Growing up, Mikaili and Khari Robertson often felt different from other kids.
They kept gallon-sized water jugs sitting by their desks at school, couldn’t play outside if it got too hot or too cold, and sometimes had to miss school because of bouts of extreme pain. It was all part of living with sickle cell anemia, a disease in which odd-shaped red blood cells make it difficult for blood to move through the body.
“It was hard not having someone understand why you can’t do certain things,” said Khari , now 20, who would avoid talking about the disease with other students.
Now college students, the brothers, with the help of their parents, who all live in Ellicott City, are trying to make it so other kids with sickle cell and their families don’t feel so isolated.
Through an organization their parents started, the Maryland Sickle Cell Disease Association, the Robertsons are helping families better understand the disease, navigate what services are available and connect them with others who live with sickle cell.
Derek and Shanta Robertson started the organization 12 years ago because of their own experiences having two of three sons with sickle cell. In recent years, they have seen big strides come from their work, including their first convention focused on the disease this past spring and legislation to better serve kids with sickle cell while they are at school or school functions.
“What we were trying to do was be proactive,” said Derek Robertson. “We had good health care and family and community support. We wanted to bring that to others.”
People with sickle cell produce blood cells that are rigid, sticky and abnormally shaped like sickles, or crescent moons. The cells tend to form clumps and get stuck in tiny blood vessels, making it difficult for oxygen to move through the body.
When parts of the body don’t get enough oxygen, it can lead to sudden bouts of pain that can last from a few hours to days at a time. The pain can be minor or people can go into a sickle crisis and experience pain so severe it limits daily activities.
There's no cure for most people with sickle cell anemia. But treatments can relieve pain and help prevent problems associated with the disease.
Extreme temperature changes can trigger a crisis. That means avoiding a cold pool. Physical trauma, such as getting hit suddenly, and overexertion can also leave a child bent over in pain, which means many don’t play football or other sports.
Mikaili and Khari said their parents were strong advocates and made sure they took precautions to prevent an episode. That is why they were frequently seen with large bottles of water to keep the blood flowing. Staying well hydrated can help prevent a sickle cell crisis.
“Khari and I were both fortunate to have a lot of great care and our parents were really on top of things,” said Mikaili, 18. “When it came down to managing our disease, it was all about getting ahead of things and taking precautions to try and avoid sickle cell episodes.”
Their parents also made sure teachers and school nurses knew to take it seriously when their sons said they were feeling pain. They let administrators know that sometimes their sons might have to miss a lot of school. And they told them to be aware that they drink a lot so may have to go to the bathroom often.
“We have heard anecdotal stories about kids not allowed to use the bathroom and they use the bathroom on themselves,” Derek Robertson said. “You have to make sure they have adequate bathroom breaks.”
Through their foundation, the Robertsons helped get legislation passed in the most recent General Assembly session that makes public schools follow a formal plan on managing a student’s sickle cell disease. The Maryland Department of Health will now develop guidelines for schools to follow.
Dr. Sophie Lanzkron, an associate professor at the Johns Hopkins School of Medicine who runs a lab focused on sickle cell research, supported the legislation.
“We want kids to be able to go to school and feel supported,” said Lanzkron, who created a registry of sickle cell patients to improve research.
Khari Robertson said he liked that the organization linked him to other kids living with sickle cell. He had mild symptoms so he tried to mask his disease from other kids as much as possible. He also refused to not let it slow his life down. But when he tried out for the basketball team, he discovered severe bone scarring in his leg, a symptom of sickle cell.
“That really opened up my eyes to why I can’t do certain things,” he said.
Khari and his brother now encourage other kids to accept the disease and know they can still have fulfilled lives. Khari plays college baseball.
Dr. Jason Fixler, a pediatric hematologist who treats sickle cell patients at the Herman & Walter Samuelson Children's Hospital at Sinai, said the disease doesn’t get a lot of attention or funding for research in part because it affects such a small number of people — just 100,000 in the United States. There is also a stigma attached to the disease for older people because one of the main treatments is narcotics. Taking painkillers is frowned upon because of the opioid crisis.
Others have argued that a racial component may play a role in the lack of attention to sickle cell disease. One in 365 African-American babies is born with the disease and 1 in 13 carries the trait that could enable them to pass it on to their children. It also tends to affect a poorer population.
Any attention organizations like the Maryland Sickle Cell Disease Association can bring to the disease is good, said Fixler, who goes to churches to talk about the disease.
“Parents can hear about other people who have children with the disease and share solutions and experiences,” he said. “I think that is helpful.”
That is one of the things the Robertson family hopes to achieve with its organization: connecting people.
“It is harder when one person is advocating for their child,” Khari Robertson said. “When they have a team around them, it is easier to get what they need.”
Nikia Vaughan met the Robertsons at a national convention on the disease. Her 6-year-old daughter and husband both live with sickle cell.
Vaughan, who lives in Baltimore, was worried about giving her daughter the drug hydroxyurea to treat the disease. Many sickle cell patients use it, but Vaughan had been given misinformation that it could cause children to develop leukemia. The Robertsons assured her that their sons took the drug safely.
“They had children with the disease,” she said. “They were able to teach me many things and dispel some myths. I call Derek and Shanta all the time. They are like family.”