Health ministers from across Africa have committed to increasing awareness about sickle cell anaemia, a debilitating, inherited blood disorder that affects millions on the continent and is particularly prevalent among those of African heritage.
The decision to “ramp up awareness, bolster prevention and care to curb the toll of sickle cell disease” was made at the 72nd World Health Organisation (WHO) Regional Committee for Africa meeting, which took place last week.
According to the WHO, about “1 000 children are born with the disease every day in Africa, making it the most prevalent genetically-acquired disease in the region”.
“More than half of these children will die before they reach the age of five, usually from infection or severe anaemia.”
The WHO’s regional director for Africa, Dr Matshidiso Moet, said that more needed to be done on the continent to “improve access to treatment and care” for patients suffering from the disease.
“Most African countries do not have the necessary resources to provide comprehensive care for people with sickle cell disease despite the availability of proven cost-effective interventions for prevention, early diagnosis and management of this condition. We need to shine the spotlight on this disease and help improve the quality of life of those living with it,” said Moet.
The organisation estimates that of the 120 million people suffering from sickle cell disease worldwide, 66% are from Africa, with more than 300 000 babies being born with severe haemoglobin disorders each year as a result.
Dr Shahroch Nahrwar, a clinical haematologist at Melomed Tokai private hospital in Cape town, said sickle cell disease, also referred to as sickle cell anaemia, “is more common in the black population because it is most prevalent in Central Africa”.
“You inherit it from your parents. It is a red blood cell disease that causes an abnormal shape, which are called sickle cells … they have the shape of a sickle, and they are very stiff sickles which can obstruct blood flow and beyond that, obstruction. You can get a lack of blood flow and hypoxemia, which means a lack of oxygen, that causes pain which we call a vaso-occlusive painful crisis,” he said.
Nahrwar said the most common symptoms included but were not limited to pain in the arms, legs, ribs, chest abdomen and back.
“Children usually present at an early stage. They can get spleen problems, painful hands, usually because of dactylitis in which you get swollen, painful hands.”
Nahrwar said the disease could be cured through a stem cell transplant from a healthy donor to replace the bone marrow where the sickle cell is being produced.
Alternatively, genetic therapy can be used. This involves the removal of abnormal genes to replace them with normal ones. He cautioned that the approaches were “fairly experimental”, despite some successes having been achieved.
Mwenda Phiri, 20, has been living with haemoglobin SS, the most common type of sickle cell disease, since he was nine months old, a result of inheriting copies of haemoglobin S gene from both parents.
“I was diagnosed with sickle cell anaemia at just nine months old after I had persistent coughing, general weakness and unusually light-skin in infancy. I was taken to the hospital and after testing it was concluded that I had a chest infection and a mild urinary tract infection. However, what was most concerning was my particularly low haemoglobin levels at about six grams per decilitre compared with normal levels of 14.
“More testing was done to rule out conditions such as HIV, malaria and leukaemia and with this testing for sickle cell was ordered and I was then diagnosed with sickle cell anaemia.”
Phiri said that he experienced his first sickle cell “crisis” when he was three, which resulted in acute chest syndrome, a complication of the disease that causes chest pain, coughing, fever and low oxygen levels, among other symptoms.
“I had my first blood transfusion and spent a fortnight in a hospital, emerging fully recovered. From the age of three until 15 I have been able to deal with most of my crises without complication.
“In 2017, at the age of 15, I suffered from complications of an appendix removal, which led to a second dose of severe acute chest syndrome and I was admitted to the ICU for 12 days.
“Since then there have been more hospital trips due to complications such as dealing with pneumonia, stomach ulcers, infections and more. I have also had an autosplenectomy, which occurs when a disease damages the spleen to such an extent that it becomes shrunken and non-functional.”
He said that although taking medication had helped him manage the disease, maintaining a balanced lifestyle while learning to cope with stress was important in preventing another “crisis”.
“Sickle cell has many triggers to a crisis and dealing with them as you grow becomes very important. A huge trigger was dealing with stress. Stress negatively affects me most of the time and learning coping mechanisms such as breathing techniques, doing yoga, practising mindfulness, being organised and well planned can be essential, especially as you get older.
“Sickle cell being an invisible illness means that I cannot directly physically see the effect that it has on my body, which comes with the backlash of many misunderstandings, such as being branded lazy when you’re chronically fatigued, being looked at as a drug seeker when the intense pain is frequent and feeling so isolated and lonely when there is no one who understands what you are going through,” he said.