most pain in a patient with sickle cell disease
to a simple vaso-occlusive crisis, treat them for
this, and not investigate further. As the case presented here demonstrates, failure to identify the
actual life-threatening process occurring in a patient with sickle cell disease presenting with pain
can result in preventable early mortality. Clinicians must approach a sickle cell patient reporting pain in a thoughtful manner, and consider a
complete differential diagnosis, including both
sickle cell disease complications and those unrelated to sickle cell disease. Knowledge of
the disease courses of the different sickle cell
genotypes is essential, and must go beyond a superficial hierarchy of severity, but rather include
an understanding of the complications each
genotype is most prone to, and at what ages.
Complete laboratory assessment, including a
comprehensive metabolic panel, should be performed on all admitted patients, not just a complete blood count. Treating pain with high-dose
opioids, while appropriate in an uncomplicated
pain crisis, can lead to ACS or even respiratory
failure in a patient with uninvestigated liver and
kidney dysfunction. The most important lesson
to remember is that even the sickle cell disease
patient who has been given the unfortunate and
pejorative label of “frequent flyer” by some providers has the potential for rapid deterioration
into multisystem organ failure and death.
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