Drugs that aim to reduce the loss of water from red blood cells in people with sickle cell disease

Sickle cell disease is an inherited condition that causes red blood cells to become sickle shaped when they lose water. This leads to a high risk of the blood vessels becoming blocked. Such blockages can cause pain, stroke and damage to organs. Recent therapies aim to stop the cells becoming sickle shaped by preventing them losing water. This review included two studies, one with zinc sulphate and the other with senicapoc. The study with zinc sulphate showed that this drug may be able to reduce the number of sickle cell crises without causing toxic effects. There were 145 participants in this study and results showed a significant reduction in the total number of serious sickle-related crises over one and a half years, mean difference -2.83 (95% confidence interval -3.51 to -2.15). However, our analysis was limited since not all data were reported. Changes to red cell measurements and blood counts were not consistent. No serious adverse events were noted in the study. The study with senicapoc demonstrated that this drug increases the red blood survival and has a role in the prevention on red blood cell dehydration in people with sickle cell disease. The higher dose of the drug was more effective compared to the lower dose. But these changes in the red blood cells did not translate into positive clinical outcomes in terms of reduction in the number of sickle cell crises. Senicapoc had a favourable safety profile. More longer-term research is needed on these drugs and others that might prevent water loss in red blood cells.