For Za’Riah, Johns Hopkins All Children’s Hospital is something of a second home.

Za’Riah, 10, has been visiting the hospital’s main campus in St. Petersburg, Florida, for about eight years and more recently has been receiving care at the Outpatient Care location in Tampa.

“We love All Children’s,” says her mother, Jai’Lynique.

Za’Riah has HbSS, commonly known as sickle cell anemia, a disease of the red blood cells that is inherited when both parents pass an Hbs gene to the child. It is marked by reduced hemoglobin levels in the body resulting in periods of fatigue, skin pallor, shortness of breath, lightheadedness, dizziness or a fast heartbeat.

The various forms of sickle cell disease are the most common inherited form of blood disorder. Sickle cell disease most commonly affects children of African descent, Hispanics and those of Caribbean or Mediterranean ancestry.

Like many, Za’Riah was diagnosed shortly after birth.

Za’Riah’s Early Care

Za’Riah was born in Spring Hill. Newborns routinely are given blood tests, including one for sickle cell disease. About 100,000 people in the United States have sickle cell, according to the National Institutes of Health.

Red blood cells carry oxygen to the body’s tissues. They generally move easily through blood vessels, but sickle cells are stiff and sticky and are often shaped like the letter C. They tend to cluster and struggle to move through small blood vessels. They can stop the movement of healthy, oxygen-carrying blood. The decrease in oxygen delivery to the tissues and organs can cause pain and organ dysfunction. 

At about age 2, Za’Riah was referred to the sickle cell program at Johns Hopkins All Children’s, one of the largest such programs on Florida’s west coast. 

Initially, Za’Riah had good luck with hydroxyurea, an oral medication commonly used to treat sickle cell. Za’Riah’s case became more complicated about four years ago when she developed asthma. She began taking a liquid steroid for the asthma, but Jai’Lynique says it’s difficult for Za’Riah to take it all at once.

“It’s hard to have two chronic conditions like sickle cell and asthma,” says Tamara New, M.D., director of the sickle cell program at Johns Hopkins All Children’s. “The child has a lot to deal with in that situation.”

A New Twist

Za’Riah has an annual test called a transcranial doppler ultrasound (TCD), which uses sound waves to detect blood flow in the brain.

Earlier this year, the TCD showed a lower rate of blood flow, which can lead to a stroke. After consultation with Johns Hopkins All Children’s pediatric neurologist Thomas Geller, Za’Riah switched from hydroxyurea to monthly blood transfusions.

“She had concerning findings on her TCD so we obtained an MRI/MRA,” New says. “This showed changes that put her at increased risk of stroke, which is why we started her on these transfusions.

“Transfusion therapy is relatively common in patients with sickle cell and monthly red cell exchanges are common for patients who have either had a stroke or in whom we are trying to prevent stroke.”

Although she still loves the hospital, Za’Riah doesn’t enjoy the transfusions.

“We are currently working with her closely to help her with her anxiety about these procedures,” says Dawn Gates, the nurse coordinator for the sickle cell clinic who Za’Riah knows as “Nurse Dawn.” 

Za’Riah will have another TCD soon to evaluate the progress, but her mother says she hasn’t had significant pain episodes for a while.

“Za’Riah is a special girl for sure,” Gates says. “She is a ray of sunshine despite all of the medical hurdles that she has had to endure.”