Describe in detail the pathophysiological process of sickle cell anemia

What is the etiology of sickle cell anemia?
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Describe in detail the pathophysiological process of sickle cell anemia

Describe in detail the pathophysiological process of sickle cell anemia
Describe in detail the pathophysiological process of sickle cell anemia
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SCA is characterized by abnormal hemoglobin synthesis, which results in the formation of sickle-shaped red blood cells (RBCs) (Kato, Steinberg & Gladwin, 2017). Because of their disfigured shape, sickle-shaped RBCs have a shortened life span, are unable to transport enough oxygen to the tissues in the body, and become trapped in the vasculature, resulting in vascular blockage, pain, and organ infarction (McCance, Huether, Brashers, & Rote, 2013). Sickle cells undergo hemolysis in the spleen or become blocked there, resulting in blood drawing and splenic vessel infarction (McCance, Huether, Brashers, & Rote, 2013). The resulting anemia initiates erythrocytes in the bone marrow and, to a lesser extent, in the liver. After reoxygenation and rehydration, most sickled erythrocytes return to their normal form (McCance, Huether, Brashers, & Rote, 2013).

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Nonreversible sickling is caused by irreversible plasma destruction rather than irreversible hemoglobin changes (McCance, Huether,

Describe in detail the pathophysiological process of sickle cell anemia
Describe in detail the pathophysiological process of sickle cell anemia

Brashers, & Rote, 2013). The plasma membrane loses some of its ability for active transport, allowing calcium ions to enter (McCance, Huether, Brashers, & Rote, 2013). Individuals with SCA, whose erythrocytes have an elevated proportion of hemoglobin S, may have up to 30% of their red blood cells become irreversibly sickled (McCance, Huether, Brashers, & Rote, 2013). Sickling is a rare, sporadic phenomenon that can be caused or exacerbated by one of the following stressors: low blood oxygen tension (hypoxemia), high hydrogen ion concentration in the blood (decreased PH), high plasma osmolarity, low blood volume, and low temperature (McCance, Huether, Brashers, & Rote, 2013).

The increased reduction of PO2 attributed to tenacious hypoxemia caused by stressors ultimately leads to sickling in the microcirculation of all cells that have Hb S in the site not through the body. Decline blood pH curtails hemoglobin’s affinity for oxygen (McCance, Huether, Brashers, & Rote, 2013). When decreased oxygen is picked up by hemoglobin in the lungs, the PO2 falls promoting additional sickling (McCance, Huether, Brashers, & Rote, 2013). Increased osmolarity of the plasma pulls water out of the red blood cells. This promotes sickling by increases the relative Hb S composition in the red blood cells (McCance, Huether, Brashers, & Rote, 2013).

Symptoms

Symptoms of sickle cell anemia commonly begin around the age of five months.
They differ from one individual to the next and evolve with time.
The following are examples of signs and symptoms:

Anemia.
Sickle cells readily disintegrate and die, leaving you with an insufficient number of red blood cells.
Red blood cells last approximately 120 days before needing to be replaced.
However, sickle cells normally die in 10 to 20 days, leaving a red blood cell shortage (anemia).

Your body can’t get enough oxygen if there aren’t enough red blood cells, resulting in weariness.

Painful episodes.
Pain crises, or periodic episodes of pain, are a common symptom of sickle cell anemia.
Pain occurs when sickle-shaped red blood cells impede blood flow to your chest, abdomen, and joints through tiny blood channels.
Bone pain is also a possibility.

The discomfort can range in intensity and linger anywhere from a few hours to several weeks.
Some folks only have a couple of pain crises every year.
Others suffer from a dozen or more pain crises every year.
A hospital stay is required in the event of a serious pain crisis.

Chronic pain affects certain adolescents and adults with sickle cell anemia, which can be caused by bone and joint degeneration, ulcers, and other factors.

Hands and feet swell up.
Sickle-shaped red blood cells limit blood flow to the hands and feet, causing edema.

Infections occur frequently.
Your spleen may be damaged by sickle cells, making you more susceptible to infections.
Vaccinations and antibiotics are regularly given to newborns and children with sickle cell anemia to prevent potentially life-threatening illnesses like pneumonia.

Puberty or delayed growth.
Red blood cells transport oxygen and nutrients throughout your body, allowing you to thrive.
In newborns and children, a lack of healthy red blood cells can limit growth and cause puberty to be delayed.

Issues with vision.
Sickle cells can clog the tiny blood arteries that supply your eyes.
This can cause vision issues by damaging the retina, which is the part of the eye that interprets visual images.

When should you see a doctor?

Sickle cell anemia is most commonly detected in children through newborn screening programs.
If you or your kid gets any of the following symptoms, contact your doctor or seek emergency medical attention straight away:

Fever.
People with sickle cell anemia are more likely to get sick, and fever is often the first sign of infection.

Severe discomfort in the belly, chest, bones, or joints that cannot be explained.

Hands and feet are swollen.

Swelling in the abdomen, particularly if the area is painful to the touch.

Pale skin or a lack of color in the nail beds.

The skin or the whites of the eyes have a yellow tinge.

Symptoms or signs of a stroke.
Call 911 or your local emergency number straight away if you develop one-sided paralysis or weakness in the face, arms, or legs; confusion; difficulty walking or talking; sudden vision changes or inexplicable numbness; or a strong headache.

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Causes

A mutation in the gene that signals your body to generate the iron-rich compound that makes blood red and allows red blood cells to transmit oxygen from your lungs throughout your body causes sickle cell anemia (hemoglobin).
The defective hemoglobin in sickle cell anemia causes red blood cells to become hard, sticky, and malformed.

For a kid to be impacted, the faulty form of the gene must be passed down from both parents.

If only one parent carries the sickle cell gene to their child, the youngster will inherit the characteristic.
People with the sickle cell trait produce both normal and sickle cell hemoglobin because they have one normal and one faulty hemoglobin gene.

They may have sickle cells in their blood, but they are usually asymptomatic.
They are, nonetheless, disease carriers, meaning they can pass the gene on to their children.

Factors that are at risk

Both parents must contain the sickle cell gene for a child to be born with sickle cell anemia.
Sickle cell anemia is most common in black people in the United States.

Complications

Sickle cell anemia can cause a variety of problems, including:

Stroke.
Blood flow to a part of the brain can be blocked by sickle cells.
Seizures, numbness or paralysis in your arms and legs, abrupt speech difficulties, and loss of consciousness are all symptoms of a stroke.
If your child exhibits any of these signs or symptoms, get medical help right once.
A stroke has the potential to be fatal.

Acute chest syndrome is a condition in which the chest muscles become inflame
This life-threatening consequence can be caused by a lung infection or sickle cells obstructing blood arteries in the lungs, causing in chest discomfort, fever, and difficulty breathing.
It may necessitate immediate medical attention.

Hypertension of the lungs.
High blood pressure in the lungs can occur in people with sickle cell anemia.
Adults are most commonly affected by this condition.
Shortness of breath and exhaustion are prominent signs of this potentially lethal illness.

Damage to the organs.
Sickle cells that obstruct blood flow to organs starve the organs of oxygen and blood.
Sickle cell anemia causes persistent oxygen deficiency in the blood.
This shortage of oxygen-rich blood can harm nerves and organs, such as the kidneys, liver, and spleen, and can even be fatal.

Blindness.
Sickle cells can obstruct the tiny blood arteries that supply your eyes with oxygen and nutrients.
This can harm your eye and cause blindness over time.

Ulcers on the legs.
Open sores on the legs are a symptom of sickle cell anemia.

Gallstones.
Bilirubin is a chemical produced by the breakdown of red blood cells.
Gallstones are caused by a high quantity of bilirubin in the body.

Priapism.
Men with sickle cell anemia may experience painful, long-lasting erections.
Sickle cells can obstruct the blood vessels in the penis, resulting in impotence.

Complications of pregnancy
During pregnancy, sickle cell anemia can raise the risk of high blood pressure and blood clots.
It also raises the chances of miscarriage, early birth, and babies with low birth weight.