Pediatric emergency medicine trisk 553

TABLE 93.6
SICKLE CELL ANEMIA: COMPLICATIONS AND MANAGEMENT


Complication

Clinical features

Management

Comments

Vasoocclusive
episode

1. Joint or bone pain 1. Mild or moderate pain IV fluid can be
2. Joint swelling
Oral or IV hydration
D5 normal
(e.g., dactylitis)
(bolus 10–20 mL/kg,
saline or D5 ½
3. Abdominal pain
then 1–1½ times
normal saline
(may mimic acute
maintenance volume)
solution
abdomen)
Analgesia
NSAID may be

NSAIDs
ibuprofen or
Oral medications
ketorolac and
such as oxycodone
should
may be sufficient
continue after
for some patients
discharge until
IV (intermittent)
pain resolves
doses of morphine,
If delayed or
fentanyl, or
difficult IV
hydromorphone
access,
Nitrous oxide plus
intranasal
oral opioids is
fentanyl may
used in some
be considered
centers
for initial
Consider admission
therapy
if
Patient-controlled

pain worsens
analgesia
inadequate oral
(PCA) may
fluid intake
include a
repeat emergency
continuous
department visits
infusion of
2. Severe pain
opioid
IV bolus 10–20
mL/kg followed by If using fentanyl,
patient may
hydration at 1–1½
require PCA
times maintenance
due to short
volume
duration of
Analgesia
action
NSAIDs
(ketorolac)
IV (intermittent
and frequent)
doses of morphine,
fentanyl, or
hydromorphone

PCA (early)
For refractory
pain: IV ketamine
at subdissociative
doses
Intermittent
Continuous
infusion
Admit unless pain
markedly reduced
and patient
tolerates oral
fluids
Priapism

Splenic
sequestration

Acute chest
syndrome

1. Prolonged
erection lasting
more than 3 hrs
2. Pain
3. May experience
difficulty
urinating

1. Oral or IV hydration
Urology may
(10–20 mL/kg bolus,
consider early
then 1–1½ times
aspiration of
maintenance)
the corpora
2. Oral pseudoephedrine
3. Analgesia as needed to
control pain
4. Consult with urology if
no relief within 3 hrs of
onset
1. Left upper
1. Immediate volume
Onset of
quadrant pain
replacement
symptoms is
2. Pallor
IV fluids
often sudden
3. Lethargy
Simple red blood cell Usually occurs
4. Splenomegaly
transfusion
before age 5,
5. May have altered 2. Admission to hospital

but may
vital signs
3. Splenectomy in
develop later in
(tachycardia,
refractory patients
patients with
hypotension)
hemoglobin SC
6. Worsened anemia
disease
with elevated
reticulocytes and
mild to moderate
thrombocytopenia
1. Chest pain
1. Antibiotic therapy
2. Oxygen
Third-generation
saturation below
cephalosporin,
patient’s baseline
consider addition of
vancomycin; if

Therapy with
steroids not
usually needed
unless patient
has a history of

Ischemic or
hemorrhagic
stroke

Hemolytic
anemia crisis

3. Symptoms of
respiratory
distress
4. New finding on
chest radiograph
5. Fever often
present

allergy to
cephalosporins,
consider quinolones
(e.g., levofloxacin)
or clindamycin,
AND
Macrolide
2. Consideration of red
blood cell transfusion
for patients with
respiratory distress
Simple transfusion if
hemoglobin <10

g/dL
Exchange transfusion
if hemoglobin ≥10
g/dL

1. Focal neurologic
deficits
Focal
weakness
Dysarthria
Aphasia
2. Increased ICP
Severe
headache
Vomiting
Bradycardia
3. Seizure
4. Impaired mental
status/coma
5. Nuchal rigidity
(hemorrhagic
stroke)
1. Fatigue
2. Pallor
3. Scleral icterus
4. Jaundice
5. Tachycardia
6. Headache
7. Fall in the
hematocrit from

1. Immediate CT
2. If stroke present,
initiate exchange
transfusion. Simple
transfusion may be
used as a bridge while
exchange is being set
up if hemoglobin <10
g/dL
3. MRI if within 2–4 hrs
of symptoms
4. 1.5–2 times volume
exchange transfusion
5. Angiography to target
management

asthma and
signs of an
asthma
exacerbation

Ischemic strokes
may be more
subtle than
hemorrhagic
strokes

1. Usually self-limited,
Transfusions

routine supportive care
usually not
2. Transfusion of pRBCs
required
if severe symptoms
(altered mental status,
impaired oxygen
delivery, significant
tachycardia)