CLEVELAND, Ohio (Ivanhoe Newswire) — A bone marrow transplant can be a life-saving procedure, but there are often many obstacles to overcome. One little miracle baby beat all the odds and now has a chance at a normal life.
Today, Denniya Rawls is a happy and healthy baby. But when she was just three months old, Denniya got very sick, and doctors told her parents she wouldn’t make it.
Robin Thornton, Denniya’s mom, said, “She wouldn’t make it overnight, and if she made it overnight, she wouldn’t make it over 24 hours.”
Denniya had a rare condition known as primary HLH. Her immune system was attacking her healthy organs and she needed a bone marrow transplant … fast!
Rabi Hanna, MD, Director of Pediatric Blood and Marrow Transplantation with Cleveland Clinic Children’s said, “Patients with this disease, if they don’t get a bone marrow transplant, they all die, unfortunately.”
But none of Denniya’s relatives was a match. And there’s a major shortage of African American donors. In fact, black patients only find an unrelated donor about 25 percent of the time. But, Denniya’s parents were thrilled when a perfect match was found within just a couple of weeks.
“It was exciting to actually know that they found the match so fast and for it to be ten out of ten. It was just a blessing,” said Dennard Rawls, Denniya’s dad.
Denniya had eight weeks of chemo before her procedure. But then another setback … her condition flared right before the transplant.
“Patients who flare their disease prior to transplant, or, immediately, less than 20 percent of them will survive,” explained Dr. Hanna.
Still, she beat the odds and now at seven months, Denniya’s going home after spending more than 100 days of her short life in the hospital.
At least three thousand people die each year because they can’t find a donor match. While blacks find a bone marrow donor only about 25 percent of the time, whites are able to find a donor about 75 percent of the time.
Contributors to this news report include: Julie Marks, Producer; Robert Walko, Editor.
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MIRACLE BABY GETS A BONE MARROW TRANSPLANT
BACKGROUND: Hemophagocytic lymphohistiocytosis, or HLH, is a life-threatening condition that can be defined as either primary or secondary HLH. Primary HLH is when the condition is inherited. Secondary HLH is used when your doctor thinks the condition may have occurred for a variety of other non-inherited reasons. Patients with primary HLH have cells of the immune system that don’t work properly. These cells become overactive, causing too much inflammation. Ordinarily, these cells should destroy infected, damaged cells of the body. In HLH, the immune system begins to damage the patient’s own tissues and organs, including the liver, brain and bone marrow where blood is made. Sometimes, doctors can see these angry immune cells “eating” other cells when they look at the bone marrow from a patient using a microscope.
HLH DIAGNOSIS AND TREATMENTS: HLH can be diagnosed only with proper blood tests including blood cell counts, liver function, infection studies, and markers of immune system activation. A bone marrow biopsy may be performed to look for microscopic evidence of HLH. Doctors may also perform a lumbar puncture to collect cerebrospinal fluid and make sure the HLH is not affecting the brain. Treatment is very important for patients with HLH because the condition is life-threatening. Doctors use treatments that suppress the immune system. Patients are usually treated with steroids, chemotherapy and/or antibody therapies that target and destroy the diseased cells. Patients may receive other medications that suppress the immune system. Additionally, doctors may give medications that help prevent and/or treat any infections. Many patients must also have their immune systems replaced by receiving a blood or bone marrow stem cell transplant.
STEPS TOWARD PROGRESS: Lisa Filipovich, MD, director of the Immune Deficiency and Histiocytosis Program at Cincinnati Children’s Hospital, says a big breakthrough in understanding the disease came in the late ’90s, when the first genetic cause was identified. People who shared the disorder’s unique set of abnormalities had a common genetic mutation that kept them from producing perforin, a protein crucial to the function of the immune system. “It began to explain why the natural killer cells and other cytolytic cells in this disease didn’t work,” said Filipovich, “but it still didn’t tell us why patients got the disease.” Because HLH is considered a rare disease, many healthcare providers are unfamiliar with its symptoms and it often goes undiagnosed for too long. “One of the problems is no one stumbles on the diagnosis soon enough. However, we have made pivotal advances in this area,” Filipovich explained. One of the advances is the use of “reduced intensity conditioning” which uses a milder biologic agent to prepare a child for bone marrow transplant. The approach has far less systemic toxicity than the usual chemotherapy. It cannot be used for every child, but for those who are eligible, it has improved long term survival to 90 percent.
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