By Daniel Wako
Having a child is perceived as happiness to parents and the community at large but how sad and painful does it feel to parents to see their children cry in pain due to a crisis caused by a condition that cannot be treated? Those are the pains that can be explained by parents who have children living with sickle cell anemia.
While marking the sickle cell anemia awareness month of September in Kenya, Dr. Rashid Aman, Health Chief Administrative Secretary, noted that the disease is still a problem in the country and Africa at large with at least 240,000 children born with the disease in the continent every year.
In Kenya, the Ministry of Health estimates that 14,000 children are born with sickle cell disease (SCD) every year. Further, due to the absence of routine newborn screening and appropriate treatment, an estimated 50-90% of those born with the condition die undiagnosed before their fifth birthday in Sub-Saharan Africa.
The disease is common across Kenya, affecting 18 counties, with high disease burden pockets in Western, Nyanza, and Coastal regions.
According to data provided by the Ministry of Health while launching the new guidelines on SCD management in 2021, in the western region, it is estimated that as high as 18% of children are born with a sickle cell trait and 4.5% will end up developing SCD.
In the lake region, it is estimated that about 17% of children are carriers of the trait with 0.6% having SCD while in the coastal region, using inpatient data, almost 1% of inpatient children have SCD and are almost 20 times more likely to die compared to admissions of other morbidities.
Sophie Uyoga, SCD researcher at Kilifi Kenya Medical Training Research Institute (KEMRI) offices, explains the disease affects the shape of the red blood cells, which carry oxygen to all parts of the body. “Red blood cells are usually round and flexible, so they move easily through blood vessels. In sickle cell anemia, some red blood cells are shaped like sickles or crescent moons. These sickle cells also become rigid and sticky, which can slow or block blood flow in the body,” Uyoga said.
She explains that at least one percent of the total number of newborns are born with sickle cell anemia in Kilifi county every year. ” Kilifi county records between 300 to 400 new cases of sickle cell anemia among newborns. This represents one percent of the total number of newborns every year. This situation is almost similar in all counties in Kenya’s coastal region,” Uyoga notes.
According to her, the disease is caused by a change in the gene that tells the body to make the iron-rich compound in red blood cells called hemoglobin. Hemoglobin enables red blood cells to carry oxygen from the lungs to other parts of the body.
“The hemoglobin associated with sickle cell anemia causes red blood cells to become rigid, sticky, and misshapen but for a child to be affected, both mother and father must carry one copy of the sickle cell gene also known as carriers, and pass both copies of the altered form to the child,” Uyoga says.
“If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one typical hemoglobin gene and one altered form of the gene, people with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin. Their blood might contain some sickle cells, but they generally don’t have symptoms. They’re carriers of the disease, however, which means they can pass the gene to their children.”
According to Dr. Wilfred Masoso, KEMRI Kilifi, SCD is prevalent in coastal, Nyanza and western regions of Kenya, probably due to malaria prevalence in these regions. He notes that sickle cell carriers have an advantage against malaria infection but when sickle cell anemia patients get infected with malaria, they are at a high risk of losing a life.
“This is because Malaria affects the red blood cells and as you already know, a sickle cell anemia patient already has a problem with the red blood cells thus when diagnosed with malaria the chance of them dying is high, but if it happens we do have special drugs that we give them,” he says.
“Sickle cells can also block blood flow to an area of the brain. This results in a stroke that can be manifested with signs like seizures, weakness or numbness of the arms and legs, sudden speech difficulties, and loss of consciousness. The other health complications that can result from sickle cell anemia are; acute chest syndrome, organ damage, pulmonary hypertension, blindness, splenic sequestration, leg ulcers, deep vein thrombosis, gallstones and pregnancy complications.”
KEMRI Kilifi office hosts sickle cell anemia clinics that help improve the health of those who turn positive when tested for the disease. ” We do not have clear data on sickle cell anemia because most facilities in our counties are not able to do this testing and that’s why we have a hospital where we do testing as KEMRI. In the event we confirm a patient has sickle cell anemia we enroll them in sickle cell clinics where they are always in contact with doctors who check and advise them on their health,” Uyoga said.
The service provided during sickle cell anemia clinics includes; routine outpatient care, ongoing pain management, medication management to lessen symptoms, blood transfusions of healthy red blood cells to treat or prevent complications, wound care, education support, and genetic counseling.
Dr. Masoso confirms that attending sickle cell clinics is not only improving the health and well-being of sickle cell anemia patients but also the understanding of the parents and the community at large about this condition.
“These clinics are really helpful because when a patient attends this clinic he/ she is examined by the doctor who at the end gives advice on what to do if it is blood transfusion or treatment of other infections it will be done from here,” he said. “The health of patients who attend sickle cell clinics and those who do not cannot be compared at all.”
Another achievement of these clinics is the successful equipping of the society and specifically, the parents with sickle cell anemia, with true and reliable information about this condition whereas initially the community used to relate this disease with witchcraft which is not true, Dr. Masoso adds.
When sickle cell anemia patients get malaria infection their health deteriorates greatly with most of them losing their lives to the disease. During sickle cell anemia clinics these patients are highly monitored not to get malaria, they are given paldril and folic acid to boost their immunity against malaria infection.
“Malaria affects red blood cells and remember a sickle cell anemia patient has an existing problem with Red blood cells. This means if they get malaria they are at risk of losing their lives. During sickle cell clinics, we give them drugs to prevent serious malaria and any other infection,” said Dr. Uyoga.
“The service is free. Coast region is among areas with high numbers of malaria cases which is a threat to sickle cell anemia patients. We have saved many lives by reducing malaria infections among sickle cell anemia patients. We also do blood transfusion in cases where a patient has a low amount of blood.”
At Kilifi county referral hospital, pain crisis management is done for patients at a free cost. This is because pain is the most common complication of sickle cell anemia and a major reason for people with the disease to go to the emergency department or hospital.
Sickled cells traveling through small blood vessels can get stuck and block blood flow throughout the body, causing pain. A pain crisis (vaso-occlusive episode or VOE) can start suddenly, be mild to severe, and can last for any length of time. Pain can occur in any part of the body but commonly occurs in the hands, feet, chest, and back.
Pain that comes suddenly and lasts for a short time is referred to as acute pain while chronic pain is daily, continuous pain lasting more than 6 months. People with sickle cell anemia can experience acute pain, chronic pain, or both. Thus, they are given opioids to reduce pain.
Like any other program Uyoga points out the high costs of doing sickle cell anemia tests, hospitals lacking equipment for sickle cell testing and treatment, and patients being unable to pay for service as the main challenges facing sickle clinics.
“Challenges are there because putting up these clinics and running them requires a lot of money and that’s why most hospitals are unable. Also, most of our patients come from humble backgrounds such that they cannot pay for services thus we opted to do them for free,” she said.
Umi Salma is a mother whose happiness of having a baby was cut short after her son was diagnosed with sickle anemia at the early age of only two years. ” Like any mother, I received my son as a bundle of joy. He was a bouncing baby with good health but this was short-lived after he started getting sick every now and then,” she said, adding that it was not easy to identify the condition that was disturbing her son, as she had to move to various hospitals without the doctors identifying the problem.
” I took him to various hospitals but the health problem was not easily identified until I was advised to visit KEMRI hospital in Kilifi, where we took a sickle cell anemia test and the results turned positive. This marked the beginning of our endless visits to this facility,” said Salma.
Even though she was happy having identified the health problem that was disturbing her son, it was painful to note that the condition could not be treated completely. ” It was still painful because someone used to tell me he will not live above 20 years but after consulting the doctors I was educated with the right information and put in sickle cell clinic scheduled on every first Tuesday of the month,” Salma said.
“My son is alive because of attending sickle cell clinics here at KEMRI. I thank God for this place, they don’t charge us anything. Unlike the other facilities where sickle cell patients pay to be treated, here services are offered for free.”
On the other side, Mary Mbeu explained that her daughter started showing signs like swelling of her legs at age of six months and when she took her testing she was confirmed to have sickle cell anemia. ” My daughter Aisha got sick when she was only six months old and when I took her to Kilifi KEMRI hospital, a sickle cell anemia test was done and the results were positive.”
Now at two and a half years old, Mbeu says her daughter is living a normal life because of attending sickle cell clinics at KEMRI. ” We have been attending this clinic since she was confirmed sick and the results are good. Looking at her now, you cannot differentiate between her and other children.”
Among the challenges that Salma and Mbeu face in ensuring their children are healthy is the cost of making frequent visits to the clinic and the high prices of drugs given to sickle cell patients.
“The challenges are many but finance is the main problem. Traveling to this facility frequently or when our children are sick requires money. Again most of the drugs given to sickle cell patients like hydroxyurea are very expensive,” Mbeu said.
“Another challenge comes with feeding these children because they require special meals that are rich in vitamins and minerals. This is expensive to achieve.”