The many faces of a sickle cell crisis

A sickle cell crisis does not introduce itself politely. Sometimes it shows up as excruciating pain. Sometimes as yellowing eyes from jaundice. Sometimes as an unexpected mass in the left side of the abdomen, the spleen swollen and struggling. Diagnosis: splenic sequestration crisis.

At other times, sickle cell slips in under the guise of a common cold. What begins with mild signs of a viral infection can suddenly turn serious as the marrow falls silent. The haemoglobin, already fluctuating at baseline, drops sharply, which can leave any sickle cell warrior severely fatigued and drained of life. Diagnosis: aplastic crisis.

And then there are the curveballs. A patient with chest pain that is not clearly cardiac, not clearly a pulmonary embolism or a pneumonia but could be all or none of the above. Diagnosis: acute chest crisis.

Another patient who suddenly cannot see well, actively bleeding at the back of the eye with worsening vision loss, silently cutting off one of life’s key senses. Diagnosis: sickle cell retinopathy.

For clinicians, these presentations can look like many different conditions, but they all fall under the same diagnosis: sickle cell. The challenge is recognising it in time.

Anyone can be born with sickle cell, but it is most common amongst people of Black African and Black Caribbean heritage in the UK. It also disproportionately affects people of South Asian, Mediterranean and Middle Eastern heritage.

At its core, the problem lies in the shape of the red blood cell. Instead of staying round and flexible, the cells change into rigid sickles. To complicate matters further, they are stickier than normal and create more inflammation. Together, these changes clog vessels, starve tissues of oxygen, and leave patients vulnerable to a cascade of complications. With such a condition, what begins as 1 symptom such as pain or tiredness can at any time become something much more dangerous: stroke, sepsis, or multi-organ damage. And although a chronic, lifelong condition, sickle cell does not always give second chances.

Why rapid recognition and treatment saves lives

This is why triage is not just a process; it is part of the treatment itself. The gold standard is clear: patients should be prioritised highly in emergency departments. They should be seen quickly, given strong pain relief within 30 minutes, as recommended by the National Institute for Health and Care Excellence, and assessed for red flags without delay. Every minute counts. Audits remind us that when those targets are missed, outcomes worsen and trust erodes. Patients remember the hours they waited in pain, long after the crisis has passed.

Specialist units: making a real difference

But there are glimmers of progress. Across England, emergency department bypass units are leading the way in areas such as London and Manchester. Patients can go straight to a specialist team instead of waiting in A&E. The difference is striking. I have seen patients walk into these units in real distress and leave hours later, comfortable and most importantly, feeling heard. One patient put it best: “It felt like someone finally understood”. That is what well-designed services can deliver, not just timely pain relief but dignity. As a doctor who oversees the day-to-day care of such a unit, I can say first-hand they provide not only a space for patients to be treated but for clinicians and healthcare staff to have the time to listen and provide patient-centred care.

Elsewhere in the country, patients may not have access to dedicated units. They may rely on general emergency departments or smaller haematology teams. But the standard of care should not drop no matter where you are. Whether you are in London, Manchester, Birmingham, or a coastal town, the principles are universal: work with empathy, spot the signs, treat quickly, escalate when needed.

Sickle cell disease is the most common inherited blood condition in the UK, and it is hiding in plain sight. It may sound like a dozen different pathologies, but it is 1 condition, and recognising it quickly can be life-saving. In some NHS trusts, sickle cell management is embedded into simulation training, ensuring it stays at the tip of the tongue, front and centre with all other differential diagnosis. In others, guidelines are displayed in every corridor. And increasingly, patients are leading the way, helping us to make the right choices in how we improve services. All we have to do is engage, listen, and act, both on the good and the bad. If we built houses only on feedback from the sunny days, we would all be freezing when the cold came. This is why their feedback holds the key.

For patients, every encounter is a test not just of the healthcare system, but of whether they are believed and respected. For clinicians, every encounter is an opportunity. The first half hour can transform not only a crisis but a life. Quick recognition is not just protocol. It is compassion. It is care. And sometimes, it is survival.

Meeting communities where they are

Sickle cell care is not just clinical, it is cultural. Many patients come from communities where mistrust of healthcare runs deep, shaped by years of not being heard. Real education and reassurance often happen outside the clinic room, in churches, schools, barber shops, the group chats and DMs (direct messages) in the everyday spaces where people feel safe to ask the questions, they might not ask a doctor. If we want to change outcomes, we must be willing to meet patients there too.

As for me, I hope to shape the future of this care from a new angle. I want to become a specialist red cell GP, building a true bridge between community and hospital care as specialist support increasingly shifts into the community. Not just in clinics, but in the places where conversations really happen. So, maybe I will catch you on the sidelines at 5-a-side football game, in the middle of a carnival dance, or on your favourite YouTuber’s podcast. Watch this space.

That is my goal, but for the rest of you clinicians who simply want to learn more, you can spot the signs of sickle cell early and understand how it should be treated by taking the free e-learning module on the NHS Learning Hub.

Publication reference BL00055