Spotting the signs of sickle cell disease and how to respond
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Spotting the signs of sickle cell disease and how to respond

Erin Dean Health journalist

Lack of knowledge of the blood disorder is common among healthcare professionals. Find out how to help your patients

Sickle cell disease, a group of disorders that alter the haemoglobin in red blood cells, affects an estimated 15,000 people in the UK, according to the Sickle Cell Society.

Emergency Nurse. 29, 5, 16-19. doi: 10.7748/en.29.5.16.s11

Published: 31 August 2021

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Picture credit: iStock

The changes it causes lead the red blood cells to become dehydrated, more rigid and less able to negotiate capillaries. These sickle-shaped cells then clump together and block the small blood vessels, leading to an episode known as a sickle cell crisis.

Without appropriate treatment, a sickle cell crisis can lead to organ damage or death.

Yet expert nurses have warned that the care received by people seeking emergency treatment for a sickle cell crisis falls below the expected standard. A 2017 survey by the Sickle Cell Society of patients and carers – designed to inform new standards on the care of adults with the disease – found poor quality of emergency compared with planned care.

Here, we examine the signs of a sickle cell crisis, the reasons behind shortcomings in care, and how nurses can advocate for their patients.

Clinical guidance from the National Institute for Health and Care Excellence (NICE) and charity the Sickle Cell Society is clear: sickle cell crisis must be treated as a medical emergency requiring prompt pain control.

Yet common problems for people in sickle cell crisis include unacceptable delays in receiving pain relief, insufficient or excessive doses, inappropriate analgesia, and stigmatising the patient as drug seeking, NICE says.

A 2015 survey found people seeking urgent care often faced unnecessarily long waits in emergency departments (ED) during an acute episode. Of 280 adults who sought emergency care for their sickle cell disorder, fewer than half (45% or 126) felt that the healthcare staff knew enough about their condition.

Seek emergency care

The survey asked adult respondents who experienced pain when accessing urgent care how quickly the emergency staff assisted in easing their pain. Of the 88 people who answered, 26 felt they were assisted quickly enough, while a further 41 received assistance, but said their pain could have been eased more quickly. But 21 of the 88 reported that the emergency staff failed to ease their pain at all.

Specialist nurse consultant Lola Oni, service director at the Brent Sickle Cell and Thalassaemia Centre, part of London North West University Healthcare Trust, is not surprised that many people with sickle cell disease have a negative experience when seeking emergency care.

Sickle cell is an inherited disorder that disproportionately affects people of African and Caribbean backgrounds. It also affects people from eastern Mediterranean, Middle Eastern, Indian and South and Central American backgrounds.

Dr Oni says racial stereotyping and poor knowledge about the condition can prevent some healthcare staff from quickly providing critical care and instead raise suspicion about their reason for requesting pain medication.

‘Despite perceptions, patients with sickle cell live in rural and remote areas, as well as cities, and young people with the condition will be at universities all over the country’

Joan Walters, lead nurse for clinical practice and education for children and young people/haemoglobinopathies, Variety Children’s Hospital

‘A lot of it is about perception,’ Dr Oni says. ‘Healthcare professionals see a patient who is a young black man or woman asking for strong opioids. The main messages seen in the media, particularly about young black men, are around knife crime and here is a patient with that ethnic and age profile asking for very strong painkillers.

‘Patients with sickle cell disease often live with severe pain for decades and have been taught to manage pain cognitively, so they may not be displaying the outward signs of being in severe pain that professionals expect. They are often not kicking the walls and screaming, but that doesn’t mean they are not in agonising pain.’

Poor care damages the trust needed for a good therapeutic relationship between patients and healthcare professionals, and feeds perceptions that people with sickle cell can be difficult patients, Dr Oni says.

Signs and symptoms of an acute sickle cell crisis

An acute sickle cell crisis in a person with sickle cell disease may be indicated by:

  • » A sudden onset of pain

  • » Infection

  • » Anaemia

  • » Other symptoms, such as a stroke or priapism (a painful erection)

  • » History of a previous crisis (or rarely, sickle cell disease may be undiagnosed)

(Adapted from NICE (2016) What Are the Complications of Sickle Cell Disease?)

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Try to manage pain at home

‘I don’t know a single person with sickle cell who doesn’t know someone who has died as a result of the disorder. So people are terrified when they are in hospital. It is not surprising if they become difficult.’

‘People with sickle cell will have tried to manage their pain at home, so by the time they reach emergency care, they will be seriously unwell. We as nurses and doctors are taught about empathy, but some staff have lost the ability to be empathetic.’

A lack of awareness also affects the standard of care. The 2008 National Confidential Enquiry into Patient Outcome and Death report found that basic nursing observations appropriately interpreted were of critical importance to the management of people with sickle cell disease – but it also found these were not always adhered to. For example, fluid balance was not well recorded in some patients and this contributed to deterioration in several patients.

Many factors can cause a crisis, including infection, dehydration, not having eaten sufficiently that day and being cold, which can cause the blood vessels to constrict, Dr Oni says. Sometimes the cause may never be known.

Staff should be aware of a serious lung condition, acute chest syndrome, in patients with an acute painful sickle cell episode, if they show signs of abnormal breathing, chest pain, fever or hypoxia. This life-threatening condition is one of the most common causes of death from sickle cell disease.

Other potential complications include acute stroke (due to blood flow to the brain being blocked during a crisis), aplastic crisis (when insufficient blood cells are made by the bone marrow), infections and osteomyelitis (infection in the bone).

Splenic sequestration, a potentially life-threatening condition in which the spleen swells, reducing the amount of red blood cells circulating in the body, is most commonly seen in children with sickle cell disease. This can cause shortness of breath, a rapid heartbeat, anaemia and abdominal swelling and pain.

Joan Walters, lead nurse for clinical practice and education for children and young people/haemoglobinopathies at Variety Children’s Hospital, part of King’s College Hospital, London, says that healthcare staff also need to be mindful of diagnostic overshadowing.

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‘People with sickle cell will have tried to manage their pain at home, so by the time they reach emergency care, they will be seriously unwell’

Lola Oni, specialist nurse consultant and service director at the Brent Sickle Cell and Thalassaemia Centre, London

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Infection presents the greatest risk to children with sickle cell disease

Picture credit: iStock

‘Sometimes other complications and conditions can be missed by too much focus on the sickle cell disease,’ she says. ‘So observations must be carried out quickly, and acted on, as with any other patient assessment.’

The leading cause of death in children with sickle cell disease worldwide is infection, so those presenting with a temperature need to be treated aggressively, says Ms Walters.

With established standards and guidelines in place, death of a child with sickle cell disease from infection in the UK is a failure, she says.

Nurses should also be aware that sickle cell disease is the leading cause of stroke in children.

‘Sometimes children can be seen as naughty, because they may be flopping around or have behavioural changes, but these can be signs of stroke,’ Ms Walters says.

Pain assessment

The Sickle Cell Society published the latest version of its guidance on the care of children in July 2019. It says that pain assessment should include the use of a validated pain assessment tool that is developmentally age appropriate, and there should be a policy in the ED regarding triage, pain assessment and length of acceptable time from arrival to administration of analgesia (not exceeding 30 minutes).

All medical and nursing staff involved in treating children for severe acute pain should have regular training in pain management and in the application of the local protocols.

Ms Walters says that making every contact count is important; when nurses see sickle cell patients it is a good opportunity to check that they are receiving and taking prophylactic antibiotics.

Sickle cell expert nurses agree that emergency care for the disorder varies substantially, and this can be due to lack of knowledge.

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A 2019 Nursing Standard investigation found one in five universities did not educate nursing students about sickle cell disease

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A 2019 investigation by Nursing Standard found one in five universities did not educate nursing students about sickle cell disease.

Of 70 UK universities offering undergraduate nursing degree courses who responded to a Nursing Standard Freedom of Information request, 15 said they did not devote any teaching time to sickle cell disease.

This echoes the findings of the All Party Parliamentary Group on Sickle Cell and Thalassaemia 2018 report I’m in Crisis. Of the 197 nursing students surveyed for the report, 71% said they had not had any formal teaching sessions about sickle cell.

How to respond to a sickle cell crisis

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Treat an acute painful sickle cell episode as an acute medical emergency Follow locally agreed protocols for managing acute painful sickle cell episodes and/or acute medical emergencies that are consistent with this guideline

Regard the patient (and/or their carer) as an expert in their condition throughout the episode Use an age-appropriate pain scoring tool to assess pain in all patients who present at hospital

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Offer analgesia within 30 minutes of presentation

Clinically assess all patients who present at hospital This includes monitoring blood pressure, oxygen saturation on air (if oxygen saturation is 95% or below, offer oxygen therapy), pulse rate, respiratory rate and temperature

Assess the effectiveness of pain relief every 30 minutes until satisfactory pain relief has been achieved, and at least every four hours thereafter, using an age-appropriate pain scoring tool, and by asking questions such as ‘how well did that last painkiller work?’ and ‘do you feel that you need more pain relief?’

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Monitor patients taking strong opioids for adverse events, and perform a clinical assessment (including sedation score) every hour for the first six hours

Follow locally agreed protocols for managing sickle cell crises as the acute painful episode resolves, to step down pharmacological treatment

Be aware of possible complications, including acute chest syndrome and acute stroke

(Adapted from NICE (2012) Sickle Cell Disease: Managing Acute Painful Episodes in Hospital)

Care remains patchy

There is a perception that care is needed mainly in larger cities, but patients with sickle cell disease live all over the UK and need expert care wherever they present, Ms Walters says.

‘Care remains patchy. Despite perceptions, patients with sickle cell live in rural and remote areas, as well as cities, and young people with the condition will be at universities all over the country.

‘If staff do not have experience or interest in sickle cell, it affects the patient experience.’

Find out more

All Party Parliamentary Group on Sickle Cell and Thalassaemia Report (2018) I’m in Crisis tinyurl.com/Sickle-Cell-Committee-Report

National Institute for Health and Care Excellence (2016) What Are the Complications of Sickle Cell Disease? tinyurl.com/Sickle-Cell-Complications

National Institute for Health Research (2015) Piloting a New Patient Reported Experience Measure for Sickle Cell Disease: a Report of the Findings tinyurl.com/NIHR-Sickle-Cell-Report

National Institute for Health and Care Excellence (2012) Sickle Cell Disease: Managing Acute Painful Episodes in Hospital nice.org.uk/guidance/cg143

Sickle Cell Society sicklecellsociety.org

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