Sickle cell disease update hails lifelong advocates, empowers a community

Sickle cell disease update hails lifelong advocates, empowers a community

For newly diagnosed sickle cell disease (SCD) patients, the outlook has never been brighter. There are two cures for the congenital condition, bone marrow transplantation and gene therapy, though these are not suitable for everyone and unaffordable (or inaccessible) for most. Individualized patient care plans are popular and encouraged. Recent work in North Carolina has aligned hospital emergency departments with patients' prognosis and care. And the community of support has never been more active.

This is especially true in eastern North Carolina and at East Carolina University, said retired Dr. Tate Holbrook.

"Eastern North Carolina is a unique place to be because we have the patients, we have the providers, we have the knowledge base, and we have the support of the rest of the country for what we're doing here."

That's one reason Jackie Cannon arrived in good spirits to the fourth Tate Holbrook Annual Sickle Cell Disease Update at Eastern AHEC earlier this month. She lost her son Romon Deshawn Cannon a few years ago at the age of 44 to complications from the disease. He was diagnosed as an infant.

One of the symptoms is sudden, severe pain, often called "crises," and sufferers in this community are called "warriors."

"When we started out 49 years ago, there was almost nothing," Cannon said. "I would pump him full of Tylenol, and I would sit and rub him for the pain, just rub him where it hurts, because there was nothing else."

Today, there are advanced drug therapies, individualized patient plans in large part self-directed by the patients, resources for pediatric patients transitioning into adult care clinics, support groups and summer camps for kids with blood disorders, and more.

"There are sickle cell counselors in every county in the state," said Holbrook, who intercede at the earliest stages for families of babies facing the new diagnosis.

The one-day conference included an overview of the Sickle Cell Disease Association of America and presentations on the sickle cell trait, navigating hospital emergency departments as a patient, a novel local patient case that resulted in a hemoglobin genetic mutation named for the city of Chocowinity (where the patient was born), an explanation of the voluntary withdrawl of the drug Voxeletor, and award presentations.

Child Life and Sickle Cell

One of those awards was induction into the Order of the Long Leaf Pine Society, the highest civilian honor bestowed by the state of North Carolina, for longtime child life specialist Jacque Sauls, who retired earlier this year.

Sauls was one of the pioneers of the child life specialist academic profession in the east.

She is the founder and director of ECU's Rainbow Services, which hosts two summer camps for children living with SCD and pediatric cancer, hemophilia and bleeding disorders - Camp Hope, Camp Rainbow - as well as the pediatric hematology/oncology child life practicum. Camp Rainbow and Camp Hope are free.

She was nominated by Coleman Hinson, programming coordinator in the Office of Faculty Advancement and Leadership Development at the Brody School of Medicine.

As a member of the order, Sauls is officially an ambassador of the Old North State and, according to decorum, entitled to propose and offer The Tarheel Toast at appropriate occasions.

"I've always hoped to be able to impact the needs of children in our state … and one need is just the need to express themselves," she said after the ceremony.

The growth of the child life specialist role and the summer camps have helped meet this need among pediatric patients. A community facing a prognosis together is stronger, more resilient, she said.

Such solidarity may have helped along a key advancement inside hospital emergency departments in the state that Sauls called out for praise.

Pain relief in minutes

Historically, emergency departments are inconsistent and too often insufficient managing sickle cell patients' crises, according to advocates. That's changing.

Emergency departments in North Carolina are now adopting standardized order sets and point-of-care tools that acknowledge sickle cell patients' pain, have on hand their individualized care plans (a collaboration between the patient and their primary care center), and streamlined evaluation and orders. The aim is to deliver tailored pain therapy as quickly as possible.

"These new [protocols] are going to have a terrific impact," Sauls said. "Instead of patients having to explain their sickle cell condition while they're in dire pain, they can move right to pain relief."

Also awarded was Dr. Todd Savitt, a longtime faculty member in the bioethics and interdisciplary studies department at the Brody School of Medicine. He received the fourth Charles W. Daeschner III, MD Excellence in Sickle Cell Care Award. Savitt is a medical historian who has written and spoken about the earliest known cases of sickle cell disease, which disproportionately affects Black Americans.

Throughout the day, speakers and audience members drew on the word "empower" as if it were the theme.

"Early on, we were taught to self-empower by the social worker Kermit Nash" at the University of North Carolina, Holbrook said. "Empowering parents to be in charge of their child, or the patient himself to be in charge. To think, 'My care plan? I've written it. It's for me. My doctor gave it to me, but I helped him create it - and here it is.'"