Sickle Cell Disease in Kids: What Parents Need to Know for Better Care

Sickle cell disease (SCD) is the most common inherited blood disorder in the U.S. It’s a genetic condition that changes the shape of red blood cells (RBCs) from rounded to rigid, sticky, and crescent-shaped. These deformed cells block blood flow, cause severe pain, trigger anemia, and damage vital organs. Early diagnosis, proper management, and increased understanding are essential for improving the patients’ health.

Who is at Risk for Sickle Cell Disease?

Sickle cell disease mainly affects people whose ancestors came from areas where malaria is or used to be common. Therefore, people with African, Mediterranean, Middle Eastern, Indian, Caribbean, or Latin American backgrounds are at higher risk. In the U.S., African Americans are the group most affected by sickle cell disease.

Additionally, sickle cell disease is passed down by parents. For a child to actually get the disease, they need to receive two faulty hemoglobin genes—one from each parent. However, if a child only gets one faulty gene and one normal one, they won’t have the full disease. Instead, they’ll have what’s called the sickle cell trait(SCT). Usually, this trait doesn’t cause symptoms, but it does mean the person can pass the gene to their children.

Symptoms of Sickle Cell Disease

Symptoms of sickle cell disease typically appear around five months old. Common symptoms include:

• Anemia: Due to sickle cells' short lifespan, children with SCD often suffer from chronic anemia. As a result, they may experience fatigue, pale skin, and slower growth.
• Pain Crises: Sickle cells can clog small blood vessels and cause sudden, sharp pain. These episodes, called pain crises, can happen in the chest, belly, bones, or joints.
• Swelling: Painful swelling in the hands and feet, called dactylitis, is often an early sign in infants. In many cases, this indicates that the sickle cells are causing blockages in smaller blood vessels.
• Frequent Infections: Sickle cells can damage the spleen and make children more prone to infections.  To help protect against this, kids need vaccinations and preventive antibiotics. 
• Acute Chest Syndrome: This condition is similar to pneumonia and can cause chest pain, fever, and breathing difficulty. 

Diagnosis of Sickle Cell Disease

Most states in the U.S. check for SCD at birth through a routine newborn blood test. Typically, a simple blood test looks for abnormal hemoglobin. If doctors suspect sickle cell disease, they perform further tests such as hemoglobin electrophoresis. This latter test can confirm the diagnosis and identify the specific type of SCD. This is the reason why early diagnosis is crucial for people suspected of SCD. Doing so allows for swift care and preventive treatment and  lowers the risk of serious complications.

Treatment and Management

While there is no universal cure for sickle cell disease, various treatments can help manage symptoms and prevent complications. Doctors can tailor treatment plans to the individual needs of each child and may include:

• Pain Management: Treat pain crises using over-the-counter pain relievers or prescription medicines. In severe cases, doctors may hospitalize patients to provide IV fluids and pain relief.
• Staying Hydrated: Drink plenty of water to help lower the chances of pain episodes. By staying well-hydrated, children can reduce the risk of cells clumping together.
• Blood Transfusions: Regular transfusions can treat severe anemia. They also help prevent strokes and other complications.
•  Hydroxyurea: This medication reduces pain crises and acute chest syndrome. Specifically, it boosts fetal hemoglobin production which stops red blood cells from sickling. Over time, this can lead to fewer complications.
• Preventive Care: Vaccinations and daily antibiotics help protect against infections. Additionally, regular check-ups and screenings catch any organ damage early.
• Stem Cell Transplant: In some cases, a stem cell transplant from a matched donor offers a potential cure. However, it comes with significant risks and isn’t suitable for everyone.

Medical Advances and Family Support

Medical advances have greatly improved the outlook for children with sickle cell disease. For instance, gene therapy aims to correct the genetic cause of the disease and may offer a cure. Additionally, new medications reduce pain episodes, lower infection risks, and improve blood flow. With the right care, many kids can live healthy, active lives.

Families play a major role in the health of children with SCD. They make sure children receive regular check-ups, follow treatment plans, and maintain healthy habits. They also offer emotional support during pain episodes and encourage a positive outlook. This support network helps manage the condition and improves the child’s quality of life.

Additionally, education and awareness are essential for families caring for children with SCD. Learning about the condition helps parents and caregivers respond to emergencies quicker and manage symptoms more effectively. Moreover, support groups and counseling provide emotional support and practical tips. These resources help families and patients cope with the challenges of this lifelong condition.

Facing SCD with Strength

Living with sickle cell disease presents many challenges. Yet, each day offers a chance to show resilience and courage. With the expertise of medical teams and the care of loved ones, facing the hardest moments becomes manageable. More importantly, managing SCD becomes easier and a healthier future becomes achievable by staying informed and taking proactive steps

Looking for more ways to support your child with SCD? Click here for helpful tips and guidance.