Aims and intended learning outcomes

The aim of this article is to raise greater awareness of sickle cell disease, its acute manifestations and the potential for rapid clinical deterioration that can occur in patients with this condition. After reading this article and completing the time out activities you should be able to:

Introduction

Sickle cell disease is the most common serious inherited genetic disorder in the UK (Pizzo et al 2011). It is a group of inherited disorders characterised by the presence of a mutated form of haemoglobin, specifically haemoglobin S (HbS), resulting in sickle-shaped red blood cells under deoxygenated conditions (Herrick 1924). These distorted red blood cells can obstruct blood flow through the capillaries causing painful vaso-occlusive crises, which are one of the most frequent and debilitating symptoms of the disease. Vaso-occlusive crises are characterised by bone pain that typically occurs in the extremities, back, joints, abdomen or chest and if not treated rapidly can lead to serious complications such as end-organ damage or acute chest syndrome (Sundd et al 2019). More than 90% of acute hospital admissions among people with sickle cell disease are due to vaso-occlusive crises (Sins et al 2017) and each crisis must be considered life-threatening.

An estimated 15,000 people have sickle cell disease in the UK with approximately 350 neonates identified as having the disease annually (Sickle Cell and Thalassaemia All-Party Parliamentary Group 2018). The prevalence of sickle cell disease across the UK varies, with London accounting for the highest distribution of people with the condition (Piel et al 2013). The highest prevalence of sickle cell disease occurs in people of African and African-Caribbean origin, although it is also prevalent in those originating from the Eastern Mediterranean, Middle East, India and South and Central America (Sickle Cell Society 2018). It is estimated that by 2050 there will be a 30% increase in the number of children born with sickle cell disease in the UK (Strunk et al 2020).

Various barriers to effective care for people with sickle cell disease have been identified, such as a lack of understanding among nurses about the complexity of the condition and how to respond appropriately (Royal College of Nursing (RCN) 2016, All Party Parliamentary Group on Sickle Cell and Thalassaemia/Sickle Cell Society (APPG/SCS) 2021). In addition, Power-Hays and McGann (2020) described how many patients with sickle cell disease have experienced racism and been marginalised when seeking emergency care at a time when they are experiencing inexorable pain. Therefore, it is essential that emergency nurses understand the wider aspects of vaso-occlusive crisis management, including the importance of providing culturally competent care and recognising the need for comprehensive triaging to improve patient outcomes.

The death of Evan Smith, a 21-year-old man who called 999 from his hospital bed (BBC News 2021), not only drew attention to the acute effects and rapid deterioration that can occur in people with sickle cell disease, but also demonstrated how sickle cell pain is still often dismissed by healthcare professionals and at an organisational level. Evan Smith’s death triggered the No One’s Listening inquiry (APPG/SCS 2021), which examined the quality of care received by patients with sickle cell disease when accessing secondary care. The inquiry identified a pattern over many years of substandard care, stigmatisation and lack of prioritisation of these patients. Optimal assessment and management of patients with sickle cell disease presenting with vaso-occlusive crises in EDs is essential to ensure such failings are not repeated.

Effects of sickle cell disease

Sickle cell disease is characterised by chronic anaemia, progressive organ damage and various acute manifestations. In addition to vaso-occlusive crises, other serious and unpredictable acute manifestations include infections, haemolytic anaemia, aplastic crisis, splenic sequestration crisis, stroke and acute chest syndrome (Table 1) (National Institute for Health and Care Excellence (NICE) 2014, De et al 2019). As a result of these severe complications, sickle cell disease has been associated with a significant reduction in life expectancy (Sins et al 2017).

Sickle cell pain can be acute, chronic or acute-on-chronic and is typically categorised as mild, moderate or severe. According to Maakaron and Taher (2021), self-management at home with bed rest, oral analgesics and hydration can be effective for people experiencing mild or moderate sickle cell pain. People with sickle cell disease tend to only present to the ED when they are experiencing severe pain and only after their attempts to self-manage this have been unsuccessful (Maakaron and Taher 2021).

It is also important to recognise that it is not only the acute complications of sickle cell disease that require timely and effective management, but also the psychosocial effects and stress associated with living with this long-term, life-threatening condition (De et al 2016). Wallen et al (2014) identified that depression, anxiety and sleep disturbances are among the most common psychological issues that coexist with acute and chronic pain. Therefore, patients with sickle cell disease may require referral to additional services such as pain specialists or psychologists (National Confidential Enquiry into Patient Outcome and Death 2008).

Assessment

Excruciating vaso-occlusive pain tends to be the most common symptom and the ‘hallmark’ of a vaso-occlusive crisis (Maakaron and Taher 2021). NICE (2014) guidance states that people who present to an ED with a painful vaso-occlusive crisis need to receive a pain assessment, a clinical assessment and appropriate analgesia within 30 minutes of their arrival. It is important that healthcare professionals ask appropriate questions to determine if the patient has taken any over-the-counter analgesics before they presented to the ED and administer further medicines based on clinical guidance and the latest evidence.

The National Early Warning Score 2 (NEWS 2) (Royal College of Physicians 2017) ‘track-and-trigger’ tool typically supports nurse triage processes and hospital admissions (Kemp et al 2020). However, during a vaso-occlusive crisis, vasodilatory compensatory mechanisms can mean that patients remain normotensive throughout the crisis (De et al 2019) which could lead to inadequate scoring. This makes the assessment process highly subjective and can result in delays in treatment escalation.

The acute manifestations of sickle cell disease can be non-specific and challenging to identify, so various investigations and medical imaging may be necessary as part of an assessment. Patel et al (2021) set out a systematic approach to a 15-minute general consultation in the ED (Table 2), which could be used to guide healthcare professionals. However, it is important to emphasise that interpretation of assessment findings will in part be subjective.

When assessing patients, it is important to recognise that the appearance of cyanosis, anaemia-related pallor and jaundice - a result of accelerated haemolysis and the excess by-product bilirubin - differs from that seen in people with a lighter skin tone or white skin. Evidence has also shown that darker skin pigmentation, combined with peripheral shut down, can decrease the accuracy of pulse oximetry during hypoxic episodes (Bickler et al 2005).

Invasive interventions such as obtaining an arterial blood gas should not be considered in children and only as a last resort in adults or in those with suspected acute chest syndrome. Nurses need to observe for evidence of sickle cell-related keloid scarring linked to overgranulation (De et al 2019) and should be aware that if this is in the lower leg region it may affect oxygen saturation (SpO2) readings taken via the toes. Patients, their family members or carers and emergency nurses must also recognise the importance of checking the patient’s mucous membranes and sclera diligently; the inside of mouth must be checked for a blue/grey tinge, which would indicate cyanosis, and the sclera for pallor which would indicate anaemia.

Pain assessment

Darbari and Brandow (2017) supported the use of objective pain assessment tools that are age and developmentally appropriate. They emphasised that such tools are necessary to assess sickle cell pain accurately, determine the effects of this pain on a person’s quality of life and investigate underlying pain mechanisms. This could lead to better understanding of sickle cell disease and improved patient outcomes.

Although Darbari and Brandow (2017) recommended a multidimensional, patient empowering reporting-outcome approach, NICE (2012) uses the unidimensional Visual Analogue Score (VAS) (Figure 1) to guide definitions of pain intensity. NICE (2012) guidelines define moderate pain as typically a VAS score of between 4 and 7 and severe pain as typically a VAS score of more than 7 but emphasise that this will not apply to all patients because pain is subjective. The VAS tool can be supplemented by using a body diagram if a patient’s pain is multifocal.

Figure 1.

Visual analogue score

TIME OUT 2

Consider how responses to pain may differ in people with lifelong pain-causing disorders compared to those who experience pain briefly. How would you assess these appropriately?

Management

Barriers to effective care in the emergency department

Swann (2021) describes how suboptimal pain assessment can have a detrimental effect on the quality of an individual’s healthcare experience. To highlight some of the specific challenges that patients with sickle cell disease may experience, it may be useful to compare their pain assessment and management with that of patients with other acute conditions who may present to the ED. For example, in the authors’ experience managing pain associated with myocardial infarction or an acute bone fracture - which is typically localised - is relatively standardised and often guided by a set pathway or protocol. However, managing sickle cell pain tends to be more complicated since it can occur anywhere in the body and may be multifocal (De et al 2019).

Sickle cell pain may also differ from the novel pain seen in other conditions. For example, patients with cancer will often experience novel pain with the onset of cancer, so their bodies will respond and cope differently to this new unpleasant sensation (Gordon et al 2005), whereas patients with sickle cell disease will be reacting to pain that has been present throughout their lives. Therefore, the authors call for nurses to actively listen to patients as the ‘lifelong pain expert’ to support improvements in their care.

Stanton et al (2010) drew attention to patients with sickle cell pain often having to cope simultaneously with excruciating pain, healthcare staff’s lack of knowledge about their condition and its complications and racial discrimination during an emergency admission. Widespread documentation and many similar reports of this type of stigmatisation and implicit bias demonstrate how these episodes of suboptimal care are not isolated but appear to be frequently experienced across EDs globally (Bulgin et al 2018). This type of negative stereotyping and prejudice has led to the coining of derogatory terms such as ‘sickler’ and ‘drug seeker’. Power-Hays et al (2020) reported that many patients with sickle cell pain ensure that they dress smartly before presenting to an ED in an attempt to avoid judgement and to receive higher quality emergency care.

In addition, patients with sickle cell disease are used to coping with significant levels of pain all their lives and have developed a diverse range of coping mechanisms that may not be perceived as conventional or evidence-based. Anecdotal examples from the authors’ clinical experience include application of cold compresses, smoking cannabis, taking warm baths or consuming alcohol. Others, who are empowered to request opioid-based treatments, could be misinterpreted due to an association with addiction and might not be believed when asked about their pain severity in acute settings (Masese et al 2019). Further, derogatory terms such as ‘A&E hopper’ are still used in relation to situations when service users are marginalised and forced to attend other EDs to obtain supplementary opioid-based analgesics to reduce their discomfort (Bulgin et al 2018). These dismissive attitudes and accusations of feigning pain are contrary to The Code: Professional Standards of Practice and Behaviour for Nurses, Midwives and Nursing Associates (Nursing and Midwifery Council 2018) and McCaffrey’s (1968) definition of pain as ‘whatever the experiencing person says it is, existing whenever and wherever the person says it does’. Patient self-reporting was McCaffrey’s care standard for evaluating pain and the authors of this article believe that a cultural shift back to this principle is required in nursing.

Curative options for patients with sickle cell disease are limited to allogeneic haematopoietic stem cell transplant, commonly known as bone marrow transplant. However, these are associated with numerous risks and complications such as graft versus host disease in which the transplanted cells attack other cells in the body (NHS England 2019). Until genetic advances such as gene therapy and gene editing become more widely available and effective, emergency admissions for vaso-occlusive crises will continue. Therefore, there is an urgent need for increased awareness of sickle cell disease among healthcare professionals to ensure that this patient group receives high-quality care (Bulgin et al 2018, APPG/SCS 2021).

Emergency nurses need to ensure clinical governance measures are benchmarked consistently against evidence-based standards such as those published by NICE (2014) and the British Society of Haematology (Qureshi et al 2018). In addition, the RCN (2016) and the Sickle Cell Society (2018) have recommended targeting improvements in sickle cell disease awareness through education and training. Clinical training investment, particularly for nurses working in emergency care settings, is also essential. Alongside specialist haematology nurses, the authors are developing a short ten-minute educational podcast on vaso-occlusive crisis management which could be shared and accessed by ED staff to promote knowledge acquisition or updates.

Prevention of vaso-occlusive crises

Nurses should be aware that many patients with sickle cell disease recognise the factors that can trigger a vaso-occlusive crisis. These triggers may include cold weather, dehydration, infection, hypoxia, vascular strain or trauma, as well as excitement, sexual arousal, fear or stress (Maakaron and Taher 2021). It is important to identify which triggers a patient commonly experiences and to avoid or manage these as appropriate.

Patients with sickle cell disease are at risk of developing folate deficiency, so commonly take folic acid supplementation. However, there are no trials showing the benefit of routine folate supplementation (Sickle Cell Society 2018). Al-Yassin et al (2012) recommended the safest clinical practice is to individualise folic acid supplementation for children and adults with sickle cell disease, based on their needs and comorbidities, while continuing to encourage concordance with more evidence-based preventative medicines such as penicillin (Sickle Cell Society 2018). Another important medicine in preventing vaso-occlusive crises is hydroxycarbamide, also known as hydroxyurea. This medicine increases levels of fetal haemoglobin (HbF), thereby decreasing the proportion of HbS as well as suppressing inflammatory responses which contribute significantly to the pathophysiology of the sickling process (Thornburg et al 2010).

Health education and preventative measures need to form part of an ongoing care plan. Again, the authors emphasise that people with sickle cell disease are the experts on their pain, so the nurse and patient need to work together to set personal objectives around maintaining their health and well-being. Collaborating to devise self-care strategies and agreeing goals will improve concordance and monitoring and could reduce or prevent future emergency admissions related to crises.

Conclusion

Vaso-occlusive crises are the most common reason why patients with sickle cell disease present to the ED. In some cases, presentation with painful vaso-occlusive crises can quickly progress to serious and life-threatening complications. Therefore, timely assessment and intervention are crucial to alleviate these patients’ pain and avoid such complications. Patients with sickle cell disease experience lifelong pain and may use various strategies to cope with this, which may make their pain appear less severe to healthcare professionals who do not see them regularly.

Often, these patients experience discrimination when accessing healthcare services, so it is important for nurses and other healthcare professionals to increase their awareness and modify their behaviours to restore patients’ confidence in services and provide optimal care. Believing in and involving patients as the experts on their pain, and involving specialist haematology teams early on, will support this process.