Every year in the UK there are avoidable deaths from sickle cell disease, and many more people are left in excruciating pain waiting for appropriate treatment.
Within health care, there are significant gaps in awareness, understanding and knowledge about what sickle cell disease is, who it affects, how symptoms present, and how non-specialist nursing staff, in any setting, can provide quality care to patients living with this blood disorder.
“All non-specialists need to understand sickle cell disease,” advises Sekayi Tangayi, a nurse consultant for the Sickle Cell and Thalassaemia based at North Middlesex University Hospital NHS Trust/Royal Free Hospital.
Every informed interaction can prevent complications, build trust and save lives
“For individuals living with this lifelong condition, every informed and responsive interaction can make a meaningful difference in preventing complications, building trust, and ultimately saving lives," continues Sekayi.
Who's listening?
The lack of adequate sickle cell treatment and support led to an All-Party Parliamentary Group inquiry. Their 2021 report, No One’s Listening, examined the level of care patients with sickle cell receive. It found that outside of specialist services, sub-standard care was particularly prevalent in general wards and accident and emergency departments. It also highlighted that health care professionals do not receive sufficient sickle cell disease training.
Here’s what you need to know to improve outcomes for your patients.
What is sickle cell disease?
Also called haemoglobinopathies, sickle cell disease is a group of complex and lifelong conditions affecting the red blood cells.
Sickle cells are crescent-shaped cells that become rigid and then die prematurely. This can block small blood vessels which causes bouts of severe pain, life-threatening infections and a range of chronic complications.
Who does it affect?
There are around 17,500 people in the UK with this inherited condition. It can affect anyone, but it’s particularly common in people with African and Caribbean family backgrounds, as well as people with Asian, Mediterranean and Middle Eastern heritages.
Are there triggers?
Cells can sickle due to cold weather, sudden changes in temperature, dehydration, stress, fever, infection, hormonal changes such as puberty or menopause, and anaesthetics.
What are the long-term symptoms?
People with sickle cell disease live with a range of symptoms, including musculoskeletal pain, eye problems, frequent infections, and chronic anaemia that can cause fatigue and shortness of breath.
Over time, sickle cell disease can cause damage to the liver, kidney, lungs, heart and spleen.
What is a sickle cell crisis?
Red blood cells in those with sickle cell disease will behave normally most of the time (red blood cells last 20-30 days), which means symptoms will come and go. But when cells sickle and get stuck to the side of small blood vessels and block them, this causes painful episodes, called sickle cell crises or a vaso-occlusive crisis.
Attacks are unpredictable and can start quite suddenly
“Early identification and understanding of complications significantly improves patient outcomes and reduces avoidable harm. Timely recognition, escalation, and intervention avoids delays which can lead to irreversible damage and even death,” warns Sekayi.
What are the common complications of a crisis?
- sickle cell acute chest syndrome (a leading cause of death)
- stroke (a risk in children as well as adults)
- overwhelming infection (such as pneumococcal sepsis)
- splenic sequestration
- acute kidney injury
- priapism (which left untreated can lead to erectile dysfunction)
- avascular necrosis.
A sickle cell crisis can affect any part of the body. These attacks are unpredictable and can come on quite suddenly, lasting for days or weeks at a time. Each time a person has a crisis, it causes damage.
Can a sickle cell crisis affect people in different ways?
How frequently these episodes occur, and the severity of symptoms, will vary from person to person. While some people can manage symptoms at home, others will need to be cared for in hospital to help manage severe pain.
Making a difference as a non-specialist
Consultant nurse Sekayi shares four ways you can improve care for patients with sickle cell.
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Recognise the patient (and their family/carers) as experts in their own condition
“No one knows their condition better than the person living with it,” advises Sekayi.
Sekayi says to always ask open questions, actively listen, and respect the patient’s lived experience. Patients will often know what works for them in managing pain, triggers, and responses to treatment.
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Escalate any concerns early on
“Even small actions can prevent serious consequences,” says Sekayi.
Her guidance is to stay vigilant and to monitor and report any deterioration using the early warning systems: MEWS, NEWS2 and PEWS.
Recognise red-flag complications and immediately report anything the patient has raised and what you have observed and are worried about. It doesn’t matter how small your concern is – early recognition and escalation can save lives.
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Understand the patient journey
“There’s more to sickle cell than what you see at the bedside,” explains Sekayi.
She says to remember that many patients face stigma, unconscious bias and systemic racism, repeated disbelief or underestimation of their pain, and time away from their loved ones, which can cause mental health struggles. Always show compassion and advocate for their needs; and consider their past health care experiences too.
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Keep learning
“Your confidence can change a patient’s experience,” says Sekayi.
Sekayi encourages all non-specialists to engage with relevant training and development, attend haemoglobinopathy workshops, and familiarise themselves with local protocols. Use your leadership skills to raise awareness in your team, make small but meaningful improvements in care and challenge unprofessional attitudes and behaviours.
Challenging health inequalities
Sekayi’s not alone in recognising the prejudice faced by many sickle cell patients. Her colleague Natasha Lewis, a nurse consultant and lead nurse for sickle cell and thalassaemia services at Homerton Healthcare NHS Foundation Trust, says: “Patients come from a wide range of cultural backgrounds, yet biases – unconscious and conscious – continue to shape their care”.
Compassionate care must always be at the heart of nursing
The No One’s Listening report also found sickle cell patients were frequently subject to “prejudicial attitudes” and “treated with a lack of respect or prioritisation” in health care settings. Overwhelming evidence suggests these attitudes are often underpinned by racism.
• Read next: Empire, racism and the NHS: the history of sickle cell disorder
Harmful experiences affect patients emotionally and physically and, in some cases, have led to preventable loss of life. “This must change,” urges Natasha.
“Patient safety and compassionate care must always be at the heart of nursing, so every individual receives equitable and respectful treatment."
Together with prompt recognition of symptoms and appropriate pain management, culturally competent communication can drastically reduce unnecessary suffering and prevent further avoidable deaths.
Further information
- Our sickle cell and thalassaemia competency framework is a guide for all health care professionals working with sickle cell and thalassaemia patients – wherever they present. It looks at the practical skills needed to provide quality, evidence-based care, and you can use these competencies to self-assess.
- The publication also details where you can access further training to support your learning and development against the competencies, including the Sickle Cell and Thalassaemia Screening e-learning module.
- The Sickle Cell Society (SCS), UK Thalassaemia Society (UKTS) and the UK Forum on Haemoglobin Disorders all offer information, support and access to further resources.
