Haematology
Sickle cell disease: from screening to crises and lifelong follow-up
Sickle cell disease is an inherited disorder of haemoglobin. Some red blood cells become less flexible, which can lead to anaemia, painful crises and organ damage. Carrying the sickle cell trait and having the disease are not the same situation.
Written by the Polycrabat Sevamed team · Published on · Updated on
In brief
- The diagnosis is based on a specific analysis of haemoglobin.
- A fever in a person with sickle cell disease requires prompt assessment.
- Follow-up aims to prevent complications, not just to relieve crises.
What happens during a painful crisis?
Blockage of small blood vessels can cause intense pain, often in the bones. The assessment also looks for infection, dehydration or an acute complication. Pain relief and hydration are adapted to the person’s condition; not all crises are treated in the same way.
Which signs call for emergency care?
Fever, chest pain, breathlessness, weakness on one side, difficulty speaking, severe abdominal pain or marked pallor require urgent assessment. Acute chest syndrome and stroke are serious complications. A “usual” crisis should not lead anyone to ignore a new symptom.
How can complications be prevented?
Vaccinations, infection prevention, monitoring of anaemia, organ surveillance and long-term treatments are individualised. Hydroxyurea may be offered depending on the form of the disease and the patient’s profile. Transfusions have specific indications and require monitoring of their risks. Certain specialised options apply to selected situations.
How should the care pathway be organised?
A record summarising the genotype, previous complications, transfusions, treatments and instructions in case of fever makes continuity of care easier. The transition from paediatric to adult care should be prepared, as should plans for pregnancy and travel.
Useful questions to ask
- Which form of sickle cell disease has been confirmed?
- What is my personal action plan in case of fever or chest pain?
- Which organs need to be checked regularly?
Frequently asked questions
Does having the sickle cell trait mean that you are ill?
No. Carrying the trait and the forms of the disease must be distinguished by testing.
Is all pain a sickle cell crisis?
No. Another cause, sometimes urgent, must be considered depending on the symptoms.
Can a person with sickle cell disease fly to receive treatment?
Often yes, after medical advice and with appropriate precautions: good hydration, protection from the cold, regular movement and, depending on the case, oxygen during the flight. A recent crisis, fever or chest pain must be assessed before departure.
This content provides general medical information. It does not replace a consultation or a personalised decision by your care team. In case of serious or sudden symptoms, contact the emergency services in your country or a doctor without delay.
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How is the disease confirmed?
Newborn screening, where available, allows early care. In children and adults, a haemoglobin study distinguishes the different forms of the disease from simply carrying the trait. Genetic counselling helps families understand how it is inherited, without presuming anyone’s choices.