Sickle cell crisis: Difference between revisions
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==Background==
==Background==
===Precipitating Factors ===
===Precipitating Factors ===
#For vaso-occlusion:
*For vaso-occlusion:
##Stress
**Stress
##Cold weather
**Cold weather
##Dehydration
**Dehydration
##Hypoxia
**Hypoxia
##Infection
**Infection
##Acidosis
**Acidosis
##Alcohol intoxication
**Alcohol intoxication
##Pregnancy
**Pregnancy
##Exertional stress
**Exertional stress
#For aplastic crisis:
*For aplastic crisis:
##Parvovirus B19 infection
**Parvovirus B19 infection
##Folic acid deficiency
**Folic acid deficiency
#For acute chest syndrome:
*For acute chest syndrome:
##Fat embolus
**Fat embolus
##Infection
**Infection
##Pain crisis
**Pain crisis
##Asthma
**Asthma


== Work-Up ==
== Work-Up ==
#CBC
*CBC
##Assess for significant anemia
**Assess for significant anemia
#Chemistry / LFT / lipase
*Chemistry / LFT / lipase
##If abd pain, may be cholecystitis, mesenteric ischemia, or perforation
**If abd pain, may be cholecystitis, mesenteric ischemia, or perforation
#Retic count (if aplastic crisis considered - rare in adults)
*Retic count (if aplastic crisis considered - rare in adults)
##Should be >0.5%
**Should be >0.5%
#CXR
*CXR
##If cough, SOB, or febrile
**If cough, SOB, or febrile
#O2
*O2
##If hypoxic; otherwise may inhibit erythopoesis
**If hypoxic; otherwise may inhibit erythopoesis
#ECG
*ECG
#T&S/T&C
*T&S/T&C
#BCx2
*BCx2
#VBG
*VBG
#UA
*UA
#Urine pregnancy
*Urine pregnancy
#Head CT/MRI
*Head CT/MRI
##If symptoms of stroke
**If symptoms of stroke


==Clinical Manifestations==
==Clinical Manifestations==
=== Vaso-Occlusive Crisis<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref> ===
=== Vaso-Occlusive Crisis<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref> ===
#Pain
*Pain
##Most common manifestation of SCA (79-91% of ED visits)
**Most common manifestation of SCA (79-91% of ED visits)
##Lower back, long bones most commonly affected
**Lower back, long bones most commonly affected
##Because anemia can precipitate a crisis, must check for acute Hb drop
**Because anemia can precipitate a crisis, must check for acute Hb drop
#Bony infarction
*Bony infarction
##More debilitating and refractory pain than past episodes
**More debilitating and refractory pain than past episodes
##Localized bone tenderness, elevated WBC
**Localized bone tenderness, elevated WBC
###Fat embolism can be complication
***Fat embolism can be complication
#Dactylitis
*Dactylitis
##Tender, swollen hands/feet
**Tender, swollen hands/feet
##May have low-grade fever
**May have low-grade fever
##Occurs in <2yr old, extremely rare >5yr old
**Occurs in <2yr old, extremely rare >5yr old
#Avascular necrosis of femoral head
*Avascular necrosis of femoral head
##Occurs in 30% of patients by age 30yr
**Occurs in 30% of patients by age 30yr
##Pts p/w afebrile, inguinal pain with weight-bearing
**Pts p/w afebrile, inguinal pain with weight-bearing


=== Respiratory Distress and Chest Pain ===
=== Respiratory Distress and Chest Pain ===
#[[Acute Chest Syndrome]]
*[[Acute Chest Syndrome]]
#PNA
*PNA
##Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. Flu
**Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. Flu
##Only need blood cx in pts ill enough to require ventilator
**Only need blood cx in pts ill enough to require ventilator
#Asthma
*Asthma
##Common in pts with SCD
**Common in pts with SCD
##Increases likelihood of chest syndrome by 4-6x
**Increases likelihood of chest syndrome by 4-6x
#Pulmonary Hypertension
*Pulmonary Hypertension
##Develops in 15-35% of children with SCD
**Develops in 15-35% of children with SCD
###Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE
***Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE


=== Abdominal Pain ===
=== Abdominal Pain ===
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=== Vaso-Occlusive Pain Crisis ===
=== Vaso-Occlusive Pain Crisis ===
#Analgesia
*Analgesia
##IV opiods prefered (Morphine or hydormorphone)
**IV opiods prefered (Morphine or hydormorphone)
###Avoid IM route (use SQ if necessary)
***Avoid IM route (use SQ if necessary)
###Avoid meperidine
***Avoid meperidine
###Use PCA pump if available
***Use PCA pump if available
###Redose in 30min if inadequate
***Redose in 30min if inadequate
###Normally admit if needs more than three doses
***Normally admit if needs more than three doses
##Use of concurrent acetaminophen encouraged, unless contraindicated
**Use of concurrent acetaminophen encouraged, unless contraindicated
#Hydration
*Hydration
##Initial bolus should be 1/2NS with goal of increasing MCV of sickled cells<ref>Guy, R. Treatment of Sickle Cell Crisis with Hypotonic Saline Clinical Research 1971; 19: 420</ref><ref>Pathogenesis and Treatment of Sickle Cell Disease H. Franklin Bunn, M.D. N Engl J Med 1997; 337:762-769</ref>
**Initial bolus should be 1/2NS with goal of increasing MCV of sickled cells<ref>Guy, R. Treatment of Sickle Cell Crisis with Hypotonic Saline Clinical Research 1971; 19: 420</ref><ref>Pathogenesis and Treatment of Sickle Cell Disease H. Franklin Bunn, M.D. N Engl J Med 1997; 337:762-769</ref>
##1.5 times maintenance w/ D5 1/2NS
**1.5 times maintenance w/ D5 1/2NS
##Normal Saline boluses for hypovolemic shock
**Normal Saline boluses for hypovolemic shock
#O2 is not useful in the nonhypoxic patient
*O2 is not useful in the nonhypoxic patient


=== Acute Chest Syndrome ===
=== Acute Chest Syndrome ===
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=== Priapism ===
=== Priapism ===
#Hydration
*Hydration
#Transfsuion and/or exchange transfusion
*Transfsuion and/or exchange transfusion
#Urology consult
*Urology consult
#If persists for >4-6hr:
*If persists for >4-6hr:
##Aspiration of corpora
**Aspiration of corpora
##Irrigate and infuse 1:1,000,000 epi solution
**Irrigate and infuse 1:1,000,000 epi solution


=== Neurologic Disease ===
=== Neurologic Disease ===
#t-PA is not recommended
*t-PA is not recommended
#Exchange transfusion urgently (within 8 hours) to decrease HbS below 30%
*Exchange transfusion urgently (within 8 hours) to decrease HbS below 30%
#Hydration
*Hydration


===Splenic Sequestration===
===Splenic Sequestration===
#Volume resuscitation
*Volume resuscitation
#Simple transfusion vs exchange transfusion
*Simple transfusion vs exchange transfusion


== Disposition ==
== Disposition ==
#Consider admission to the hospital if:
*Consider admission to the hospital if:
##Acute chest syndrome is suspected
**Acute chest syndrome is suspected
##Sepsis, osteomyelitis, or other serious infection is suspected
**Sepsis, osteomyelitis, or other serious infection is suspected
##Priapism, aplastic crisis, hypoxia
**Priapism, aplastic crisis, hypoxia
##WBC >30K
**WBC >30K
##Plt <100K
**Plt <100K
##Pain is not under control after 2-3 rounds of analgesics in ED
**Pain is not under control after 2-3 rounds of analgesics in ED
##<1yr old
**<1yr old
#Consider discharge if:
*Consider discharge if:
##Pain is under control and patient can take oral fluids and medications
**Pain is under control and patient can take oral fluids and medications
##Ensure appropriate oral analgesics are available
**Ensure appropriate oral analgesics are available
##Provide home care instructions
**Provide home care instructions
##Ensure resource for follow-up
**Ensure resource for follow-up


== See Also ==
== See Also ==
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*[[Aplastic crisis]]
*[[Aplastic crisis]]


== Source ==
==References==
<references/>
<references/>


[[Category:Heme/Onc]]
[[Category:Heme/Onc]]

Revision as of 16:32, 30 May 2015

Background

Precipitating Factors

  • For vaso-occlusion:
    • Stress
    • Cold weather
    • Dehydration
    • Hypoxia
    • Infection
    • Acidosis
    • Alcohol intoxication
    • Pregnancy
    • Exertional stress
  • For aplastic crisis:
    • Parvovirus B19 infection
    • Folic acid deficiency
  • For acute chest syndrome:
    • Fat embolus
    • Infection
    • Pain crisis
    • Asthma

Work-Up

  • CBC
    • Assess for significant anemia
  • Chemistry / LFT / lipase
    • If abd pain, may be cholecystitis, mesenteric ischemia, or perforation
  • Retic count (if aplastic crisis considered - rare in adults)
    • Should be >0.5%
  • CXR
    • If cough, SOB, or febrile
  • O2
    • If hypoxic; otherwise may inhibit erythopoesis
  • ECG
  • T&S/T&C
  • BCx2
  • VBG
  • UA
  • Urine pregnancy
  • Head CT/MRI
    • If symptoms of stroke

Clinical Manifestations

Vaso-Occlusive Crisis[1]

  • Pain
    • Most common manifestation of SCA (79-91% of ED visits)
    • Lower back, long bones most commonly affected
    • Because anemia can precipitate a crisis, must check for acute Hb drop
  • Bony infarction
    • More debilitating and refractory pain than past episodes
    • Localized bone tenderness, elevated WBC
      • Fat embolism can be complication
  • Dactylitis
    • Tender, swollen hands/feet
    • May have low-grade fever
    • Occurs in <2yr old, extremely rare >5yr old
  • Avascular necrosis of femoral head
    • Occurs in 30% of patients by age 30yr
    • Pts p/w afebrile, inguinal pain with weight-bearing

Respiratory Distress and Chest Pain

  • Acute Chest Syndrome
  • PNA
    • Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. Flu
    • Only need blood cx in pts ill enough to require ventilator
  • Asthma
    • Common in pts with SCD
    • Increases likelihood of chest syndrome by 4-6x
  • Pulmonary Hypertension
    • Develops in 15-35% of children with SCD
      • Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE

Abdominal Pain

  • Differential Diagnosis
    • Pain crisis
      • 3rd most common site of pain crisis
      • Sudden onset of poorly localized abdominal pain
        • May have tenderness, guarding; should not have rigidity/rebound
    • Gallbladder disease (stones) is common; may occur as early as 2-4yr old
      • RUQ pain, jaundice, anorexia, tender hepatomegaly, fever
      • Bilirubin level higher than usual (>4mg/dL)
    • Acute hepatic sequestration
      • Labs are variable
      • US or CT shows diffuse hepatomegaly

Infection

  • Across all ages, infection is leading cause of death
    • Increased prevalence of meningitis, PNA, arthritis, osteo
  • Children aged 6mo to 3yr at greatest risk for sepsis
  • Parvovirus B19
    • Can cause several different syndromes:
      • 1. Erythema infectiosum ("slapped cheeks" rash)
      • 2. Gloves and socks syndrome
        • Well-demarcated, painful, erythema of hands and feet
          • Evolves nto petechiae, purpura, vesicles, skin sloughing
      • 3. Arthropathy - symmetric or asymmetric, knees and ankles
      • 4. Aplastic crisis
        • Reticulocyte count drops 5d post-exposure, followed by Hb drop
        • Can cause serious anemia which lasts for 2wk

Musculoskeletal Infection

  • Pts w/ SCD have increased rates of bone and joint infection
    • Difficult to distinguish from bony infarcts
      • High fever is more typical of infection
      • Limited range of motion is much more typical of infection
      • Left shift is unique to infection
      • ESR is unreliable
      • May require bone scan or MRI to definitely distinguish infection from infarct

Splenic Sequestration

  • Major cause of mortality in <5yr old
  • Labs: Hb drop, no change in bili, normal to incr retic count
    • 2 types: major and minor
      • Major
        • Rapid drop of hb (>3pt)
        • Pallor, LUQ pain, splenomegaly
        • Can progress w/in hours to AMS, hypotension, CV collapse
      • Minor
        • More insidious, smaller drop in Hb

Neurologic Disease

  • CVA is 250x more common in children with SCD
    • 10% of children suffer clinically overt stroke
    • 20% found to have silent CVA on imaging
  • Increased rate of cerebral aneurysm and ICH

GU

  • Priapism
    • Occurs in 25% by age 20
  • Papillary necrosis
    • Hematuria w/o casts or pyuria

Treatment

Anemia

  • Transfusion
    • Indications:
      • Aplastic crisis
      • Sequestration crisis
      • Hb <6 w/ inappropriately low retic count
      • Hb <10 w/ acute crisis
    • Transfuse 10 mL/kg over 2hr period

Vaso-Occlusive Pain Crisis

  • Analgesia
    • IV opiods prefered (Morphine or hydormorphone)
      • 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
  • Hydration
    • Initial bolus should be 1/2NS with goal of increasing MCV of sickled cells[2][3]
    • 1.5 times maintenance w/ D5 1/2NS
    • Normal Saline boluses for hypovolemic shock
  • O2 is not useful in the nonhypoxic patient

Acute Chest Syndrome

Priapism

  • Hydration
  • Transfsuion and/or exchange transfusion
  • Urology consult
  • If persists for >4-6hr:
    • Aspiration of corpora
    • Irrigate and infuse 1:1,000,000 epi solution

Neurologic Disease

  • t-PA is not recommended
  • Exchange transfusion urgently (within 8 hours) to decrease HbS below 30%
  • Hydration

Splenic Sequestration

  • Volume resuscitation
  • Simple transfusion vs exchange transfusion

Disposition

  • Consider admission to the hospital if:
    • Acute chest syndrome is suspected
    • Sepsis, osteomyelitis, or other serious infection is suspected
    • Priapism, aplastic crisis, hypoxia
    • WBC >30K
    • Plt <100K
    • Pain is not under control after 2-3 rounds of analgesics in ED
    • <1yr old
  • Consider discharge if:
    • 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

References

  1. ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  2. ↑ Guy, R. Treatment of Sickle Cell Crisis with Hypotonic Saline Clinical Research 1971; 19: 420
  3. ↑ Pathogenesis and Treatment of Sickle Cell Disease H. Franklin Bunn, M.D. N Engl J Med 1997; 337:762-769
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