|Tackling the secret to fighting off sickle cell disease|
|100,000 Americans impacted by malady|
|Published Monday, September 23, 2019 2:39 pm|
Sickle Cell Disease never stops.
September is SCD awareness month, but what happens during the other 11?
“It is a really difficult disease to have, because it is lifelong, and it is really difficult to predict when you are going to have issues,” said hematologist/oncologist Dr. Paulette Bryant of the St. Jude Affiliate Clinic at Novant Health Hemby Children’s Hospital, who specializes in SCD. “It is painful, and it can affect every part of your body from your brain to your kidneys to your lungs.”
SCD is defined as “a group of inherited red blood cell disorders,” according to the Centers for Disease Control and Prevention. A healthy red blood cell is round and responsible for carrying oxygen throughout the body. When the disease is present, red blood cells take the form of a sickle—a C-shaped farm tool—becoming hard and sticky, which can clog blood flow. These cells die quickly, resulting in a constant shortage of red blood cells. The disease often causes incredible pain, infection, acute chest syndrome, as well as stroke. These symptoms become worse with age.
“What we have found is we can shepherd these kids through to 17-18 [years old], and we think we’re doing a great job, but once they get older, they have more issues,” Bryant said. “Yes, all older people have more issues than they did when they were young, but if you have sickle cell, it can be almost life threatening in your 20s or 30s versus living to be much, much older.”
SCD impacts approximately 100,000 Americans, occurring in one out of every 365 African American births. Bryant explained how a newborn screening tests for SCD. If a baby is diagnosed, preventative treatment can begin around nine months old.
“The test goes to the state, which often goes to a community organization and then they contact us, or Atrium or Levine, and say, ‘this child has SCD,’” Bryant said. “From that point we begin educating the parents. They often don’t have problems until they are six or eight months. There are treatments for prevention that we can start, like Hydroxyurea at nine months of age, which has made a huge difference on how these patients grow up. SCD patients when I was a baby hematologist used to be skinny and sick and they can’t go to school. When they are on Hydroxyurea they are healthy, they are thriving, and they are in school and doing well.”
Helping SCD patients accomplish normal milestones is part of Bryant’s mission as a pediatrician.
“The goal is graduate from high school, get a job, go to college, contribute to your community, and most patients can,” Bryant said. “There are some who are so sick that they cannot do that, but Hydroxyurea and with another medicine called Endari, we’re learning about the disease. We’re learning about the red cells. We’re learning about how the white cells can connect with it, and we’re also learning how good health and lifestyle changes can make a difference with SCD. We’re also learning from the adults what they see.”
Treating a child with SCD vastly differs from treating an adult.
“We are also learning as pediatricians and adult doctors how to work together,” Bryant said, “so when we hand the patient off, Dr. Miller knows everything about that patient and shepherd them through the difficult part of being an adolescent and young adult.”
Said Novant Health Hematology’s Dr. David Miller, who specializes in the treatment of SCD: “A lot of doctors and patients just expect to treat with pain medication. There are lots of complications that evolve over time.”
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