Comprehensive Sickle Cell Disease Program

Adults with childhood blood disorder no longer outgrow care. The Comprehensive Sickle Cell Disease Program follows a patient over a lifetime—the first of its kind in the Upstate.

ToyAnita Jones was diagnosed with sickle cell disease (SCD) at age 6, more than 25 years ago. As she transitioned from a child to a teenager and then an adult, she was forced to wrestle not only with her complex medical condition, but also with a transition from familiar doctors who knew her story to a new set of doctors who were starting fresh.

“I felt different and misunderstood enough as a child who couldn’t run or play the same way her friends did,” Jones said. “Leaving the doctors who knew me as soon as I became a young adult made it even harder. Not to have to transition would have meant everything to me.”

“The worst feeling for patients with a chronic illness is the thought of leaving a health care provider who knows them well,” said Alan Anderson, MD, a pediatric hematologist/oncologist with GHS Children’s Hospital. “Our vision was to serve upstate patients with sickle cell disease and ensure they’d never need to go elsewhere. We wanted to create a lifespan clinic, and that’s just what we’ve done.”

The comprehensive program launched in summer 2018. According to the National Institutes of Health, SCD is an inherited blood disorder that alters the shape and longevity of red blood cells, leading to symptoms ranging from swelling of the hands and feet, fatigue, pain and infection to stroke, organ damage and red blood cell shortage, which often leads to transfusions. The illness disproportionately affects the African American community, Dr. Anderson noted, and also can affect those of Hispanic descent.

Over the last 20 years, Dr. Anderson recounted that pediatric SCD patients have seen a “drastic improvement in survival,” thanks to advances in monitoring hemoglobin, fighting infection and maintaining everyday health. “We now see that over 96 percent of children with SCD live at least to age 18.” For patients transitioning from pediatric to adult care, however, the statistics are less encouraging.

“Admission rates to the ER and hospital rise, mortality rates increase and overall compliance with health maintenance, including visits to subspecialists, go down markedly,” Dr. Anderson stated. Patients often lack a true medical home, he added, because few care options focus on sickle cell; the effects are costly both physically and economically. The Centers for Disease Control and Prevention estimates that costs for hospital stays related to SCD were roughly $488 million in 2004 alone.

“In patients with SCD, changes in temperature, decline in oxygen levels and other causes of stress to the body can lead to a change in the shape of the red blood cells and ultimately block blood flow to critical areas,” Dr. Anderson noted. “The biggest outward manifestation is pain, and sickle cell patients often need narcotic pain meds to treat it. We see many who use the ER to receive the help they need in medical crises.” That isn’t the ideal model of care, he pointed out, but few hospital systems have offered other solutions. “We needed to fill that gap.”

Change fueled by community support

Fresh off two years in Botswana in southwestern Africa serving and studying patients with blood disorders and cancers, Dr. Anderson returned to Greenville in 2017 with a renewed determination to deliver care to an underserved population. He served as medical director of GHS Pediatric Hematology/Oncology before moving abroad and had quite a challenge awaiting him upon his return: Open the system’s first lifespan sickle cell disease program.

Dr. Anderson made a case for the program, demonstrating the need for seamless SCD care and its worth for area patients. After assembling a community advisory board of political and religious leaders, along with concerned members of the health care sector and African American community, Dr. Anderson led discussions about the lack of robust care for sickle cell patients transitioning into adulthood.

“They saw the value of having a dedicated team of providers follow patients throughout the continuum of their chronic illness,” Dr. Anderson remarked. “It’s a travesty in the American health care system to have life expectancies going down for a chronic illness. It shouldn’t be happening, and we intend to change things.”

A huge relief: the value of seamless care

At 21, Jones became pregnant. The difficulty of managing her disease was compounded by the challenge of having to walk through her history over and over with doctors who didn’t focus on sickle cell.

“At my old practice, they knew and trusted me,” she recalled. “I didn’t have to go to the ER as often. They helped me manage my pain or get transfusions when I needed them. I lost all that when I left.”

This experience drives Jones to support young sickle cell patients and their families, walking them through the highs and lows that can come with a chronic disease. She attends clinic support groups and mentors patients who experience the symptoms, both medical and social, she did at that time.

“My family support system was strong,” she acknowledged, “but I still could have used a peer or the advice of someone who had been in my shoes.”

“With sickle cell, you have symptoms inherent to your condition, and as a young adult, you also have things inherent to that age range, like a feeling of invincibility, lack of compliance, confusion about how to access health care for yourself and other social pressures,” Dr. Anderson explained. “You have few medical homes that focus on all those facets. Outside of medicine there is a stigma associated with sickle cell and its symptoms. It’s a great deal to manage at any age.”

Jones agreed, remembering hospital visits when she was questioned or viewed “as someone just showing up to ask for pain meds. If you don’t know my diagnosis or my background, you see me as someone I’m not.”

Conversely, she said, “Dr. Anderson’s staff just gets it. I can walk right in and explain my level of pain, how many bags of fluids I think I need, what medicines have worked for me before. They take me and my symptoms seriously, and they take me at my word. It’s a huge relief.”

ToyAnita Jones, center, with two of the medical team (Alan Anderson, MD, and Katie Muschick, BSN, RN, PCNS) who have given her a medical home that specializes in sickle cell disease.

Filling a void and information gap

In addition to providing sickle cell patients a lifelong medical home and a place where they feel understood, Dr. Anderson aims to educate them. “We want our patients to understand newer modification agents available, preventive health recommendations and the value of knowing where to get IV fluids or pain meds when they travel. We discuss things that will keep them out of the ED or hospitals where there is less likely to be knowledge about SCD and how to manage it.”

“At 32,” Jones admitted, “I’ve learned the importance of slowing down, taking fluids and meds, and decreasing my symptom crises before they come. When I was younger, I didn’t see it that way. But I believe what the clinic is doing now can help the next generation avoid that mindset.”

Dr. Anderson’s team hopes to close the information gap for the community at large as well.

“The social stigma of a disease driven by pain is real,” Dr. Anderson said. “We train our patients to know what works for them. But when they request the medicine they need, they are labeled as drug-seeking, coming in and immediately requesting something from providers. The only way to combat that stigma is to educate those outside of the sickle cell community about what they face.”

Here for a lifetime

In the months and years ahead, the clinic intends to add a new provider, expand care options, and continue to partner with community leaders for advocacy and awareness.

“Dr. Anderson’s team does more than care for us physically. This kind of spiritual and mental support is something I’ve always wanted,” Jones emphasized. “You see these kinds of coordinated efforts for cancer and other diseases, but the sickle cell community has never experienced that. Lifelong care is going to benefit everyone—those behind me and those older than me. It’s so much more than a doctor’s office for us.”

Jones is heartened by the knowledge that, whatever comes in the years ahead, she will have a medical home. Dr. Anderson concluded, “I tell my patients that as long as you’re here and you need us, we’ll be with you.”