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KAPI‘OLANI’S SICKLE CELL DISEASE TREAT- MENT FOR PEDIATRIC PATIENTS.

BY THE TIME HE WAS FOUR YEARS OLD, GRIF HAD ALREADY EXPERIENCED A STROKE, A RESULT OF HIS SICKLE BETA THALASSEMIA, ACCORDING TO HIS MOTHER, TAYLOR PIERCE.

With the sickle cell disease treatment for pediatric patients program—only available at Kap‘iolani Medical Center for Women and Children—Grif has been able to manage his disease.

Dr. Jasmin Jensen of the Pediatric Hematology-Oncology department at Kapi‘olani explains that sickle cell dis- ease is a condition where a patient’s red blood cells “get ‘stuck’ in a sickle shape.” These abnormal cells damage blood vessels and can block blood vessels.

“These abnormal blood cells die early, which causes a shortage in the number of red blood cells, so patients have anemia,” Jensen explains. “A sickle cell ‘crisis’ is a potentially life-threatening emergency that happens when a lot of the patient’s red blood cells suddenly get stuck in the sickle shape.” This can cause pain, breathing difficulties and even stroke. Even when not in a crisis, patients are at risk for kidney failure, chronic sores, vision loss and breathing disorders.

Grif is a patient who experienced a sickle cell crisis.

“He had a full left-side-brain ischemic stroke when he was just four years old,” recalls Taylor. “He spent two years in and out of the ICU at Kapi‘olani. He also sustained a second hemorrhagic stroke about seven months after his initial stroke.”

Taylor explains that Grif was diagnosed with Moya Moya and, due to the damage to his brain, developed epilepsy and apraxia of speech. “He is considered non-verbal, but he doesn’t let that hold him back from communicating,” Taylor says, adding that he regained his vision and hearing while at Kapi‘olani.

There are various treatments for sickle cell disease, including medications. Jensen explains that during a crisis, a combination of treatments, including blood transfusions and medications are used to help the patient.

“In order to avoid any further strokes, he receives regular, chronic, blood exchange transfusions,” Taylor says. “Donor blood is quite literally saving his life!”

The sickle cell patients at Kapi‘olani receive red blood cell exchange transfusions via the hospital’s apheresis program. All of the current patients have had previous strokes. Without the apheresis program, they would receive monthly ‘simple transfusions.’

“Sometimes this is sufficient, but for many patients, it does not lower the levels of harmful sickle cells enough to really protect them from serious complications,” Jensen explains.

“[Grif] has been receiving red blood exchange transfusions for a few years now and is doing great,” Jensen says. “He tolerates his procedures really well and he hasn’t had a major sickle cell crisis for more than two years.”

Jensen points out that Grif is not letting sickle cell disease hold him back. He attends school and the family has taken a few trips together.

Jensen says that the apheresis program is growing and will offer new therapies in the future. “I am so fortunate to be able to work with a wonderful team, our apheresis program has worked together to meet many challenges over the last few years and their teamwork is why we are now able to offer life-saving therapies like red blood cell exchange transfusions for patient’s like Grif.”

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