"If I'm not teaching this child how to live, then I'm teaching her how to die."
Soon after Lisa Berry-Hughes had her third child, Briana, she was told her newborn daughter had sickle cell disease and probably wouldn't live to be 6 months old. Today, Briana is 14, enjoys singing and dancing, and takes an active role in her own health care. But the journey has not been an easy one.
As soon as Lisa heard her daughter's diagnosis, she began researching sickle cell anemia. Briana is the first and only child in the immediate and extended family to have the disease. As Lisa delved into numerous books on the subject, she soon found that, "Briana wasn't by the book." While Lisa had read that symptoms usually appear around 6 months of age, Briana proved that wrong when she had her first sickle cell crisis at 1 month old.
Sickle cell anemia is an inherited disorder of hemoglobin, the protein that gives red blood cells their red color and carries oxygen to the tissues. The abnormal hemoglobin makes the usually round red blood cells rigid and distorted into sickle, or crescent, shapes. At times, these stiff, crescent-shaped red blood cells can plug up small blood vessels and decrease blood flow to various parts of the body. This leads to anemia, unpredictable pain episodes, and sometimes, other serious medical complications.
For Briana, the pain began as an infant with a cerebral blockage where a tremendous amount of excess blood was surrounding her brain. Lisa had been referred to Cincinnati Children's Hospital Medical Center shortly after Briana's birth. For the next five years, Briana was in and out of Cincinnati Children's for treatments related to her disease. "The first five years were rough," Lisa says. "Briana was very susceptible to colds and sickness." Finally, when she turned 6, Briana celebrated a birthday at home rather than in the hospital.
Finding – and Giving – Support
From the beginning, Lisa learned to reach out for help. She got in touch with the Sickle Cell Parent and Family Network, a support group that meets at Cincinnati Children's. From other parents and caregivers, she began learning more about the disease and how to ease Briana's pain. Finding support through such a group, Lisa says, is crucial for anyone dealing with a chronic illness. "You not only release, but you also get back," she says. And with three other children at home to care for, Lisa certainly needed an outlet.
Having such assistance from others has allowed Lisa to offer her daughter the strength and support she needs. "I realized if I'm not teaching this child how to live, then I'm teaching her how to die. I chose to teach her how to live," Lisa says. This includes helping Briana become independent as she grows up. She's taught Briana about her medications, including what their names are, what they are for, how much of each she needs to take and at what times. This makes it easy for Briana to go on a sleepover since she can take care of herself.
Briana herself also has played an influential role in the sickle cell community. She has spoken publicly about sickle cell to others, and participates in a teen forum yearly. This education is all part of what Lisa refers to as "building bridges. It's preparing her for adulthood."
A Long Road
At various times, Briana has been on morphine, codeine and OxyContin. She was in and out of a wheelchair for more than two years. For another two years, she had to be hooked up to a pump nightly to eliminate excess iron from her blood. That process required Lisa to stick Briana with a needle every night. (Briana is now on an experimental drug to eliminate iron from her blood.) She also has the added burden of other medical conditions including epilepsy, asthma and Attention Deficit Hyperactivity Disorder (ADHD). She continues to visit Cincinnati Children's monthly for blood transfusions. Lisa feels those are really helping, and she is thrilled to report that Briana is on far fewer medications now than in the past.
Through all of this, Briana has struggled, but she hasn't let the disease hold her back. "She's been stressful and angry," Lisa says. "She feels like she's missed out on a lot. But she also understands where she's been and she's making plans for the future." Part of those plans include pursuing becoming a vet, a dream Briana has nurtured over the years as numerous pets have crept their way into the family's care. Briana also fuels her passion for dance by participating on dance teams at her school and church.
What Lies Ahead
Lisa emphasizes that because blood runs through the entire body, sickle cell anemia affects every organ in the body. In the past, these patients were expected to live to their mid-20s or early 30s. But Lisa knows some individuals in their late 50s and early 60s who are surviving sickle cell. Briana's progress causes her to be optimistic. She also is encouraged by the progress made in the field over the past several years, and she is a true advocate for those who suffer from the disease.
"We need to support sickle cell not just because it affects my family," Lisa says, "but because it may be affecting someone else who needs the same information, help and support that I needed when Briana was diagnosed."
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