Sickle Cell Complications in Children

Sickle cell disease (SCD) is a lifelong genetic disorder that affects red blood cells, causing them to take on an abnormal crescent or sickle shape. These abnormally shaped cells can lead to numerous sickle cell complications, especially in children who are still undergoing vital stages of growth and development. 

The condition occurs when a child inherits two abnormal hemoglobin genes, one from each parent. If both parents carry the sickle cell trait, there is a 25% chance their child will develop SCD. The disease can present as sickle cell anemia (HbSS), the most severe form, or other variants such as HbSC or HbSβ-thalassemia.

How is sickle cell disease diagnosed?

In Nigeria, newborn screening for sickle cell disease is not widely implemented, but with early diagnosis, it is possible to effectively manager the condition. Blood tests, such as hemoglobin electrophoresis or high-performance liquid chromatography (HPLC), can detect abnormal hemoglobin types in children. These tests should ideally be done shortly after birth to ensure early intervention and proper care.

Sickle Cell Complications in Children

Children with sickle cell face a variety of complications that can affect their physical and cognitive development. These complications include:

1. Pain Crises (Vaso-Occlusive Episodes)
   • What it is: These painful episodes are the most common and distressing sickle cell complications. They occur when sickle-shaped cells block blood flow to various parts of the body, causing severe pain.
   • Impact: For children, these crises can result in school absences, limited physical activity, and frequent hospital visits. The unpredictable nature of pain crises can take a toll on their emotional well-being as well.
   • Prevention: How to prevent sickle cell crises in a child involves regular hydration, avoiding extreme temperatures, managing stress, and preventing infections that could trigger a crisis.
2. Acute Chest Syndrome
   • What it is: This life-threatening condition is similar to pneumonia and occurs when sickle cells block blood flow in the lungs.
   • Impact: Acute chest syndrome can cause breathing difficulties, fever, and severe chest pain, and requires immediate medical attention. It is one of the leading causes of death among children with SCD.
   • Prevention: Avoiding respiratory infections through vaccination and prompt treatment of any signs of infection are key strategies.
3. Stroke
   • What it is: Sickle cell increases the risk of stroke in children, particularly due to blocked blood vessels in the brain.
   • Impact: Strokes can cause lasting physical and cognitive impairments, affecting a child’s ability to learn, speak, and move.
   • Prevention: Regular transcranial Doppler (TCD) screening for at-risk children, combined with blood transfusions, can significantly reduce the risk of stroke.
4. Delayed Growth and Puberty
   • What it is: Due to the reduced oxygen-carrying capacity of sickle cells, children with SCD often experience delayed growth and puberty.
   • Impact: Slower growth can affect a child’s self-esteem, especially if they fall behind their peers in terms of physical development.
   • Prevention: Regular nutritional support, supplementation, and healthcare monitoring can help improve growth outcomes for children with SCD.
5. Frequent Infections
   • What it is: Sickle cell compromises the spleen, an organ critical for fighting infections, making children more susceptible to bacterial infections like pneumonia and meningitis.
   • Impact: Infections can escalate rapidly and become life-threatening, especially in areas like Nigeria, where access to healthcare can be limited.
   • Prevention: Regular use of prophylactic antibiotics, vaccination against common infections, and timely medical intervention for fevers are vital preventive measures.

Sickle Cell in Nigeria: A National Health Challenge

Nigeria bears the largest burden of sickle cell disease globally, accounting for more than 50% of the world’s cases.

Research has shown that about 50 million people are live SCD globally and Nigeria is the epicentre zone. The country has about 4-6 million people living with the disease (1 in every 4 Nigerians has a sickle cell trait).

Children with SCD in Nigeria often face delayed diagnosis, limited access to specialized care, and a lack of consistent medication. Many succumb to complications before the age of five, primarily due to infections or lack of proper crisis management.

How to Prevent Sickle Cell Crisis in a Child

Preventing crises and sickle cell complications is a big challenge for parents and caregivers, but there are strategies that can significantly reduce the frequency and severity of complications.

1. Hydration: Keeping children hydrated helps prevent the sickle cells from clumping together and causing blockages. Encourage regular water intake, especially during physical activities or hot weather.
2. Avoiding Temperature Extremes: Extreme cold or heat can trigger pain crises. Keeping children warm in cold weather and cool in hot weather is essential.
3. Managing Stress and Infections: Emotional and physical stress, including infections, can worsen sickle cell symptoms. It’s crucial to manage stress levels and promptly treat any signs of illness, especially fevers.
4. Medications and Supplements: The regular use of medications like hydroxyurea can reduce the number of pain crises by increasing fetal hemoglobin, which doesn’t sickle. In addition, folic acid supplements support red blood cell production.
5. Vaccinations: Ensure children receive vaccines against common infections like pneumonia, meningitis, and influenza. These illnesses can exacerbate sickle cell complications.
6. Routine Health Checkups: Regular visits to a hematologist can help monitor a child’s health and manage complications before they become severe. Screenings such as transcranial Doppler ultrasounds help detect stroke risks early.

Living with Sickle Cell

Despite the challenges, advancements in healthcare and better management practices offer hope for a better quality of life. With early diagnosis, effective management of complications, and preventive strategies, many children with SCD can lead fulfilling lives.

In Nigeria, improving public awareness about SCD and providing more accessible healthcare services are essential for reducing the burden of the disease. Organizations and governments must work together to ensure that children born with sickle cell can access the care they need.

Genetic Awareness in Nigeria: Taking Steps to Prevent Sickle Cell Disease

Understanding how to prevent sickle cell crises and ensuring timely treatment for complications are critical to help children live healthier, happier lives. Even if you’re not a parent, being informed about your genetic status empowers you to contribute to prevention and management of SCD in Nigeria. Education, awareness, and early detection are key in creating a healthier future for generations to come.

Related: Sickle Cell Disease in Nigeria: Can we talk about Genetic Testing?