Sickle Cell Disease: Warrior tells his story of resilience

When Dancan Ekaya was born, his parents, Rukia Achieng and Richard Simiyu noticed some worrying symptoms in their child.

According to Dancan, his parents noticed that he was not only crying a lot, his body and limbs also seemed swollen.

These symptoms, according to Children Sickle Cell Foundation (CSCF) Chairperson Selina Ogweno are the early signs of the disease in infants, along with frequent infection, yellowing of the eyes (jaundice) and the most significant symptom which almost every patient experiences is periodic episodes of extreme pain.

Finally, in 2001, when he was three months old, Dancan was diagnosed with sickle cell anaemia, one of a group of inherited disorders known as sickle cell disease.

"My mother did not believe this at first. She was reluctant to accept the reality during the first hospital visit so she took me to another hospital where she was given the same information. From there, my mother accepted the diagnosis, comforting herself that indeed everything happens for a reason," Dancan narrates.

"I am the only child with sickle cell anaemia in a seven-member family and it is now 22 years later," says Dancan who is now a third-year Communication and Media student at Rongo University.

According to the Centers for Disease Control (CDC), Sickle Cell Anaemia develops when one's body makes an abnormal form of the protein (haemoglobin) in the blood that carries oxygen.

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The abnormal haemoglobin (HbS), causes red blood cells to change their crescent structure and become like sickles, they clump together and blood vessels cause pain and damage to tissues.

Sickle cell disease, according to the CDC, is a genetic condition that is present at birth. It is inherited when a child receives two genes-one from each parent - that code for abnormal haemoglobin.

In Kenya, according to World Health Organization (WHO), 14,000 children are born with sickle cell every year and, on a global scale, over 300,000 children are born with it.

Varying experiences

In the absence of routine newborn screening and appropriate treatment, an estimated 50-90 per cent of those born with the condition die undiagnosed before their fifth birthday in Sub-Saharan Africa, according to the Ministry of Health. Therefore, early diagnosis and treatment are important and a person with the disease can live a full, active life.

The symptoms and complications of SCD are different for each person and can range from mild to severe, and its management is focused on preventing and treating pain episodes and other complications.

According to Ebrahim Adamjee, the Medical Head of Specialty Medicines and Programmes in Sub-Saharan Africa at Novartis, SCD symptoms can be disruptive.

For example, it can cause swelling in the hands and feet, which is caused by sickle-shaped red blood cells blocking blood circulation. People with the disease can also experience damage to organs such as the spleen, increasing an individual's vulnerability to infections.

As a result, infants and children with SCD commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections like pneumonia.

In addition to the above symptoms, SCD can lead to delayed growth or puberty because red blood cells provide the body with oxygen and nutrients necessary for growth, and a shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.

Vision problems can also occur, as sickle cells can plug the tiny blood vessels that supply the eyes, damaging the retina and causing vision problems.

Living with SCD

Duncan says living with the disease has not been easy. His education, for one, has been full of interruptions.

"From my early years in school, nursery to class four, my school life was on and off due to this condition. There was a time I stayed in the hospital for two months. The hospital became my home. Another time in 2010 when I was in Class Four, I missed a full year of school. The result was poor grades and I had to repeat Class Four the following year," he says.

He says that he didn't really understand what sickle cell was until Form Four when he learnt about Genetics in Biology class.

Dancan says that pain from a crisis can occur in any part of his body but most commonly the chest, back, joints, arms and legs. These crises have been difficult moments for him and this mostly happens in the evening hours.

Dancan is one of many people with sickle cell disease who have been fighting and standing up against stigma.

He recalls as if it were yesterday when a close relative made hurtful statements to his mother that Duncan should die while still young because he wrongly believed that no single SCD patient could live more than 18 years.

"As long as you follow the doctor's advice and take drugs as recommended, you will live a normal life. The deeply entrenched notion that the disease is akin to a death sentence has been the biggest challenge in the society," Duncan says.

"Here in school some friends who I expected would feel sympathy were in the forefront in making my life miserable. When I complain about pains, whether I'm in school or elsewhere, instead of people helping me to sort out the issue, some say that I'm faking, others say it is as a curse or a result of witchcraft," Duncan says.

"But I appreciate the few who have been standing with me, especially whenever I'm in a pain crisis. A big shout out to them!" he says adding that his parents and some of his friends and coursemates have been a big help since they understand his condition.

Duncan says he is unable to attend lectures when he is going through a pain crisis.

"I thank God I have never missed any cat or examination because of my condition," he says.

Managing the condition, he says, is also expensive.

"My parents are not employed but they are forced to take care of me and other siblings who need the same attention. Reality hits me hard when no money is unavailable to even buy drugs and food," Dancan says.

"One tablet, for example, hydroxyurea, costs at Sh50 and, according to doctors' prescriptions, you are supposed to take two tablets a day. When multiplied by 7 days a week, I tell you that's very expensive keeping in mind there are other drugs to buy," he says.

"The condition is indeed a big problem, especially to poor households, because the patients need regular check-ups, alongside the expensive drugs," Dancan says. "This is why many SCD patients are urging the government and other stakeholders to make it easier for them to access the drug."

Indeed, according to Vincent Owino, a medical doctor, it takes Sh3,000 - 3,500 a month to put a patient on medication and that is beyond the reach of most vulnerable families.

Dancun says that in addition to providing free or subsidised drugs, the government should create awareness about the disease to eliminate the myths and stigma associated with it.

"Society should take this condition as a genetic one . The perennial myths and misconceptions surrounding the disease should be done away with. Also, let fellow patients follow the doctors' rules,.They should stick to drugs and avoid possible triggers and they need to accept they are suffering after they are positively diagnosed. Let us support people living with sickle cell disease so that, in future, we can have warriors like me," Dancan says.

Change needed

Dancan urges the government to create more SCD clinics, especially in the prone areas like Coastal, Nyanza and Western regions.

"You will find that there are few SCD clinics in these counties, and where they are available, not enough has been done to educate and sensitise the staff in the diagnosis and care of patients," Dancan says.

CSCF Chairperson Ms Ogweno says that, in order to silence this genetic condition, genetic counseling to couples who are about to get married should be done and health facilities should be equipped in order to manage SCD.

"Healthcare workers should also be regularly trained on new trends of managing it and newborn screening should be availed to all public facilities," she says.

Novartis's Mr Adamjee says that the establishment of facilities with relevant experts such as hemato-oncologists is vital and capacity building of multidisciplinary teams involved in the care of SCD patients in endemic areas is also essential.

"Setting up robust referral networks that can link health facilities to Sickle Cell Disease Centers of Excellence is crucial in ensuring that patients receive appropriate care, and education of the public to reduce stigma around sickle cell disease is necessary for the provision of adequate support for patients and their families," he says.

Dancan says that despite the challenges he has been facing, he felt inspired to stand up for many people living with SCD by creating awareness using various foundations where he is a member and advocate. These foundations include King Walubokho Foundation (KWF), Bungoma Sickle Cell Foundation, Warrar Shujaaz, and Sickle Cell Awareness Foundation (SCAF) Kenya.

Work to do

"I have had the chance to spread awareness on the disease and mentor others on how to take care of themselves while living with sickle cell disease," Duncan says. He adds that the organisations also conduct hospital and community visits where they demystified myths around this genetic condition.

"We coach and encourage patients suffering the same condition that, this is not the end of life. I'm 22 years now and I'm not going anywhere anytime soon. The main thing one can do to is to reduce the chances of experiencing painful episodes (sickle cell crises) by avoiding possible triggers. Drink plenty of fluids, wear warm clothing and avoid sudden temperature changes such as swimming in the cold," Dancan says.

"I also ensure I include all food groups in every meal even though it's sometimes expensive to maintain, keeping in mind I'm still a student," he says.

"What I can say is that having this genetic condition is not a death sentence. Let patients live normal life. I have passed through all these challenges but I am still alive and strong. My education and career are on track. I am a good writer and I do media production. If I had given up from the early days, I would be nowhere. I am proud to be known as an SCD warrior," Dancan says.