The signs and symptoms of
sickle cell anemia are gonna vary.
Fortunately, sickle cell crisis does not
occur in infants under 5 to 6 months of age.
The child will still genetically have sickle cell
disease but does not show any of the signs and symptoms.
After birth, the fetal hemoglobin is still
in circulation preventing a sickle cell crisis.
However, once the fetal hemoglobin
is replaced with adult hemoglobin,
usually around 5 to 6 months of
age, a sickle cell crisis is possible.
The severity of symptoms can vary
and can present as fussiness in babies.
Your patient may have jaundice because remember the sickled
cells do not live as long as the normal red blood cells.
A regular red blood cell lives about 120 days but in
sickle cell anemia, remember they die after 10 to 20 days.
When they die, this waste product called bilirubin
is released from the destroyed red blood cells
and it's going to start accumulating causing
jaundice or the yellowing of the skin or eyes.
The patient can also have classic symptoms of anemia
including frequent dizziness or lightheadedness,
fatigue or malaise, irritability, pale skin or
pallor and delayed growth and development
and this is thought to be due to
the lack of oxygen to the organs.
Signs of symptoms of a complication
involving sickle cell is called hand-foot syndrome
and this is when sickled cells are gonna block
the small blood vessels in the hands and feet.
This causes pain swelling and a fever.
This can affect one or both
hands, and feet can also be affected.
This condition typically last 1 to 2 weeks and it's
seen most often in kids 6 months to 2 years old.
The treatment for this involves hyperhydration
or increasing fluids, and pain medication.
A vaso-occlusive crisis means there is a reduced
blood supply to the organ and tissues called ischemia
and this is going to cause
severe and acute pain.
The signs and symptoms of vaso-occlusive
crisis depend on the organ affected.
In the lungs this is called
acute chest syndrome.
In the brain, it can cause a stroke.
In the eyes, it can cause retinopathy.
The bones can develop avascular necrosis.
The kidneys can show a nephropathy,
and the penis can show priapism.
Acute chest syndrome is a vaso-occlusive
crisis that occurs in the pulmonary vasculature.
This is a medical emergency and the
patient can die without immediate treatment.
The patient will likely present in
some form of respiratory distress
and maybe using their
accessory muscles of respiration
or they may have nasal flaring to
try to bring more oxygen into the lungs.
Due to the massive inflammatory process,
there will likely be leukocytosis on the CBC
which is an increased
white blood cell count.
The diagnosis of acute chest syndrome is made when
a patient has a new infiltrate on their chest x-ray
and at least one of the
following other symptoms:
cough, wheezing, chest pain, tachypnea
which is an increased respiratory rate, or fever.
There are other complications of acute
chest syndrome including atelectasis
and this can happen when the
patient has a lot of pain in their ribs
or if they have a vertebral infarction
which is decreased blood flow in the spine.
This can cause a lot of pain with deep breathing
so the patient's gonna take more shallow breaths.
They're gonna have a decreased
respiratory drive and cough.
A true thromboembolism can happen and
this is due to the activated endothelial lining.
There is a reduced red blood cell flexibility and
this is going to increase the red cell adherence.
This can cause the vaso-occlusion.
A fat embolism can be triggered
due to a bone marrow infarction
and infections are another
trigger of acute chest syndrome.
These are the common infections
that can trigger acute chest syndrome:
Chlamydia pneumoniae, Mycoplasma pneumoniae, Staph.
aureus, Strep. pneumoniae, Respiratory Syncytial Virus,
the parvovirus, rhinovirus which is the common
cold and influenza and parainfluenza viruses.
Another component of sickle cell crisis
is called acute splenic sequestration crisis
and this is a medical emergency that can lead
to death unless prompt treatment is carried out.
It is usually due to a rapid enlargement of the spleen as
a result of trapping the circulating blood in the spleen.
This causes a drastic reduction in the
blood flow to the other organs of the body
and there will be a life-threatening anemia.
This typically occurs in
children under 2 years of age.
The child will have a rapid enlargement
of their spleen called splenomegaly
as a result of the circulating
blood going straight to the spleen.
This causes hypovolemia as there's a drastic reduction
of the blood flow to the other organs of the body
because most of that blood volume
becomes trapped in the spleen.
This is harder to recognize in a patient
than those who have external bleeding
because no blood is leaving the body but
the patient is still going to get hypovolemic
as that blood available to maintain
their hemodynamic stability is decreased.
You'll see a drop in the hemoglobin
level of greater than 2 g/dL.
The patient will also develop reticulocytosis
which is an increased reticulocyte count
as the body tries to churn
out more red blood cells.
The platelet count will begin to drop.
The patient may or may not have a
fever and the patient will experience pain.
The patient's then going to develop a
life-threatening and circulatory collapse.
Death can occur in less than 30 minutes
if the situation is not urgently corrected
with blood transfusions and a splenectomy.
Aplastic crisis is characterized by
an acute drop in the hemoglobin level
and a markedly reduced number
of reticulocytes in the blood.
This occurs when a patient with sickle cell disease is
infected with a certain common viral or bacterial pathogen.
The most common causes of infection are
the human parvovirus B19 or Fifth disease
and this is characterized by fever, malaise
and red cheeks that have a slapped cheek look.
It can also be caused by other infection
such is Strep. pneumoniae, Salmonella,
other strep infections and the Epstein-Barr virus.
During an aplastic crisis, the bone marrow
has impaired production of red blood cells
and this causes a rapid and
life-threatening drop in hemoglobin.
Hemolysis is gonna continue but the bone marrow is not
able to churn out new cells fast enough to compensate.
The marrow will recover in about 7 to 10 days
and then red blood cell production will continue.
The management of aplastic
crisis is with a blood transfusion.
Patients can also develop a stroke and this is the
most common central nervous system manifestation.
The patient may experience weakness on
one side of the body also known as hemiparesis,
and seizures may occur.
After the stroke, the patient may
have cognitive and behavioral effects
and children may have a
decreased school performance.
Sickle cell anemia patients are susceptible to infections as a
result of the inability of their spleen to function properly.
There is also a defect in their complement system
which is used to eliminate harmful microorganisms
and it's an important
component of immune function.
This defect in the complement system causes an inability
to deal with encapsulated microorganisms in the body
leading to infection.
Patients with sickle cell anemia
are at increased risk for: pneumonia,
meningitis and bone infections.
Patients with sickle cell disease
also experience chronic recurrent pain,
chronic pains associated with depression
and other psychological disorders.
Compared with the general population, patients with
sickle cell disease have a higher prevalence of depression.
Here you can see the patient with
sickle cell disease is having daily pain.
This is gonna contribute to a lower mental
health related to their quality-of-life score
and lower physical health because it hurts.
In turn, this patient'sgoing to also
have a greater medical resource use
and this is gonna result
in higher healthcare costs.