haemoglobin disorders that are born globally each year.
These staggering statistics, according to health experts, has ranked Nigeria as a black sheep among the comity of nations.
But the Nigerian government seems unconcerned about the country’s status as the global epicentre of the disease with successive administrations showing no clear signs of commitment to its elimination.
Today, as the world gathers to mark the 2021 edition of the annual World Sickle Cell Day towards raising awareness about the disease, there is no evidence of any activity on the part of the Nigerian government to solidarise with those living with the disease.
This year, relevant individuals and governmental and non-governmental organisations will assemble in the United States of America (USA) to mark the day with the theme; “Shine the Light on Sickle Cell.”
However, without any significant activities lined up to sensitise the populace on the danger inherent in ignorance about the disease, Nigeria continues to grope in the dark for imaginary solutions.
A PREMIUM TIMES’ recent letter seeking relevant information on the disease, which was addressed to the Federal Ministry of Health, Nigeria’s government department in charge of health issues, was yet to be replied by the ministry.
Also, the ministry’s official in charge of family health, the department responsible for family health issues, Salma Kolo, however, said the department of hospital services is responsible for sickle cell matters.
Mrs Kolo said; “I’m yet to receive your letter on my desk. However, I will direct you to the director of hospital services if you can come to the ministry on Monday. That is the department in charge of sickle cell disease.”
About sickle cell disease
According to Medscape, a multilingual online platform providing continuing medical education for physicians and health professionals, sickle cell denotes all genotypes containing at least one sickle gene, in which haemoglobin “S” makes up at least half the haemoglobin present.
The organisation further categorises major sickle genotypes to include those with haemoglobin “SS” which is otherwise called sickle cell anaemia and regarded as the most common form, and another with homozygote for the “S” globin with usually a severe or moderately severe phenotype and with the shortest survival.
There are also others with haemoglobin S/b-0 thalassemia which means double heterozygote for haemoglobin “S” and b-0 thalassemia, which is said to be indistinguishable from sickle cell anaemia, and the haemoglobin S/b+ thalassemia, which is said to be “mild-to-moderate severity with variability in different ethnicities,” among others.
By its foundation, sickle cell anaemia is an inherited form of anaemia, described as a condition in which there are not enough healthy red blood cells to transport adequate oxygen throughout the body.
Experts say sickle cell anaemia can lead to many complications such as stroke, acute chest syndrome, pulmonary hypertension, organ damage, blindness, leg ulcers, pregnancy complications, among others.
For instance, it is believed that sickle cells can block blood flow to an area of the brain, igniting stroke signs such as seizures, weakness or numbness of arms and legs; sudden speech difficulties, and loss of consciousness.
According to the World Health Organisation (WHO), approximately five per cent of the world’s population carries trait genes for haemoglobin disorders, mainly sickle-cell disease and thalassaemia.
According to the global health body, more than 300,000 babies with severe haemoglobin disorders are born globally each year, but 75 per cent is said to be found in Sub-Saharan Africa.
Nigeria carries 66 per cent of the burden in the region, the American Centre for Disease Control and Prevention (CDC) states.
Experts further added that the majority of the children with the most severe form of the disease die before the age of 5, usually from an infection or severe blood loss.
According to a report from the World Health Organisation (WHO), 24 per cent of the Nigerian population are carriers of the mutant gene and the prevalence of sickle-cell anaemia is about 20 per 1,000 births.
In countries such as Cameroon, the Republic of Congo, Gabon, Ghana and Nigeria, the prevalence is between 20 to 30 per cent while in some parts of Uganda, the prevalence is said to be as high as 45 per cent.
Meanwhile, a study published on thelancet.com shows that “Nigeria has the highest birth prevalence of sickle cell disease in the world, with an estimated 150,000 annual births of babies with sickle cell anaemia, the most common form of sickle cell disease.”
An earlier report by this newspaper also quoted the WHO 2014 statistics, saying that; “at least 100,000 babies die from the disorder in Nigeria every year.”
And like other sub-Saharan African countries, the inherited blood disorder is said to be the underlying cause of about 1 in 12 newborn deaths.
The grim statistics is said to have qualified Nigeria as the number one sickle-cell endemic country in Africa.
Efforts to stem the tide?
Annette Akinsete is a consultant public health physician, who, like many other Nigerians, is unimpressed with the Nigerian government’s poor attention to the elimination of the disease in the country.
Ms Akinsete, who doubles as the chief executive officer of Sickle Cell Foundation Nigeria, a non-governmental organisation that is focused on preventive and curative measures for sickle cell disease, said she cannot beat her chest that Nigeria is committed to global policies and programmes aimed at beating the scourge.
In an exclusive interview with PREMIUM TIMES, the campaigner said Nigeria has not invested enough in SCD.
She said; “The government comes up with policies and plans but they need to work with advocates for implementation. NGOs are the ones picking the slack for the government. We want the government to come up with comprehensive policies that take care of people with sickle cell.”
The senator representing Ebonyi North Senatorial District, Sam Egwu, once corroborated Mrs Akinsete while leading a debate on a bill to prevent, control and manage sickle cell anaemia in Nigeria.
Mr Egwu, an agronomist and former Ebonyi State governor, who rued the prevalence of the disease in the country, said the situation has risen to an alarming proportion, but that “little is known about it and far little is done about it.”
Abiola Morakinyo, an SCD sufferer, lamented what he described as the victims’ harrowing experiences for “diseases brought upon them by others.”
He lamented the social challenges such as stigmatisation facing the patients, saying Nigeria is one of the very few countries where medications taken regularly by the SCD patients are not available for free.
“The disease is prevalent among the poor due to illiteracy and poor awareness campaigns by the government. But the poor cannot afford regular medications such as folic acid,” Mr Morakinyo told PREMIUM TIMES on the phone.
The sickle cell prevention, control and management bill, which was sponsored by Mr Egwu during the eighth parliamentary calendar, scaled the second reading in 2020.
The main provision of the bill seeks to prevent persons who are carriers of AS and SS genotype from marrying others carrying similar genotypes.
The sponsor said the bill seeks to curb preventable massive deaths and avoidable hardships caused by the disease, and that such will be achieved “by placing a statutory duty on the federal government to engage in and encourage the prevention, control and management of the occurrence, spread and effect of sickle cell anaemia.”
He said the legislation will enable the Ministry of Health to direct, coordinate and supervise the prevention, control and management of the disease, while it will also be empowered to accredit reputable public and private hospitals and medical clinics across the country, including the rural areas, to function as accredited participants in the prevention, control and management of the disease in Nigeria.
The lawmaker explained that the passage of the bill will ensure that experts and other concerned parties are committed to the prevention and control of sickle cell disease in the country through adequate sensitisation and counselling towards facilitating uniformity and standardisation of care for those living with the disease.
Mr Egwu said; “It will ensure that these campaigns, sensitisation and services are taken to the rural areas across the nation where the majority of the citizens are ignorant of this disease and do not carry out the relevant tests to ascertain their genotype before getting married.
“Proper sensitisation of the rural dwellers and accessibility of the blood testing services, which this Bill seeks to enhance, will lead to an appreciable reduction in the number of fresh cases in a short time.”
However, since May 2020, when the bill scaled the second reading, with a committee tasked to report back to the house by June, nothing has been heard of the bill one year after.
But the sickle cell advocate, Ms Akinsete, has criticised the bill, saying it is not what should be prioritised.
She said; “Those are not the kind of things we are looking out for because they are thinking of how to prevent sickle cell. Let’s focus on those who are there already. In terms of prevention, it’s just to make people know what sickle cell is and that’s why we have World Sickle Cell Day. This can help people make informed decisions but you cannot force your decision down their throat.
“Let them make the decision to either marry each other or not. But it is best they know before they get to that level of marriage.”
She also suggested that Nigeria should have enough counsellors in primary healthcare centres (PHCs) in every local government area in the country to prevent sickle cell disease. “Until that happens, I won’t say Nigeria has done enough.”
Health Commissioner speaks
The commissioner for health in Osun State, Rafiu Isamotu, has said by virtue of Nigeria’s population and the nature of the disease, it is easier to guess that Nigeria has the larger percentage of the global cases of SCD.
He, however, explained that since he joined the Osun State government to man the health ministry about eight years ago, he has focused on public campaigns and awareness drives to limit the prevalence.
Mr Isamotu, a paediatrician, said; “I want to believe that we are not doing enough awareness regarding this. The less people with sickle cell trait marry each other, the less (the) chance of giving birth to children with sickle cell.”
He said the state government is working on a major collaboration aimed at screening newborns of sickle cell, which he said would kick off in October in the state.
“We have concluded arrangements on how we would start newborn screening of sickle cell. Exactly what is done in Europe and the USA. By the grace of God, by October this year we would start. We are also recommending Hydroxyurea as part of our treatment regime for sickle cell kids in the state,” the commissioner added.
Mr Isamotu, while pleading with his colleagues across the 35 other states of the federation and the federal capital territory to step up the efforts to address the disease’ prevalence, noted that the partnership targeted at newborn screening would be with Global Sickle Cell Alliance, Inc.
Mrs Akinsete noted that in Nigeria, when people with sickle cell disease appear in hospitals for treatment, they hardly receive the required attention.
She said unlike other patients with other forms of complaints, those with SCD require pain relief and rehydration.
Mrs Akinsete said; “So you need to set up a drip for them and observe them for the day. For the usual daily medication, it’s folic acid every day. Folic acid is hematinic; that is one thing that helps to build blood.
“When we say they have a shortage of blood, sometimes doctors prescribe iron tablets for them but that is wrong. People with sickle cell should not be given iron tablet. Iron tablet is what we give people with shortage of blood but we don’t do that for sickle cell. This is because they already have excess load of iron in their bodies. Sometimes when we give them blood transfusion, we give them something to remove the iron. We also give malaria prophylaxis and that is very important.”
She said because Malaria attacks the red blood cells, and that sickle cell patients already have problems with their red blood cells, “this means that the blood they have will be compromised. So we give them prevention medication for malaria, like proguanil and daily doses of folic acid.”
Mrs Akinsete said at her clinic in Lagos, such medications are given free “because if we don’t, most families cannot afford these medicines every day.”
“In our country, the health insurance scheme is still in its infancy. When patients go to the hospital, they are told that their insurance does not cover these medicines. So until the government steps in with a proper policy, backed by law, the people with sickle cell should be treated free. At least folic acid and proguanil are preventive drugs and what we are pushing for is that they get these medicines free every day of their lives,” Mrs Akinsete added.
She also spoke about a new medication called hydroxyurea, saying it was initially found for cancer treatment before it was repurposed for sickle cell.
Because it increases the level of fetal haemoglobin, she said taking this drug makes people with haemoglobin “S” come up and they begin to behave like AS because they would no longer have a crisis.
She added; “So the discovery of hydroxyurea has been a big boost to the management of sickle cell. Although many doctors are still wary of prescribing it because it’s a new drug. But we are prescribing it in our clinic as other countries are also using it.
“Some people talk about infertility and dark nails as some of the side effects but we are following them based on science and research findings. The other treatment used for children with high risk of stroke is hydroxyurea. In fact, before this treatment, it was blood transfusion for every three weeks. But that’s almost impossible in Nigeria because we hardly have enough blood in our blood bank.”
The Osun State health commissioner corroborated Mrs Akinsete, and added that in science, as long as the merits far outweigh the demerits, the drugs are safe to use.
Mr Isamotu, however, did not confirm the infertility claim, saying it is a drug adopted by his state. “Apart from routine care of giving prophylactic folic acid and penicillin antibiotics to all kids with diagnosed sickle cell, we also follow them up in the clinic till their teen years.”
There is significant progress made by scientists in the developed world to combat the scourge of the disease through a new innovation called gene therapy.
In 2017, France recorded a milestone when a 13-year-old boy with sickle cell disease became the world’s first patient to be successfully treated with gene therapy.
The teenager was said to have remained free from sickle crises and other signs of the disease 15 months after his treatment, which was conducted as part of Phase One of two clinical trials.
Meanwhile, Africa also received a boost when in 2020, the United States’ National Institutes of Health and the Bill & Melinda Gates Foundation announced plans to invest $200 million in the development of gene-based cures for sickle cell disease and HIV.
Previously regarded as experimental, gene therapy has now evolved with a team of scientists in Germany and Iran said to have noted in a September 2019 review that, at least, 20 gene therapy products have already been approved.
In Nigeria, the Sickle Cell Foundation has established a centre for bone marrow transplant in collaboration with Lagos University Teaching Hospital (LUTH).
World Sickle Cell Day
Since 2008, World Sickle Cell Awareness Day has been held annually marked across the world.
The decision followed a resolution passed by the General Assembly of the United Nations, recognising SCD as a public health concern.
The choice of the date, June 19, is in commemoration of the date the resolution was passed.
It is aimed at increasing public knowledge and raising awareness of Sickle Cell Disease (SCD) and the struggles people with the disease and their families go through.
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