Most with a sickle-cell crisis present with severe, acute bone pain secondary to ischaemic bone marrow necrosis. Get an urgent haematology consult
- Also see topic on Sickle cell. Follows an acute pain crisis
- Seen in 50 percent of people with SCD
- Commonest with HbSS or HbS-ß0 thalassemia
- Triggered by vaso-occlusive crisis (VOC) and
- Chest Infection/Pneumonia
- Fat embolism from necrotic bone marrow.
- Pulmonary infarction
- New Rib|Sternal|Thoracic vertebral pain
- Bilateral chest signs - crepitations
- Increased resp rate > 20/min
- Hypoxia, Falling Hb concentration
- FBC: anaemia and leucocytosis and elevated reticulocytes
- LFT: elevated bilirubin
- CXR: Bilateral infiltrates
- Sickle solubility test: positive
- Electrophoresis: HbS±HbC or HbD with no HbA (except in HbS/ß+ thalassaemia).
- Pneumococcal septicaemia
- Splenic sequestration
- Erythrovirus infection associated with marrow aplasia
- Acute folate deficiency.
- ABC, respiratory support, HDU bed,Aggressive pain relief
- Exchange blood transfusion to reduce the amount of HbS to <20%.
- Maintenance of oxygenation: consider CPAP or IPAP
- Intravenous antibiotic therapy may be needed.
- Prevent: transfusion and Hydroxycarbamide/Hydroxyurea