Trials and tribulations with Sickle Cell Disease

Dar es Salaam. Yusuph Gesase, learnt that he was living with Sickle Cell Disease (SCD) when he was in Standard Four—about eleven years ago. The 19-year-old is the last born in a family of five children—all diagnosed with SCD, except one of his siblings.

Before he understood his condition, Yusuph says, “I could see my parents take me to the clinic regularly but I didn’t know the reason.” At that age, he says, something known as Sickle Cell was still not in his vocabulary.

“I wouldn’t bother about what I was suffering from. I am blessed with good and caring parents who were taking care of me in every step of the disease and life,’’ says the Form Six student at Kibaha Secondary School in Pwani Region.

Today, as he narrates his story of tribulations and triumphs with SCD, he recalls how he has been able to cope with the disease throughout his life; while still pursuing his education dreams. He is attending his regular clinic visits at Muhimbili National Hospital (MNH).

His family has had a share of the ordeal. Yusuph’s father, Mr Waigama Gesase, a resident of Ubungo suburb in Kinondoni District tells Your Health that two of his five children have died of SCD.

“Out of the three surviving children, only one is free from the disease. It’s Yusuph and his sister who are living with SCD today,’’ he says during an interview in Dar es Salaam.

When the family realised that their children were being diagnosed with SCD, screening for the disease was not a priority to them. Little did Mr Gesase and his wife know that they were “carrying sickle cell genes.”

“At first, my family didn’t bother to test for sickle cell traits. We didn’t see the reason to do so because in our family background, we couldn’t recall any of our parents or relatives who had SCD,’’ he says.

“Doctors told us that a child is born with sickle cell disease because of the genes carried by both parents. This gave us confidence to go and test. It’s then that we realised that we had the sickle cell traits.”

“I thank God that all my three surviving children have never blamed us [the parents] for passing over the sickle cell genes to them,” he says as he banks all his hopes on Yusuph who is defying all the odds to beat off the challenges of living with SCD.

“This scenario has also taught us many lessons. Families need to be thoroughly screened for sickle cell traits, and if possible before marriage,” suggests Mr Gesase

“Whenever Yusuph is going through any painful crisis, he keeps giving us hope that we, his parents, should not worry about his sickness. He usually tells us that he would get better,” says Mr Gesase as he praises his son for the initiatives he has put up to raise awareness on SCD in various communities in Tanzania.

As Yusuph climbs his education ladders, he believes SCD should never be the reason for people to lose hope and their dreams of attaining higher education.

He did his ordinary level Secondary education at Marian Boys Boarding School in Dar es Salaam. He recalls that when he was in Form One and Two, everything went well in his school routines. However, when he reached Form Three, his elder sister died of SCD. That was in 2013.

“After losing my sister, I got stressed. I started experiencing painful crises and somehow failed to cope. I could experience stomach pains and swollen legs.” This scenario affected his school routines. “It’s here that I began being an on-and-off student in school,” says Yusuph.

He tells Your Health that at one moment, he would be two weeks in school and other two weeks admitted in the hospital or staying at home for supportive care. At one moment, he says sadly, that he was admitted about four times in eight months.

He further narrates that the year that his sister died was a very difficult one for him. “I could not concentrate on my studies. I always thought it was my turn to die,’’ he recalls the sad moments.

“Although the school where I was studying was good and the teachers understood my situation, I could not handle the situation. It reached a point where some family members started convincing my parents that I should be removed from a boarding school,” says Yusuph.

In this situation, both his parents and Yusuph agreed on one thing: That Yusuph should not be transferred from his usual school, instead, he should continue studying at the same school.

Yusuph says he also believed it was the right thing for him because through boarding school, he become stronger studied harder and finally, he passed with a division one in his Form Four national exams.

Later, when Yusuph was selected to join Kibaha Secondary School for advanced level education, the fear to die of sickle cell began haunting him. He was frequently experiencing painful crises.

However, one day, while at home, he stumbled on a book in a shelf. It was about sickle cell disease.

In the course of reading, he says, on sentence appeared shocking. It said, “…thousands of children die of sickle cell worldwide before celebrating their fifth birthday and most of deaths occur in Africa.”

According to him, the journal also talked about how governments in Africa were not taking the problem seriously.

He says that most statements made in the book were by people from Europe and America. They explained that 90 per cent of children with sickle cell die every year and only 10 per cent of survive from sickle cell.

“This made me thank God having survived and celebrated my fifth birthday. This pushed me to realize that I could do some essential things on sickle cell disease to help other people suffering from the same condition, “he said.

Upon finishing reading the book, he decided to share his idea of becoming a sickle cell ambassador with the doctor who was treating him. He says that his doctor supported the idea. The doctor advised him to involve other people in the prospective project by then.

Following his doctors’ advice he tried his avenues and was able to meet the Chief Executive Officer (CEO) of the Tanzania Sickle Cell Disease Alliance; to request for support. Fortunately, says Yusuph, the CEO got interested and promised to support.

Yusuph and his fellow students now formed a sickle cell foundation at Kibaha Secondary School. They now provide public health education about the disease during morning speeches at school.

He says that when he formed the foundation, he started with only 30 ambassadors who were writing feature stories on sickle cell, but currently they have extended to other 17 secondary schools in Tanzania—with more than 400 ambassadors giving awareness on sickle cell countrywide.

“Currently, children die before their fifth birthday because some parents have wrong perception about the disease instead of taking the children for early interventions. We have found out that some parents opt to take the children to traditional healers,’’ says Yusuph.

Globally, statistics show that 312,307 children are born with sickle cell every year, however, 76 per cent of children are born in Africa and this indicates that 237,253 are born with sickle cell every year, according to the World Health Organization (WHO).

However, Tanzania has one of the highest annual births of Sickle Cell Disease (SCD), data further shows. Without interventions, up to 90 per cent of affected children are predicted not to survive beyond childhood, experts warn.

Interventions such as newborn screening and enrolment into comprehensive care programmes with prompt diagnosis and treatment of complications and prevention of infections by vaccination and oral penicillin has had remarkable impact on improving survival in SCD patients in Europe and the Americas.

Unfortunately, the introduction of interventions in Africa has been hampered by lack of research evidence.

Currently, Muhimbili University of Health and Allied Sciences (Muhas) has developed a framework for research, which is integrated into healthcare provision with development of policies to improve healthcare.

The government in collaboration with sickle cell stakeholders recently launched a sickle scan diagnostic rapid test which helps to detect the disease in three to five minutes.

Medomix Business Development Director Bob Kabugi said that the new invention would help to detect sickle cell diseases (SCD) immediately the baby is born.

“In the past, the available methods of diagnosis of SDC were expensive, time consuming, difficult to interpret results and for one to take results it took long,” he said.

The new technology does not require electricity so it could be used in remote areas. It does not also require highly skilled personnel to interpret the results and it has a 99 per cent of specificity and sensitivity in detecting the disease.

In view of this, he said that sickle scan will revolutionalise the survival prospects for children born with SCD in resources-limited areas, who would not otherwise have celebrated their 5th birthday.

Deputy Minister for Health, Community Development, Gender, Elderly and Children Dr Hamis Kigwangalla says that in Tanzania about 8,000 to 11,000 children are born with sickle cell every year, among those children about 15 to 18 per cent are have the traits. Therefore, in Tanzania and other African countries, the survival rate for children with sickle cell is only 50 per cent to 10 per cent especially in Sub-Saharan Africa, adds Dr Kigwangala.

Tanzania Sickle Cell Disease Alliance Director General Dr Deogratius Soka tells Your Health that lack of adequate training for health practitioners to handle sickle cell patients was still a challenge in the country.

According to him, sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body.

People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

Since most of health practitioners fail to offer services according due to lack of enough training, he suggests that the government should establish a policy that insists teaching sickle cell in schools.

He said that currently, no school in the country teaches about sickle cell compared to other diseases.