Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Table 1

Approaches to the management of sickle cell disease and its complications.

Approach	Definition
(1) Supportive management	Management intended to maintain the essential requirements for good health such as balanced diet, sleep, hydration, and folic acid
(2) Symptomatic management	Management targeted to alleviate the symptoms of the disease as they occur. These include blood transfusion for symptomatic anemia, analgesics for pain, and antibiotics for infections
(3) Preventative management	Approaches to prevent the occurrence of complications of the disease. These include things like vaccination, avoidance of stressful situations, Hb F induction with hydroxyurea or other agents, and transfusion to prevent the recurrence of stroke
(4) Abortive management	Major purpose of this approach is to abort painful crisis thus preventing them from getting worse or precipitating other complication. The only promising abortive approach has been nitric oxide
(5) Curative therapy	This is the ultimate goal of all inherited disorders. This has already been achieved in SCD by stem cell transplantation. Gene therapy is another challenging goal