Pathology of sickle cell disease explained

Haemoglobin normally helps red blood cells carry oxygen and remain flexible as they pass through narrow vessels. In sickle cell disease, altered haemoglobin can make cells curve into a sickle shape, especially during reduced oxygen, dehydration, infection or physical stress. These cells may stick together and slow circulation. The resulting tissue irritation can cause episodes of joint or bone pain. Because sickled cells are removed from the bloodstream sooner than healthy cells, the body may have fewer cells available to carry oxygen, contributing to fatigue and pale skin. The condition is inherited; it is not caused by something a person did.

A clinician may review pain episodes, tiredness, infections, breathing symptoms and family history. A blood test can examine haemoglobin and the appearance or type of red blood cells; further testing may clarify the inherited pattern. Drink regularly, follow the personal plan from your sickle cell team, keep recommended appointments and take prescribed medicines as directed. Ask about vaccines, infection prevention and safe activity. Premarital screening can help couples understand inherited blood conditions before having children. Useful background reading includes Sickle Cell Disease, Fatigue, Joint Pain, Pale Skin, Blood Test and Premarital Screening.

Seek urgent medical help for severe or worsening pain, fever, chest pain, difficulty breathing, sudden weakness, confusion, a seizure, severe headache or marked dehydration. In a child, unusual sleepiness, poor feeding or breathing difficulty also needs prompt assessment. These symptoms can signal infection, reduced blood flow or another complication and should not be managed by waiting for a routine appointment.

Ask which sickle cell pattern you have, how to recognise your early warning signs, what to do during a pain episode, and which medicines or vaccines are appropriate. You can also ask whether relatives should be offered testing and how screening may affect family planning.