Sickle Cell Anaemia

Key facts

• Sickle cell anaemia is an inherited disorder in which red blood cells carry an abnormal haemoglobin that makes them rigid and crescent-shaped instead of soft and flexible.
• These misshapen cells snag in small blood vessels, cutting off blood flow and causing recurring painful episodes, chronic anaemia, and gradual damage to organs including the spleen, kidneys, and lungs.
• Hydroxycarbamide, also known as hydroxyurea, is the best-established medicine for reducing how often these crises happen.
• A painful erection lasting more than four hours, sudden severe headache, vision loss, or one-sided weakness needs emergency care immediately.

What is actually going on

Sickle cell anaemia is inherited, passed down through genes that code for haemoglobin, the protein in red blood cells that carries oxygen. The abnormal form it produces makes red cells stiff and crescent-shaped rather than the flexible disc shape that normally slides smoothly through blood vessels. These rigid cells snag in small vessels and block blood flow, producing recurring vaso-occlusive crises, painful episodes that can strike almost any part of the body, alongside chronic anaemia and progressive damage to organs including the spleen, kidneys, and lungs.

Reducing crises with medicine

The main goal of long-term management is to cut the frequency and severity of vaso-occlusive crises. Hydroxycarbamide is the best-established medicine for this: it raises levels of foetal haemoglobin, a form that does not sickle, and has been shown to reduce hospital admissions and extend life expectancy. Because sickle cell anaemia sits within the broader field of blood disorder management, it often appears alongside medicines listed under oncology support.

Practical steps that help

Staying well-hydrated, avoiding extreme temperature changes, and keeping up with vaccinations (the spleen is often damaged early, raising infection risk) are practical steps that complement medical treatment.

When to seek emergency care

Anyone experiencing a sudden severe headache, vision loss, one-sided weakness, or a painful erection lasting more than four hours should seek emergency care immediately; each can signal a serious complication of the disease.

This page is educational and does not replace advice from a doctor or pharmacist who knows your health history.