Inherited Blood Disorder in Children

Sickle cell anemia is the most common and severe type of inherited blood disorder. Children with sickle cell anemia have inherited hemoglobin S genes from both parents. The hallmark of the disease is sickle-shaped red blood cells. Instead of healthy, round red blood cells moving smoothly through your child’s body, the sickle-shaped red blood cells are sticky and tend to clump together, causing blockages in small blood vessels.

Sickle cells also have a shorter lifespan than healthy red blood cells, causing them to “clog” in the bloodstream as they die off, instead of being naturally filtered out by the spleen. Fewer healthy red blood cells can cause anemia and also damage your child’s spleen, which is a critical component of filtering infections out of the blood.

Symptoms, Diagnosis and Outlook

Children with two sickle cell genes are born with the disorder, although they typically won’t start showing symptoms until they are a few months old. Children with one sickle cell gene are healthy, but will be sickle cell carriers – meaning they can pass it on to their future children one day.

Sickle cell symptoms can include:

  • Acute chest syndrome: the sickle cells clump together in the lung blood vessels and causes dangerous pneumonia-like symptoms (violent cough, fever and pain)
  • Anemia: the most common sickle cell symptom causing your child to be pale and lethargic
  • Jaundice: children with sickle cell anemia can have the telltale yellow coloring of jaundice in their eyes and skin. Jaundice is caused by the quickly-dying sickle blood cells not being filtered out of the body fast enough, causing bilirubin to build up
  • Sickle Cell Crisis: this dangerous symptom is caused by the sickle cells getting stuck in small blood vessels, blocking blood flow and causing pain in the arms, legs, chest, fingers or toes
  • Pooling: when sickle cells clump together in the spleen, they can get stuck there. This build-up slows down other red blood cells from moving, causing a sudden and dangerous drop in hemoglobin

Many hospitals now include hemoglobin testing as part of your child’s routine newborn screening. Diagnostic tests to determine if your child has sickle cell anemia include blood tests, a physical exam and a comprehensive family history.

At Penn State Children’s Hospital, our team of pediatric hematologists are here to help your child – and your entire family – every step of the way. We know that early diagnosis is critical to your child’s overall health, and our multidisciplinary team will work together to develop a treatment plan to help prevent some of the severe complications of sickle cell anemia.

Our Experts in Care

The pediatric hematology experts at Penn State Children’s Hospital are committed to providing our patients with comprehensive, multidisciplinary sickle cell anemia care.

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Why Choose Penn State Children’s Hospital for Care

Comprehensive Sickle Cell Anemia Program

The Pediatric Comprehensive Sickle Cell Anemia Program at Penn State Children’s Hospital offers robust diagnosis, treatment and ongoing management for sickle cell patients. Our expert team cares for more than 150 affected children and their families through preventive care and therapy for both ongoing symptoms and acute complications. Our goal is to help your child learn to manage life with sickle cell disease – and we’re here to provide support and resources to make that happen.

Commitment to Research

Penn State Children’s Hospital frequently participates in clinical trials on conditions like sickle cell anemia. For more information on clinical trials at Penn State Children’s Hospital, please visit StudyFinder.

Support Groups

Support groups provide children and their families an opportunity to connect with others in similar situations. Learn more about the support groups offered at Penn State Health Children’s Hospital.

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