Sickle cell fight is in Dot family's blood

The same is true in health care. If a sickle cell patient with health insurance has a stroke or an episode of acute chest syndrome while at home, Mrs. Reid said, it is not automatic for care to be provided at the site of the episode, or even immediately upon arrival at the hospital. In her experience, sickle cell patients, as well as their pediatricians and hematologists, often have to fight to have such conditions recognized as emergencies.

“We are lobbying legislatures to create laws to show that this actually is a real disease and a real blood disorder in order for patients to get the services they need without it being a fight,” Mrs. Reid said. “It has to be automatic.”

To further their efforts at Boston’s Children’s Hospital, the Reids have urged the formation of a Consumer Advisory Board made up of doctors, nurses and several families of children with the disease. Together, the board created protocols aimed to streamline the care that sickle cell patients receive in the emergency room and during inpatient visits.

While the Reids say there is still plenty of work to be done, the situation is better than it was when Damali was growing up with sickle cell disease.

“When I was growing up, I had a really, really difficult time,” Damali said. “I was in and out of the hospital three or four times a year, maybe even more, and for very long periods of time.”

That made her battle very different from the one her brother has fought.

“When I was Amiel’s age, it wasn’t as easy to get access to certain medications as it is now,” she said.

One such medication is hydroxyurea, a drug that has proven to decrease the number of sickle cell attacks by stimulating the bone marrow to create more fetal hemoglobin, which prevents red blood cells from taking the sickle shape and reduces the risk of a sickle cell crisis. Dr. Ellis Neufeld, Amiel’s hematologist at Children’s Hospital Boston, introduced the Reids to the medication and suggested to Damali’s doctor that he give Damali the same treatment.

The presence of hydroxyurea throughout Amiel’s life does not mean his experience with sickle cell disease has been less severe or painful. His parents recall a period when Amiel was in and out of the intensive care unit for nearly three straight months. But they said the medication changed his life, from one full of constant suffering to a more tolerable and routine lifestyle.

“It saved Amiel’s life,” Mrs. Reid said.

And he’s making the most of it. Last night, he appeared on an episode of the PBS series “NOVA scienceNOW” with Dr. George Daley of Children’s Hospital Boston’s stem cell program. The TV program discussed sickle cell disease, as well as Daley’s research on how one’s own skin cells can be used to create stem cells to possibly treat diseases like sickle cell.

Amiel’s starring roles don’t stop there. On Sept. 6, he will play the piano at a gala boat cruise along Boston Harbor organized by the Community Sickle Cell Association of Boston. The Reids assisted in setting up the event, where scholarships will be awarded to students with sickle cell disease who are going to college.

“As more and more people are becoming aware of sickle cell disease, they are realizing it truly is a broad disease that affects many kinds of people,” Mrs. Reid said. “We gotta get in front of these diseases.”

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