Therapeutic targets
- Crisis avoidance
- Treatment of organ damage
Therapy recommendations
- Adequate fluid intake and administration of analgesics (painkillers) during pain crises.
- Administration of hydroxyurea (hydroxyurea) to treat pain crises. This can reduce both the number and intensity of pain crises and the number of episodes of acute chest syndrome (ATS; acute clinical picture characterized by fever, cough, chest pain (chest pain), tachypnea (excessive respiratory rate), leukocytosis (increase in the number of white blood cells), and pulmonary infiltrates/signs of inflammatory processes in the lungs) and reduce mortality (death rate)
- In suspected sepsis (blood poisoning) antibiotic administration with special consideration of pneumococci; in suspected osteomyelitis with consideration of salmonellae
- Transfusion therapy (administration of blood transfusions) Indications: aplastic crisis (complete suppression of blood formation for a limited time), large splenic sequestration (“seepage of a large amount of blood in the spleen“), acute thoracic syndrome (ATS) and before surgical interventions (raise Hb to 10 g/dl!).
- Bloodletting to reduce high viscosity: about 35-40% of HbSC patients benefit. Bloodletting for the remaining phenotypes: Hb values > 10 g/dl and symptoms such as increased pain, hearing loss, tinnitus (ringing in the ears) or vertigo (dizziness) occur.
- Hydroxyurea (synonym: hydroxycarbamide): prophylaxis of pain crises and acute chest syndrome (ATS).
- See also under “Other therapy.”
Further notes
- Patients with serious cardiovascular disease or those who have had a previous myocardial infarction or apoplexy should avoid daily doses of 2,400 mg or more of ibuprofen. Increased cardiovascular risk was not found at doses up to 1,200 mg per day.
- In patients in Africa, hydroxyurea did not increase susceptibility to malaria or other infections: Incidence of malaria infections actually decreased from 46.9 to 22.9 per 100 patient-years (IRR 0.49; 0.37-0.66); nonmalarial infections decreased from 142.5 to 90.0 events per 100 patient-years (IRR 0.62; 0.53-0.72).
- The drug voxelotor decreased hemolysis in patients with sickle cell disease in a phase III study. When treated with voxelotor, Hb levels increased to greater than 10 g/dL in 41% of treated patients (versus 9% in the placebo group).Mode of action: Preventing the polymerization of hemoglobin S (HbS) in erythrocytes by increasing oxygen binding to the hemoglobin molecule.
