What are the symptoms of sickle cell disease?
When do the symptoms of sickle cell disease first appear?
The first symptoms of sickle cell disease usually appear from the age of 5 to 6 months.
All babies develop foetal haemoglobin whilst in the womb and are born with it. This haemoglobin normally carries oxygen, so the baby shows no symptoms at birth. The baby then gradually begins to produce what is known as ‘adult’ haemoglobin. This process takes around 4 to 6 months, after which the first symptoms will appear.
Learn more: What should people with sickle cell disease avoid?
What are the clinical signs of sickle cell disease?
The first clinical signs in children are often painful episodes (vascular occlusive crises) resulting from the blockage of blood vessels by deformed red blood cells. In addition, signs observed include swelling of the hands and feet (hand-foot syndrome), an enlarged spleen which the doctor can detect during a consultation, and a yellow rather than white colour of the iris.
Other signs in children also include pale mucous membranes, a consequence of anaemia, and growth retardation.
One of the most significant risks during childhood is the risk of infection; therefore, it is essential to ensure that children receive their full course of vaccinations as soon as possible and also to have antibiotics available to treat infections.
Pain is a characteristic symptom of sickle cell disease. It results from the blockage of blood vessels by deformed red blood cells. This pain can be severe (lasting from a few hours to a few days) and is often triggered by changes in temperature, dehydration, or a lack of oxygen. It can also develop into what is known as chronic pain (lasting several weeks or months).
These circulatory problems can cause damage to various organs (liver, spleen, brain, lungs, bones, eyes, etc.)
Laboratory tests are essential to confirm the diagnosis and manage patients.
Learn more: Why do people with sickle cell disease need to drink plenty of water?
How does sickle cell disease progress?
The progression of sickle cell disease varies greatly from one patient to another; some patients experience periods of relative well-being interspersed with acute, painful crises, whilst others suffer from persistent chronic symptoms.
Without treatment, sickle cell disease can lead to serious complications such as damage to the bones and joints, lung, heart and kidney damage, as well as complications affecting the spleen and liver…
The progression of the disease depends partly on adherence to prescribed treatments and also on the precautions taken in daily life to prevent crises from occurring.
Learn more: How to live with sickle cell disease?
References ___________________________________________________________
Organisation Mondiale de la Santé. (2020). Drépanocytose.
National Heart, Lung, and Blood Institute. (2022). What Is Sickle Cell Disease?
Centers for Disease Control and Prevention. (2021). Sickle Cell Disease.