Sickle Cell Disease

Sickle cell disease (SCD) is best understood as three linked problems rather than as pain alone: chronic haemolytic anaemia, intermittent vaso-occlusion, and progressive endothelial or vascular injury. A patient may arrive with severe pain, but the immediately dangerous complication may instead be sepsis, acute chest syndrome, stroke, splenic sequestration, or another process requiring a different first action. The practical sequence is therefore: identify the SCD genotype and baseline, look for the complication causing today’s change, treat time-sensitive threats without waiting for perfect diagnostic certainty, and use disease-modifying therapy and surveillance to reduce the next event. Canadian practice also requires attention to regional newborn-screening coverage, off-label hydroxyurea use, immunization against encapsulated organisms, and access to specialist transfusion or cellular-therapy programmes.