By Sharyn Jackson
The Des Moines Register
DES MOINES, Iowa - Time, for Isaiah Newsome, is measured by the pulses and beats of sickle cell anemia.
Every day, sometimes twice a day, he dips into a baggie full of pill bottles to manage his pain, heal his organs and protect his already fragile health. Every three weeks, he gets a transfusion of new blood to replace his dead, misshapen cells. Every two months, he has a procedure, called an infusion, to give strength to his bones, weakened to the level of an 80-year-old woman. Out of every school year, his mother estimates that he's missed half of his classes.
Time, for Isaiah, has been reconfigured by this genetic blood disease, all because two mutated genes came together 16 years ago.
"It's not the best," Isaiah said. "It hurts a lot. You're in pain a lot of the time."
Through it all, the Des Moines North High School student has found strength in his family, his faith and his creativity. But most of all, from blood. It takes seven to nine units each transfusion - 120 to 150 units a year - to keep healthy, globular red blood cells flowing through Isaiah's veins in place of the crescent-shaped cells that form traffic jams in his vessels, starving his organs of oxygen and causing excruciating pain.
Ethnicity and race can play a factor in blood type, so for patients with a disease like sickle cell, which in the United States affects mostly African Americans, the blood pool is limited. For patients who receive blood regularly, antibodies build up and make the pool of donors their bodies will accept even smaller.
Blood exchange and a daily medication, hydroxyurea, are the main forms of treatment for sickle cell anemia. Otherwise, patients like Isaiah rely on pills to dull the pain. When the pills aren't enough, he goes to the hospital for intravenous painkillers.
"These are the only disease-modifying measures we have," said Dr. Nick Fustino, a pediatric hematologist at Blank Children's Hospital in Des Moines, where Isaiah is treated.
"Unfortunately, sickle cell research is not well-funded. It is not glamorous," Fustino said. "So despite the fact that we've known about this disease a little over 100 years now, the research is only just getting going, in my opinion."
One reason that research on sickle cell treatment has lagged is because of its status as a rare disease that affects only a small population. But rarer diseases have gotten exponentially more funding from the National Institutes of Health, according to a 2004 study in the journal Pediatrics. Some say sickle cell's perception as an African-American disease is why.
"It is the most common rare disease out there, and way more common than cystic fibrosis," said Paula Tanabe, an associate professor at Duke University's School of Nursing. "But it hasn't received the funding for some reason, and many people, of which I am one, do believe that racism is a part of one of the reasons it has not gotten enough attention."
The disease carries a stigma that gets worse as its sufferers age, Tanabe said. "It's not a disease like juvenile diabetes where the general public can relate to that," Tanabe said. "Health care providers pretty much acknowledge this is a bad disease for kids, and then they turn into an 18-year-old African-American male, and come to the emergency room and ask for opioids, and it's just, 'They're addicted.'"
Disproportionate funding for sickle cell disease research is one of the Sickle Cell Disease Association of America's main advocacy concerns, said communications specialist Elizabeth Samolis.
Another direct result of lack of funding, advocates say: a lack of specialists who treat the disease.
A team of pediatric hematologists at Blank Children's Hospital treats 30 to 40 juveniles with the disease in central Iowa, Fustino said. Nurses who specialize in sickle cell disease are on staff, as well as psychologists and social workers.
"Both the physical and mental toll is pretty significant in many patients with sickle cell disease," Fustino said. "In addition to hospitalizations, the day to day challenges of sickle cell disease often get overlooked, especially at home, where patients can have daily pain."
Isaiah described what he feels as "a dull aching pain that's there 24/7." He said he tries to hold it in until the pain is too excruciating to remain silent.
"That's the kind of kid he is," said Susan Hetherington, Isaiah's learning enhancement teacher at North High School. "He'd come in here looking horrible, and you could look at him and know he was miserable, in pain. But he still comes to school as much as possible. He's a tough kid. He likes to persevere."
The experience, however, is lonesome. Isaiah doesn't have any friends with the disease, and his mother, Charice Williams, doesn't have other parents she can relate to. And though many states have a chapter of the Sickle Cell Disease Association of America, Iowa does not.
"Isaiah doesn't have any peers. He doesn't have anyone to bounce his feelings or experiences off of that can truly relate to what he goes through," Williams said. "I'm here, I'm with him, I'm around, but I can never say that I can feel what he's feeling, and that's sad to me."
"When you're watching the nurses constantly poke him for IVs and blood work, and the medicine's not working and he's frustrated because he's been there for so long, it's a mental challenge," she said. "It's a challenge to stay strong and upbeat, but you just have to."