Quick read
Key points
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Sickle cell disease is an inherited condition affecting red blood cells, causing them to become rigid and crescent (sickle) shaped instead of round and flexible. These misshapen cells can block blood flow, causing pain, and break down more quickly, leading to anaemia.
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Topic-specific prevention and risk reduction will be added during the article-specific evidence audit.
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You experience a sudden episode of severe pain, known as a sickle cell crisis
01 · Know
What is Sickle cell disease?
Sickle cell disease is an inherited condition affecting red blood cells, causing them to become rigid and crescent (sickle) shaped instead of round and flexible. These misshapen cells can block blood flow, causing pain, and break down more quickly, leading to anaemia.
It's present from birth and requires lifelong management. People with sickle cell disease can experience episodes of severe pain called 'sickle cell crises', along with increased risk of infections and other complications.
02 · Understand
Clinical context
Self-care advice
People with sickle cell disease should have regular follow-up with a specialist haematology team to monitor the condition and manage complications.
03 · Act
When to see a doctor
- 1You experience a sudden episode of severe pain, known as a sickle cell crisis
- 2You develop a fever, which can indicate a serious infection in someone with sickle cell disease
- 3You have chest pain or breathing difficulty, which needs urgent assessment
- 4You notice signs of worsening anaemia, such as increasing fatigue or pale skin
- 5You're planning a pregnancy or travel and want advice specific to sickle cell disease
04 · Prevent & manage
Practical steps
Take these self-care steps
These measures can help ease symptoms, support recovery, and reduce the chance of things worsening — but they don't replace medical advice if symptoms are severe or persistent.
- 1Stay well hydrated.Good hydration helps reduce the risk of a sickle cell crisis by keeping blood flowing more easily.
- 2Avoid known triggers.Extreme cold, dehydration, high altitude, and excessive exertion can all trigger a crisis for some people.
- 3Take prescribed medication and vaccinations as advised.This includes pain management plans and vaccines to reduce infection risk, which is heightened in sickle cell disease.
- 4Manage pain promptly during a crisis.Following your agreed pain management plan and seeking help early can prevent a crisis becoming more severe.
- 5Attend regular specialist reviews.Ongoing care from a haematology team helps monitor for complications and adjust treatment as needed.
Evidence
References
BarnMD Health Intelligence uses authoritative and clinically relevant sources appropriate to the topic. References are shown so readers can inspect the evidence directly.
- HSE: Sickle cell disease ↗
Medical information notice
Health education, not individual medical advice
BarnMD Health Library provides general health education for public health literacy. Reading this article does not establish a doctor-patient relationship and this information is not a diagnosis, prescription, or personalised treatment plan. If you have symptoms or concerns about your health, seek appropriate advice from a qualified healthcare professional. If you develop emergency warning signs, seek urgent or emergency medical attention.
BarnMD Bottom Line
Sickle cell disease is a lifelong condition, but with good management, many people lead full lives while reducing the frequency and severity of complications.
Fever in someone with sickle cell disease should always be treated as a medical emergency, as infections can become serious very quickly.