Vaso-occlusive pain crisis
Background
Precipitating Factors
- Stress
- Cold weather
- Dehydration
- Hypoxia
- Infection
- Acidosis
- Alcohol intoxication
- Pregnancy
- Exertional stress
Clinical Features[1]
- Pain
- Most common manifestation of SCA (79-91% of ED visits)
- Lower back, long bones most commonly affected
Differential Diagnosis
Sickle cell crisis
- Vaso-occlusive pain crisis
- Bony infarction
- Dactylitis
- Avascular necrosis of femoral head
- Acute chest syndrome
- Asthma
- Pulmonary hypertension
- Gallbladder disease
- Acute hepatic sequestration
- Infection
- Parvovirus B19
- Splenic sequestration
- CVA
- Cerebral aneurysm and ICH
- Priapism
- Papillary necrosis
Evaluation
- CBC
- Because anemia can precipitate a crisis, must check for acute hemoglobin drop
- Chemistry / LFT / lipase
- If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
- Retic count (if aplastic crisis considered - rare in adults)
- Should be >0.5%
- T&S/T&C
- Urine pregnancy
- CXR
- If cough, shortness of breath, or febrile
Management
- Analgesia
- IV opiods prefered (morphine or hydromorphone)
- Avoid IM route (use SQ if necessary)
- Avoid meperidine
- Use PCA pump if available
- Redose in 30min if inadequate
- Normally admit if needs more than three doses
- Use of concurrent acetaminophen encouraged, unless contraindicated
- IV opiods prefered (morphine or hydromorphone)
- Hydration
- Controversial to bolus IVF, rehydration can lead to atelectasis, pulmonary edema, and acute chest syndrome. Normal saline may also promote hyperchloremic metabolic acidosis, further precipitating sickling [2] Recent studies in children has shown potential worse pain control and increased admission associated with normal saline boluses [3] [4]
- oral rehydration, consider D5 1/2 NS at maintenance rate
- There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[5]
- O2 if hypoxic; otherwise may inhibit erythopoesis
Anemia
- Transfusion PRBCs 10 mL/kg over 2hr period
- Indications:
- Aplastic crisis
- Sequestration crisis
- hemoglobin <6 with inappropriately low retic count
- hemoglobin <10 with acute crisis
Disposition
Consider admission
- Acute chest syndrome is suspected
- Sepsis, osteomyelitis, or other serious infection is suspected
- Priapism, aplastic crisis, hypoxia, splenic sequestration
- WBC >30K
- Platlet <100K
- Pain is not under control after 3 rounds of analgesics in ED
- <1yr old
Consider discharge
- Pain is under control and patient can take oral fluids and medications
- Ensure appropriate oral analgesics are available
- Provide home care instructions
- Ensure resource for follow-up
See Also
External Links
References
- ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
- ↑ Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
- ↑ Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1)
- ↑ Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019.
- ↑ Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4