Acute presentations of sickle cell disease
assessment
- Signs of local and systemic infection
- Baseline and current Hb
- CXR if fever, chest pain, hypoxia
- CT head if stroke suspected
management
Oxygen
Hydration
Transfusion aiming Hb >50
vaso-occlusive crises - most common
| Headline | |
|---|---|
| Etiology | sickled cells → tissue infarction. Precipitated by dehydration, hypoxia, infection. |
| Clinical presentation | Severe pain |
| Diagnostic investigations | - may not reflect in Hb or retics acutely |
| Management | - poor evidence for any specific analgesic strategy - follow pain ladder; unlikely opioid naive - avoid entonox - rehydration |
acute chest syndrome - most deadly
| Headline | |
|---|---|
| Etiology | |
| Epidemiology | |
| Clinical presentation | Hypoxia, chest pain Ddx: every other cause of chest pain and hypoxia... |
| Pathogenesis | Lung infarction |
| Diagnostic investigations | |
| Management | Empirical abx? (this is not necessarily a septic problem) |
others
- hand-foot syndrome - small bones of hands and feet
- aplastic crisis - associated with parvovirus B19
- acute splenic sequestration
- Priapism
- avascular necrosis