As a child, Mikayla wanted to be a pediatrician, but when her newborn daughter, Amoura, was diagnosed with sickle cell disease, her interests evolved.

Amoura, who turned 6 in August, had her diagnosis confirmed at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida, where she has been treated ever since.

Mikayla studied and became a registered nurse, currently working in critical care at Sarasota Memorial Hospital, which has collaborated with Johns Hopkins All Children’s for decades. While she likes the fast pace of critical care, Mikayla’s experience with Amoura causes her to aspire to work with cancer and sickle cell patients in the future.

“Early on, I wanted to work with kids and be a pediatrician,” Mikayla says. “But as I’ve learned about cancer and sickle cell, I’d like to work with kids like that and help them have a bright future.”

Early Challenges

Amoura was diagnosed at Lakewood Ranch Medical Center through a routine “heel stick” blood test given to newborns.

Mikayla was told to take her to Johns Hopkins All Children’s for confirmation. The hospital has one of the largest sickle cell programs on the west coast of Florida. Sickle cell disease is the most common inherited form of blood disorder, mostly affecting children of African descent, Hispanics and those of Caribbean or Mediterranean ancestry. It is a disease of the red blood cells that is inherited when both parents pass an Hbs gene to the child.

“I knew people who had it,” Mikayla says. “I even knew some people died early from it, and I knew it caused pain and missed school.” 

Amoura’s first year was challenging. Any time she had a fever of 101 degrees or more, Mikayla had to bring her to the hospital, often to stay for a couple of nights. This happened every two to three months that first year.

“Patients with sickle cell are at increased risk of infection from some forms of bacteria, therefore when they are first diagnosed, we start them on twice daily penicillin prophylaxis antibiotics,” says Tamara New, M.D., director of the sickle cell program at Johns Hopkins All Children’s. “Also, fever of 101 F or higher is always a medical emergency for these patients. When they are young infants, this often means being hospitalized on IV antibiotics while we wait to see if they have a serious infection. As kids get older, it still means coming to the hospital right away for fever but not necessarily needing to remain hospitalized overnight.”

As Amoura got older, the care became less complicated. She takes hydroxyurea, a common oral medication for sickle cell, and it generally has controlled her symptoms well. She visits the sickle cell clinic at All Children’s every two to three months, eagerly anticipating the prize she receives after her blood draw. She also has an annual test called a transcranial doppler ultrasound (TCD), which uses sound waves to detect blood flow in the brain.

“I am in a unique and blessed position here at Johns Hopkins All Children’s because I get to meet these kiddos at birth and watch them grow up alongside of their parents,” says Dawn Gates, nurse coordinator for the sickle cell clinic. “With our population of patients, we become like extended family members.” 

A Crisis

Aches and pains are part of the sickle cell experience and, in some cases, they can be quite severe.

Amoura has been fortunate to not experience many crisis events, which occur when the sickle-shaped red blood cells that mark sickle cell disease block blood flow in tiny vessels and cause pain throughout the body.

Amoura experienced a crisis a few months before her sixth birthday that was coupled with pneumonia and acute chest syndrome, a complication of sickle cell disease that can potentially be fatal. She received her first blood transfusion as a result.

“A vaso-occlusive crisis or vaso-occlusive episode is when the sickled cells get trapped in the blood vessels and cause a blockage of blood flow and decreased blood flow to the tissues causing damage,” New says. “This can be reversible damage (such as pain) or irreversible (such as the blockage that causes stroke). Amoura handled her transfusion well.

“Sickle cell is a chronic lifelong condition with peaks and valleys — periods that are very challenging and periods that are stable — every person has a slightly different journey.”

Moving Forward

Although sickle cell is a chronic disease that Amoura likely will have for life, Mikayla looks forward with optimism both personally and professionally.

She has allowed Amoura to be part of some clinical research studies designed to improve sickle cell care, and she continues to advance her nursing career.

Mikayla is grateful to the sickle cell team — especially Gates, nurse practitioner Carrie Gann and patient care technician Judith Celestin — for supporting her from her time as a new mother to every stage along the way to become a full-fledged nurse on her own.

“They have been there since day 1,” Mikayla says. “They ask about us, and I’ve gotten to know them. It’s a real personal relationship. It feels like a family there.”