Juvenile Myelomonocytic Leukemia: Arianna’s Story

“We were told so many times that we might be able to go home and it kept not happening,” says Katrina, Arianna’s mom. “It was only when we got to the car and put her in her car seat, that it hit us — this is really happening. We’re taking her home.”

Today, Arianna is cancer-free. The sassy and expressive toddler will need ongoing monitoring and treatment, but for now, her mom says, “I just want her to be a kid, to be happy and enjoy life.”

Signs of trouble

Arianna’s birth on Oct. 15, 2014, was joyfully uneventful and her first year was filled with typical milestones like rolling over, babbling, and reaching for toys. She lived with her parents, Katrina and Matt, and her grandparents.

When Arianna was about 1 year old, her family noticed a change. First, she had frequent low-grade fevers and occasional vomiting — which her family chalked up to teething — but then her behavior changed too. Arianna cried and slept a lot more than usual, and refused to eat except to nurse.

Arianna’s parents took her to a pediatrician who said the baby had a virus and to come back if symptoms persisted. The family came back the next day, and the next. “We knew she wasn’t herself,” Katrina says. “Something was wrong.”

Katrina and Matt were worried and wanted answers. The pediatrician suggested bloodwork which could pinpoint any potential problems.

“The lab called me the next day and told me to take Arianna to the nearest hospital immediately,” Katrina says. The pathologist suspected cancer.

In shock, Katrina collapsed before forcing herself to pack a bag and take Arianna to a local hospital. After a day of testing and still no closer to a diagnosis, the hospital transferred Arianna to Children’s Hospital of Philadelphia (CHOP).

Cancer in her blood

Once at Children’s Hospital, a multidisciplinary team of emergency room doctors, oncologists, hematologists nurses and others worked together to stabilize Arianna and diagnose her condition. She’d become severely anemic and required eight blood transfusions.

Within hours, doctors determined Arianna had leukemia, the most common form of cancer in children. However, it took several days and a battery of tests to determine she had juvenile myelomonocytic leukemia (JMML), a rare cancer of the blood that mostly affects children younger than 2 years old.

“We absolutely knew we were in the best place for Arianna’s care — especially because her type of leukemia is so rare,” Katrina says.

Starting treatment

Oncologists and attending physicians in CHOP’s Cancer Center, Leukemia and Lymphoma Program and Blood and Marrow Transplant Program (BMT) guided Arianna’s care. Together, the nationally recognized pediatric experts developed a plan to treat Arianna’s condition.

The first step was induction chemotherapy — to reduce her cancer burden while clinicians searched for an appropriate bone marrow donor. Because Arianna is biracial, her family thought finding a 100 percent match would be difficult, but within a few months, six potential donors were identified and one was chosen.

On March 10, 2016, Nancy J. Bunin, MD, Medical Director of the Blood and Marrow Transplant Program, performed the bone marrow transplant on 18-month-old Arianna.

For the first few weeks post-transplant, Arianna responded extremely well. She ran around the hallways of the Hospital, danced and returned to the happy toddler her family remembered before she got sick. “The nurses called it the honeymoon phase,” Katrina says.

Aftereffects

Soon though, the aftereffects of the transplant and chemotherapy caught up to Arianna and hit her hard. “Arianna lost her hair. She stopped eating. She was vomiting and had diarrhea, and developed a rash all over her body,” Katrina says.

Arianna had developed graft-versus-host disease (GVHD), a complication associated with some transplants. The newly transplanted donor cells were attacking Arianna’s existing cells.

GVHD can affect the skin, liver, intestinal tract and other organs. In Arianna, the disease attacked her skin and digestive tract.

The next few months were an emotional roller coaster for Arianna’s family. Arianna experienced numerous setbacks. She had episodes of sepsis, pneumonia, a bowel blockage, and needed a ventilator to breathe. After each medical set back, Arianna rallied, but the ongoing wear and tear on her young body began to take its toll. Her lungs and liver weakened and hospice care was being considered.

Miracles happen

While Arianna’s family tried to accept that they might lose her, the toddler’s condition inexplicably began to improve. Arianna was suddenly more awake and she was happy.

A few days later a doctor, with tears in her eyes, approached Katrina. Arianna’s latest X-ray showed her condition had significantly improved. Katrina was ecstatic, yet dumbfounded: how could this happen?

“The doctor said ‘sometimes you don’t ask questions, you just accept it for the miracle that it is,” Katrina says. And so, she did.

Follow-up X-rays confirmed Arianna’s improvement, and a few weeks later, the toddler was weaned off the ventilator. She began physical and occupational therapy to regain some of the muscle mass she’d lost during her long illness.

On April 12, 2017, Arianna finally went home after spending more than a year in the Hospital.

Looking ahead

Today, Arianna is doing well. She loves the outdoors, spending time with her family and her favorite Disney characters, Minnie Mouse and Moana. The toddler has a strong personality that’s reflected in her hearty belly laughs and stern “I mean business” looks.

“Her spunk is what has gotten her through so much of her journey,” Katrina says. Arianna will continue to receive ongoing monitoring through the Blood and Marrow Transplant Program at CHOP, and an intestinal transplant is likely in her future.

“We can’t say enough about how absolutely incredible CHOP was to Arianna and our family,” Katrina says. “They are family. We’ve laughed, we’ve cried, and we’ve watched Arianna grow into this adorable, sassy toddler who you can’t help but love.”

Arianna is a Patient Ambassador for the 2017 Parkway Run & Walk, CHOP’s largest fundraiser for pediatric cancer research and care. The goal of the Parkway Run is to create a future where no child is sidelined by a cancer diagnosis.