Interesting Case Rounds
Rebecca Burton-MacLeod
R5 (yikes!), Emerg Med
July 5th, 2007
Case
21M presents to ED c/o CP
X ~4 hrs
Retrosternal, radiated bilaterally
Not pleuritic
+ SOB +nauseas; not dizzy, no palpitations
Also c/o generalized abdo pain, and pain to
bilateral thighs (present x2days)
History cont’d
No previous similar episodes, but
sometimes his legs “have pain”
Pt moved from India about 2mos prior,
++ language barrier !
PMHx: “healthy”
Meds: none
Allergies: none
Further hx
Questions ?
Physical exam
HR 115, BP 128/72, RR 26, Temp 37.9, sats
92% r/a
Pt ++ uncomfortable, tearful
CNS—A+O
CVS—N heart sounds, PPP
Resp—lungs clear
Abdo—generalized mild tenderness, no guarding,
no rebound, + BS
MSK—c/o ++ tenderness to palpation bilateral
thighs; no erythema or swelling noted
Investigations
??
Investigations
CXR
EKG
CBC, lytes, Cr
EKG
Bloodwork
Hgb 77
Plt 185
WBC 31
Lytes, Cr N
“Have you ever been told you
have anemia before?”…
Sickle cell disease
Autosomal recessive
disease
Caused by substitution
of valine for glutamine
in Hgb beta-chain
(Hgb S)
Sickle cell distribution
Sickle cell crisis
Vaso-occlusive crisis
Aplastic crisis
Splenic sequestration
Acute chest syndrome
Infections
Case cont’d
Pt received:
IV N/S 1L bolus; 2x maintenance fluids
IV morphine 5mg boluses to total of 20mg
Oxygen by n.p.
Case cont’d
Called back to see pt as noted to be ++SOB
Sats now 85% on r/a
Clinically—wheezes and crackles heard
bilaterally on auscultation of lungs
Temp now 38.7C
ABG—on 8L O2: pH 7.24 /CO2 51 /O2 50;
lactate 12
Any thoughts ?
Acute chest s/o
Defined as:
New infiltrate on CXR
Associated with new symptoms (fever, cough, sputum
production, dyspnea, hypoxia)
Occurs in 50% of SCD pts; recurrent in 80%
Preceded by vaso-occlusive crisis in half of
patients
Leading cause of death for pts with SCD
Risk factors for ACS
Young
Homozygous sickle-cell genotype
Fever
Winter months
Surgery
Previous ACS events
AVN of bones
High Hgb levels
Low fetal Hgb levels
High steady-state leukocyte counts
Pathophysiology
Infection
Fat embolism
Thromboembolism
Vaso-occlusive sickling erythrocytes
Hypoventilation due to rib/sternal infarction and
pain
Hypoventilation due to narcotic administration
Pulmonary edema due to fluid overload
Sign / symptoms
~25% of adults have other symptoms of pain (usually lower
extremities)
Mgmt
Identify and treat precipitating factors
Maintain/improve oxygenation
Prevent further alveolar collapse
Maintain adequate fluid volume
Control pain
Treat underlying infection
Oxygenation
Administer O2 to keep PaO2 ~70-100
Pts may require simple, or exchange
transfusions (allow removal of Hgb S) if
severe anemia
Incentive spirometry, CPAP, or intubation/
mechanical ventilation with PEEP
Indications for exchange tx
Evidence of rapid clinical deterioration
SaO2 <80% despite aggressive ventilatory
support
Serial decline in SaO2
Worsening/unstable vital signs
Persistent RR >30/min
Maintain fluid volume
Replenish dehydration with N/S
For further fluid volume, use D5W/0.25N/S
Hypotonic fluids preferred as allows water to
enter RBC
Causes osmotic swelling and reduced tendency
for sickling
Treat underlying infection
2nd-3rd gen cephalosporin +/- macrolide
Bacteremia in ~3.5% of cases of ACS
Strep pneumoniae
H. flu
Staph aureus
Salmonella
Enterobacter
Clostridia
Other therapies
DBRCT of IV dex in peds (n=43) showed SS
shorter hosp stays, dec blood transfusions, dec
clinical deterioration
Multiple case reports and animal studies of nitric
oxide improving pt symptoms (lack of NO
bioavailability in SCD)
Poloxamer-188 (non-ionic surfactant) may shorten
duration of ACS and length of hospitalization
(n=43)
Questions ?
References
Rosens
Ballas SK, et al. Safety of purified poloxamer 188 in SCD.
Hemoglobin. 2004.
Bernini JC, et al. Beneficial effects of IV dexamethasone in children
with mild to mod severe ACS complicating SCD. Blood. 1998.
Gladwin MT, et al. The ACS in SCD. Am J Resp Crit Care Med.
1999.
Kararmaz, A et al. ACS in a patient with SC anemia successfully
treated with erythrocytopheresis. Int J Emerg Inten Care Med. 2006.
Machado RF. Nitric oxide based therapies in SCD: the evidence
continues to mount. Crit Care Med. 2007.
Vichinsky EP, et al. ACS in SCD: clinical presentation and course.
Blood. 1997.
Yale SH, et al. ACS in SCD. Postgrad Med. 2000.