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• To understand why vaso-occlusive crises in sickle cell disease should be treated as a medical emergency
• To increase your awareness of why patients with sickle cell disease often receive suboptimal care
• To acknowledge the need for staff education on sickle cell disease so that patients receive appropriate care
Most patients with sickle cell disease (SCD) need support from healthcare services to manage their condition, including painful vaso-occlusive crises. Vaso-occlusive crises should be treated as a medical emergency, but the quality of the care patients receive when they present to the emergency department (ED) is often suboptimal. This article reports the findings of a literature review on the views of ED nurses and doctors about patients with SCD. The review included four studies, all of which had been conducted in the US, demonstrating that research on the topic is limited. The review found mostly negative views, including the belief that patients misuse pain medicines and demonstrate drug-seeking behaviours. Racial bias, widely recognised as a negative influence on the care of patients with SCD, was not mentioned in any of the studies. Staff education regarding SCD is required to ensure patients receive the care they need when they present to the ED.
Emergency Nurse. doi: 10.7748/en.2023.e2171
Peer reviewThis article has been subject to external double-blind peer review and checked for plagiarism using automated software
Correspondence Conflict of interestNone declared
Jerman H, Chang Y-S (2023) Sickle cell disease: healthcare professionals’ views of patients in the emergency department. Emergency Nurse. doi: 10.7748/en.2023.e2171
Published online: 15 August 2023
Sickle cell disease (SCD) is a potentially life-threatening condition (Dormandy et al 2018) and one of the two main types of haemoglobin disorders, the other being thalassaemia (World Health Organization 2021). Haemoglobin disorders are genetic blood diseases characterised by the inheritance of a genetic mutation of red blood cells from both parents, who are usually themselves healthy. Approximately 5% of the world’s population carries trait genes for haemoglobin disorders and more than 300,000 children with severe haemoglobin disorders are born every year (World Health Organization 2021). SCD is particularly common among people with an African or Caribbean background (NHS 2022).
There has been extensive research into treatments for SCD in recent decades, but none of the newer treatments have been officially approved or recommended, so they are not widely used (Gardner et al 2016, Salinas Cisneros and Thein 2020). However, there are recommended treatments for managing the symptoms and complications of SCD (Bartolucci and Galactéros 2012, Aich et al 2019). Many patients with SCD experience chronic pain and take opioids for much of their life (Smith et al 2015). SCD also causes many complications and these often require hospital admission. Aljuburi et al (2012) calculated that, in 2009-10, the overall rate of admission to hospital for SCD as a primary diagnosis in England was 33.5 in 100,000 population.
The most common reason why patients with SCD attend hospital is an episode of painful vaso-occlusion (Abd Elmoneim et al 2019, Zakaria et al 2021), also called vaso-occlusive crisis, sickle cell crisis or acute sickle cell pain. Patients with SCD have at least one vaso-occlusive crisis per year on average (Taylor et al 2010). The pain caused by a vaso-occlusive crisis is usually severe. It can sometimes be managed at home, but patients often need to attend hospital so that they can receive stronger analgesia (Rizio et al 2020). Vaso-occlusive crises account for 90% of hospital attendances among patients with SCD (Sickle Cell Society 2021).
Complications of vaso-occlusive crises include leg ulceration, stroke, pulmonary hypertension and priapism (Hebbel 2014). If vaso-occlusion occurs in larger blood vessels, particularly coronary vessels, the complications are more severe (Vichinsky et al 2000, van Tuijn et al 2010, Nur et al 2020, Friend et al 2023). Vaso-occlusion in the pulmonary vessels can result in acute chest syndrome, which often leads to cardiac arrest and death. Acute chest syndrome is the most common cause of death among people with SCD (Vichinsky et al 2000, Friend et al 2023).
The timely treatment of vaso-occlusive crises is likely to reduce the risk of further complications and death (Osunkwo et al 2020, Sickle Cell Society 2021), so patients who undergo a vaso-occlusive crisis must be promptly and adequately assessed and treated. In its guideline on managing acute painful episodes of SCD in hospital, the National Institute for Health and Care Excellence (NICE) (2012) states that acute sickle cell pain should be treated as a medical emergency and recommends administering analgesia within 30 minutes of presentation.
However, it appears that patients with SCD going through a vaso-occlusive crisis often do not receive urgent assessment and treatment in the emergency department (ED) (Sickle Cell Society 2021). This could be partly due not only to high numbers of patients in the ED coupled with staff shortages, particularly of nurses (Glassberg 2017, Scammell 2019), but also to the fact that healthcare professionals often underestimate the pain associated with a vaso-occlusive crisis (Linton et al 2020).
It is essential that patients with SCD seek medical support when they need it (Gardner et al 2010), but there are several barriers that prevent them from doing so, one of which is how they are viewed by healthcare professionals working in the ED (Kanter et al 2020). However, as far as the authors of this article are aware, there are no published literature reviews on the views of healthcare professionals working in EDs regarding patients with SCD.
To synthesise the literature on the views of healthcare professionals working in EDs regarding patients with SCD.
A systematic search was conducted in January 2022 in three databases, the British Nursing Index, the Cumulative Index to Nursing and Allied Health Literature (CINAHL) and MEDLINE. The search strategy had been developed in collaboration with a university librarian. To ensure all relevant terms would be searched for, a thesaurus search had been conducted and medical subject headings (MeSH) were included. Free-text searching was used, as was truncation to ensure all possible variations of keywords would be found (Salvador-Oliván et al 2019). Table 1 shows the keywords used in the database searches.
Only primary research studies published in the previous 20 years, in peer-reviewed journals and in English were considered. Only studies concerning adult patients and healthcare professionals working in hospital EDs were considered.
The database searches retrieved 394 studies. Removing duplicates left 269 studies. Applying inclusion and exclusion criteria left 179 studies. The first author manually checked the abstracts of these 179 studies for relevance, which left 17 studies. The first author then read the full texts of these 17 studies, after which there were four studies remaining for inclusion in the review.
Quantitative data were converted into qualitised data – that is, a descriptive summary (Tashakkori and Teddlie 1998, Sandelowski 2000, Driscoll et al 2007, Collingridge 2013) and thematic synthesis (Thomas and Harden 2008) was used to synthesise the qualitative and qualitised data.
Table 2 gives an overview of the four included studies.
• Patients with sickle cell disease (SCD) are prone to vaso-occlusive crises, which warrant urgent treatment
• SCD predominantly affects people of African or Caribbean origin and racial bias been shown to negatively affect patient care
• Barriers to patients with SCD seeking medical support include how they are viewed by emergency department (ED) staff
• In four US studies, ED nurses and doctors were shown to hold mainly negative views of patients with SCD
• Education could support ED staff to better understand why patients with SCD present to the ED
In their study, Ratanawongsa et al (2009) developed the Positive Provider Attitudes toward Sickle Cell Patients Scale, which comprises three sections exploring:
• Participants’ attitudes towards patients with SCD compared with other conditions.
• Whether participants agree or disagree with a list of statements about patients with SCD.
• How likely is it, according to participants, that patients with SCD would over-report pain or misuse drugs.
Ratanawongsa et al (2009) acknowledged that their study only provided preliminary evidence for the reliability and validity of their scale and that its validity needed to be further explored.
Freiermuth et al (2014, 2016) and Jenerette et al (2015) used the General Perceptions about Sickle Cell Patients Scale employer by Haywood et al (2011), in which participants’ responses to questions are used to calculate levels of positive and levels of negative attitudes. Here are examples of questions:
• ‘What percentage of patients with SCD are drug seeking when they come to the hospital?’
• ‘What percentage of patients with SCD are frustrating to take care of?’
• ‘What percentage of patients with SCD are satisfying to take care of?’
• ‘What percentage of patients with SCD are easy to empathise with?’
Freiermuth et al (2014) also used the Medical Condition Regard Scale (Christison et al 2002) and the findings obtained were very similar to those obtained with the General Perceptions about Sickle Cell Patients Scale (Haywood et al 2011).
The four studies did not always present the data their findings were based on. Only findings supported by data are included here. Findings common to all studies but not supported by data – for example, healthcare professionals’ view that patients with SCD who undergo a vaso-occlusive episode tend to exaggerate their pain – are not included here. Conversely, findings not common to all studies but supported by data – for example, uneasiness with the care provided by other staff, which was mentioned in three of the four studies – are included here.
The thematic synthesis of data generated three main themes:
• Belief that patients misuse pain medicines.
• Frustration with providing care.
• Uneasiness with the care provided by other staff.
All studies identified a belief among healthcare professionals that patients misuse pain medicines and demonstrate drug-seeking behaviours. In Ratanawongsa et al (2009), there was an overall belief among participants that patients misuse, and do not follow medical advice regarding, pain medicines. Jenerette et al (2015) found that most participants believed patients do not use pain medicines appropriately. Freiermuth et al (2014) found that most participants believed patients with SCD attending the ED have an opioid addiction.
Freiermuth et al (2014, 2016) found that, overall, nurses had greater levels of negative attitudes than doctors. In Freiermuth et al (2016), the belief that patients are addicted to pain medicines was significantly more common among nurses. Freiermuth et al (2016) also noted that the overall decline in negative attitude scores over time was more pronounced among doctors.
One of the items on the General Perceptions about Sickle Cell Patients Scale (Haywood et al 2011) relates to healthcare professionals’ levels of frustration: participants are asked to rate ‘how frustrated [they] are with providing care to adult patients with sickle cell disease’. The Positive Provider Attitudes toward Sickle Cell Patients Scale developed by Ratanawongsa et al (2009) comprises a similar item: participants are asked to rate their agreement with the following statement: ‘This patient is frustrating to look after.’
In all studies, participants had high levels of frustration. In Jenerette et al (2015), a large number of participants had high ratings on the ‘frustration’ item of the scale, which correlated with comments in the surveys such as ‘It is very hard to treat all of the patients without doubt due to their behaviour’.
In the two studies conducted among nurses and doctors, nurses had higher levels of frustration than doctors (Freiermuth et al 2014, 2016). Freiermuth et al (2014) reported that the longer participants had been practising, the higher their frustration levels were, and identified a correlation between level of frustration and perceived patient addiction to pain medicines.
Another item on the General Perceptions about Sickle Cell Patients Scale (Haywood et al 2011) relates to healthcare professionals’ uneasiness with the care provided by other staff. Participants are asked to rate ‘to what extent [they] feel bothered by the way other staff look after SCD patients’. In Freiermuth et al (2014, 2016) and Jenerette et al (2015), participants had high levels of uneasiness with the care provided by other staff.
In the two studies by Freiermuth et al (2014, 2016), uneasiness with the care provided by other staff was significantly higher among doctors than among nurses. In Freiermuth et al (2016), uneasiness with the care provided by other staff was not influenced by participants’ length of practice and it did not decrease in parallel with the increase in the levels of positive attitudes towards patients.
In the four included studies, the views of healthcare professionals working in the ED regarding patients with SCD were mainly negative and negative views were, overall, more common among nurses than among doctors. The studies all mention an acute need for staff education on SCD. Staff education was the intervention used by Freiermuth et al (2016) in their longitudinal quality improvement study, which showed that negative attitudes decreased and that positive attitudes increased among participants over time. This finding suggests that participants’ initial negative attitudes were due to lack of understanding of, and education about, the disease. Puri Singh et al (2016) showed that a brief video-based educational intervention can improve the attitudes of ED healthcare professionals towards patients who undergo a sickle pain crisis.
A lack of understanding can cause or contribute to bias (Kaur et al 2020). In its inquiry into failures of care and avoidable deaths of patients with SCD in secondary care, the all-party parliamentary group on sickle cell and thalassaemia (APGSCT) outlined that there is a consensus, among patients, that healthcare professionals working in EDs and on general wards have low levels of awareness and knowledge of SCD, leading to suboptimal care (Sickle Cell Society 2021). Other findings included inadequate training, negative attitudes towards patients and inadequate investment in care for SCD (Sickle Cell Society 2021). Patients have reported negative experiences of care in EDs (Haywood et al 2009, Bemrich-Stolz et al 2015, Abdallah et al 2020, Kanter et al 2020), including stigmatisation and allegations of drug-seeking behaviour (Sickle Cell Society 2021).
Healthcare professionals’ perception that patients demonstrate drug-seeking behaviours has been shown to be more prevalent regarding patients with SCD than patients with other genetic chronic conditions such as cystic fibrosis or haemophilia (Grosse et al 2009, Wakefield et al 2017, Bulgin et al 2018), despite the fact that the prevalence of opioid addiction is not higher in patients with SCD than in other patient groups (Field 2017). This erroneous perception can negatively affect the care provided to patients; for example, healthcare professionals may be less willing to prescribe opioids if they believe patients are addicted to them.
All four studies had been conducted in the US, a country that since the 1990s has been experiencing a sharp increase in the rates of deaths caused by overdoses of prescription and illicit opioids (Centers for Disease Control and Prevention 2022). The US opioid epidemic is likely to have influenced the views of the doctors and nurses involved in the four included studies. This is supported by Ruta and Ballas (2016), who demonstrated that patients with SCD have become ‘guilty by association’ due to the ongoing opioid epidemic, despite the fact that the number of deaths by overdose is significantly smaller in people with SCD than in the general population. None of the four included studies mention the US opioid epidemic.
Racial bias is another factor that none of the four studies mentions but that potentially influenced the views of participants. This would echo DeLaune et al (2020), who wrote that ‘studies done in the USA suggest that many health-care providers have an unconscious preference for white patients and negative attitudes toward people of colour’ and that this can manifest as ‘domineering language and speech, poor communication, negative patient-perceived interactions, mistrust in the patient-physician relationship, stigmatizing language in the medical record, and substandard treatment recommendations’.
As explained above, SCD predominantly affects people of African or Caribbean origin (NHS 2022) and racial bias been shown to negatively affect the care patients with SCD receive (Bulgin et al 2018, Power-Hays and McGann 2020, Sickle Cell Society 2021). Racial bias has been shown to affect patients with SCD more than patients with any other disease (Power-Hays and McGann 2020) and according to Bulgin et al (2018), SCD is the only genetic disease affected by racial bias. In its inquiry, the APGSCT noted that ‘the experience of people living with sickle cell is that the failings in treatment and the lack of understanding outlined in this report show deep inequality in the healthcare system’ (Sickle Cell Society 2021).
Data synthesis may be susceptible to bias from the person undertaking it (Campbell et al 2020), particularly when grouping data into themes. Furthermore, if the person who conducts the literature review also undertakes clinical work – which was the case of the first author (HJ) of this article – they are likely to have views that could influence the review process. Only articles in English were included, which could have limited the amount of relevant research found. Only four relevant studies were found, all of which had been conducted in the US (including three in North Carolina) and two of which had the same lead author. Some of the findings did resonate with HJ’s clinical experience, but because of the limitations of this literature review the findings cannot be generalised.
The literature emphasises the importance of staff education and training to enhance the care of patients with SCD in EDs (Haywood et al 2009, Glassberg 2017, Menaa et al 2017, Kaur et al 2020). Education about SCD could be useful to address negative views among healthcare professionals, including that patients misuse pain medicines, and to address healthcare professionals’ frustrations and uneasiness with the care provided by others. It could support ED staff to better understand why patients with SCD present to the ED and to recognise the importance of urgent assessment and treatment. The lack of research on the topic of this literature review, in the US and across the English-speaking world, is significant. Future research could include studies of patients’ views regarding the care they receive as well as further studies exploring the views of healthcare professionals.
SCD is a common inherited blood disorder which predominantly affects people of African or Caribbean origin. Patients with SCD often present to the ED with a vaso-occlusive crisis, which can have life-threatening consequences and warrants urgent assessment and treatment. According to the findings of this literature review, nurses and doctors working in EDs tend to hold negative views of patients with SCD, including the belief that patients misuse pain medicines and demonstrate drug-seeking behaviours. However, these findings are based on a small number of studies (four), all of which had been conducted in the US. None of the studies mentioned racial bias despite the fact that it is a well-known factor in the suboptimal care patients with SCD receive. Education on SCD could support ED staff to better understand why patients with SCD present to the ED and why they need urgent assessment and treatment.
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