One of the key mainstays of managements of
these patients is by giving immunizations.
Remember, these children are at increased
risk for encapsulated organisms.
This is because their
spleens are ineffective.
So, we need to give both the 13 valent
and 23 valent pneumococcal vaccines.
Also, we typically give them
influenza vaccines to try to prevent
chest crisis as a result
of viral infection.
Last, these children should get
meningococcal vaccines as well
since meningococcus is also
an encapsulated organism.
We will usually treat patients with
a mainstay of two medications.
One is they will often early in
childhood go on penicillin prophylaxis
as a way of preventing
Secondly, we try to put these
patients on hydroxyurea.
Hydroxyurea will increase the percent of
fetal hemoglobin that is in the blood.
This can reduce a number of complications
including acute chest and pain crises,
so these patients should
be always on hydroxyurea.
There are other treatments we could try.
In severe cases, one could
try a stem cell transplant,
which is a challenging
and complex care
and requires immunosuppression, so we
don’t want to do that all the time.
The other thing we will often do in
acute crisis is we will do transfusions
to try and keep their blood levels high and
don’t let them become profoundly anemic.
So these patients have
a myriad of problems
in addition to just the
sickling of the cells
and we’re going to screen
for these problems.
So we need to make sure we’re
watching their blood pressure
and that they’re not
If they develop, we
need to treat it.
We watch for cerebral complications.
We will screen for retinopathy.
These patients should have an eye
doctor that they see regularly.
We watch for chronic lung disease.
We will keep an eye out for
their pulmonary hypertension
and they may get
One thing I like to emphasize
is the role of poverty
in the management of
sickle cell disease.
It’s unfortunate in the U.S. that
there is a maldistribution of poverty
among patient populations who have
more risk for sickle cell disease.
Because unlike other developed
countries in the world,
we don’t have an equal access
to the health care system.
It’s unfortunate that these
very patients who are poor
sometimes are getting
a less good care.
Perhaps, for example, they are less likely
to get access to their hydroxyurea.
So it’s important that we
have outreach programs
that can reach out to poor
patients with sickle disease.
One thing that happens in the
hospital setting fairly frequently
is dealing with an acute
So it’s very important in patients
during an acute vaso-occlusive crisis
that they get
This can prevent the endothelial
damage from the sickled cells.
We typically give these
patients hypotonic fluid,
as these patients may have problems with
sodium load because of renal dysfunction.
We will often transfuse for
things like splenic sequestration
or if they have an aplasia of their red
blood cells as a result of an infection.
But what’s key around these
patients is pain management.
If we’re unclear as to how
best to manage these patients,
we will often consult
a pain specialist.
Sometimes these patients have
registries within the hospital
and they know what
works well for them.
Another thing that I would
point out as a side point
is although these patients are
at risk for increased clots,
we do not routinely do
prophylaxis for clots.
That’s my summary of sickle
cell disease in children.
Thanks for your attention.