Sickle Cell Disease

St. Louis Children’s Hospital

Olivia's Story

Olivia is a sweet and funny child who radiates pure joy around anyone she meets. She is always the first one to step up and help others any way she can. Described as a firecracker by her dad, Olivia is full of personality, loves math and to have fun with her tightknit family that includes her 16-year-old brother and parents, Keionna and Maurice.

Before Olivia was born, her parents knew there was a chance she would inherit sickle cell disease, a blood disorder that prevents the body’s organs and tissues from getting the oxygen they need to function properly. Both of her parents are carriers of the sickle cell gene, giving Olivia a greater chance of being diagnosed with the disease. Her newborn screening results came back abnormal, and the pediatrician called her parents to not only request a redraw of Olivia’s screening, but a blood test from both parents as well.

At just 6 weeks old, Olivia’s doctors at St. Louis Children’s officially diagnosed her with the chronic illness. Olivia’s mom was in tears for her daughter because she grew up seeing loved ones struggle with debilitating effects of the disease. There are four main types of sickle cell, and Olivia’s is Hemoglobin SS disease, the most common and most severe, because patients with this variation experience the worst symptoms at a higher rate. While her parents were devastated, they knew the Sickle Cell Disease Program at St. Louis’ Children’s would provide the best and most consistent care for Olivia.

Starting around age 1, doctors discovered that Olivia developed an enlarged spleen, one of the common complications of the disease. Known as splenic sequestration, sickled red blood cells collect in the spleen, causing it to become enlarged and painful. While no immediate action was needed, doctors kept a close watch on her. During a routine medical appointment when she was 3, her care team found her hemoglobin levels were dangerously low and her spleen was even more enlarged.

Olivia was immediately admitted to Children’s for a blood transfusion and to have her spleen removed. A few weeks after surgery, doctors worried about further complications and ordered a specialized ultrasound to evaluate blood flow to her brain that came back abnormal. A subsequent MRI revealed white spots on Olivia’s brain — she had suffered what’s known as a silent stroke due to her extremely low hemoglobin.

The next course of treatment was a series of blood transfusions every six weeks. Unfortunately, after three rounds, Olivia began to develop antibodies against the donor blood. Unable to find a new donor, Olivia was placed on medication to boost her hemoglobin levels.

There are always risks for Olivia. Due to COVID-19, she is currently doing virtual schooling, which prevents her from coming into contact with germs that could make her sick. Like many sickle cell patients who suffer from weakened immune systems, Olivia takes penicillin twice a day to prevent bloodstream infections. She also comes to St. Louis Children’s every three months for routine screenings to make sure she is staying healthy.

Now almost 10-years-old, Olivia, who loves to watch YouTube videos and sketch, is living a fulfilling childhood — something her parents didn’t know would be possible. While she has undergone surgery, numerous blood transfusions and medications to control the risks of further organ and joint damage, asthma and more, Olivia and her family know the outcomes have been better than they ever imagined.

How CMN Funds Help Olivia and Kids Like Her:

Programs that bring animals into the hospital allow kids to take their minds off the medical procedures and focus elsewhere. For Olivia, that meant redirecting her during years of routine blood draws as she developed a fear of needles. Thanks to funds from CMN, Olivia could focus on petting a furry friend next to her instead of paying attention to the nurses during anxiety-provoking procedures. Olivia was able to face her fears but direct her attention elsewhere, making the process much more manageable.