It's a blood disorder that used to carry a death sentence for children before their 18th birthday.
Today, breakthroughs in treatment for Sickle Cell Anemia are allowing people to live well into their thirties and forties, if not longer. However, the care for this disease as an adult isn't always available. Doctors in Louisville are making sure that's not the case.
"It's just painful sometimes," Jalen Matthews said.
Matthews, 20, was diagnosed with Sickle Cell Anemia as a baby. She had a stroke at just 11 years old as a result, leaving her with paralysis in her left arm.
"Some days were harder than others," Matthews said.
Sickle Cell Disease (SCD) affects an estimated 100,000 people in the U.S, according to the Centers for Disease Control. It's a congenital blood disorder that can cause chronic pain, multi-organ failure and stroke.
Unlike healthy red blood cells, those with Sickle Cell are curved, which can slow or stop vital blood flow and the oxygen our body needs.
Matthews is a student at the University of Kentucky and travels home to Louisville every six weeks for treatment, at Norton Children's Hospital.
"It's exhausting. You have a lot of doctor's appointments, you have to miss out on certain things," Matthews said.
Tethered to a machine, she undergoes a red blood cell exchange, where her so-called bad blood is removed, as new, healthier blood is pumped in. The hours-long process will be one she goes through the rest of her life.
Related: Growing up with sickle cell disease
Doctors are working to make sure she gets the care she needs long-term, something that's not always available to adults with SCD.
"It wasn't long ago before the 1970's people didn't make it out of childhood," Dr. Shawn Glisson, a Hematology-Oncology physician at Norton Cancer Institute said. "Sickle Cell patients can live into their 30's and 40's, but often run into trouble. Most people have died by age 51. The oldest person we have with Sickle Cell is in her 70's."
Together, doctors and nurses make sure kids are prepared for life on their own before they're transferred out of pediatric care, around 18 years of age. But not everyone's ready at that stage. A new transition program put in place by Norton Healthcare and the University of Louisville is making the process even smoother.
"Now, it's their responsibility. They should know the medicines they need to take and need to take the medicines without prompting and be responsible for all the specialist visits," Dr. Ashok Raj, a Pediatric Hematology-Oncology physician at Norton Children's Hospital said.
Dr. Raj was Matthew's doctor for 17 years before she made the switch. He works alongside Spencer Moorman who helps transition kids under their care, with a new curriculum.
"They've gone through the motions, but they don't really understand what it is," Moorman, a clinical social worker, with UofL Physicians said.
"We try to practice with them, speaking up, asking questions, making your own appointments," Moorman said. "School is a huge issue. A lot of these kids, they fall through the cracks. They're penalized for being absent."
Right now, both kids and adults with Sickle Cell Disease can attend monthly support programs, a resource to help keep them on track.
For adults with Sickle Cell who don't stay vigilant with their treatment, Dr. Raj says the survival rates are not as good. Currently, there are 22 adults going through the transition program at Norton Children's.
Matthews may be an adult, but her mom stays close by, soaking in all the moments they can.
"I just try to live my best life everyday. I always say, I'm not here for a long time. I'm here for a good time," Matthews said.
Matthews plans to graduate from UK next May and move to Atlanta, home to one of the largest Sickle Cell centers in the country.
While this transition program is specifically geared toward sickle cell patients, it's a great reminder to all parents with younger children going through a chronic illness to begin the transition process early.
Did you know...
It is estimated that:
SCD affects approximately 100,000 Americans.
SCD occurs among about 1 out of every 365 Black or African-American births.
SCD occurs among about 1 out of every 16,300 Hispanic-American births.
About 1 in 13 Black or African-American babies is born with sickle cell trait (SCT).