Sickle cell anemia in children: causes, treatment and more

Sickle cell disease is the most serious form of sickle cell disease (SCD). Sickle cell anemia in children occurs when a child has a lower level of healthy red blood cells than expected. The condition is present from birth, although symptoms do not appear in early childhood.

SCD is a group of genetic disorders that cause abnormal hemoglobin proteins in red blood cells. When symptoms begin to appear, a child may experience complications ranging from mild to life-threatening.

Managing SCD requires early diagnosis, a healthy lifestyle, and quality medical care from a hematologist and other sickle cell experts.

Read on to learn more about sickle cell anemia in children, including the causes, treatment, and diagnosis of the condition.

CDS is the result of a genetic mutation that causes abnormal hemoglobin. Hemoglobin is a protein found in red blood cells that gives them their color and helps carry oxygen throughout the body.

Sickle cell anemia, a form of SCD, occurs when a person has fewer healthy blood cells than expected. There are also other forms of this disease, but sickle cell disease is the most common.

It happens when a person inherits two copies of an S protein gene, HbSS. This gene produces an abnormal form of hemoglobin, which causes sickle cell disease and stiff red blood cells.

In a child with SCD, the red blood cells become hard and sticky. The structure of red blood cells also changes and resembles a sickle – an agricultural tool – or the letter C. Doctors call these cells “sickle cells”.

Sickle cells clump together and cannot move through blood vessels as easily as typical red blood cells. As a result, the blood flow is blocked and causes pain in the child.

Sickle cells also die faster than typical red blood cells and can damage the spleen, an organ responsible for filtering infection from the blood. As a result, the child may be more prone to serious infections.

SCD is purely a genetic condition.

Sickle cell disease is usually the most severe form of the disease. It occurs when a person inherits two copies of a gene that codes for hemoglobin ‘S’, an abnormal form of hemoglobin.

Other types of SCD involve the inheritance of one gene for protein S and a different gene for another atypical type of hemoglobin.

However, lifestyle and health factors can influence the severity of a person’s symptoms. For example, high altitude areas make it harder for the body to absorb oxygen, which can exacerbate symptoms.

Sickle cell trait

Children can inherit only one copy of a gene that carries a sickle cell mutation from a parent. When this happens, they have the sickle cell trait, which means they are carriers of SCD and can pass the disease on to future generations.

Sickle cell trait usually causes no symptoms. Very rarely, a person may experience difficulty breathing and other serious complications during strenuous exertion.

How can a child have sickle cell disease if neither parent has it?

A child can have sickle cell disease even if neither parent has it. This happens when both parents have the sickle cell trait, which means they are carriers of SCD, and each passes on a mutated gene to their child.

The increase in newborn screening means parents are likely to know if they are carriers.

However, it is possible to be a carrier and not know it, especially if a person:

  • never tested
  • has no family history of SCD
  • was adopted and does not know his family history

Any child can be born with SCD. However, the condition is much more common in areas with high malaria exposure.

Researchers believe that the sickle cell trait may offer significant protection against malaria. A person born in areas with high rates of malaria or descended from people born in these areas can I have a much higher risk of having the sickle cell trait. This includes people whose ancestors are:

  • Sub-Saharan Africa
  • the Mediterranean
  • Middle East
  • South Asia

According to researchapproximately 1 in 500 African Americans have SCD and 1 in 12 carry the mutation.

Learn more about sickle cell anemia in African Americans.

Babies have no symptoms during the first 6 months of life. This is because they retain hemoglobin from the fetal stage, which prevents the complications of sickle cell disease.

Symptoms tend to appear as babies get older and the sickled red blood cells stick together. This can cause blood clots and blood flow problems and prevent blood from carrying oxygen throughout the body.

Symptoms of SCD include episodes of pain, which doctors may call pain attacks, and anemia.

Signs of anemia include:

Doctors usually Diagnose sickle cell disease during routine newborn screening.

Other aspects of the diagnosis may include taking a family history and a physical exam. A doctor may also order other hemoglobin tests, such as electrophoresis. This uses an electric current to move red blood cell molecules through a gel based on their size and electrical charge.

Routine screening

A drop of blood from a heel prick can show that a person has an abnormal hemoglobin protein. This screening can also identify a child’s type of SCD. For example, a child with sickle cell disease will have two copies of the HbSS embarrassed.

A child who does not have newborn screening may get a diagnosis later, during a routine blood test at a doctor’s appointment. Diagnosis in adulthood is not typical, as survival into adulthood is unlikely without treatment.

Treatment focuses on managing symptoms as they appear and preventing them from getting worse.

Doctors can recommend a treatment plan that includes:

  • make sure the child has all their vaccinations
  • antibiotics to prevent infection
  • iron supplementation
  • pain medication
  • regular eye exams

Some medications they may prescribe include:

  • Hydroxyurea: This medication helps manage painful episodes.
  • Voxelator (Oxbryta): This helps to reduce the sickling of blood cells.
  • Crisanlizumab (Adakveo): This IV medication helps make red blood cells slippery.
  • L-glutamine: This oral medication reduces pain.

People may also receive blood transfusions for severe anemia.

Management may depend on the severity of the disease, the type of sickle cell disease, how often the child has complications, and how well caregivers and children follow preventive measures. Caregivers who help their child follow a healthy lifestyle can help reduce some effects of the disease.

However, it is not always possible to completely prevent a complication from occurring.

Sickle cell disease can affect virtually every aspect of health and development. Common Complications to understand:

Caregivers should explain SCD to their child in age-appropriate terms.

This may involve letting the child know that the condition is not their fault, that they will need ongoing medical care, and that there are things they can do to improve and protect their own health. .

Some strategies for helping a child live with SCD include:

  • enroll them in a sickle cell program at a hospital or clinic that regularly treats children with sickle cell anemia
  • help them find a support group
  • highlighting role models with SCD, such as celebrities Miles Davis and Santonio Holmes, as proof that the condition need not undermine a full and enriched life
  • encourage older children to start managing their condition by giving them feedback on treatment and giving them the opportunity to ask their doctor questions

When to call a doctor

Caregivers of children with sickle cell disease should work with a doctor who specializes in treating this disease in children.

A person should contact a doctor if:

  • a child’s symptoms worsen
  • a child experiences complications from the treatment
  • a child appears sick or in pain
  • a baby’s development is off track

If a child has severe pain or difficulty breathing, caregivers should take them to the emergency room immediately.

Caregivers of children with sickle cell disease may fear that the future is uncertain. However, with proper medical care, children can and do live long and healthy lives with this condition.

Comprehensive newborn screening is essential to diagnose sickle cell disease. After that, parents or caregivers should see a hematologist who specializes in sickle cell disease.

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