Sickle Trouble
Experts believe only one in two children with sickle cell disorder live past age 10. Up to 16% of all deaths in children under age five could be from the condition. But after five decades dealing with sickle cell disorder, Adebola Benjamin believes the difficult experience was manageable. “I have gone through the hard part,” […]
Experts believe only one in two children with sickle cell disorder live past age 10. Up to 16% of all deaths in children under age five could be from the condition.
But after five decades dealing with sickle cell disorder, Adebola Benjamin believes the difficult experience was manageable.
“I have gone through the hard part,” says the founder of Sickle Cell Support Centre, who turned 55 in November.
But she was wrong. The consequences of the treatments she underwent to deal with crises that plagued her childhood started to show up, she says.
“It is only recently that the medical community discovered the pain relievers they gave us from the time we were children causes ulcer, right from your oesphagus to your rectum.”
Her latest experience with a burst ulcer was terrible, she continues, after an older ulcer opened in March.
“It is by divine intervention that I am talking to you today. I had internal bleeding. All the blood ran into my intestines and everything.”
Tears choke her voice as recalls resigning her civil-service job to start the foundation.
Dealing with SCD is as easy as getting information, and sifting fact from fiction. In the condition, normal red blood cells lose their round shape, become “sickled” and unable to transport oxygen.
“To make a difference, we need to know what’s the best treatment,” says Dr Baba Inusa, core founder of Sickle Cell Cohort Research (SCORE) and lead consultant at SCD and Thalassaemia Centre at London’s Guy’s and St Thomas.
An estimated 300,000 children world over are born with SCD—nearly half of them in Nigeria, says Inusa.
“But until recently it wasn’t possible to make a diagnosis when the children are born.” That meant no prior information to prepare parents to deal with a child with SCD—from birth and until they are in school.
Few schools figure SCD children as special-needs candidates, and policies hardly detail safety and health for SCD children in school. SCORE has adapted a policy guide by the Economic and Social Research Council, hoping to push it into use in Nigerian schools.
The policy details good practice that recognizes special needs of SCD children: support programmes and extra lessons to enable SCD children catch up on school when crises force them to be absent from school; unfettered access to water (dehydration is problematic) and toilet (they pee frequently); less strenuous exertion during physical exercise; protection from malaria to which SCD kids are vulnerable; adequate environment temperature; individual health care plan.
“We have large number of patients but also we have a responsibility to make sure the services they receive are best for them,” says Dr Inusa. “That is why we want to make sure people managing these patients know exactly what to do.”
Secondary school student David Joshua is still in the thick of treatment. His last crisis—the bout of illness and pain that comes with SCD—was only days before his last school exams.
And he’s not the only one in pain, says his mother Ngozi Favour. “Any time the crisis comes, with pains. When I mean pain, I mean pain. Once he’s crying, my heart is…I’m fed up. I don’t know what to do,” she says.
That’s only one child, and the family has two children with SCD. The condition is easily inherited, and experts believe full genotype knowledge before child bearing is important.
“If we don’t know what our genotypes are, we run a risk of passing SCD onto our children, and this is because up to a quarter of us in Nigeria carry the sickle cell gene,” says Dr Oluseyi Oniyangi, consultant paediatrician at National Hospital, Abuja.
Among every 100 Nigerians, up to 25 may carry the gene. By Mendelian law of inheritance, “if two people who are carrying the sickle cell gene decide to get married and have children, they run a 25% risk of having [an SCD] child with every pregnancy,” says Dr Oniyangi.
The atmosphere most likely to help the cause is open disclosure, proper information and the right skills to deal with SCD.
“We want [this] policy in schools so that these children are not alienated and people can understand their condition,” says Dr Olufunke Lawson, SCORE trustee and consultant paediatrician at Zankli Medical Centre.
“I believe Nigeria needs to do a little bit more,” says Benjamin. “The time that my life has been in danger and I was saved were times I have been abroad. I was treated free. The nurses and doctors were trained to handle SCD. But in Nigeria, it is not like that.”