Sickle cell crisis: Difference between revisions
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==Background== | |||
* Sickle cell disease is the most common monogenetic disorder worldwisde affecting 30 million people | * Sickle cell disease is the most common monogenetic disorder worldwisde affecting 30 million people | ||
* Disease can manifest with vaso- | * Disease can manifest with vaso-occlusion(pain), acute chest syndrome, stroke, aplastic anemia, priapism | ||
==Precipitating Factors== | ==Precipitating Factors== | ||
* For vaso-occlusion: | |||
* For vaso-occlusion: | |||
** cold weather | ** cold weather | ||
** hypoxia | ** hypoxia | ||
| Line 13: | Line 13: | ||
** emotional stress | ** emotional stress | ||
** pregnancy | ** pregnancy | ||
** exertional | ** exertional stress | ||
* For aplastic crisis: | * For aplastic crisis: | ||
** parvovirus B19 infection | ** parvovirus B19 infection | ||
| Line 20: | Line 20: | ||
** fat embolus | ** fat embolus | ||
** infection | ** infection | ||
** pain | ** pain crisis | ||
** | ** asthma | ||
==Diagnosis== | |||
Insert | Insert | ||
== | ==Work-Up== | ||
* CBC / stat hemaglobin | |||
* CBC / | |||
** Assess change in anemia | ** Assess change in anemia | ||
* Chemistry / LFT / lipase | * Chemistry / LFT / lipase | ||
| Line 37: | Line 35: | ||
** If cough, sob, or febrile | ** If cough, sob, or febrile | ||
* O2 | * O2 | ||
** If pain crisis | ** If pain crisis or hypoxic; otherwise may inhibit erythopoesis | ||
* ECG | * ECG | ||
* T&S/T&C | * T&S/T&C | ||
* BCx2 | * BCx2 | ||
* | * VBG | ||
* UA | * UA | ||
* Urine | * Urine pregnancy | ||
* Head CT/MRI if symptoms of | * Head CT/MRI if symptoms of stroke | ||
==DDx== | |||
* ACS | * ACS | ||
* anemia (acute, chronic) | * anemia (acute, chronic) | ||
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* pulmonary embolism/infarction | * pulmonary embolism/infarction | ||
* rheumatic fever | * rheumatic fever | ||
* | * sepsis | ||
* stroke (ischemic or hemorrhagic) | * stroke (ischemic or hemorrhagic) | ||
* UTI | * UTI | ||
* | * URI | ||
==Treatment== | |||
* Abd Pain | * Abd Pain | ||
** Surgery if abdominal injury | ** Surgery if abdominal injury | ||
| Line 83: | Line 79: | ||
==Disposition== | ==Disposition== | ||
* Admission if: | * Admission if: | ||
** Acute Chest Syndrome - pain/pulmonary infiltrates from infection or pulm infarct | ** Acute Chest Syndrome - pain/pulmonary infiltrates from infection or pulm infarct | ||
** Stroke, priapism, serious bacterial infection, aplastic crisis, hypoxia, acidosis | ** Stroke, priapism, serious bacterial infection, aplastic crisis, hypoxia, acidosis | ||
** Unable to tolerate PO or inadequate pain control, abnormal vital signs | ** Unable to tolerate PO or inadequate pain control, abnormal vital signs | ||
* | ** Consider if pregnant (in crisis), or uncertain dx of SCD | ||
* Consider if pregnant (in crisis), or uncertain dx of SCD | |||
==See Also== | ==See Also== | ||
Acute Chest Syndrome | Acute Chest Syndrome | ||
==Source== | ==Source== | ||
DONALDSON 6/07 | DONALDSON 6/07 | ||
| Line 226: | Line 94: | ||
TUNG 7/10 | TUNG 7/10 | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Revision as of 17:43, 14 March 2011
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