Having conquered her 21 years deadline, Regina Atatsi is among the millions of people around the world living with Sickle Cell Disease (SCD).
Her dream of becoming an athlete has been aborted because of the severe chest pains she suffers anytime she tries running.
Battling the disease for 30 years, Ms Atatsi, who discovered her ailment at an early age, has had to live on folic acids and multivitamins until recently when she was also put on hydroxyurea, a medicine used to treat SCD.
Her ordeal began at age nine when she fell ill, was rushed to a private hospital and immediately referred to the Korle Bu Teaching Hospital, where she was diagnosed with the disease.
According to her, the disease is the worse pain anyone could ever experience. “When I have sickle cell crisis, there is nothing I can do on my own and this makes it even more painful because you have to depend on relatives, friends and nurses to help you get around,” she said.
Ms Atatsi had her own fair share of disapprovals and discriminations including death “predictions” as a result of her condition but her faith factor has kept her going.
Despite these pressures and her ordeal, she is a SCD warrior, married with two kids (both AS) and has plans to begin her own business soon.
Ms Atatsi also firmly believes that SCD is not a death sentence as dreams could still be achieved, “just persevere.”
Dr Mary Ansong, Founder, International Sickle Cell Centre (ISCC) happens to be a carrier of the sickle cell trait and has lost two loved ones, a relative and a friend from SCD.
Speaking in an interview with this reporter last Wednesday in Accra, she explained that SCD is an inherited blood disorder that one is born with, adding that everyone inherits two sets of hemoglobin genes, one from the mother and another from the father and that gave the red blood cells (RBCs) the ability to carry oxygen to different parts of the body.
However, in SCD, Dr Ansong said the RBCs, which normally have a round, flexible, doughnut shape become banana shaped. The sickle-shaped red blood cells are therefore hard and sticky, and this blocks blood flow in tiny blood vessels.
She stated that this results in pain (which can be very severe) as well as other complications like stroke, visual disturbances or even blindness, and damage to affected body organs like the spleen, liver, heart, lungs and kidney.
Dr Ansong also noted that SCD is caused by a slight change, called a point-mutation, in the beta-globin gene which is found in hemoglobin.
According to Verywell Health, a health propagation brand, the most common form of SCD is called hemoglobin SS. The other major types are: hemoglobin SC, sickle beta zero thalassemia, and sickle beta plus thalassemia.
Hemoglobin SS and sickle beta zero thalassemia are the most severe forms of sickle-cell disease and are sometimes referred to as sickle cell anemia. Hemoglobin SC disease is considered moderate and in general, sickle beta plus thalassemia is the mildest form of sickle-cell disease.
Signs of SCD include pain, fatigue, infection and stroke while treatment includes blood transfusions, penicillin, hydroxyurea, bone marrow transplantation and immunisations.
The Centers for Disease Control and Prevention indicates that SCD affects approximately 100,000 Americans. It says that SCD occurs among about one out of every 365 Black or African-American births and occurs among about one out of every 16,300 Hispanic-American births. Also, about one in 13 Black or African-American babies is born with sickle cell trait (SCT).
SCD is a major public health problem with over 200,000 babies born per year with the disease in Africa. Approximately 80 per cent of all children born with SCD are in sub-Saharan Africa.
In Ghana, the United States National Library of Medicine states that SCD is a significant public health burden with two per cent (about 15,000) of newborns affected by SCD; one in three Ghanaians has the hemoglobin S and/or C gene.
The Sickle Cell World Assessment Survey (SWAY) surveyed patients on the impact of the disease on their daily lives. A total of 2,145 patients from 16 countries completed the survey from April 3 to October 4, 2019. The survey included patients from high-income (HI) and low-middle-income (LMI) countries, and used a seven-point Likert scale, with scores of five, six, or seven indicating high impact or high severity.
Of the participants surveyed, the majority indicated that SCD affected their emotional wellbeing (60%). Many patients also reported feeling frustrated with putting up with symptoms (58%), and worries about worsening disease (58%). In addition, the majority of patients reported avoidance of intense physical activity (62%).
A total of 53% of patients felt that SCD limited them to certain careers, 44% reported that SCD has prevented them from attending work, and 46% reported that SCD reduced attendance at school.
Fatigue, bone aches, and headaches were the three most commonly reported symptoms (41%, 38%, and 25%, respectively). Patients who experienced these symptoms were more likely to respond that the disease had a high impact on their lives or wellbeing. Fatigue was the most common symptom in patients before the survey (65%).
Dr Ansong indicated that SCD is prevalent in the country, with one in three Ghanaians being carriers of the AS or AC genotype and 18,000 children born this year with the disease.
Unfortunately, she said many parents only discovered their sickle cell genotype after the birth of a precious child with the condition.
“Nonetheless, SCD is not a death sentence. With early diagnosis, treatment, education and a supportive community, people living with the condition can enjoy full and healthy lives,” she added.
As such, Dr Ansong advised that having Hb electrophoresis test done was the first step in knowing one’s sickle cell genotype. The second step is to seek genetic counselling to provide you the needed information about the various reproductive choices you can make to protect the health of your future children.
She emphasised that one could achieve his or her dreams and aspirations once they understood the disease, the triggers of pain episodes and how their body handles certain stressors.
“This helps you put the necessary checks in place to avoid the triggers of a sickle cell pain episode. Having a supportive community that love you and are equally knowledgeable about the condition also helps a lot.
Dr Ansong emphasised that unlike before the 1990s, early diagnosis and the right medical care now allowed people living with SCD to survive into adulthood, adding that the oldest patient she knew in the country living with the disease was 98 years old, “other patients are in their 60s, 70s, 80s and 90s.”
She underscored that healthcare disparities and a lack of education led to the death of her relative and friend from SCD, as such, she encouraged all Ghanaians to educate themselves on the condition.
Dr Ansong also used the opportunity to appeal to well-meaning individuals and organisations to pay more attention to SCD to help the millions of lives that are affected directly and indirectly by the condition.
BY ABIGAIL ARTHUR