How do we diagnose sickle cell anemia?
Well genetic counselling is available for parents
known to have sickle cell disease or trait.
Diagnosis of sickle cell anemia is also done as part
of the routine newborn screening done in most states.
There are benefits to early diagnosis.
These patients can have
prevention and relief of pain episodes.
You can try to prevent infections,
organ damage and strokes.
You can try to treat anemia
and control complications
and these patients can be offered
counselling, prophylaxis, increased immunizations
and follow-up information regarding research.
Managing a patient with
sickle cell disease is complex.
The patient may need the bed rest
during an acute vaso-occlusive crisis
and increased fluid therapy - this is key.
Yyour patient may need one and a half or
two times their normal maintenance.
A blood transfusion of packed red cells
can help in the setting of severe anemia
and analgesics are used to relieve pain.
There should be a really low threshold in treating a
patient with sickle cell anemia who has an infection.
Antibiotics are usually started early as the
patient's immune system is already compromised.
A splenectomy is the surgical removal of the spleen and this
is done in order to prevent recurrence of certain complications.
Your patient may need supplemental oxygen
and this oxygen is gonna be administered
because remember the red blood cells
already have dysfunctional hemoglobin
and this makes a decreased
oxygen carrying capacity.
Supplemental oxygen can help
manage the hypoxia and crisis symptoms.
To help prevent a crisis, patients are
gonna want to try to avoid infections.
They're also going to want to decrease their exposure
to cold because of the vasoactivity that happens.
Same thing goes with stress.
Patients are gonna want to be non smokers
and they want to avoid becoming dehydrated.
You'll have a really low threshold for
rehydrating a patient with sickle cell disease.
A patients with sickle cell disease can
go into a crisis in a hypoxic environment
so they're gonna avoid going to high
altitude areas or maybe climbing tall mountains.
Patients with sickle cell anemia can also prevent
a crisis by taking prophylactic antibiotics.
All children's sickle cell disease you should
take a penicillin twice a day until at least age 5
to prevent pneumococcal sepsis.
You want to be sure your patients with sickle
cell disease are up-to-date on their vaccinations
and this decreases the likelihood that
they'll get these vaccine-preventable diseases
including influenza and pneumococcal.
Children with severe types of sickle cell disease
should be offered primary stroke prevention
with annual transcranial Doppler scans
and blood transfusions when resources allow.
Hydroxyurea is a medication, it's a myelosuppressive
agent that's the only effective drug
proven to reduce the
frequency of painful episodes.
It raises the hemoglobin level and decreases
the rate of painful episodes by about 50%
Education is key in preventing a
crisis as this is a lifelong chronic disorder.