1. John
Martinelli,
MSIII,
SGUSOM
DATE:
9/28/13
Pediatrics,
Case
1:
Sickle
Cell/Aplastic
Crisis
Identifying
Data:
N.S.
is
an
8
year
old,
English
speaking,
African-­‐American
female
who
presented
to
the
SBMC
ED
with
her
mother
on
the
evening
of
9/10/13.
She
was
immediately
admitted
to
the
SBMC
pediatric
floor
and
subsequently
discharged
on
9/20/13.
DOB:
1/4/05
Chief
Complaint:
During
the
ED
visit,
N.S.
complained
primarily
of
a
painful
lower
back
describing
it
as
“hurts
so
bad
it’s
hard
to
move”.
History
of
Present
Illness:
N.S.
has
a
known
history
of
sickle
cell
disease
with
positive
HbSC.
One
day
prior
to
this
admission,
N.S.
began
experiencing
severe
and
increasing
diffuse
deep
lower
back
pain,
which
began
radiating
to
both
proximal
lower
extremities.
She
described
this
pain
as
increasing
hour-­‐to-­‐hour
and
was
similar
to
past
episodes,
which
twice
required
hospitalization.
On
the
same
day
prior
to
admission,
the
pain
became
progressively
generalized,
beginning
with
non-­‐specific
headache
and
then
chest
pain
as
well
as
low-­‐grade
fever.
She
was
given
Tylenol
at
home
without
relief.
Per
her
mother,
N.S.
did
not
experience
vomiting,
diarrhea,
upper
respiratory
symptoms,
respiratory
distress,
change
in
urination,
neurologic
abnormalities,
or
behavioral
changes
associated
with
her
current
illness.
In
the
ED,
it
was
recommended
N.S.
be
admitted
for
painful
sickle
cell
crisis.
Past
Medical
History:
N.S.
was
born
full
term
via
spontaneous
vaginal
delivery,
weight
7lbs
8oz.
She
did
show
evidence
of
neonatal
jaundice,
which
was
successfully
treated
with
phototherapy.
She
was
first
diagnosed
with
HbSC
sickle
cell
disease
based
on
newborn
screening
and
has
been
followed
every
six
months
alternating
between
NBIMC
and
SBMC.
She
was
hospitalized
two
times
at
SBMC
in
the
past
12
months
for
recurrent
painful
sickle
cell
crises.
She
has
not
required
ACS,
PICU,
blood
transfusions,
or
intubation
at
any
time.
N.S.
also
has
a
history
of
mild
persistent
asthma
that
occasionally
required
albuterol
and
pulmacort
for
control;
however,
she
has
been
asymptomatic
for
more
than
a
year.
In
addition,
ENT
saw
her
for
obstructive
sleep
apnea
secondary
to
tonsillitis
for
which
tonsillectomy
&
adenectomy
was
recommended,
however,
the
mother
refused.
She
is
current
with
all
immunizations
including
polyvalent
Prevnar
23.
Medications:
Folic
Acid
(not
compliant)
Upon
Admission:
IVF
Maintenance:
D5
1/2NS,
62ml/hr.
KCL:
20meq,
15ml,
PO
BID.
Toradol:
10mg,
0.33ml,
IV
Push
Q6H
PRN
(moderate
pain)
Morphine:
2mg,
1ml,
IV
Push
Q3H
PRN
(severe
pain)
Tylenol:
220mg,
6.88ml,
PO
Q4H
PRN
(fever)
Rocephin:
1,100mg,
27.5ml,
55ml/hr,
IV
Push
(Sepsis
Prophylaxis)

2. Allergies:
NKA,
NKDA
Family
History:
Mother
and
sister
have
a
history
of
asthma.
Mother
is
HbSA
and
father
is
HbAC.
Social
History:
N.S.
lives
at
home
with
her
mother
and
older
sister.
There
are
no
pets
and
no
smoking
at
home.
Her
mother
feels
confident
she
is
eating
a
well
balanced
diet
and
states
N.S.
is
active
and
often
plays
outside
with
friends.
She
attends
school
regularly
and
performs
well,
although
she
occasionally
has
some
reading
difficulty.
Review
of
Systems
(upon
admission):
General:
Distress
due
to
lower
back
and
proximal
bilateral
leg
pain.
Skin:
Warm,
pink,
moist,
without
pallor.
Eye:
No
history
of
eye/vision
problems
or
change
in
vision.
HENT:
Normocephalic.
Cardiovascular:
No
history
of
cardiovascular
disease
or
symptoms.
Pulmonary:
No
asthmatic
symptoms
x
1
year.
Lymphatics:
Does
not
report
swelling
or
tenderness.
Gastrointestinal:
No
history
of
GI/abdominal
problems
or
nausea/vomiting/diarrhea.
Genitourinary:
No
change
in
urinary
frequency
or
color.
No
urinary/sexual
developmental
concerns.
Musculoskeletal:
No
history
of
musculoskeletal
disorders,
weakness,
trauma,
or
fractures.
Neurologic:
No
history
or
signs/symptoms
of
neurologic
disorders.
Hematologic:
Known
HbSC.
Endocrine:
No
known
history
or
symptoms
suggestive
of
endocrine
disorders.
Psychiatric:
AAOx3,
appropriate
affect.
No
history
of
psychiatric
illness.
Physical
Exam
(upon
admission):
Vitals:
BP:
96/56
PR:
116
(elevated)
RR:
24
Pulse
Ox:
97%
RA
T:
97.3
General:
Distress
due
to
lower
back
and
proximal
bilateral
leg
pain.
Skin:
Warm,
pink,
moist,
without
pallor.
Eye:
PERRLA
(-­‐)APD,
EOM’s:
Full
(-­‐)Diplopia,
CVF:
360degs
OU,
(+)Conjunctival
Pallor
OU,
(+)RR
OU.
Deferred
dilated
retinal
exam,
r/o
sub-­‐clinical
sickle
cell/fan
retinopathy.
N.S.
reports
no
change
in
vision.
HENT:
Normocephalic,
moist
oral
mucosa,
(-­‐)pharyngeal
erythema/edema/exudate,
(-­‐)otitis
b/l.
Cardiovascular:
RRR,
good
peripheral
pulses,
normal
peripheral
perfusion,
no
edema.
Pulmonary:
Clear
to
auscultation
with
symmetric
air
movement
bilaterally.
2

3.
Lymphatics:
No
cervical,
axillary,
or
inguinal
lymphadenopathy.
Gastrointestinal:
Soft,
non-­‐tender,
without
evidence
of
organomegaly
or
splenomegaly.
Genitourinary:
Deferred.
No
change
in
urinary
frequency
or
color.
No
pain
on
urination.
Musculoskeletal:
Normal
range
of
motion.
Normal
strength
in
all
extremities.
(+)
DTR
4/4
bilaterally.
Neurologic:
CN
II
–
XII
intact,
normal
&
symmetric
muscular
tone,
normal
balance
and
gait,
PERRLA
(-­‐)
APD,
EOM’s
Full
(-­‐)
Diplopia,
CVF
Full
360degs
OU.
No
evidence
of
neurologic
deficit.
Hematologic:
Known
HbSC,
depressed
Hgb
(7.5),
low
Reticulocytes
(0.3%),
elevated
WBC
(13.2).
Endocrine:
No
goiter,
myxedema,
exophthalmos,
tremor,
hirsutism,
or
evidence
of
abnormal
sexual
development.
Psychiatric:
AAOx3.
Appropriate
affect.
Labs
(upon
admission):
WBC:
13.2
Na:
143
K:
3.0
Gl:
141
Retic:
0.3%
Hgb:
7.5
Cl:
105
HCO3:
22
Sickle
Cell:
1+
Platelets:
459
BUN:
6
Cr:
0.41
Target
Cell:
2+
ALT:
14
Tbili:
0.9
AST:
34
Tprotein:
7.7
ALP:
116
Albumin:
4.5
(-­‐)
Urine
Cx
(-­‐)
Blood
Cx
(+)
Parvo
B19
Isolated
Differential
Diagnosis/Assessment:
Based
on
N.S.’s
past
medical
history,
pain,
fever,
decreased
Hgb,
low
reticulocyte
count,
elevated
WBC,
along
with
a
positive
Parvo
B19
isolate,
a
diagnosis
of
vaso-­‐occlusive
HbSC
sickle
cell
crisis
exacerbated
by
Parvovirus
B19
leading
to
aplastic
crisis
is
most
likely.
However,
in
the
absence
of
this
patient’s
past
medical
history,
other
diagnoses
must
be
considered
based
on
similar
initial
clinical
presentations.
Examples
include
acute
anemia’s
from
sudden
hemolysis
or
hemorrhage.
A
leukemia
or
blast
crisis
leading
to
diminished
erythropoiesis
is
another
consideration.
Osteomyelitis
can
also
present
with
fever
and
penetrating
pain.
Regarding
sickle
cell
disease
with
or
without
Parvo
B19
infection,
one
must
be
prudent
with
respect
to
possible
associated
comorbidities.
Vaso-­‐occlusive
acute
chest
syndrome
with
possible
rib
infarction,
sepsis
due
to
splenic
failure,
splenic
sequestration,
congestive
heart
failure
due
to
prolonged
compensatory
tachypnea,
and
possibly
pericarditis
can
occur.
Vaso-­‐occlusive
cerebrovascular
accidents
may
occur
producing
neurologic
sequelae.
Asymptomatic
or
sub-­‐clinical
sickle
cell/fan
retinopathy
must
also
be
ruled
out
to
prevent
progressive
or
sudden
visual
loss.
3

4. Plan:
In-­‐patient
treatment
for
N.S.
includes
IV
hydration,
pain
management,
as
well
as
initial
sepsis
antibiotic
prophylaxis.
In
the
absence
of
positive
blood
and
urine
cultures,
continued
antibiotic
therapy
is
not
indicated.
Understanding
anti-­‐viral
treatment
does
not
exist
for
Parvovirus
B19
infection,
in-­‐patient
supportive
care
with
careful
monitoring
of
Hgb
and
reticulocyte
levels
is
paramount.
With
evidence
supporting
clearance
of
the
virus
via
normalized
laboratory
values,
only
then
will
it
be
appropriate
to
consider
discharge.
After
10
days
in-­‐patient,
N.S.
finally
improved
and
was
discharged.
Her
follow-­‐up
care
includes
scheduled
visits
with
both
her
pediatrician
and
hematologist.
Her
mother
was
reminded
of
the
potential
importance
of
folic
acid
supplementation
as
well
as
occasional
OTC
pain
management
for
minor
symptoms.
However,
it
was
emphasized
to
immediately
report
to
the
ED
if
there
is
any
question
of
recurrence
or
future
signs/symptoms
of
repeat
crises.
4