What’s in a name?

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Rightly or wrongly, stereotyping is engrained in physicians from when they start medical school. Consider the classic medical exam questions regarding a South Asian gentleman with a cough. Students are almost conditioned to start recalling their knowledge of tuberculosis before they have even finished reading the question. Stereotyping saves lives. Following Occam’s razor of common things being common means that a patient attending A&E with fever and hypotension is likely to receive potentially life-saving antibiotics even before rarer diagnoses have been excluded. However, one of the toughest skills of being a doctor is recognising when our conditioned stereotypes are hindering our ability to provide good care. 

Race and health are often linked. This might be to illustrate the varying prevalences of different diseases within different ethnic groups. Occasionally, treatment for a certain disease will vary by the patient’s ethnicity (such as choice of anti-hypertensive in the under 55s). Nevertheless, Covid-19 has demonstrated that not all generalisations based on race and disease are valid. In the first few weeks of the pandemic, when it was first realised that ethnic minorities in the UK and USA seemed to be much more likely to die from the disease, speculation regarding genetic susceptibility to the novel virus was rife. Yet ultimately, as is often the case, it appears that socio-economic factors, rather than genetics, explain these tragic differences. 

Yet sickle cell disease is one condition where there is undoubtedly a correlation between ethnicity and the likelihood of having the disease. Sickle cell disease (SCD) is caused by a mutation on the eleventh chromosome, affecting the gene that codes for one of the protein molecules in haemoglobin. The results of this mutation are abnormally ‘sickle-shaped’ red blood cells which cause an array of complications: from episodes of vaso-occlusive crisis (severe pain due to restricted blood flow to different parts of the body); strokes; anaemia and splenic infarction leading to infection susceptibility. The life expectancy of a baby born in the UK with sickle cell disease is less than 60 years. In order to have sickle cell disease, a child must inherit a mutated copy of the gene from each of their parents. Inheriting just one copy of the mutated gene has been shown to be protective against malarial infection. This selective advantage has meant that the sickle cell trait gene remains prevalent in areas of the world where malaria is endemic, most notably in Sub-Saharan Africa. Consequently, the vast majority of people with SCD are of African origin.

Until I began working as a doctor, I had only a biochemical view of sickle cell disease. I had learned about the genetics behind the disease and how these manifest as symptoms. However, as my medical school was in a predominantly white area of the UK, I encountered very few patients with SCD during my clinical placements. I did not regularly care for patients with sickle cell until I started to work in London, a couple of years after I had graduated from medical school. What struck me then was the negative attitude that some of my medical colleagues exhibited towards patients that we encountered with the disease. Patients with SCD most commonly presented to hospital complaining of severe pain due to vaso-occlusive crisis. Despite the clearly established pathology that explained their presentation, I found that many of my colleagues would question whether the patients were really in as much pain as they claimed. Strong analgesia would be prescribed reluctantly and often only after delay and discussion about ‘opioid seeking behaviour’. This is where I was first introduced to the term ‘sickler’.

A ‘sickler’ is a patient with sickle cell disease. It is often used in discussion between medical professionals as a faster way of saying ‘a patient with sickle cell disease’. Patients with SCD, like any other patient group with a chronic, incurable condition, can be difficult to manage. Seeing a patient return multiple times to A&E with the same complaint is frustrating for any clinician who takes satisfaction in curing their patient’s ailments. Sadly, SCD is something that cannot be ‘cured’. Patients have to live their lives knowing that the next painful vaso-occlusive crisis could occur at any moment. As is often the case with people who unfortunately have had to grow up in and out of hospital, a significant number of patients with SCD have behavioural or mental health issues that add to the challenges of managing their condition. Yet I did not see doctors show the same level of frustration towards patients suffering from other chronic conditions that often led to similar mental health problems; such as patients with type 1 diabetes, childhood cancers or inflammatory bowel disease.

In a 2013 study by Glassberg et al1, 655 Emergency Medicine physicians were surveyed on their use of the term ‘sickler’, their attitudes towards patients with SCD and the frequency with which the physicians adhered to evidence-based guidelines when providing analgesia to patients presenting in vaso-occlusive crisis. It was found that doctors using the term ‘sickler’ were more likely to harbour negative attitudes towards patients with SCD. 50% of the physicians surveyed admitted to using the term ‘sickler’ either “frequently” or “always”. Within the group of physicians who “frequently” used the term ‘sickler’, there was a statistically significant reduction in adherence to evidence-based guidelines when managing vaso-occlusive crisis pain, compared to those physicians who either “rarely” or “never” used the term ‘sickler’. A further study found a similar association; medical practitioners with negative attitudes towards individuals with SCD displayed lower adherence to treatment guidelines for managing vaso-occlusive crisis2. A 2010 study carried out in Chicago3 found that patients attending the emergency department in vaso-occlusive crisis were having to wait for an average of 80 minutes for analgesia, 30 minutes longer than the average wait time for patients presenting to the same department complaining of renal colic; despite the fact that patients in vaso-occlusive crisis reported higher average pain scores on arrival to the department than those with renal colic. In a 2020 survey of patients with SCD, two-thirds of respondents reported that they had delayed attending an emergency department for care during a vaso-occlusive crisis because of previous negative healthcare experiences and stigma around their disease4. However, it is not just on the ‘shop floor’ that these negative attitudes manifest. In 2007 Solomon5 reviewed a range of medical textbooks providing educational information about analgesic treatment regimes for patients in vaso-occlusive crisis. He found that less than 40% of the books sampled noted that opioid addiction was infrequent in this population of patients. However, over 90% of the textbooks reviewed offered such assurance when discussing treatment for cancer-related pain.

I associate the term ‘sickler’ with the frustration that healthcare professionals have towards managing patients with SCD. I do not use the term ‘sickler’ and am uncomfortable when I hear my colleagues use it. Tellingly, I have never heard a medical professional use the term ‘sickler’ in front of a patient with SCD.  When I consider the reasons behind why SCD patients are stereotyped in the way that they are by healthcare professionals, I can’t help but focus on the fact that most ‘sicklers’ are black. It is much easier for someone to empathise with a condition that they can relate to. I suggest that for predominantly white physicians with predominantly white middle class family and friendship groups, it is much more difficult to empathise with a condition that affects mainly black people than a disease that could occur to either themselves or a loved one.

After the Lockdown Summer of 2020, with the re-emergence of the ‘Black Lives Matter’ movement and the introduction of the term ‘unconscious bias’ into the mainstream, it is an apt time to discuss the dangers of using the term ‘sickler’. The term trivialises what is a severe and life-limiting genetic condition. It promotes the idea that patients presenting to A&E with severe pain in vaso-occlusive crisis are opioid seeking. This has led to patients receiving delayed or inadequate care. I know that most doctors I have worked with do not consider themselves as racist. Yet when we as medical professionals, consciously or subconsciously, act upon stereotypes that we have developed in our careers, to impact negatively upon patient care; then that is a form of racism. Whilst the term ‘sickler’ continues to be used amongst healthcare professionals, the stereotypes attached to the term will propagate through to future generations of doctors and medical students. It is not racist to feel frustration at a patient with sickle cell disease that is demanding treatment. However, it is racist to allow that frustration to impact upon the treatment that that patient, or the next one, receives. 

References

1. Glassberg, J., Tanabe, P., Richardson, L. & Debaun, M. Among emergency physicians, use of the term "Sickler" is associated with negative attitudes toward people with sickle cell disease. Am. J. Hematol. 88, 532–3 (2013).

2. Glassberg, J. A. et al. Emergency provider analgesic practices and attitudes toward patients with sickle cell disease. Ann. Emerg. Med. 62, 293-302.e10 (2013).

3. Lazio, M. P. et al. A comparison of analgesic management for emergency department patients with sickle cell disease and renal colic. Clin. J. Pain 26, 199–205 (2010).

4. Abdallah, K. et al. Emergency Department Utilization for Patients Living With Sickle Cell Disease: Psychosocial Predictors of Health Care Behaviors. Ann. Emerg. Med. 76, S56–S63 (2020).

5. Solomon, L. R. Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void. Blood 111, 997–1003 (2008).

About the authors

  • Alex Gibbs works in East London as a doctor training to specialise in Emergency Medicine. When not working, Alex likes to savour the rare occasions that Newcastle United win a football match.

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