What should you know about effects of sickle cell disease?

In sickle cell disease, some red blood cells become rigid and crescent-shaped. They can break down earlier than usual and may obstruct circulation in small vessels. This can reduce oxygen delivery and produce anaemia, fatigue, pale skin, jaundice or breathlessness. Blocked blood flow can cause sudden episodes of severe pain in bones, joints, chest or the abdomen.

Over time, the condition may affect the lungs, kidneys, eyes, bones or spleen. The pattern differs between people. Dehydration, infection, cold exposure, stress or low oxygen can trigger problems, although an episode can occur without an obvious trigger.

Follow the care plan from your haematology team, take preventive medicines as prescribed and keep vaccinations and scheduled checks up to date. Drink regularly, dress for cold weather, pace strenuous activity and seek advice early for fever or infection symptoms. Keep a record of pain, fatigue, medicines and possible triggers.

A blood test can help assess anaemia and other blood changes. Premarital screening can help couples understand inherited haemoglobin traits before planning a family. Ask which tests and specialist reviews apply to you.

Seek emergency care for chest pain, trouble breathing, sudden weakness or numbness, confusion, severe headache, a seizure, severe persistent pain, a high temperature, marked pallor or unusual sleepiness. A painful erection lasting several hours is also urgent. Do not wait for a routine appointment when symptoms are sudden, severe or different from your usual episodes.

Which symptoms suggest a pain crisis or infection? How should I use my pain plan? Which organs need monitoring, and should relatives or a partner consider a blood test or premarital screening?