How To Treat Sickle Cell Anemia

What is Sickle Cell Anemia?

Sickle cell anemia is a blood disorder. It's something that happens with the hemoglobin in red blood cells — the protein that distributes oxygen all over your body.

In sickle cell anemia:

• Red blood cells are no longer round and pliable; they become shaped like a sickle (such as a crescent moon).
• These sickle-shaped cells may clump, obstruct blood flow, and tend to break up easily.
• This results in issues such as pain attacks (crises), anemia (decreased red blood cell count), weakness, damage to the organs, and an increased risk of infections.

It's a genetic disorder, so you inherit it from your parents via their genes.

Sickle Cell Anemia Symptoms

These are the primary symptoms of sickle cell anemia:

• Weakness and tiredness (due to anemia)
• Sickle cell crises — intense pain usually in the chest, joints, arms, and legs
• Hands and feet swelling
• Recurring infections (since the spleen becomes damaged)
• Retardation of growth in kids and adolescents
• Vision impairment (if blood vessels in the eyes get clogged with sickled cells)
• Breathlessness or dizziness at times

Symptoms typically begin between 5–6 months of life in infants.

Sickle Cell Anemia Causes

The cause for sickle cell anemia is a mutation in the gene that instructs your body to produce hemoglobin (the oxygen-carrying part of red blood cells).

This is how it occurs:

• Hemoglobin is usually referred to as Hemoglobin A.
• In sickle cell anemia, the body produces abnormal haemoglobin known as Hemoglobin S.
• Hemoglobin S makes red blood cells rigid, sticky, and sickle-shaped.
• These cells will clog blood flow and fragment too early, causing anemia.

You will inherit the disease if you receive the faulty gene from both of your parents. If you receive it from only one parent, you have sickle cell trait, which is that you don't typically get symptoms but may pass the gene on to your kids.

Sickle Cell Anemia Diagnosis

Sickle cell anemia is diagnosed by a few easy tests:

• Blood test: A normal blood sample is drawn to test for abnormal hemoglobin (hemoglobin S).
• Hemoglobin electrophoresis: This special test divides various forms of hemoglobin and indicates whether you have hemoglobin S.
• Newborn screening: In most countries, infants are tested at birth as part of routine newborn screening.
• Prenatal testing: While pregnant, physicians can test the baby's genes by analysing amniotic fluid or placenta samples.

Physicians can also screen for:

• Red blood cell count
• Blood oxygen levels
• Organ function (if sickle cell anemia is already problematic)

Sickle Cell Anemia Complications

Sickle cell anemia may cause a number of complications, some of them mild and others severe:

• Stroke: Sickled blood cells have the ability to clog blood vessels in the brain.
• Acute chest syndrome: Obstruction in the lungs results in chest pain, coughing, and shortness of breath — it's an emergency.
• Organ damage: Kidneys, liver, spleen, and heart can be damaged with time.
• Blindness: Clogged blood vessels in the eye can lead to loss of vision.
• Gallstones: Resulting from the breakdown of too many red blood cells.
• Leg ulcers: Slow-healing sores.
• Pulmonary hypertension: Elevated blood pressure in the lungs, leading to breathing difficulties.
• Infections: The spleen becomes weakened, and the body is unable to resist infections effectively.
• Delayed growth and puberty: Because of insufficient oxygen and nutrients arriving in the body.
• Priapism: Persistent, painful erections in men, resulting from blocked blood flow.

How to Treat Sickle Cell Anemia

Sickle Cell Anemia (SCA) is treated to lessen symptoms, avoid complications, and enhance the quality of life. Even though the disease does not have a universal cure, some medications may help considerably diminish the consequences of the disease. In other instances, bone marrow (stem cell) transplant is a cure.

How to treat sickle cell anemia: Main Goals

• Avert sickle cell compilations
• Treat anemia and pain
• Prevent infections
• Consider chronic issues
• Enhance blood supply and circulation of oxygen

How to treat Sickle Cell Anemia: Standard Treatments

Medications

Hydroxyurea

• It enhances the production of fetal hemoglobin (HbF) that diminishes the red cells sickling.
• Less pain, less arrival to hospitals.
• Unchecked pregnancy.

Pain-relief medications

• Mild pain- acetaminophen or NSAIDs
• Excruciating pain: opioids in medical care

Fibre supplements Boosters of flour-based supplements

• Aids in the formation of red blood cells that are healthy

Antibiotics (e.g. penicillin)

• Administered to children so as to prevent infections, particularly pneumonia

L-glutamine (Endari)

• Lessens oxidative stress and crises rate

Voxelotor (Oxbryta)

• Increases the levels of hemoglobin; minimizes hemolysis

Crizanlizumab (Adakveo)

• Monoclonal antibody to lessen incidences of pain attack

Blood Transfusions

Used to:

• Manage severe anemia
• Prevent stroke (in children at high risk of stroke especially)
• Operation planning
• Complications in pregnancy management

Stem Cell or Bone Marrow Transplant

• It is the only cure for SCA
• Sickle cell-making bone marrow is replaced with healthy donor marrow
• The most successful results are achieved in matched siblings donors
• Possible complications: rejection, infection, chronic immune suppression

Prevention Care and Lifestyle

• Stay hydrated
• Avoid extremes of temperatures
• Health care providers should treat infections promptly.
• Receive routine vaccines (pneumococcus, meningococcus, flu and hepatitis)
• Frequent meetings with hematologist
• Food (no over-exertion) and exercise

Observation and Treatment of Complications

• Eye test: this is a test to identify damage to the retina
• The tests of kidney and liver functions
• Transcranial Doppler (TCD): in children - as an indicator of the risk of stroke
• Cardiac tests: of pulmonary hypertension

New and Emerging Therapies

• Gene therapy: the sickle gene is corrected with the help of gene editing (e.g., CRISPR), in experimental trials
• New drugs under development: attacks the molecular basis of sickling at its roots

Sickle Cell Anemia in Children

Sickle cell anemia in kids usually begins to develop symptoms at around 5 to 6 months of age. Here's what occurs:

Early signs:

• Swelling of the hands and feet (referred to as dactylitis)
• Irritability or fussiness due to pain
• Frequent infections (due to the spleen being involved)
• Slow growth and delayed puberty

As they develop:

• Kids can experience painful sickle cell crises (sudden attacks of pain)
• They can become easily tired and weak (due to anemia)
• They may develop jaundice (yellow color of the skin and eyes)
• They are in danger of severe issues such as strokes or acute chest syndrome

Special treatment for children involves:

• Regular visits
• Preventive vaccinations against infections
• Daily antibiotics (occasionally) to guard against bacteria
• Drinking plenty of water and staying away from extreme temperatures
• Medications such as hydroxyurea to minimize crises

Sickle Cell Anemia Management

Sickle cell anemia management aims at preventing complications, alleviating symptoms, and enhancing quality of life. Here's how it's typically performed:

Medications:

• Hydroxyurea: Reduces the frequency of pain crises and blood transfusions.
• Pain medications: For the treatment of sickle cell crises.
• Antibiotics (such as penicillin): Prescribed to children to avert infections.
• Folic acid supplements: To assist in the formation of new red blood cells.

Blood transfusions:

• Employed to treat severe anemia, avoid stroke, or avert complications.

Bone marrow or stem cell transplant:

• The only possible cure, but it's only available to younger patients with a matching donor.

Lifestyle advice:

• Drink lots of fluids.
• Avoid getting too hot or too cold.
• Try to cope with stress and have plenty of rest.
• Have all the recommended vaccines.
• Have a healthy, balanced diet.

Check-ups:

• To detect and deal with problems early, such as lung problems or damage to organs.

Sickle Cell Anemia Crisis

A sickle cell crisis (also referred to as a pain crisis) occurs when sickle-shaped red blood cells clog up blood vessels to areas of the body, resulting in sudden and intense pain.

Here's what you need to know:

• Pain can occur anywhere but most frequently in the chest, arms, legs, and back.
• The pain can be mild or so severe that it requires hospital treatment.

Crisis triggers include:

• Dehydration
• Infection
• Cold weather or sudden change in temperature
• Stress
• High altitudes (less oxygen)

In a crisis, treatment is centered on:

• Relieving pain (with medicines)
• Hydration (IV fluids if necessary)
• Treating infection
• Oxygen therapy if breathing is compromised

Some individuals have regular crises, but others may have them infrequently. Early treatment can really cut down on how often they occur!

Sickle Cell Anemia Prevention

Sickle cell anemia cannot be totally prevented since it's a genetic disorder. But some measures can help prevent risks and complications:

Genetic counselling:

• Those with a family history can be tested before conception to know the risks.

Early diagnosis:

• Screening of newborns can detect sickle cell early, so treatment begins before issues arise.

Preventive healthcare:

• Regular visits to the doctor
• Vaccinations (to avoid infections)
• Daily antibiotics for young kids
• Taking medications such as hydroxyurea to decrease pain crises

Healthy lifestyle:

• Stay well-hydrated
• Avoid very cold or hot temperatures
• Follow a healthy diet
• Manage stress
• Avoid tobacco and high elevations

Education and awareness:

• Being aware of the symptoms and taking action can stop crises from worsening.

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