What should you know about sickle cell anemia and sickle cell disease?

Sickle cell disease describes a group of inherited haemoglobin conditions. Sickle cell anaemia commonly refers to the form in which a person has two haemoglobin genes that produce substantial sickling. The altered cells can become rigid and obstruct small blood vessels, reducing oxygen delivery to tissues. This can lead to episodes of joint or bone pain, fatigue, and pale skin, although symptoms and severity vary between people.

A diagnosis is based on testing rather than symptoms alone. A blood test can identify haemoglobin types and help separate sickle cell disease from carrier status or another cause of anaemia. A clinician may also review previous episodes, family history, and current medicines.

If you have a new or uncertain result, arrange a review with a clinician familiar with haemoglobin disorders. Discuss a plan for pain episodes, hydration, vaccinations, infection prevention, and regular monitoring when relevant to your diagnosis. Share the result with close relatives and consider premarital screening or genetic counselling when planning a family. Never assume that iron will correct anaemia unless iron deficiency has been confirmed.

Get urgent help for severe pain, fever, chest pain, breathing difficulty, sudden weakness, confusion, vision change, or a new severe headache. A painful erection that does not settle also needs urgent assessment. Seek advice promptly for worsening fatigue, yellowing of the eyes, dehydration, or an infection because sickle cell disease can make complications develop quickly.

Ask which sickle cell type is present, how the diagnosis was confirmed, and which symptoms should trigger your personal emergency plan. Ask whether relatives or a partner should have a blood test, how often follow-up is needed, and which treatments or preventive measures fit your health history.