mEq/L. Liver function tests are not obtained.
After 3 doses of hydromorphone, he falls asleep.
He is not in a monitored bed, and intravenous
fluids, while ordered, are not started. At 6: 30 AM
the day after admission, he cannot be aroused
on a routine vital sign check; he has an SpO2 of
60%, a blood pressure of 80/60 mm Hg, and
heart rate of 148 beats/min. A rapid response is
called, and naloxone is administered along with
oxygen by face mask and several fluid boluses. His
systolic blood pressure increases to 100 mm Hg
from a low of 70 mm Hg. His SpO2 increases to
92%, and he is arousable and alert, although he
reports 10/10 leg pain. His abdomen is noted to
be distended and tender.
The patient is opioid tolerant and has received equivalent doses of opioids in the past
without excess sedation. He may have liver dysfunction making him unable to metabolize opioids effectively. His hemoglobin and platelets
continue to decline, raising concern for splenic
sequestration versus sepsis. Failure to place him
on a monitor allowed his hypoxia to continue
for an unknown amount of time, placing him
at high risk for developing ACS. Lack of intravenous hydration while he was too sedated to
drink likely exacerbated his sickling.
At 9: 20 AM, a CBC is obtained and reveals a
hemoglobin of 4. 8 g/dL and a platelet count of
44,000/µL. Two units of stat O negative blood
are administered, and preparations are made
to administer an exchange transfusion. A liver
panel is obtained 3 hours later, which reveals an
AST level of 1200 U/L and an ALT level of 1050
U/L. His bilirubin is 10 mg/dL, and his lactate
dehydrogenase level is 1800 U/L. His urine is
dark and is positive for bilirubin and ketones.
He is transferred to the intensive care unit. A
chest X-ray shows pulmonary congestion. Hematology/oncology is consulted.
He receives a 7-unit red blood cell exchange,
which reduces his HbS to 11%. He continues to
be hypotensive, and requires norepinephrine to
support his blood pressure. Antibiotic therapy is
started. His creatinine concentration rises to 2. 3
mg/dL, potassium is 7. 8 mEq/L, and bicarbonate is 12 mEq/L. He is placed on hemodialysis.
Computed tomography of the chest and abdomen reveals lower posterior lung infiltrates
and a grossly enlarged spleen. He requires intubation. He is given a diagnosis of ACS in addition to kidney failure, liver failure, and “sickle crisis.” He continues to require daily to twice daily
transfusions to maintain a hemoglobin of 7 to
9 g/dL, and his abdominal distension increases.
As his condition worsens, surgery is consulted to
discuss a liver transplant. He is deemed to not be
a surgical candidate, and he passes away 6 days
after entering the hospital. The immediate cause
of death is listed as vaso-occlusive crisis, with ACS
and sickle crisis listed as contributors.
If vaso-occlusive crisis is used to indicate a
pain event, it is not an accurate cause of death.
Pain is one of the most distressing complications
of sickle cell disease, and frequent pain events
are associated with early mortality, 4,80 but they
are not in themselves fatal. ACS is the number
one cause of death in sickle cell disease, 4 and it
likely contributed to this patient’s death. Sickle
crisis is a vague term that should not be used
in this context. Causes of death should include
splenic sequestration and multisystem organ
failure. Multisystem organ failure in sickle cell
disease often responds to aggressive transfusion
therapy, which this patient received.116–118
Sickle cell disease is a complex chronic dis-
ease that impacts almost every organ system in
the body. Clinicians may be inclined to attribute