When a 26-year-old man declared he would die in hospital, it was difficult to believe. There was no gunshot wound, no visible signs of trauma, nor was there an apparatus attached to him indicating he was under critical care. However, like he predicted, 20 minutes later, after calling his mother, the father to a two-year-old took his last breath. He died in the arms of Kayla Mortimer, the new president of the Bahamas Sickle Cell Association’s (BSCA).
“I could not sleep for a week after that,” recalled Mrs Mortimer. “Every time I closed my eyes I would hear his voice, see his face.”
Since last September, 12 people, including that 26-year-old man, have died from the inherited red blood cell disorder known as sickle cell anaemia, haemoglobin S or SS disease, the association reported last week.
Sickle cell disease has robbed families of their youngest members, but in more common scenarios, the disease has taken a higher number of mothers from children, many of them toddlers, said the organisation, which has just over 200 members in New Providence, Grand Bahama, Abaco, Exuma and Eleuthera.
Most sickle cell warriors – the association’s name for its members – suffer from sickle cell crises. The pain can range from manageable to excruciating, brought on by misshapen red blood cells getting stuck in their blood vessels, blocking the life-sustaining fluid from reaching parts of the body. Over time it can result in bone and organ damage.
Still, even in the face of life-threatening conditions, some put off going to the emergency room, knowing that once there they could likely face another battle – convincing staff they are not drug addicts faking symptoms to score painkillers.
“Unfortunately, it seems there are some medical personnel who are not as knowledgeable about the disease and it shows in the level of care they provide,” said the association’s new vice president, Nadine Sawyer, whose daughter is afflicted with sickle beta plus thalassemia (SB+ thalassemia), a “mild” form of the sickle cell disease.
An inherited condition, the “plus” indicates that a patient’s blood has a below normal amount of haemoglobin. What is present is the abnormal haemoglobin called haemoglobin S (sickle haemoglobin). This differs from sickle beta 0 thalassemia, in which a person has no normal haemoglobin.
When she began making hospital visitations in January, Mrs Sawyer, who utilises private care for her daughter, came face to face with obstacles sickle cell warriors confront in a public health system setting – a lack of prompt patient care and challenges with the availability of all medication.
In battling a disease that gets complicated very quickly, every second counts. The eponymous sickle shaped blood cells do not deliver oxygen to organs and tissues like healthy blood cells do, which can cause serious damage over time.
While recognising the public health system has the mammoth task of caring for the uninsured and under-insured masses seeking medical services, the BCSA said healthcare practitioners must remain ever mindful of the fact that off-the-chart pain drives sicklers to seek emergency treatment in hospital.
Ideally, the BSCA would like to move the Bahamas closer to worldwide norms where sickle cell patients receive treatment within 20 minutes. In many cases, sicklers in crises wait hours for pain relief.
The association would also like to see more healthcare providers educated specifically on the disease, especially those on call in the Accident and Emergency Department.
“We have to raise awareness not only in the medical community, but also with teachers, employers and the general public at large that we live in pain every day, but we can live and be productive citizens,” said Mrs Mortimer, whose newly minted college graduate daughter is a sickler.
Treatment, she said, must be specialised for each warrior as no two sicklers’ experience is the same.
Also topping the new board’s agenda is the launch of a national registry to determine the number of individuals afflicted with the disease and carriers of the sickle cell trait.
“Sickle cell disease is not a death sentence. We have a 70-year-old warrior in Freeport,” said Mrs Mortimer. “Creating the right environment makes a difference.”