One of the dreaded complications of sickle cell disease happens when sickle-shaped red blood cells cause large amounts of blood to pool in the spleen. It happened to Karson George of Canton in August 2019, when he was 9 months old.
Normal red blood cells are round and flexible, allowing them to flow easily through the vessels. Sickle cells are rigid and stick together, hampering normal flow of oxygen-carrying blood through the body. In Karson’s case, the sickle cells blocked the blood flow out of his spleen, causing blood to become trapped in the organ. The condition is called splenic sequestration. His spleen swelled, while lack of blood flow robbed his vital organs of oxygen. He passed out and almost died before arriving at Akron Children’s Hospital by helicopter.
Karson spent about a week in the hospital. Today at age 2, his disease is under control. His mother, Jessica, says the ordeal made her more vigilant than ever about taking steps to prevent medical complications. The inherited blood disorder can cause a range of problems over the years, including organ failure, stroke, debilitating pain and bone and eye damage. Treatments have come a long way in preventing these complications.
“If you meet my son, you wouldn’t believe he has sickle cell disease,” Jessica says. “Since that happened, he’s been excellent. He runs and plays around like any normal 2 year old.”
A key medication is hydroxyurea, which can decrease several complications. A number of other drug treatments may be needed, as well as antibiotics, extra vaccinations and transfusions.
“It’s nothing to play with, I’ll tell you that,” says Jessica, who also has a son Derrick, age 3. “Keeping up with medical appointments is very important. Medications are very important. That’s what I learned.”
Children at 9 months begin taking hydroxyurea for the rest of their lives, says Joe Delagrange, hematology/oncology social worker in Akron Children’s Sickle Cell Program.
“Sickle cells cause damage and inflammation throughout the body, and this medication prevents the damage,” Delagrange says. “I tell parents we’re not necessarily treating for tomorrow or next week, we’re treating for next year, for 5, 10 years down the road.”
A complication such as Karson’s is not always evident until it becomes critical, says Lauren Beck, pediatric nurse practitioner in the Showers Family Center for Cancer and Blood Disorders.
The Sickle Cell Program teaches parents how to feel for an enlarged spleen and detect other problems, she says. Akron Children’s serves about 150 sickle cell patients from 14 counties in Akron and at the Beeghly Campus in Boardman.
An estimated 100,000 people in the United States live with the disease. Most are Black or Hispanic-American.
Preventive treatments have improved life expectancy for people with sickle cell disease
“There are now four medications that have been FDA approved for prevention of sickle cell complications,” Beck says. “For a long time, we only had one, hydroxyurea. If they’re taking their medications and coming to their appointments, these kids are living longer and have a better quality of life.”
September is Sickle Cell Awareness Month. For more information or to request an appointment, contact the Showers Family Center for Cancer and Blood Disorders at (330) 543-8580.