Sickle Cell Disease: Background information when reading A Kind of Madness

Sickle Cell Disease

This article relates to A Kind of Madness

In the story "Milk and Oil" from Uche Okonkwo's collection A Kind of Madness, Soty, a girl befriended by the main character Chekwube, has sickle cell disease. This fact is revealed to Chekwube slowly through certain habits and rituals that seem part of a foreign and sometimes strangely privileged world: Soty avoids the sun, drinks a glass of milk every day, and receives oil massages at home.

Sickle cell disease (SCD) is an inherited blood disorder affecting the hemoglobin, the protein in red blood cells. The hemoglobin of people with SCD causes cells to be formed in a way that prevents them from effectively carrying oxygen to tissues. This results in the blockage of blood vessels and the movement of blood, and can cause a number of symptoms and complications. These include anemia ("sickle cell anemia," sometimes used interchangeably with SCD, refers to a common manifestation of the disorder); pain from blockages known as "pain crisis"; eye, kidney, and bone damage; acute chest syndrome (which resembles pneumonia); and stroke. While gene therapies that may be able to cure SCD are beginning to become a possibility for some, it is still generally considered a lifelong condition and treatment is usually focused on alleviating pain and preventing fatal complications.

SCD disproportionately affects people of African descent and is most common in sub-Saharan Africa. Nigeria, where Okonkwo's story takes place, is the country with the highest reported rate of SCD; 2 to 3% of the population is affected, and 25% carry sickle cell trait, a single copy of the inherited gene that causes the disorder. To be born with the disease, a person needs to inherit one of these genes from both parents, meaning that people with sickle cell trait do not have SCD but can pass it on if they have a child with someone else with the gene. Navigating the possibility of giving birth to a child with SCD has become a fraught and complex part of Nigerian life. It is often a topic of dicussion before dating or marriage, and those with sickle cell disease or trait face discrimination.

Meanwhile, many Black people with SCD in countries where they experience systemic racism must deal with a painful condition in a society and healthcare system that subjects them to life-threatening disparities. In the United States, around 100,000 people have SCD (out of 8 million cases worldwide), with Black people accounting for over 90%. Sickle cell patients frequently need treatment for pain episodes, and many Black Americans with the condition (along with many Black Americans in general), report being disbelieved about their level of pain or need for medication. This results in them being less likely to go to the emergency room during episodes, which leads to delays in necessary medical intervention that increase the likelihood of organ and tissue damage, shortening life expectancy. SCD is also a condition that can put people at higher risk for complications from Covid-19. This makes it one among many factors in how Black Americans have and continue to be disproportionately affected by the virus and obstacles to protections from it, such as proposed and existing anti-mask legislation in an atmosphere where disabled and health-conscious Black people fear being targeted by police.

The antiblack and eugenicist precedents behind existing inequities like these raise questions about access to emerging therapies for curing SCD, which make use of gene-editing technologies. While these therapies look promising, they are currently extremely expensive, and racism in healthcare often affects who can receive effective treatment beyond economic factors, as those who have historically been abused and put in danger by medical experimentation have good reason to distrust it.

In an op-ed for Sapiens, Nigerian medical anthropologist Morenike Samuel suggests that the therapies now available in the US are unlikely to provide relief to Africans with the disease. She shifts the focus towards improving social conditions, writing, "Sickle cell anemia cannot be eliminated with state-of-the-art medical treatments only available to the privileged. The burden of disease shouldn't be borne alone by patients and their loved ones. Government and communities have a responsibility to make living with sickle cell bearable—even convenient."

A banana-shaped sickle cell among normal red blood cells, courtesy of Anatomy and Physiology, Connextions, CC BY 3.0 DEED

Filed under Medicine, Science and Tech

This article relates to A Kind of Madness. It first ran in the June 5, 2024 issue of BookBrowse Recommends.