Carolinas Emergency Medicine Residency

Sickle Cell Anemia Related Emergencies

12/14/2017

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Vaso-Occlusive Crises

Vaso-occlusive episodes are from tissue ischemia and infarction from intravascular sickling
1. 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:
1. IVF: D5 ½ NS @ 1.5 maintenance or fluid bolus in dehydrated or hypotensive pt
2. 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
1. 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:
1. CBC, leukocyte differential, reticulocyte count (aplastic anemia vs. splenic sequestration), U/A
2. If you think they may need an RBC transfusion, send a cross-match
3. ABG for hypoxemia
4. CXR - May be normal initially
5. BCx
Tx:
1. Oxygen
2. Oral hydration preferred
  1. If IV: hypotonic fluids at rate 1.5x maintenance (overly aggressive fluids can worsen ACS)
3. Pain meds but don’t decrease resp drive too much
4. Abx: empiric Abx for CAP
  1. Ceftriaxone 50mg/kg and a macrolide
  2. Common concurrent infections: chlamydia, mycoplasma, viral, strep. pneumo, staph aureus, H. flu
5. Bronchodilators: nebulized B2-adrnergic agonists
6. Chest physiotherapy
7. Transfusion for:
  1. Severe acute anemia
  2. PaO2 < 70 mmHg
  3. Oxygen saturation drop of 10% from baseline
Exchange transfusion for
1. 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
2. Suspected or confirmed fat or bone marrow embolism (from boney infarct)

Hematologic crises

Acute Splenic Sequestration Crises
1. Spleen traps much of the circulating blood volume
2. Presentation:
  1. Usually occurs in kids b/c of splenic infarction that develops later in life
  2. Severe: sudden-onset LUQ pain, pallor, lethargy
  3. Minor episodes: insidious onset of abdominal pain, slowly progressive splenomegaly, and a more minor fall in Hb level
3. Dx and Differential:
  1. CBC shows profound anemia
  2. Normal to elevated retic count
4. Tx: transfusion with RBCs
Aplastic Episodes
1. Caused by viral infections (usually parvovirus B19), bacterial infections, folic acid deficiency, or bone-marrow suppressive drugs
2. Presentation: gradual onset of pallor w/out pain or jaundice
3. Low hemoglobin w/ decreased or absent reticulocytosis
4. Tx:
  1. Transfusion for:
    1. Hb <6
    2. A drop in Hb by 3
    3. Symptomatic
Hemolytic Crises
1. Bacterial or viral infections can also precipitate increasing degree of active hemolysis
2. Usually sudden onset
3. Dx: Anemia with marked increase in reticulocytes
4. Tx: treat underlying infection

Infections
1. Due to functional asplenia → deficient antibody production and impaired phagocytosis → bacterial infections, esp. encapsulated organisms, pose a serious threat
  1. Leading cause of death
2. Dx: CBC and cultures
3. Tx:
  1. Children receive penicillin ppx until age 5
  2. Ill appearing children < 1 year old treated empirically w/ Abx against S. pneumoniae and H. influenza → ceftriaxone 50mg/kg
  3. 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
    1. If not meeting these criteria → admit for Abx and obs

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Avoiding Risk in Neurovascular Emergencies - Dr. A. Asimos

12/14/2017

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The “Blood Can Be Very, Very Bad” mnemonic should be utilized to systematically review any brain CT (Blood, Cisterns, Brain, Ventricles, Vessels, Bone).
The reflex exam is an essential part of the motor exam (especially when the differential diagnosis includes both a polyneuropathy and a myelopathy) as the presence of hyperreflexia will suggest a myelopathy, while hyporeflexia will suggest a polyneuropathy.
In someone with a sudden onset of diminished mental status and bilateral weakness, an acute basilar occlusion must be included in the differential diagnosis.
Both spinal cord compression and a polyneuropathy are frequently heralded by paresthesias, so these processes should always be acknowledged in a patient presenting with bilateral paresthesias.

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Concussions - Dr. S. Fling

12/7/2017

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Definition: Trauma-induced alteration in mental status with or without loss of consciousness
Concussions occur as a result of neurometabolic destruction at the cellular rather than structural level, which is why our current modalities of imaging are negative.
Concussive symptoms are divided into 4 categories: Cognitive, physical, emotional, sleep-related
Patients who are at increased risk of prolonged concussive symptoms include those with severe initial symptoms, teenagers, females, and those with previous concussions
Beware secondary impact syndrome which occurs when a patient re-injures their brain before full recovery from previous injury
As far as ED management, history is key along with a thorough physical exam including a neuro exam. Do not forget to check for gait instability, which is the most specific indicator of concussion
From your neuro exam, use PECARN to determine if a CT scan is indicated to determine structural injury
Discharge instructions are key. The Big 5 include:

Cognitive rest first 24-48 hours after injury
More benefit in uninterrupted sleep
Graded return to sport protocol
Symptom free without meds before full return to play
Set Expectations

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Sickle Cell Anemia Related Emergencies

Avoiding Risk in Neurovascular Emergencies - Dr. A. Asimos

Concussions - Dr. S. Fling

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