The sickling process in relation to clinical manifestations.

Knowledge of the detailed molecular mechanisms of the sickling process has led to an understanding of factors precipitating crises and hence allowed a reasoned approach to therapy. At present amelioration of the symptoms of sickle cell disease rests primarily on the avoidance and prompt treatment of the basic precipitating factors of the sickling process--fever, hypoxia, acidosis, and dehydration. Over the years many drugs have been claimed to have anti-sickling properties but have not stood the test of time. The current partial success of cyanate stems directly from this molecular knowledge. As further details of the site of intermolecular binding involved in the sickling process become available the possibility of developing more specific anti-sickling drugs becomes clearer.