Therapy

In the case of homozygous carriers, an attempt can be made to integrate the cultivation of normal erythrocytes in the body with an allogenic stem cell transplant. For this purpose, blood-forming stem cells are transferred to a sibling or a stranger, which then take over the (correct) blood formation. This is also done, for example, in the case of malignant blood diseases such as leukemia. All in all, a person affected should avoid oxygen deficiency and, in case of a hemolytic crisis, receive adequate intensive medical care. This includes, for example, adequate fluid substitution or a blood transfusion (to ensure that the oxygen transport in the blood is restored).

Prognosis

Due to its genetic cause, sickle cell anaemia is in principle an incurable disease. Only the attempt of a stem cell transplantation can be undertaken. Depending on the level of development in the country of the affected person, people have a lower life expectancy. Hemolytic crises, for example, caused by infections, are most likely to lead to death from organ failure. Regular vaccinations and prompt hospital care help to improve the prognosis.

Special features during pregnancy

Only homozygous patients show problems during pregnancy due to their sickle cell anemia: The increased risk of thrombosis should be mentioned first and foremost, but this does not require drug treatment such as anticoagulation (coagulation inhibition), but only close monitoring (twice as often as in healthy people).Patients with sickle cell anemia do not have an increased risk of abortion or premature birth, the children only have a lower birth weight compared to the average. Absolute contraindication (contraindications) for pregnancy is if the affected person has already suffered severe complications from her disease such as strokes and chronic insufficiency of heart, lungs or kidney. Furthermore, it has been observed in sickle cell patients that for unknown reasons they suffer certain pain episodes during pregnancy. Here, analgesic (pain-relieving) therapy is used as in other pregnant women. The study situation of pregnant sickle cell patients and their children is still incomplete and needs further comprehensive research in the future.