So how does hemoglobin SS disease,
our classic presentation
of sickle disease,
how does it present?
The most important thing
that we should know about
is the vaso-occlusive
This is common.
Patients with hemoglobin SS disease
will present with extraordinary pain.
They often have to be treated with
large amounts of morphine for comfort.
And this is a complicated problem because
they may become addicted to morphine
and it may be difficult to distinguish
the pain crisis from non-pain crisis.
It’s important to know which patients
have sickle cell disease though
because pain is never
a good option.
These patients also are at
increased risk for stroke.
Remember, these sickled cells
can cause microvascular trauma
which can setup a
So we need to be aware of these
patients having sickle cell disease
because they can have
stroke even in childhood.
It’s common for patients
to have an infarction
of their spleen or
This usually happens very early in
life from the first several years.
These patients may
develop a large spleen,
but then eventually that
spleen will contract down
and be essentially ineffective.
Acute chest is a significant problem.
This is when cells get
sickled inside the lungs.
The problem is it can cause a fever
and it can be clinically very challenging
to distinguish acute chest from pneumonia.
So they often end up on
antibiotics as well.
Patients may have myocardial
dysfunction from clots,
literally having a
Patients may develop priapism.
Any erection that lasts more
than 4 hours must be attended to
and generally, you have to do --
you have to call urologist who then
can draw the blood out of the penis.
It’s very complex and
challenging to deal with.
Patients may have an aplastic crisis.
In other words, they may have a moment
when they stop making red blood
cells inside their bone.
This may be a result of,
say, a viral infection.
Additionally, patients can develop
osteomyelitis or bone infections.
Normal patients can develop
So the reason I bring this up is that
because these patients are asplenic,
they are at risk for unusually,
a salmonella osteomyelitis.
In these patients, probably group A strep
is still the most common organism,
but when we see them
with an osteomyelitis,
we have to think of expanding
our antimicrobial coverage
beyond the usual culprits which are
group A strep and Staph aureus.
Patients with hemoglobin SS
disease eventually will develop
chronic condition as well such as avascular
necrosis of joint such as the hip.
They may develop cholecystitis
from frequent red blood cell
turnover resulting in gallstones
biliary tree disease.
Patients may develop hearing loss.
They may develop leg ulcers from
inadequate perfusion of the extremities.
They develop retinopathy.
They also may develop renal failure
from sludging within the kidneys.
They may develop liver cirrhosis
and if the lungs continue to
get acute chest syndrome,
they may eventually develop
a pulmonary hypertension.