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,
bloodstream infections, 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.