Together on a Precarious Walk: Sickle Cell Disease

Like many 4-year-old girls, Kynnedi Sturges is curious, talkative and likes to play with dolls. “Dolls with hair,” her mother, Domonique Sturges, said, “because she thinks she can do hair.” Unlike most other 4-year-old girls, Kynnedi has sickle cell disease, which affects a person’s red blood cells, causes debilitating pain and can lead to other serious complications. Four years ago, fear of complications, like stroke, severe anemia or vision problems, overshadowed Domonique’s thoughts. Now, she focuses on ensuring Kynnedi experiences the joys of childhood. “I want her to live a normal life and succeed at everything she wants to do. I just want her to be happy,” Domonique said. “She’s bigger than sickle cell. That’s not going to hold her down.”

From Day One

Within the first few days of life, newborns in Arkansas are screened for nearly 30 health conditions, including cystic fibrosis, hypothyroidism and sickle cell disease. The sooner conditions like these are identified, the sooner treatment can begin. Receiving the news can be shocking to parents, especially if there has been no family history of the disease or no signs of it in pre-natal screenings.

Domonique received the call about the sickle cell diagnosis about two weeks after Kynnedi was born. "I broke down. It was fear because I didn't know the extent of it. I didn't know anything about the disease, so I was afraid."

Treatment often begins with educating parents about the condition and preparing them for how to support their child. Domonique’s primary source of education and support came from the state’s only sickle cell specialty nurse, Angela Mull, BSN, RN, RNP, CPN and Carolyn Saccente, M.D., a pediatric hematologist and oncologist at Arkansas Children’s Hospital. Saccente is also an associate professor in the Department of Pediatrics at the University of Arkansas for Medical Sciences. Mull said Domonique reacted to the diagnosis like many parents, “fearful and tearful.”

Domonique's fear was amplified by the fact that Kynnedi has Hemoglobin SS (HbSS), the most severe type of sickle cell disease. Understanding the disease, including its risks and treatments, is the first step in overcoming fear.

"From day one, it's the teaching," Mull said. She helps parents like Domonique face their fears head-on. She shares some hard truths, like the pain crises Kynnedi will likely experience. Other sickle cell patients describe a pain crisis as feeling like thousands of tiny shards of glass are flowing through your veins or you're being "poked or stabbed by little, pointy iron nails."

Domonique appreciates Mull's direct and honest communication. "There are some difficult things that we have to talk about regarding the disease and what we will face throughout Kynnedi's life, but nurse Angela Mull has always been an open door. She gave me her cell phone number. No question is a dumb question. With anything I'm worried about, I've always been able to call her at Arkansas Children's."

Building Immunity

Like most mothers, Domonique's first instinct was to shield her child from the triggers that can cause a pain crisis or other complications. Kynnedi's weakened immune system makes her susceptible to the flu, RSV and COVID-19, so her mother avoids large crowds and gatherings with lots of children. Some triggers are less obvious, like susceptibility to salmonella from picking up lizards, snakes, frogs or turtles or helping prepare chicken in the kitchen. The Centers for Disease Control and Prevention recommends those with sickle cell disease also avoid:

• getting too cold or too hot (especially challenging in an Arkansas summer)
• getting dehydrated
• high-altitude places, like climbing mountains or flying in planes

Mull said it's just as important to focus on what children and families can do as what they can't, because reasonable exposure to others strengthens the physical immune system and develops emotional resilience. "To be part of the community, they have to build an immunity," Mull said. "That's what I help facilitate with these parents - [overcoming] that internal emotion that their child has to be wrapped in a plastic bubble."

Building emotional immunity means learning how to overcome other's ignorance of the disease. Sickle cell disease impacts around 100,000 people in the U.S. - a relatively small number, which means many people aren't familiar with the precautions those with the disease must take. Sometimes, people - including family members and friends - dismiss Domonique's concerns as overprotective. Those dismissals were frustrating for Domonique, who describes herself as someone who used to be "a very timid person."

Domonique has developed strategies and communication skills for dealing with others and has also learned how to cope with the internal struggles of being a mother to a child with a chronic disease. "As a parent, it hurts," she said. "It makes me feel defeated sometimes because I can't take it from her. But I have always felt that God has us go through things for a reason. Kynnedi is a blessing [to me]. And, if she can be a blessing to me, she can be a blessing to other people."

Advocating

Over the past four years, the desire to let Kynnedi have a full and happy childhood and be a blessing to others has motivated Domonique to overcome her shyness. "I'm more verbal," she said. "When it comes to my child, of course, I have to be able to speak up. We're going to do what's best for Kynnedi no matter what."

Doing what's best for Kynnedi includes driving to ACH in Little Rock from their home in Camden, Arkansas every three months for checkups. The nearly 4-hour round trip is worth it to Domonique because she trusts the care team. Speaking up for Kynnedi means being able to explain to employers the importance of making the drive to ACH. It also means being compassionate when explaining to others why Kynnedi won't attend a family event or a celebration with lots of children.

"Kynnedi's mother is one of the best educators in her community about sickle cell," Mull said. "Domonique can talk about sickle cell not just with expertise but with empathy."

Mull believes Kynnedi is learning how to advocate for herself by watching her mother talk with others about sickle cell disease. Kynnedi is also learning how to share the burden of a chronic illness by watching her mother lean on Mull and the care team at Arkansas Children's Hospital during discouraging moments.

Mull and Domonique are both stubbornly optimistic about Kynnedi’s future.

"It's a precarious walk that they walk every single day of their life," Mull said. "The important thing is that there are so many new treatments out there and therapies that we can do."

It's a sentiment Domonique shares. "Life changes every day," she said. "There might be a cure for it. We're hoping for one."

Children like Kynnedi and her mother, Domonique, are one reason Arkansas Children’s is committed to bringing the highest level of care and the most advanced treatments to the state and region.