Hope for Adrian
At just 6 months old, Adrian McKinney II experienced his first episode of unpredictable, unbearable pain. As his mother, Torie, held him in her arms, she knew there was nothing she could do. Along with her husband, Adrian, she worried for their precious, only child.
“We were at church and he could not be consoled,” Torie recalls. “I was holding him and there was nothing that could get him to stop crying.”
Adrian II has sickle cell disease (SCD), a genetic blood disorder that causes chronic episodes of extreme pain, breathing problems, organ damage and many other complications. The McKinneys knew their son had SCD, but nothing would prepare them for their first sickle cell crisis.
The McKinneys quickly brought Adrian II to Cincinnati Children’s where he was admitted and given a blood transfusion. At that moment the gravity of their son’s situation really hit home. Because both Torie and Adrian are carriers of the sickle cell trait, they knew there was a one-in-four chance that Adrian II would have SCD, but they did not fully understand how dramatically their lives would be impacted.
A Life-Changing Diagnosis
After the first sickle cell crisis, the McKinney family’s life suddenly became a whirlwind of frightening episodes of illness that kept Adrian II at the hospital almost more than he was at home.
“Adrian II was hospitalized every six to eight weeks until he was 2 years old,” Torie explains.
Like many children with SCD, Adrian II has had very serious complications, including an elevated heart rate, high fevers and acute chest syndrome. At one point, Adrian II was placed on a ventilator when he developed acute chest syndrome, which progressed to the even more dangerous condition of acute respiratory distress.
Before Adrian II was born, both Torie and Adrian worked as engineers. After her maternity leave, Torie went back to work part-time, but ultimately decided to stay home with Adrian II.
“We could plan for some of the doctor appointments, but then there would be unexpected emergency visits and inpatient stays. We didn’t want to overuse the flexibility our employer was giving us,” Adrian says, noting their top priority has always been Adrian II’s health.
Adrian II will continue to visit the hospital monthly for blood transfusions to prevent the reoccurrence of acute chest syndrome. And as he gets older, he’ll have to be more cautious than other kids. “On a typical sunny day, Adrian II can’t stay out and play all day like other kids because he can become dehydrated. When he starts to swim, we’ll have to make sure the water is warm enough because cold can bring on a sickle cell crisis,” Adrian explains.
“It’s difficult, but we’re going to make it through and so will Adrian II,” Torie adds. “Adrian II is a joy to our lives and a testament to the work that all the people here at Cincinnati Children’s do.”
Cincinnati Children's: A Leader in Innovative Treatments
Sickle cell disease affects 100,000 people in the United States and millions worldwide. And nearly half of those suffering are children, like Adrian II. SCD causes healthy, normally round, red blood cells to become rigid, sickle-shaped crescents, which clog small blood vessels, decreasing the oxygen and blood flow through the body. The result is a host of issues ranging from fatigue, chronic pain and infections to strokes, organ damage and shortened life expectancy.
For decades, the dedicated team at the Comprehensive Sickle Cell Center (CSCC), now within the Cancer and Blood Diseases Institute at Cincinnati Children’s, has been working hard to improve treatments. As a national leader in sickle cell research and clinical care, Cincinnati Children’s was one of the first hospitals in the country to screen newborns for SCD and has pioneered treatments that are now used nationwide, including using penicillin to reduce infant mortality due to infections and complications from SCD. Cincinnati Children’s was also one of the first hospitals in the world to cure SCD with a bone marrow transplantation.
Now scientists at Cincinnati Children’s are on the brink of a gene transfer therapy breakthrough that could end the suffering caused by SCD and help ensure a healthy future for Adrian II and others like him.
“With the knowledge gained at Cincinnati Children’s and the research that has been conducted, the center will soon find a way to alleviate the pain our patients endure daily. Cincinnati Children’s always rises to a challenge, and this is an important challenge to conquer,” says Clinton Joiner, MD, PhD, director of the Division of Hematology and the CSCC at Cincinnati Children’s.
Punam Malik, MD, program leader for the Hematology and Gene Therapy Program and director of the Translational Core Laboratories at Cincinnati Children’s, is the lead investigator of the cutting-edge research that has stopped the formation of sickled red blood cells in the laboratory. The groundwork for this promising trial has taken decades to complete. The approach uses stem cells from the patient’s own body. The cells are taken from the person’s bone marrow then manipulated so they produce red blood cells that can’t sickle. They are then reintroduced into the patient’s body. Correction of sufficient bone marrow cells has the potential to be a “one-time” therapy for SCD.
For the McKinneys, that prospect is miraculous.
“One of the most disappointing things is that there are limited treatments and the disease has been around for at least 100 years,” Torie says. She and Adrian are encouraged by the work Cincinnati Children’s is doing to end the suffering associated with the disease, suffering for the patient and the entire family.
To help accelerate this important work, a group of community members have formed the Sickle Cell Community Advisory Committee at Cincinnati Children’s to raise awareness about the disease and to generate financial support for the research being done at the medical center.
“We are truly optimistic that Cincinnati Children’s – with the support of our community – will make this new research a reality so that other families won’t have to go through the same struggles and pain that we have endured,” says Torie.
Community Leaders Join the Fight Against Sickle Cell Disease
Mitchel Livingston, PhD, vice president for student affairs and chief diversity officer at the University of Cincinnati, and his wife, Carol, attended an informal meeting where they met Adrian McKinney II, a 3- year-old with sickle cell disease (SCD). Immediately, they were taken with the small child’s big personality.
“You could feel Adrian’s energy in the room.” Mitchel says. “But what struck me was that tomorrow it could be different. He could be in the hospital.”
The Livingstons knew they wanted to do something to help the many children who suffer from SCD.
Together with other community leaders, they helped form the Sickle Cell Community Advisory Committee at Cincinnati Children’s to start discussions within the community about the devastating disease.
Silence about SCD has led many to believe that it is no longer a threat. However, the genetic disorder is still afflicting millions of people worldwide, causing pain, breathing difficulties, organ damage and other complications that can strike at any time. According to Clinton Joiner, MD, PhD, a world-renowned expert on sickle cell disease and director of the Comprehensive Sickle Cell Center at Cincinnati Children’s, approximately 500 people die of SCD and its complications each year, and about half of patients die by age 45.
Janet Reid, managing partner at Global Novations and a member of the committee, knows this all too
well. She has a form of the disease called sickle cell thalassemia and, at 57, she feels very lucky to be alive.
“I am proud of all I have accomplished in my life despite having sickle cell thalassemia,” Janet says. “My only regret is that I didn’t talk about it earlier.”
Janet and her colleagues hope to raise awareness about the devastating effects of sickle cell. The group is also working to raise funds to support groundbreaking research at Cincinnati Children’s.
Punam Malik, MD, program leader for the Hematology and Gene Therapy Program and director of the Translational Core Laboratories at Cincinnati Children’s, has been working for more than a decade to lay the foundation for a promising gene transfer therapy trial. Using stem cells from the patient’s own body, the novel approach appears to prevent the sickling of red blood cells altogether in the laboratory. The gene transfer therapy will be the first of its kind in the nation for SCD.
Carl Satterwhite, president of River City Furniture, joined the committee after witnessing Adrian II’s remarkable spirit and learning more about the disease. His business made a financial donation to Cincinnati Children’s to support the research efforts.
“While I knew sickle cell disease was still around, I thought it was a very minimal issue,” Carl says. “After learning more about the disease, it was important for me to get my business involved. My employees also care about finding a cure, so it was a natural partnership.”
To learn more about how you can help advance treatments for sickle cell disease, contact Beth Sims at firstname.lastname@example.org or 513-636-5285.
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