Sickle cell crisis

• 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 Acute Chest Syndrome]

Source

DONALDSON 6/07

SOTELO�10/09

TUNG 7/10

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