Sickle Cell disease

Sickle cell disease is a hemoglobin disease, which results in a deformation of the red blood cells which take a crescent shape (or sickle). These abnormal red blood cells block small blood vessels, causing pain (especially in the bones), which can lead to what is called vaso-occlusive crises. The abnormal red blood cells are destroyed, resulting in anemia. Finally, patients are susceptible to infections.

Sickle cell anemia is a serious genetic disease that affects us all. Sickle cell disease is the most common genetic disease in the world, affecting tens of millions of people. In sub-Saharan Africa, nearly 2% of newborns have sickle cell disease. In European capitals, 1 in 2,000 people are born with the disease. In France, the number of annual births of children with sickle cell disease is estimated at 350. Sickle cell disease has now become the leading genetic disease in France, with around 20,000 patients

The sickle cell patient must drink abundantly, especially during physical exertion and hot weather. He must limit cold baths and cover himself as soon as the temperature is low. If he has a fever, it should be brought down quickly. He should avoid as much as possible air travel and altitude above 1500 meters. Prevention of factors triggering attacks (cold, altitude, infections, dehydration)

Treatment of vaso-occlusive crises:

  • analgesics and oxygenation
  • Folate supplementation (folic acid = vitamin B9)
  • Preventive treatment of pneumococcal and meningococcal infections by vaccination
  • Blood transfusion in case of deep anemia or severe infection
  • Bloodletting transfusion to reduce the proportion of hemoglobin S

Most people with sickle cell disease can live normally with good prevention and inexpensive treatment (painkillers, folates and antibiotics).

When sickle cell disease patients have frequent complications (painful vaso-occlusive crises repeated in the year, splenic sequestrations, acute thoracic syndrome), other treatments are available. These treatments can in some cases significantly improve the disease, but are never trivial and are only offered when the disease is considered severe. These are hydroxyurea, transfusion programs, and possibly bone marrow transplant.

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