Sickle cell disease

Red blood cells that change shape and block vessels. The commonest inherited condition in the UK, and its pain is routinely disbelieved.

What it is

Sickle cell disease is the most common inherited condition in the UK, affecting around 17,000 people, most of African and Caribbean heritage.

A change in haemoglobin makes red cells become rigid and crescent-shaped under stress. They block small blood vessels, which starves tissue of oxygen and causes a sickle cell crisis: pain that patients consistently describe as among the worst there is.

The 2021 No One's Listening report by the All-Party Parliamentary Group found substandard care, negative attitudes and racism affecting sickle cell patients in the NHS, and it linked those failures to avoidable deaths. Any entry about this condition that leaves that out is incomplete.

Signs you might notice

A crisis: severe pain, most often in the back, chest, arms or legs, coming on over hours.

Fever, which in sickle cell is an emergency because of the risk of overwhelming infection.

Chest pain and breathlessness, which may be acute chest syndrome and is life-threatening.

Anaemia, jaundice, and fatigue between crises.

Sudden weakness, speech change or vision loss, because stroke risk is significantly raised, including in children.

How it can affect day-to-day life

Pain relief is the flashpoint. People arrive in agony, request the opioid dose their own care plan specifies, and are treated as drug-seeking. That is the specific failure the parliamentary report documented.

Every person with sickle cell should have an individual care plan, and it should be followed rather than second-guessed by whoever is on duty.

Crises are triggered by cold, dehydration, infection, stress and sudden temperature change, so ordinary things such as a cold waiting room genuinely matter.

Supporting someone well

Believe the pain and treat it fast. NICE requires pain relief within 30 minutes of presentation in an acute crisis, and that standard exists because it was routinely missed.

Carry and present the individual care plan, and advocate for it firmly.

Keep warm and hydrated. Avoid sudden cold, and plan for swimming, air conditioning and winter.

Treat fever as an emergency, always.

Take penicillin prophylaxis and keep vaccinations current, because the spleen does not work properly.

Ask about hydroxycarbamide, which reduces crises substantially and is not offered to everybody who would benefit.

Ensure children have transcranial doppler scanning to assess stroke risk.

Where to get help

Ask for care from a specialist haemoglobinopathy centre, not general services alone.

The Sickle Cell Society provide advocacy and support, and their material on challenging poor care is practical.

Severe pain, fever, chest pain or breathlessness needs emergency assessment.