Sickle cell crisis

Vaso-occlusive Crisis

• 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

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

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 diseasestones are 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

Respiratory Distress and Chest Pain

• DDX
• Acute chest crisis
  • Due to pulmonary ischemia and infarction; complication of PNA
  • Pleuritic chest pain, cough, fever, dyspnea, hypoxia, rales
  • CXR findings may be minimal; should not dissuade treatment
• 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

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 postexposure, followed by Hb drop
      • Can cause serious anemia which lasts for 2wk

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
    • Symptomatic anemia
      • Due to aplastic crisis, sequestration
    • Hb <6 w/ inappropriately low retic count
    • Hb <10 w/ acute crisis
  • 10 mL/kg over 2hr period
  • If require transfusion but Hb >10 then perform exchange transfusion

Vaso-occlusive pain crisis

• Analgesia
• Gentle hydration
  • 1.5 times maintenance w/ D51/2NS
  • Only use NS boluses for acute dehydration or hypovolemic shock
• O2 is not useful in the nonhypoxic patient

Disposition

• The decision to admit or discharge is often difficult, and is usually made with input from the patient, family, and hematologist. Children requiring two or more doses of narcotic medications in the ED are likely to require admission. Patients discharged home should be instructed to take NSAIDs if no contraindications exist and may require an oral narcotic as well. Hydrocodone (Lortab®, Vicodin®) at a dose of 0.15 to 0.2 milligram/kg done every 3 to 4 hours is generally an effective choice, and comes in a solution (Lortab®) for younger children.

Because pneumonia is frequently a precipitant or complicating factor, give empiric antibiotic treatment with a third-generation cephalosporin and macrolide. For patients whose clinical status deteriorates despite all of these measures, transfusion (simple or exchange) is indicated. Generally a PaO2 below 70 mm Hg or an oxygen saturation that has fallen >10% from baseline in a chronically hypoxic patient should be a trigger for transfusion. Steroids are not beneficial.

Respiratory distress associated with fat emboli may respond to prompt transfusion and/or exchange transfusion.

Other crises, such as renal infarct and right upper quadrant syndrome and papillary necrosis, are usually treated with hydration, analgesia, and other supportive measures.

Treat priapism with the use of hydration, analgesia, and transfusion and/or exchange transfusion. Additionally, urologic consultation and aspiration of the corpora with a 23-gauge needle may be necessary, followed by irrigation and administration of a 1:1,000,000 epinephrine solution if the priapism continues for more than 4 to 6 hours.

Alteplase (t-PA®) is not recommended for children and has no role in the management of stroke related to SCA urgent exchange transfusion to decrease the percentage of HbS below 30%. There is currently no consensus opinion on the management of blood pressure

Well-appearing children >1 year old with isolated fever and no signs of sepsis may be candidates for discharge if they are stable for a 4-hour period of observation, have good follow-up, and do not meet the high-risk criteria detailed in the Fever section above. Give a dose of ceftriaxone before discharge pending culture results. Stable children with identified source for fever (e.g., strep throat, otitis media) can be discharged with disease-specific treatment. Patients <1 year old, or those meeting high risk criteria or demonstrating signs of sepsis require admission. A third-generation cephalosporin (ceftriaxone, 100 milligrams/kg/d, one dose, not to exceed 4 grams/d) is frequently adequate empiric treatment pending culture results, but areas of high rates of resistant S. pneumoniae may consider the addition of vancomycin (10 milligrams/kg IV every 6 hours) as well

Common Outpatient Medications and Treatments

Due to the risk of bacteremia, children are maintained on oral penicillin through age 5 years old. Beyond 5 years of age, there does not seem to be any additional benefit from penicillin.14 Folate supplementation is standard due to the increased turnover of red blood cells

Background

• Can manefist as vaso-occlusion (pain), acute chest, CVA, aplastic anemia, priapism

Precipitating Factors

1. For vaso-occlusion:
   1. Stress
   2. Cold weather
   3. Dehydration
   4. Hypoxia
   5. Infection
   6. acidosis
   7. alcohol intoxication
   8. pregnancy
   9. exertional stress
2. For aplastic crisis:
   1. parvovirus B19 infection
   2. folic acid deficiency
3. For acute chest syndrome:
   1. fat embolus
   2. infection
   3. pain crisis
   4. asthma

Diagnosis

Insert

Work-Up

1. CBC / stat hemaglobin
   1. Assess change in anemia
2. Chemistry / LFT / lipase
   1. If abd pain, may develop cholecystitis, mesenteric ischemia, or perforation
3. Reticulocyte count (if aplastic crisis considered) - rare in adults
4. CXR
   1. If cough, sob, or febrile
5. O2
   1. If pain crisis or hypoxic; otherwise may inhibit erythopoesis
6. ECG
7. T&S/T&C
8. BCx2
9. VBG
10. UA
11. Urine pregnancy
12. Head CT/MRI if symptoms of stroke

DDx

1. ACS
2. anemia (acute, chronic)
3. appendicitis
4. cholecystitis
5. gout
6. hepatitis
7. meningitis
8. osteomyelitis
9. pancreatitis
10. PID
11. pneumonia
12. priapism
13. pulmonary embolism/infarction
14. rheumatic fever
15. sepsis
16. stroke (ischemic or hemorrhagic)
17. UTI
18. URI

Treatment

1. Abd Pain
   1. Surgery if abdominal injury
2. Aplastic Anemia
   1. Exculde reverible causes and tranfuse for severe anemia (Hb <6-7)
3. Pain crisis
   1. IVF, pain meds, O2 if hypoxic
4. Priapism
   1. Pain meds, exchange tranfusion before surgery, urology consult
5. Acute chest syndrome
   1. Admit if infiltrate, treat pain, give IV Abx
6. Stroke
   1. Head CT +/- LP, IVF, keep HbS <30% total blood vol

Disposition

1. Admission if:
   1. Acute Chest Syndrome - pain/pulmonary infiltrates from infection or pulm infarct
   2. Stroke, priapism, serious bacterial infection, aplastic crisis, hypoxia, acidosis
   3. Unable to tolerate PO or inadequate pain control, abnormal vital signs
   4. Consider if pregnant (in crisis), or uncertain dx of SCD

See Also

Acute Chest Syndrome

Source

Tintinalli