SCD is a severe, inherited hematological disease.
Sickle cell (SC) is named after a C-shaped farming tool called a “sickle,” with a curved, sharp edge for cutting wheat, reflecting the shape of the affected cells.
When the cells become sickle-shaped, this is called “sickling.”
A person with sickle cell disease (SCD) will have symptoms; other people have sickle cell trait (SCT). They will have no symptoms but be carriers of the disease.
The symptoms range from being mildly affected and largely free from pain, to frequent and severe pain. Until now, doctors cannot predict who will be severely affected.
Although the SCT carrier is not affected in everyday life, the presence of SCT must be considered when individuals are planning to have children.
History of SCD awareness
The sickle cell shape was first noted in patients in the US in 1910, although the condition had existed in Africa for at least 5,000 years. The pain was then understood to be caused by blockage in the blood vessels. In 1949, SCD was found to be caused by abnormal hemoglobin, referred to as “hemoglobin S.”
SCD was one of the first diseases to be understood fully at the biochemical level, when researchers learned that the abnormal hemoglobin was changing shape due to a single amino acid error in hemoglobin S.
In the early 1970s, SCD was already of some interest to the biomedical community, but there was little practical help for those afflicted. Prominent in textbooks, it was virtually absent in the consciousness of US health care professionals.
Despite early discovery of the underlying molecular cause of the disease, progress in patient care remained slow, partly due to the difficulty of treating it, and partly for social reasons.
Civil rights movement raises awareness
In the US, SC mostly affects black people, for whom health research and treatment were long neglected.
In the 1960s-70s, a civil rights journal noted that 50,000 black people had SCD and 2 million had SCT. They pointed out that money raised for SC in the US was far less than for, say, cystic fibrosis, which mainly affects white populations.
The civil rights movement brought a better chance of treatment for people with SCD.
In 1970, the Black Panther group claimed that neglect of SC by white, middle-class scientists, doctors and government bureaucrats amounted to genocide by white America against its black population.
They announced the creation of a free testing program and a research foundation, with the aim of finding a cure.
In the summer of 1971, President Richard M. Nixon reacted by announcing that he would request $6 million from Congress to combat the disease.
As the need for improved treatment became recognized, the Sickle Cell Disease Association of America was founded, which later helped establish the Sickle Cell Anemia Control Act of 1972. This allotted government health funds for screening, research and treatment programs.
In 1983, the federal government recognized National Sickle Cell Awareness Month, now held annually in September.
Stigma of SCD
Despite progress since the 1970s, stigmatization of patients with SCD has continued, due to multiple challenges, not only socioeconomic, but also because of the difficulties of managing the chronic illness while accessing and navigating the health care system.
In 2010, Coretta Jenerette and colleagues investigated the stigma faced by young adults in the US with SCD.
The authors define health-related stigma as a form of devaluation, judgment or social disqualification of individuals, based on a health-related condition, resulting in discrimination in work, at schools and within families. It impacts all aspects of life, whether physiological, psychological or social.
Since people with SCD often come from disadvantaged backgrounds, even timely, quality health care may be restricted. For example, there are reports of physicians withholding pain control treatment, due to suspicions of drug abuse. The impact of the stigma can be lifelong, instilling a sense of exile from the healthier world and disgrace due to health encounters and lack of support.
The researchers called for more interventions to tackle this problem.
Myths and misconceptions about SC
The Sickle Cell Association of Ontario, Canada, lists a number of myths and misconceptions that contribute to the stigmatization of people with SCD. We will look at some of them.
Myth #1: SCD affects only Africans and their descendants
SC affects millions of people worldwide, not only Africans. Some 60-80% of people affected in the US are estimated to be black, but other races are affected.
Fast facts about SC prevalence
- The exact number of people living with SC in the US is unknown
- The CDC put the figure at around 3 million, with many unaware of their status
- SCD is believed to affect 90,000-100,000 Americans.
People whose ancestors came from sub-Saharan Africa are more likely have it, and it occurs among Hispanics, Asians, Indians and people of Mediterranean and Middle Eastern descent.
Interestingly, the Centers for Disease Control and Prevention (CDC) note that SC is more common among people from places with high malaria prevalence; people with SCT are believed to be less likely to develop severe forms of malaria.
SCD is thought to affect 1 in 500 black people and 1 in 36,000 Hispanic-Americans. SCT could be present in 1 in 12 black people.
The CDC and National Institutes of Health (NIH), together with seven states, are now cooperating on a Registry and Surveillance System for Hemoglobinopathies (RuSH) to establish how many people are living with SC and how the disease impacts their health.
On the next page, we look at ways of dealing with and overcoming sickle cell.