Vaso-Occlusive Crises
Pain Crises
Acute Chest Syndrome – consolidation on CXR AND 1 of these: fever; >2% ↓SpO2, PaO2<60, tachypnea, increased work of breathing, CP, cough, wheezing or rales
Hematologic crises
- Vaso-occlusive episodes are from tissue ischemia and infarction from intravascular sickling
- Bones, soft tissue, viscera, and CNS can all be affected
Pain Crises
- Usually classic in location, character, and severity
- Triggers: stress, cold, dehydration, hypoxia, anemia, infection, or no cause
- In infants – first sign may be dactylitis (swelling of hands or feet) due to ischemia of metatarsal/metacarpal nutrient vessels
- Treatment:
- IVF: D5 ½ NS @ 1.5 maintenance or fluid bolus in dehydrated or hypotensive pt
- Pain medications:
- Acetaminophen for mild pain
- NSAID for mild to moderate pain
- Toradol 15mg (30mg not proven to be better)
- Opioids for moderate to severe pain, typical initial doses include:
- Morphine 0.3mg/kg PO
- Morphine 0.1 - 0.15mg/kg IV/IM
- Dilaudid 0.06 - 0.08 mg/kg PO
- Dilaudid 0.015 - 0.020 mg/kg IV/IM
- Reassess response in 15-30min and may repeat with ¼ to ½ initial dose
- Home: hydroxyurea increases % of fetal hemoglobin
- Transfusion for acute drop in hemoglobin from baseline, or Hgb < 5
Acute Chest Syndrome – consolidation on CXR AND 1 of these: fever; >2% ↓SpO2, PaO2<60, tachypnea, increased work of breathing, CP, cough, wheezing or rales
- Due to ischemia and infarction, usually a complication of PNA or as a result of bone marrow or fat emboli
- Presenting symptom: pleuritic chest pain, dyspnea, fever, cough
- Ask immunization history, esp.: pneumococcal and H. influenza type B
- Dx:
- CBC, leukocyte differential, reticulocyte count (aplastic anemia vs. splenic sequestration), U/A
- If you think they may need an RBC transfusion, send a cross-match
- ABG for hypoxemia
- CXR - May be normal initially
- BCx
- Tx:
- Oxygen
- Oral hydration preferred
- If IV: hypotonic fluids at rate 1.5x maintenance (overly aggressive fluids can worsen ACS)
- Pain meds but don’t decrease resp drive too much
- Abx: empiric Abx for CAP
- Ceftriaxone 50mg/kg and a macrolide
- Common concurrent infections: chlamydia, mycoplasma, viral, strep. pneumo, staph aureus, H. flu
- Bronchodilators: nebulized B2-adrnergic agonists
- Chest physiotherapy
- Transfusion for:
- Severe acute anemia
- PaO2 < 70 mmHg
- Oxygen saturation drop of 10% from baseline
- Exchange transfusion for
- Severe acute chest syndrome and past history of requiring vent support: useful to prevent intubation and it can decrease duration of vent support if patient already intubated
- Suspected or confirmed fat or bone marrow embolism (from boney infarct)
Hematologic crises
- Acute Splenic Sequestration Crises
- Spleen traps much of the circulating blood volume
- Presentation:
- Usually occurs in kids b/c of splenic infarction that develops later in life
- Severe: sudden-onset LUQ pain, pallor, lethargy
- Minor episodes: insidious onset of abdominal pain, slowly progressive splenomegaly, and a more minor fall in Hb level
- Dx and Differential:
- CBC shows profound anemia
- Normal to elevated retic count
- Tx: transfusion with RBCs
- Aplastic Episodes
- Caused by viral infections (usually parvovirus B19), bacterial infections, folic acid deficiency, or bone-marrow suppressive drugs
- Presentation: gradual onset of pallor w/out pain or jaundice
- Low hemoglobin w/ decreased or absent reticulocytosis
- Tx:
- Transfusion for:
- Hb <6
- A drop in Hb by 3
- Symptomatic
- Transfusion for:
- Hemolytic Crises
- Bacterial or viral infections can also precipitate increasing degree of active hemolysis
- Usually sudden onset
- Dx: Anemia with marked increase in reticulocytes
- Tx: treat underlying infection
- Infections
- Due to functional asplenia → deficient antibody production and impaired phagocytosis → bacterial infections, esp. encapsulated organisms, pose a serious threat
- Leading cause of death
- Dx: CBC and cultures
- Tx:
- Children receive penicillin ppx until age 5
- Ill appearing children < 1 year old treated empirically w/ Abx against S. pneumoniae and H. influenza → ceftriaxone 50mg/kg
- Well-appearing children >1 year with temp <40C, WBC 5-30, platelets >100,000 and Hgb >5, no CXR infiltrate → single dose ceftriaxone, 4 hours observation, and 24 hour follow-up
- If not meeting these criteria → admit for Abx and obs
- If not meeting these criteria → admit for Abx and obs
- Due to functional asplenia → deficient antibody production and impaired phagocytosis → bacterial infections, esp. encapsulated organisms, pose a serious threat