Sickle Cell Disease; Roadside to Resus

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Sep 15, 2025

Explore the complexities of sickle cell disease, a hereditary blood disorder impacting millions worldwide. Discover how abnormal hemoglobin leads to painful crises and serious complications like acute chest syndrome and stroke. Delve into recent Best Practice Guidelines aimed at improving emergency care and patient outcomes. Learn about the genetic differences between sickle cell trait and disease, and the evolutionary link to malaria. The discussion emphasizes tailored management approaches and the critical need for effective communication in ongoing patient care.

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INSIGHT

Systemic Failures Prompt New Standards

Sickle cell care failures have caused avoidable deaths and systemic issues in UK hospitals.
RCEM best practice guidelines and the 2021 report “No One’s Listening” demand clearer standards and early haemato‑logical involvement.

INSIGHT

How Sickling Drives Vaso‑Occlusion

Hemoglobin S polymerizes when deoxygenated, deforming red blood cells into sickles.
Repeated sickling damages membranes so cells become permanently sickled and obstruct microvasculature.

INSIGHT

Hemolysis Amplifies Vaso‑Occlusion

Sickle cells promote endothelial activation, leukocyte/platelet aggregation and intravascular hemolysis.
Free hemoglobin scavenges nitric oxide, causing vasoconstriction that worsens vaso‑occlusion.

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a focus on its acute presentations and the care we can deliver to improve outcomes for our patients.

Sickle cell disease (SCD) is a lifelong inherited blood disorder that affects over 15,000 people in the UK, and millions worldwide. It’s caused by the production of abnormal haemoglobin molecules, which distort red blood cells into a crescent, or “sickle,” shape. These rigid cells can block small blood vessels, leading to painful vaso-occlusive crises and organ damage.

While the condition has long been most prevalent in parts of Africa, the Middle East, the Mediterranean and India, today it’s a global health issue, and one we encounter regularly in UK emergency care. Tragically, failings in care have too often led to avoidable harm. The 2021 parliamentary report “No One’s Listening” laid bare some of these cases, highlighting missed opportunities, poor awareness, and systemic issues that cost lives, such as the death of Evan Nathan Smith.

So why are we revisiting this now? In 2024, RCEM published new Best Practice Guidelines on managing sickle cell disease in the ED. These provide clear, evidence-based standards for recognition, triage, analgesia, infection control, and safe discharge. In this episode, we take you through the key elements;

Pathophysiology – how a genetic mutation drives sickling, vaso-occlusion and inflammation.
Clinical presentations – from painful crises and acute chest syndrome, to stroke, anaemia, infection, priapism and pregnancy-related complications.
Recognition and triage – why timely pain control within 30 minutes is a must, and how to spot red flags.
Investigations and treatment – including the role of reticulocytes, the importance of knowing a patient’s baseline haemoglobin, and principles of analgesia, transfusion, oxygen, and supportive care.
Discharge and ongoing care – ensuring safe, joined-up planning, and involving haematology and specialist pathways wherever possible.

The take-home message? Every sickle cell crisis is a medical emergency. We need to listen to patients, escalate early, involve haematology, and deliver care that meets the standards they deserve.

Once again we’d love to hear any thoughts or feedback either on the website or via X @TheResusRoom!

Simon, Rob & James