Sickle cell crisis: Difference between revisions
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==Clinical Features==
==Clinical Features==
=== 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)
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**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
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***Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE
***Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE


=== Abdominal Pain ===
===Abdominal Pain===
*Pain crisis
*Pain crisis
**3rd most common site of pain crisis
**3rd most common site of pain crisis
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**Consider treatment with ciprofloxacin and bactrim in ill-appearing
**Consider treatment with ciprofloxacin and bactrim in ill-appearing


=== Infection ===
===Infection===
*Across all ages, infection is leading cause of death
*Across all ages, infection is leading cause of death
**Increased prevalence of meningitis, PNA, arthritis, osteo
**Increased prevalence of meningitis, PNA, arthritis, osteo
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****Can cause serious anemia which lasts for 2wk
****Can cause serious anemia which lasts for 2wk


=== Musculoskeletal Infection ===
===Musculoskeletal Infection===
*Pts w/ SCD have increased rates of bone and joint infection
*Pts w/ SCD have increased rates of bone and joint infection
**Difficult to distinguish from bony infarcts
**Difficult to distinguish from bony infarcts
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***May require bone scan or MRI to definitely distinguish infection from infarct
***May require bone scan or MRI to definitely distinguish infection from infarct


=== Splenic Sequestration ===
===Splenic Sequestration===
*Major cause of mortality in <5yr old
*Major cause of mortality in <5yr old
*Labs: Hb drop, no change in bili, normal to incr retic count
*Labs: Hb drop, no change in bili, normal to incr retic count
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****More insidious, smaller drop in Hb
****More insidious, smaller drop in Hb


=== Neurologic Disease ===
===Neurologic Disease===
*CVA is 250x more common in children with SCD
*CVA is 250x more common in children with SCD
**10% of children suffer clinically overt stroke
**10% of children suffer clinically overt stroke
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*Increased rate of cerebral aneurysm and ICH
*Increased rate of cerebral aneurysm and ICH


=== GU ===
===GU===
*Priapism
*Priapism
**Occurs in 25% by age 20
**Occurs in 25% by age 20
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{{Sickle cell DDX}}
{{Sickle cell DDX}}


== Diagnosis==
==Diagnosis==
*CBC
===Work-up===
**Assess for significant anemia
''Based on clinical presentation, but may include:''
*Chemistry / LFT / lipase
*CBC (assess for significant anemia)
**If abd pain, may be cholecystitis, mesenteric ischemia, or perforation
*Reticulocyte count (<0.5% suggests aplastic crisis - rare in adults)
*Retic count (if aplastic crisis considered - rare in adults)
*Metabolic panel, lipase (if c/o abd pain)
**Should be >0.5%
*CXR (if c/o cough, SOB, or fever)
*CXR
**If cough, SOB, or febrile
*O2
**If hypoxic; otherwise may inhibit erythopoesis
*ECG
*ECG
*T&S/T&C
*BCx2
*VBG
*VBG
*UA
*UA
*Urine pregnancy
*Pregnancy test
*Head CT/MRI
*CT Brain (if sx of CVA)
**If symptoms of stroke
 
===Evaluation===
*Generally a clinical diagnosis
*Certain syndromes require imaging/labs for confirmation (see below)


== Treatment ==
==Management==
=== Anemia ===
===Anemia===
*Transfusion
*Transfusion
**Indications:
**Indications:
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**Transfuse 10 mL/kg over 2hr period
**Transfuse 10 mL/kg over 2hr period


=== Vaso-Occlusive Pain Crisis ===
===Vaso-Occlusive Pain Crisis===
*Analgesia
*Analgesia
**IV opiods prefered (Morphine or hydormorphone)
**IV opiods prefered (Morphine or hydormorphone)
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**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 normoxic patient


=== Acute Chest Syndrome ===
===Acute Chest Syndrome===
*See [[Acute Chest Syndrome]]
*See [[Acute Chest Syndrome]]


=== Priapism ===
===Priapism===
*Hydration
*Hydration
*Transfsuion and/or exchange transfusion
*Transfsuion and/or exchange transfusion
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**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%
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*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
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**Ensure resource for follow-up
**Ensure resource for follow-up


== See Also ==
==See Also==
*[[Acute Chest Syndrome]]
*[[Acute Chest Syndrome]]
*[[Aplastic crisis]]
*[[Aplastic crisis]]

Revision as of 03:27, 12 March 2016

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

Clinical Features

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

  • 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
  • Bacterial gastroenteritis
    • Increased risk for salmonella
    • Consider treatment with ciprofloxacin and bactrim in ill-appearing

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

Differential Diagnosis

Sickle cell crisis

Diagnosis

Work-up

Based on clinical presentation, but may include:

  • CBC (assess for significant anemia)
  • Reticulocyte count (<0.5% suggests aplastic crisis - rare in adults)
  • Metabolic panel, lipase (if c/o abd pain)
  • CXR (if c/o cough, SOB, or fever)
  • ECG
  • VBG
  • UA
  • Pregnancy test
  • CT Brain (if sx of CVA)

Evaluation

  • Generally a clinical diagnosis
  • Certain syndromes require imaging/labs for confirmation (see below)

Management

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
    • Consider Diphenhydramine (25 or 50mg PO)
  • 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 normoxic 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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