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

Abdo pain in sickle cell

  • gallstoens are common
  • all the usual infective causes
  • sequestration syndromes
  • mesenteric syndrome

others

  • hand-foot syndrome - small bones of hands and feet
  • aplastic crisis - associated with parvovirus B19
  • acute splenic sequestration
  • Priapism
  • avascular necrosis