Sickle cell crisis: Difference between revisions
(Created page with "==Background== * Sickle cell disease is the most common monogenetic disorder worldwisde affecting 30 million people * disease can manifest with vaso-occlusion (pain), acute-che...") |
No edit summary |
||
| Line 3: | Line 3: | ||
* 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-occlusion (pain), acute chest syndrome, stroke, aplastic anemia, priapism | ||
| Line 19: | Line 19: | ||
* pregnancy | * pregnancy | ||
* exertional stress | * exertional stress | ||
* | * For aplastic crisis: | ||
* parvovirus B19 infection | * parvovirus B19 infection | ||
* folic acid deficiency | * folic acid deficiency | ||
| Line 39: | Line 39: | ||
* CBC/ | * CBC / stat hemaglobin | ||
* | * Assess change in anemia | ||
* | * Chemistry / LFT / lipase | ||
* | * If abd pain, may develop cholecystitis, mesenteric ischemia, or perforation | ||
* O2 | * 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 | * ECG | ||
* T&S/T&C | * T&S/T&C | ||
* BCx2 | * BCx2 | ||
* | * VBG | ||
* UA | * UA | ||
* | * Urine pregnancy | ||
* Head CT/MRI if symptoms of stroke | * Head CT/MRI if symptoms of stroke | ||
| Line 60: | Line 64: | ||
* appendicitis | * appendicitis | ||
* cholecystitis | * cholecystitis | ||
* | * gout | ||
* hepatitis | * hepatitis | ||
* meningitis | * meningitis | ||
| Line 79: | Line 83: | ||
* Abd Pain | * Abd Pain | ||
* Aplastic Anemia | * Surgery if abdominal injury | ||
* Pain crisis | * Aplastic Anemia | ||
* Priapism | * Exculde reverible causes and tranfuse for severe anemia (Hb <6-7) | ||
* Acute chest syndrome | * 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 | |||
| Line 92: | Line 101: | ||
* 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 | * Unable to tolerate PO or inadequate pain control, abnormal vital signs | ||
* Consider if pregnant (in crisis), | * Consider if pregnant (in crisis), or uncertain dx of SCD | ||
Revision as of 23:42, 1 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