Destigmatizing Sickle Cell Disease

0

On World Sickle Cell Awareness Day, June 19, 2018, the Nigerian show Your View hosted an interview with genetics professor Cyril Otoikhian, who asserted that “sickle cell is a death sentence,” hence the need to redirect energy and resources toward prevention. Professor Otoikhian went on to suggest that money invested by parents into the healthcare of children with Sickle Cell Disease (SCD) is a waste, drawing upon Biblical verses (“my people die for lack of knowledge,” Job 36:12) to invalidate individuals’ hopes for improved prognosis and quality of life.

Otoikhian’s argument—that trying to stop SCD is better than trying to manage it—is based on the false idea that, over time, White people excluded the SCD gene through careful choice of mates. He stated, “I think that was what the White did, which we did not get used to on time and that is why it was zoned to Africa, or Black race. But recent statistics have shown that across the whole world there are evidence of sickle cell [sic]”. Not only is this assertion misleading, it is also dangerous, as it racializes the already-existent stigma surrounding SCD.

SCD is a genetically inherited condition in which erythrocytes become sickle-shaped after oxygen release and often clump together blocking small blood vessels. Intermittent crises can occur as a result, which involves excruciating pain in various parts of the body. Individuals with SCD are often also plagued by opportunistic infections such as pneumococcal disease. In 2010, a study confirmed that sickle cell developed as a protective trait against malaria. The study highlighted that Black people, by means of historically living in regions with high malaria endemicity, had increased vulnerability regarding the sickle trait. According to this finding, Otoikhian’s argument that White people have historically better strategized to exclude the sickle trait holds no weight.

Otoikhian did raise two valid points. He emphasized the need for genetic counseling and the need to curb reliance on the hopes for a divine miracle cure. But then he swiftly reverted to his un-empathetic stance: “if we want to find a way out of sickle cell then we must drop sentiment.” He added, “somebody that is dying and you know that the person is dying you don’t need to give an empty hope to that person…I’m not that type of Scientist [sic].”

Otoikhian’s foundation’s mandate is simple: “don’t bring them to life.”

His Erikpe-Ijaiye Otoikhian (EO) Foundation is exploring an alternative solution for SCD prevention: enacting laws that punish parents with AS (carrier) or SS genotypes who produce a child with SCD. The foundation’s mandate is simple: “don’t bring them to life.”

In Otoikhian’s proposal that death is the only option for those with SCD, societal attitudes toward the condition (and Blackness, by extension) are speaking loudly and clearly. These attitudes have wide-reaching effects; research is beginning to suggest that such attitudes can significantly worsen outcomes for those living with the condition. According to Bulgin and Tanabe (2018), cultural stigma causes stress, depressive symptoms and anger, affects social interactions, and exacerbates pain and self-harming behaviours among individuals with SCD. While significant and much-needed, this research offered only one suggestion for de-stigmatizing SCD which was aimed at nurses recognizing their own biases. In the wake of Otoikhian’s comments, it’s evident that the lack of a strategy for SCD de-stigmatization needs to be addressed.

For this reason, I designed a survey completed by 24 individuals who have SCD (21) or carry it (3) about their experiences with stigma and their ideas toward improving cultural attitudes. The ethnicity of this cohort included 21 Black, two mixed race, and one Middle Eastern individual. Seventeen participants currently live in Nigeria, three in the UK, two in the USA, one in South Africa, and one in Bahrain.

As captured by the data above, SCD is a disease that primarily affects Black individuals. Incidentally, just over 70% of participants resided in Nigeria where Professor Otoikhian is based. With consent, respondents shared their specific symptoms and detailed experiences with stigma. Those with SCD stated that they regularly experience general pain and joint pain, fatigue, discomfort, and one case of stroke, while one of three carriers stated that they live with “a lot of knee pains.”

Over 66% reported stigma. One individual noted, “If being exempted from getting involved in something I felt I could have partook in without falling sick is a stigma, then yes.” Interestingly, one mixed race carrier noted facing stigma “in a secondary sense” that was directed towards their mother who was told that “sickle cell and its related conditions were…a ‘Black’ disease.” This case highlights how incidence of chronic disease can be weaponized against Blackness.

Amongst the 16 respondents who indicated they had faced stigma, certain themes prevailed:

Childhood bullying: Peers regarded the child with SCD as a “witch,” and the child was told that they would die early.

Discrimination when accessing healthcare: Some respondents noted confusion in medical staff regarding SCD, while others faced abuse from medical staff. One noted that staff thought “my pain isn’t as bad or that I’m there for the drugs.”

Workplace discrimination: Participants reported that they are excluded from particular activities because of being viewed as fragile. One reported, “I always feel worthless and underutilized…it’s really hurtful sometimes and stigmatizing. I always feel unwanted. Eventually I was sacked because of it and I’m discouraged to look for another job for fear of the same.” Another respondent noted, “Being exempted in an obvious way from certain workplace activities and subsequently being laid off from work for not being part of said activities, and other little mistakes where a query would have sufficed.”

Awkwardness in romantic relationships: One respondent reports being told by a partner that “(SCD) was long, tiresome, and something he didn’t want to deal with.”

Verbal abuse in different settings: The highly problematic and derogatory term “Sickler” is often used to address people living with SCD. Also, participants reported being told that they “would soon die” because people with SCD “don’t last.” They observe being branded as “lazy” or “fragile.”

The second part of this survey, on de-stigmatisation, was intended to host an online brainstorming session of sorts. All respondents quoted here gave their consent to be named. The suggestions made by the respondents fell into four themes:

World Health Organization (WHO) petition: Respondent Zulaha Hadejia suggested that community members “Get a petition with as many signatures as possible to the WHO banning the word “Sickler” and replacing it with the often-used “Warrior.”

SCD-specific awareness campaigns: According to participants, awareness campaigns could include SCD focus groups and seminars held in health centers where the general public is welcome. People need to know that Warriors can live just as long as those without the condition with good health management. They also need to know that SCD is non-communicable.

Mo Babawale offered, “I believe if the public understands how and what we feel, stigma can be reduced. Media, TV precisely i.e. movies mostly portray people living with SCD as weak…If society can change the narrative surrounding us, and portray us as passionate and intelligent, a lot of us will be more confident in ourselves, and be willing to put ourselves out there.” One way in which awareness can be raised through media is to include a character with SCD in a children’s TV program, much like the HIV-positive character Kami, who was included in Takalani Sesame (the South African version of Sesame Street).

Positive Role of employers: SCD can be a physical disability that, much like other chronic diseases, should not be used to discriminate against anyone in the workplace or elsewhere. Employers need to understand that people living with SCD are capable of deciding which tasks their bodies can handle. Maryam Awaisu recalled, “As a kid, stigma was in the form of people deciding for me what I automatically couldn’t do.” Alagbu Bagzton suggested that openness between employers and employees might promote confidence in the employee when taking sick days during crises without the fear of being viewed as lazy, which could have downstream consequences on employment.

Genetic counseling: One respondent wishes that people knew “that though SCD is avoidable, people shouldn’t be judged for their choices (of a partner).” Another respondent is of the opinion that we should have both aggressive grassroots and nationwide sensitization awareness campaigns for intending couples and their counselors (who are mostly religious leaders in the Nigerian context) on the implications of the AS and SS genotypes having children.

Poignantly, Uchenna Stella Kingsley-Francis emphasizes that “everyone dies…it isn’t how long [one lives], but how well.” Contrary to what Otoikhian claims and what wider society may believe, those living with SCD can live rich and meaningful lives.  

Photo Credit: ariyo olasunkanmi/Shutterstock.com

Share.

About Author

Furaha Asani is a postdoctoral Research Associate at the University of Leicester, having obtained a PhD in the field of Immunology. While her career has focused heavily on research she is a passionate writer, focusing on issues surrounding healthcare, higher education, and pop culture in science, all with the theme of equality and equity. Furaha also dabbles in creative writing. Her aim is to create engaging content that evokes interest in a non-specialist audience.

Comments are closed.

Subscribe Today

Join our mailing list to receive the latest news and updates from our team.

You have Successfully Subscribed!