Seven-year-old talks about life with sickle cell disease

By Maureen Gilmer, IU Health senior journalist, mgilmer1@iuhealth.org

Calina Jones-Mckinzie is ready for her close-up.

The 7-year-old patient at Riley Hospital for Children strikes a model-like pose – legs crossed and head turned to the side – when she sees the cameras.

“Action,” she says, beaming as her mother shakes her head nearby and laughs.

Calina’s personality is almost too big for her body, but her joy is infectious.

The second-grader is at Riley to see her team of specialists in the sickle cell clinic, a home away from home since she was a newborn.

Sickle cell disease is an inherited blood disorder that causes red blood cells to become hard and sticky. When they clog up blood vessels, the blood cannot bring oxygen to the body’s tissue. This often results in serious infections, chronic pain and organ damage.

In the United States, it is estimated that sickle cell disease affects approximately 100,000 Americans and occurs in about one in every 365 Black or African-American births.

RILEY OFFERS COMPREHENSIVE CARE

Riley’s Pediatric Sickle Cell Program, the largest in the state, benefits from comprehensive and multidisciplinary care to minimize trips for families and maximize assessments and interventions provided, according to program director Dr. Seethal Jacob.

“This way they can not only see the sickle cell specialist, but they can also meet with a psychologist, a pulmonologist and a neurologist to address concerns or complications that arise as part of sickle cell disease,” Dr. Jacob said.

Calina and her mom, Jeszma Jones, travel from northern Indiana to see Dr. Jacob and the sickle cell team three to four times a year. That means five to six hours in the car each time, but Jones says the journey is worth it.

“I absolutely love Riley. And so does Calina. Every time I tell her we are going to Riley, she gets super excited. This morning she woke me at 1 o’clock in the morning ready to go.”

This visit might have been a little different because Calina knew she was going to be photographed. Dressed in a bright yellow shirt with a unicorn on the front, a white skirt and tennis shoes, she bounds into the exam room full of energy.

“She’s a character,” her mom says. “Full of life, loves to laugh and to help people.”

She takes piano and singing lessons, likes to draw and watch old cartoons. And she loves baking with her nana.

But there’s more.

PUSHING THROUGH PAIN

“She’s also very determined. She’s a fighter. When she has a pain episode, she still tries to push through it, which is good but sometimes she tries to push herself a little too much,” Jones said.

Pain is part of sickle cell disease. It can be debilitating and lead to a host of other complications, but fortunately for Calina, it is manageable for the most part with heat therapy, ibuprofen and drinking lots of water.

She misses out on some popular childhood activities though, like swimming and playing in the snow because extreme cold and heat can cause her fingers and limbs to swell and pain to flare up.

She loves to play in the water, so her mom fills up a small wading pool at the home they share with Jones’ mom and lets the sun warm it before Calina can get in.

When the first snow falls, Calina would love to make a snow angel and a snowman, but special gloves and a snowsuit don’t give her enough protection.

Calina is also susceptible to colds and viruses, so COVID-19 terrifies her mom.

“Before COVID, we were already wiping things down, but now we’re triple cautious,” she said. “It is super scary. I really don’t want her to have to go to the hospital. I pray a lot.”

Jones is vaccinated against the virus, and she can’t wait for her daughter to receive that protection as well.

“Once they approve it for her age (CDC approval could come by next month), she will definitely be getting the vaccine.”

“MY LIFE WITH SICKLE CELL”

Calina is holding court in the exam room before Dr. Jacob comes in.

“This is my life with sickle cell,” the young patient says as she launches, unprompted, into a narrative about her disease.

“Well, my life with sickle cell is kind of bad. I don’t like sickle cell, but it’s a part of my body, so I can’t control it. This is my routine,” she continues. “First, the aching pain – every single night and day. It will hurt, literally hurt.”

When her mom asks her what she does for the pain, she talks about her heating pads, the medicine (ibuprofen) she takes and the water she drinks.

“I try to keep my body cool because if I get too hot, I will have aching pains on my foot, my arms or legs. My school is literally like a fire-breathing dragon,” she said.

Calina’s school has been having problems with its heating and air-conditioning system, and the heat that gets trapped in the building causes her asthma to flare up and leads to pain episodes.

Lucky for her, she has moved to a new school, thanks to her mom’s relentless advocacy. It’s something she is teaching her daughter as well.

“We have these conversations at home, and sometimes she gets emotional,” Jones said. “I tell her, ‘I understand your frustrations, but it’s something you’re going to have to live with.’

“The faster she gets used to acknowledging and coming to terms with it, the better it will be when she gets to adolescence because I don’t want her to feel like she’s different from other kids or maybe blame herself for her illness.”

MAKING HERSELF HEARD

Jones allows her daughter to hear her when she is making calls on her behalf, knowing that it’s important for her to learn to advocate for herself as she gets older.

“I want Calina to tell doctors what’s happening and make herself heard.”

Advocacy did not come naturally to Jones.

“I was a teen mom, so I had to step into that adult role and figure out how to navigate the medical side of the disease,” she said. “I didn’t know anything about sickle cell. I wish more people were aware of sickle cell. You may not look sick, but you never know what people are feeling on the inside and the pain they’re dealing with.”

When Dr. Jacob steps into the room, Calina sits up straight and talks to the physician about school, animals and her pain. The physician uses her stethoscope to listen to Calina’s lungs and heart.

When Calina asks to reverse roles, Dr. Jacob waits while her patient holds the medical device up to her white coat.

“Hear anything?” Dr. Jacob asks.

“It’s a healthy heart,” Calina responds, with her trademark smile.

Asked what she looks for during these visits, Dr. Jacob said one of the big things they pay attention to is whether there have been any hospitalizations or emergency room visits in the preceding months.

“That tells us if there are changes in disease severity,” she said. “We keep track of how many pain episodes kids have in a year. We know that the more hospitalizations they have, the more severe the disease is. Three or more hospitalizations for pain in a year increases the chances for them to have additional complications.”

Those complications can include anemia (requiring blood transfusions), jaundice, severe pain, stroke and organ failure. That’s why regular visits with Riley’s sickle cell team are so important, Dr. Jacob said.

“Her mom’s dedication to getting Calina the best care possible, and advocating for her all along the way, has made all the difference in Calina’s care.”

ACCESS TO CARE LIMITED FOR SOME

The family’s story illustrates the disparity in access to sickle cell care, not only in Indiana, but throughout the United States, Dr. Jacob said, as compared to other rare, inherited diseases.

She knows that traveling several hours to Indianapolis can be a hardship.

“Studies estimate that fewer than 70% of children with sickle cell disease receive comprehensive care, and only 20% experience effective care coordination between primary and subspecialty providers.”

While families like Calina’s may find accessing expert, comprehensive care for sickle cell a burden, it is a tradeoff they are willing to accept.

“They are willing to endure this just to have a team knowledgeable about the disease caring for their child,” Dr. Jacob said.

Riley instituted telemedicine for sickle cell care even before the pandemic and has recently received funding from the National Institutes of Health to expand to other parts of the state.

“We want our families to walk away from a visit with us feeling they have a team that cares about and supports them, while also receiving high-quality, evidence-based medical care,” Dr. Jacob said.

September is Sickle Cell Awareness Month, and Dr. Jacob wants to use this opportunity to send a message to the community:

“We need others to know that despite all we do at Riley, patients with sickle cell disease are a distinct population experiencing healthcare disparities and inequities in our nation,” she said.

“We need improved awareness about the disease within our communities, as well as increased funding and support for sickle cell centers across the country. And we need Hoosiers to stand with us to advocate for a population that has been neglected for far too long.”

Photos by Mike Dickbernd, IU Health visual journalist, mdickbernd@iuhealth.org