- “I have learnt that if SCD is detected and diagnosed early, especially if that’s done at a very early stage in newborn children, it has better outcomes in patients with the disease,’’ he tells Your Health in an interview.
Through his experience in taking care of patients with Sickle Cell Disease (SCD) over the years, Dr Deogratius Soka, a specialist, says he has dealt with cases of people who report to the hospital with irreversible, and at times life-threatening complications—a situation he believes could have been avoided through New Born Screening (NBS).
“I have learnt that if SCD is detected and diagnosed early, especially if that’s done at a very early stage in newborn children, it has better outcomes in patients with the disease,’’ he tells Your Health in an interview.
SCD means a group of blood disorders which are inherited from a person’s parents. The most common being sickle-cell anaemia (SCA); which results in an abnormality in the oxygen-carrying protein haemoglobin (hemoglobin S) found in red blood cell.
Dr Soka’s recommendation points to the medical benefit of early detection of the disease, yet there is a social aspect—hidden in real life stories of people who have lived with SCD for many years.
For Ms Arafa Salim, 30, a resident of Dar es Salaam who is living with the disease, knowing the genetic status in early stages—or even at adult stage—comes with additional benefits later in life.
She believes that knowing the diagnosis and keeping the records of one’s genetic status on sickle cell, helps to keep away the social dilemmas which could arise in times of marriages.
On top of enduring the pains and tribulations of living with the disease, she recalls her story with Your Health of how she had to choose between forming a bond and not passing her sickle cell gene to another generation.
She is the fourth born in a family of seven children. But in her entire family, she is the only one living with SCD. She was diagnosed with the disease at the age of eight months.
Ms Salim says she did not want to take chances when it came to marriage plans, given the lessons she had learnt through several years of living with disease.
Having learnt through doctors, that sickle cell can be passed on to the next generation through a family tree; Ms Salim had lost hope in marriage, at one moment in life. She believed that marrying a person with sickle cell traits meant that the sickle cell genotype would increase the chances of her giving birth to a child with SCD. However, she finally found a way out.
She decided to marry a man who had accepted to screen for Sickle Cell traits and later they had children.
How she played the screening cards
Experts say that for one to have a trait of sickle cell, this person must be having a defective oxygen-carrying protein in blood, known as Hemoglobin S. Hemoglobin A is not defective.
A person with traits AA has no chance of passing on the sickle cell disease. If this person has a child with someone who has sickle cell disease, then the offspring will have the trait.
But they must be taught on how to avoid passing the disease on to grandchildren.
But if the person has AS, that means they are carrying the trait of sickle cell. Here, this person has the potential of passing the disease to children in case this person mates with a person with a sickle cell patient (SS).
Here, there is a 50 chance that in every pregnancy the disease can be passed on.
This is how Ms Salim played her cards—being cautious, praying and seeking knowledge about this condition before she could go ahead to marry.
“Knowing your partner’s traits is very important, especially before tying the knots. As a person with sickle cell, I knew how to go about it. It helps partners to plan on how they can play with the probability of giving rise to children without sickle cell,’’ she says.
This, Ms Salim says, is the knowledge she has acquired through interacting with experts as she runs her initiative—the ‘Sickle Cell Disease Patients Community of Tanzania’.
She is spreading the message to others. “My career has been dictated by the desire to help others suffering from segregation because of the lack of knowledge about the disease and not by the disease itself,’’ she tells Your Health.
“If everyone knows their Sickle cell trait and they are willing to share the results, it would help partners to understand each other and avoid passing over the traits to further generations,’’ she says.
But for Dr Soka, if health facilities in Tanzania provided New Born Screening (NBS) services, the benefits would go beyond—to avert complications, disability and death.
He says that patients who end up at hospitals with complications of SCD which were not detected in the early stages are in that situation because their diagnosis was missed at lower level health facilities.
“The lack of NBS services in health facilities in Tanzania has created a big challenge because; only a few centers can give a definitive diagnosis of SCD,’’ he explains.
“As a result of the shortage of NBS services, most patients are diagnosed with SCD at a very late stage—when they are already facing complications such as stroke,’’ he explains further.
Extent of benefit from NBS
NBS has been shown to reduce deaths in children under the age of five by 70 per cent in high income countries but no country in Africa has introduced the screening services as a health intervention.
The disease is now a recognised cause of childhood mortality; such that without medical intervention; up to 90 per cent of the patients who are affected die in childhood.
Tanzania ranks high in Sickle Cell cases. Recent estimates suggest that 10,742 under 5 years of age SCD children die annually in the country, representing 6.6 per cent of overall under five deaths.
According to studies, the country has one of the highest numbers of annual births of SCD children (11,000), ranking 5th after Nigeria, DRC, India and Angola
Due to the ominous data and advocacy campaigns of stakeholders, the government has included SCD as a priority condition in the strategy for management of non-communicable diseases (NCDs), revealed Dr Soka, who is the Chief Executive Officer of Tanzania Sickle Cell Disease Alliance ( TANSCDA)
From the year 2015 to 2016, Muhimbili University of Health and Allied Sciences (Muhas), ran a new born screening program for SCD; funded by UKAid through the Human Development Innovation Fund (HDIF).
Through a technique known as isoelectric focusing (IEF), 4,002 newborns were screened at Muhimbili National Hospital (MNH) and Temeke District Hospital.
Findings showed that thirty one children, (0.8 per cent) with SCD (SS), 504 (12.6 per cent) with sickle cell trait (AS) as well as 27 with other blood disorders (0.7 per cent), and 3,440(85.9 per cent) were SCD negative (AA).
Dr Soka says that out of the 31 SCD newborns, 28 have been enrolled for comprehensive healthcare and the programme has established state-of-the art clinical research laboratory at MUHAS.
He believes this programme is clear proof that introducing NBS in Tanzania is feasible and would greatly contribute to the health of children and adults later in life.