Sickle cell crisis

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Background

  • Sickle cell disease is the most common monogenetic disorder worldwisde affecting 30 million people
  • Disease can manifest with vaso-occlusion (pain), acute chest syndrome, stroke, aplastic anemia, priapism


Precipitating Factors

  • For vaso-occlusion:
  • cold weather
  • hypoxia
  • infection
  • dehydration
  • acidosis
  • alcohol intoxication
  • emotional stress
  • pregnancy
  • exertional stress
  • For aplastic crisis:
  • parvovirus B19 infection
  • folic acid deficiency
  • For acute chest syndrome:
  • fat embolus
  • infection
  • pain crisis
  • asthma


Diagnosis

Insert


Work-Up

  • CBC / stat hemaglobin
  • Assess change in anemia
  • Chemistry / LFT / lipase
  • If abd pain, may develop cholecystitis, mesenteric ischemia, or perforation
  • Reticulocyte count (if aplastic crisis considered) -rare in adults
  • CXR
  • If cough, sob, or febrile
  • O2
  • If pain crisis or hypoxic; otherwise may inhibit erythopoesis
  • ECG
  • T&S/T&C
  • BCx2
  • VBG
  • UA
  • Urine pregnancy
  • Head CT/MRI if symptoms of stroke


DDx

  • ACS
  • anemia (acute, chronic)
  • appendicitis
  • cholecystitis
  • gout
  • hepatitis
  • meningitis
  • osteomyelitis
  • pancreatitis
  • PID
  • pneumonia
  • priapism
  • pulmonary embolism/infarction
  • rheumatic fever
  • sepsis
  • stroke (ischemic or hemorrhagic)
  • UTI
  • URI


Treatment

  • Abd Pain
  • Surgery if abdominal injury
  • Aplastic Anemia
  • Exculde reverible causes and tranfuse for severe anemia (Hb <6-7)
  • Pain crisis
  • IVF, pain meds, O2 if hypoxic
  • Priapism
  • Pain meds, exchange tranfusion before surgery, urology consult
  • Acute chest syndrome
  • Admit if infiltrate, treat pain, give IV Abx
  • Stroke
  • Head CT +/- LP, IVF, keep HbS <30% total blood vol


Disposition

  • Admission if:
  • Acute Chest Syndrome - pain/pulmonary infiltrates from infection or pulm infarct
  • Stroke, priapism, serious bacterial infection, aplastic crisis, hypoxia, acidosis
  • Unable to tolerate PO or inadequate pain control, abnormal vital signs
  • Consider if pregnant (in crisis), or uncertain dx of SCD


See Also

Acute Chest Syndrome


Source

DONALDSON 6/07

SOTELO 10/09

TUNG 7/10

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