Nigeria with the highest burden of the sickle cell disease globally is still slow in curbing the preventable disorder. There is no legislation, no national coverage of the genotype screening, and no active policy to aid in controlling and managing the disease. People living with this inherited blood disorder are left to struggle with serious health complications, discrimination and high financial burden lifelong. Rebecca AKINREMI reports.

AFTER hours trying to endure the crippling pain in his bones, Ikechukwu Oleka needed to be rushed to a hospital. His agony was particularly high around the waist and he had to be quickly admitted at the medical emergency unit. Having lived with sickle cell disease for 25 years, he thought it was the usual crisis. But the pain did not subside, even after he was discharged three days later. 

Sickle cell disease (SCD), also called SS genotype or sickle cell disorder, is a lifelong and serious inherited blood disorder but it is preventable. It comes with dreadfully painful complications commonly known as sickle cell crisis where patients’ bodies are afflicted with sudden episodes of severe pain. Not only do the patients experience pain, but they also suffer from other miserable complications.

“It can virtually affect every part of the body,” said Bello Ibrahim who is a Senior Registrar at the National Hospital’s Hematology Unit.

He noted that sickle cell disorder could lead to stroke, eye problems, protruding forehead, swollen gums, heart diseases and acute chest syndrome. “It can also affect the hip bone causing the patient to limp while walking,” he said.

Oleka learnt of his sickle cell status in 2011. “A series of tests, which include a genotype test was done after I became ill in my 100 level at the University. That was how I became aware I have SS genotype,” he said.

Now 28, Oleka noted that his parents later confessed that they got to know he was SS at age four, though it seemed they were ignorant of the disease.

“Whenever I’m in pain at my young age, my parents used hot water and Robb [ a popular ointment] to massage me and I will be given paracetamol tablets to swallow, then I cry myself to sleep. Sometimes, I wake up and the pain is gone. Other times, I wake up, still in pain,” Oleka narrated his painful life experience.

After the unforgettable pain refused to go in February 2016, he was asked to do a scan of his body which was later carried out at the National Orthopedic Hospital, Igbobi-Lagos. “They said I had Avascular Necrosis of the femoral head at the left leg and I required surgery,” Oleka said.

Simply, the disease had affected Oleka’s hip bone and has reached advanced stage. Avascular Necrosis (AVN) according to the WebMD, an American health information site, is a condition that happens where there is loss of blood to the bone. An interruption of the blood supply causes the bone to die. It most often affects the hip, but can also affect the shoulder, knees, and ankles.

“At that point, I was asked to start using a walking stick, pending the time of the surgery,” said Oleka.

Efosa Obamwonyi, Senior Registrar at Orthopedic and Traumatic Unit of the Obafemi Awolowo University Teaching Hospital said Oleka’s condition would cause him to have pain and difficulty in walking. He added that the length of two legs would become unequal thereby causing the patient to use crutches. To get back to health, Efosa said the painful “hip joint would be replaced surgically” with an artificial joint often made from metal and plastic components.

Oleka was troubled about his condition but was worried more when the hospital asked him to provide N1.7 million for the surgery. “I knew I don’t have such money. So I was worried more about how to raise it,” he said.

For 18 months, Oleka bore the burden of using crutches, and with excruciating pain on the hip.

“I was using walking stick between March 2016 to September 2017 when I had the surgery at Igbobi,” he said.

The hip replacement surgery was done twice.

“The first surgery was on a Tuesday. After the surgery, they discovered that there was a mistake in what was done. So I had to be moved back to the theatre for a correction surgery done on Friday of the same week,” Oleka recalled.

He is currently attending physiotherapy sessions four times in a month because he still drags his left foot after the surgery. Before the surgery, the young man also suffered from other complications. Earlier in 2012, he was diagnosed with Acute Chest Syndrome (ACS).

“I was having pain in my chest. I was not really doing fine,” he said with a sign of weakness in his voice.

Acute chest syndrome is one of the most serious problems that people with sickle cell disease can have, according to a journal by Joseph Maakaron, a UK-based Internal Medicine expert. He said adults with the ACS need a higher rate of blood transfusions and longer hospitalisation as it causes severe chest pain and breathing difficulty.

“ACS is an acute pulmonary illness and an important cause of death in SCD,” as stated in Nigeria’s National Guideline for the Control and Management of SCD published in 2013.

“The pains are very painful. Every time I stress myself, I just broke down with crises,” Oleka narrated adding that he spent not less than three days anytime he is hospitalised. “And I treat myself out-of-pocket.

“It is harsh. It is a great financial burden on me and my parents, and the effect is really telling on my parents especially,” he said, stressing his financial inadequacy. 

Every month, Oleka’s priority is to set aside not less than N13,000 for his drugs. That will cover his daily medications and a four-time physiotherapy session per month. But in the case of emergencies, he seeks charities.

A disease that affects academic performance

The four people living with sickle cell disease that this reporter interviewed at different times all complained about their low academic performances in both their secondary and tertiary educations.

Mayowa Tijani, 28, who lives with the disorder told The ICIR he wrote both English and Mathematics papers on his sickbed during his Senior School Certificate Examination (WASSCE).

“I was brought in an ambulance from the medical centre to write the exam in the school sickbay. I wrote the exam in a poor state of health. And when the result came out, I didn’t do so well,” he recalled.

When he proceeded to the University of Ibadan for a degree, Mayowa said he would not try to read when semester exams were coming. “When the stress is too much, I break down,” he added.

Another SS-genotype person, simply identified as Greg, lamented the tough time he had at the university. Greg told this reporter his problem became complicated when he decided to stay in the halls of residence provided by the Obafemi Awolowo University, Ile-Ife where he obtained his bachelor’s degree.

“The hostel rooms were crowded. and my health status affects my academics terribly. There are lots of times I missed my exam, classes, assignments, and tests because I was not feeling fine,” Greg said. He recalled that his colleagues used to question his studentship due to constant absenteeism.

Oleka who finished from the University of Benin also had to extend a four-year course to five. “I didn’t graduate with my colleagues,” he noted. He missed two courses when writing the final year examination.

Stigmatising the victim of sickle cell disease

“Stigmatisation is what we are experiencing every day,” said 27-year-old Bukola who soon realised she was not like every normal kid in her childhood. She told The ICIR that her colleagues while in secondary school would not want to associate with her, considering that she could suddenly become sick.

Moving ahead in life, Bukola noticed people describe her directly or indirectly as a weak person. “Sometimes it is embarrassing. Why do I actually have to be this way?” Bukola queried.

“When I was in secondary school, my mates called me all sorts of names,” Tijani recalled his experience of the stigmatisation. He noted how he was usually called “a sickler”. He also said some romantic relationships for marital purposes could not succeed because of his genotype status.

Like Oleka, Mayowa, Greg, and Bukola, there are millions of other Nigerians living with sickle cell disease. The American Centre for Disease Control and Prevention (CDC) states that slightly above 300,000 babies globally are born with severe sickle cell disease (SCD). And Nigeria alone, according to the 2015 strategic plan to fight non-communicable disease in the country, has between 150,000 and 200,000 babies born each year with SCD. This indicates that the country bears more than 50 per cent of the global burden and has the world’s highest burden of SCD.

According to the World Health Organisation, African Region, SCD which is regarded as a black disease, causes the normal round shape of red blood cells to become a banana-like shape, sickled. With the primary function of the red blood cells to transport oxygen to body tissues and carbon dioxide to the lungs, the round type can move easily through the blood vessels. But in people with SCD, their new shape prevents the free movement of the blood cells. Thus, adequate oxygen is not transported in the body. This causes pain, extreme fatigue and damage to bones, muscles, and organs on any part of the body.

A child born with SCD is birthed by parents who both carry a sickle-cell trait known as AS genotype. If both AS and AS marry each other, there is a higher prospect that their child will be born with the disease. And around one in four people in Nigeria has the AS genotype, according to the Ministry of Health.

There are over 100,000 babies that die before their fifth birthday due to sickle cell, as stated in Nigeria’s strategic plan.

No national coverage of genotype screening, no legislation

Joshua Datakun, Communication Officer at the Sickle Cell Foundation (SCAF) noted how many Nigerians are ignorant of their genotypes before marriage. He blamed the situation on the inadequate provision of the screening facilities for the haemoglobin test.

“There should be more centres where people could easily have their genotypes test done,” Joshua lamented about the testing facilities which do not have national coverage and cannot be found in the primary healthcare centres. And it costs about N3,000 in the few hospitals where available.

“It is important to know the genotype of a newborn baby,” said the NHA doctor, Bello Ibrahim. To reduce the mortality rate of children born with the disease and avoid any complications, he said Nigerians should be able to screen themselves to know their genotypes.

“Most of our patients only realise they have sickle cell when brought in sick to the hospital.” He said, unlike before, when genotypes testing is done two to three times to ascertain one’s genotype, there is a newer method to help determine the genotype correctly at once.  But the “new method that gives accurate results is only found in the tertiary health centres”.

Nigeria with a high rate of the preventable disease is yet to have legislation for the disease. In 2017, there was a bill sponsored by Ahmed Salau Ogembe who represented Kogi Central Senatorial district in the eighth assembly and Ovie Omo-Agege, his colleague representing Delta Central. The bill has sought to provide mandatory haemoglobin-genotype screening test before marriage and before newborn babies can be registered. But the bill has never gone beyond the second reading.

The objectives of the suspended bill were threefold: “To establish a clear legislative framework for effective management of sickle cell disease; to avoid human anxieties, pain, and death associated with the disease and; to improve the lives of citizens who live with it”.

The bill did not pass into law despite campaigns held by some organisations, including Nigeria Health Watch. Senami Ohimokhare of the Jephthah Ohiomokhare Foundation also launched a campaign to raise awareness of the bill in 2017 titled “What’s Your Type?”

Only Anambra State succeeded in enacting a law on sickle cell. According to the law enacted in May, no religious body or marriage registry “shall perform solemnisation of Holy Matrimony”, without presentation of a sickle cell disease prevention certificate from the intending couple. As punishment, parents, guardians, as well as priests, pastors and marriage registries that perform the marriage without genotype test, risk going to jail.

Low budget hampers policy implementation—Ministry of Health

In a bid to reduce the prevalence of sickle cell disease in the country, the federal government in 2015 launched a strategic plan for non-communicable diseases, which included halting the rise in the prevalence of SCD in the country by 2025.

“Sickle cell disease (SCD) is another major NCD that is given priority in this plan of action as Nigeria has the highest burden of SCD in the world,” the plan stated.

However, Alayo Sopekan, National Desk Officer of SCD at the Federal Ministry of Health, confirmed to The ICIR that Nigeria has not even achieved five per cent reduction of SCD since 2015 when the plan was launched.

Sopekan blamed the implementation hitch on the inadequacy of funds obtained yearly from the federal government.

“The budgetary allocation is very far from what is expected of a country that is having the highest burden of the disease; that children are dying every now and then from the disease.

“And that is one of the things we have been shouting, talking, making noise about,” Sopekan said.

He said it was during the late President Musa Yar’Adua’s administration (2007-2010) that large amounts of money were approved for sickle cell through the Millenium Development Goals (MDGs).

“We were able to do a lot at that time. We developed a policy on non-communicable disease including sickle cell disease, a national guideline for the control, prevention, and management of the disease in the country, said Sopekan. He also said the government was able to establish six zonal sickle cell Centres which are found in Federal Medical Centres in Gombe, Nasarawa, Kebbi, Lagos, Bayelsa, and Ebonyi states.

“When Yar’ Adua died and Goodluck Jonathan came in,  the programme was not funded. So there is nothing anybody can do.

“Even this government (Muhammadu Buhari administration) when they came in they have not really funded it. They have not. I think it is now they put little thing there for sickle cell in the budget. We are saying that is not even enough. That one is just like a drop in the ocean. So that is a challenge that we are having,” he narrated.

On the genotype screening, Sopekan informed the reporter that new technology has been presented to the government which can “actually screen people of their genotypes from anywhere, even if a child is born at home”. Sopekan said the government is undergoing an independent clarification to determine the functionality of the point-of-care testing of sickle cell disease.

When The ICIR checked through the budgetary allocation between 2015-2019, N146 million was provided to support the treatment of sickle cell disorder, though we cannot ascertain how much was released.

Only the 2017 and 2018 budgets included money for “awareness campaign on social-consequences of sickle cell disorder” only for the Northcentral region: N20 million in 2018 and N50 million in 2017. In 2019, no money was budgeted for the awareness of sickle cell in the country. 

Other monies budgeted included the purchase of equipment to establish data repository and data registries for SCDs and also for tobacco. These monies were included in 2018 at N10 million and in 2019 for N16 million.

Going down to 2016, nothing was in the budget relating to sickle cell disease in the year. While in 2015, N50 million was accorded for a NIPRD special project for medicinal products for the management of malaria, sickle cell, diabetics, ebola, and Lassa fever.

Nigerian has developed drug to manage SCD, but it’s yet to be mass-produced

A made-in-Nigeria drug with the generic name, “Niprisan”, was developed at the National Institute for Pharmaceutical Research and Development (NIPRD) in 1998. The drug’s license was given to an international organisation for its mass production, with the brand name of NICOSAN, but it went bankrupt.

Meanwhile, last year April, Professor Isaac Adewole, former Minister of Health announced that the federal government has given NIPRD approval to license May and Baker Plc, Nigeria’s first pharmaceutical company, to mass-produce the drug.

“What we have done is to reactivate the product and it will now be marketed in Nigeria through this agreement and we believe that the marketing and production in Nigeria will bring a lot of comfort to millions of Nigerians who are infected with sickle cell gene,” said Adewole in 2018.

A year after, the drug is yet to be commercialised.

Olobayo Kunle, a professor and director of the institute told The ICIR that NIPRD is “providing all the intellectual, technical and logistics support required to ensure that the company is able to promptly make the drug available to the market”.

The institute can not provide reasons why the drug is yet to be on the market, and only May and Baker PLC knows why, he said.

If the drug had been in use, it will have reduced the frequency of crisis and pains, thereby improving the quality of life of the people living with the disorder, he added.