Throughout medical school and into residency, Edward “Donnell” Ivy, MD, MPH, wanted to become a pediatrician, so he could help children avoid the suffering he’d experienced as a child with sickle cell disease (SCD). But the training didn’t quite match up with his expectations.

“It’s not enough to have all these good therapies to treat a disease if you’re not able to actually deliver those therapies to the patients who need them,” Ivy, the chief medical officer of the Sickle Cell Disease Association of America (SCDAA), told Medscape Medical News. “As an individual with sickle cell disease, I decided to dedicate my life to improving sickle cell care that goes beyond only working in the lab or the clinic. There’s so much systemic work to be done.”

The more Ivy learned about the US medical system, the more he realized that public health was his true calling. After switching from clinical medicine, he earned a master’s degree in public health, then did a stint at the National Heart, Lung, and Blood Institute, where he worked on the agency’s 2014 SCD guidelines. “There are newer guidelines from ASH [American Society of Hematology] and others, but I’m still proud of what we accomplished with that guideline,” he said in an interview at the SCDAA annual convention in Chicago in October. “The goal was to increase the number of providers that were available to assist in the treatment of individuals with sickle cell disease.”

Then came Ivy’s time spent at the National Health Research Services Administration, where he continued his work to improve life for people with SCD and other diseases. “We moved to a regional approach, where we tried to decentralize the knowledge around treating sickle cell disease and get more providers involved in that.” 

‘Life-Changing’ Hydroxyurea

Ivy’s childhood experiences always loomed large. Along with multiple pain crises each year, he experienced avascular necrosis (AVN) in both hips, starting at around age 8. He soldiered on through adolescence. While he was in college, a pain crisis with symptoms of acute chest syndrome (ACS) changed everything.

The treating physician recognized that ACS requires treatment by a specialist and transferred Ivy to The University of North Carolina at Chapel Hill, where he received hydroxyurea for the first time. “Hydroxyurea has literally been life-changing for me,” he said. “I went from having three or four crises requiring hospitalization each year to not having any crises at all.” 

After starting hydroxyurea, Ivy went from being an inconsistent student to making the dean’s list every semester, then attending medical school at East Carolina University in Greenville, North Carolina. “I was able to go to medical school in large part because of hydroxyurea,” he said. “That and coordinated care for SCD transformed my life.”

While in medical school, Ivy realized he could no longer postpone the dual total hip replacement surgeries that he’d needed for more than 10 years. “I’d had a severe limp for years, but the pain became too much during my surgery rotation, when I had to stand all day on bad hips,” he said. “I couldn’t help but take pain medicine because it was excruciating. Restful sleep was impossible.” 

For Ivy, his lived experience shows the challenges of improving care for people with SCD. “It’s not enough for us to just prescribe medicine and call it a day. We must figure out how we address those social determinants of health,” he said. “That means figuring out how to build a system that addresses the needs of a population that has historically had to deal with racism, underfunding, poverty, and unequal access to care.” 

That means hematologists should work to increase uptake of clinical guidelines and proven therapies like hydroxyurea where appropriate, Ivy said. He encourages hematologists to look for ways to partner with primary care clinicians. For example: By forging ties so that, once the hematologist prescribes and titrates hydroxyurea, the primary care provider can gain confidence with routine management of the patients.

“Every sickle cell patient should see a hematologist at least once a year. If they’re well managed, then maybe that annual visit is enough, and their primary provider can manage things the rest of the year,” Ivy said. “If they’re not well managed, that’s going to include seeing the hematologist more frequently. But we also need a care coordination effort that involves the hematologist, primary care, social workers, other allied health professionals, and even community health workers to help with transportation issues and other barriers in the community.” 

Ivy added, “We need hematologists to step up and take the lead with coordination where needed.”

Ivy sat down with Medscape Medical News recently. The following interview has been edited for clarity.

How does the reduced emphasis on careers in classical hematology affect the patient population with SCD?

We know that training programs tend to emphasize hematology-oncology as opposed to classical hematology. We also know that reimbursement is better in hematology-oncology. But there aren’t enough classical hematologists to adequately treat the 100,000 Americans with SCD. As a field, we need to approach this creatively to ensure patients get the care they need.

How can sickle cell specialists coordinate with other hematologists to improve care of patients with SCD?

I can appreciate that there are hematologist-oncologists who prefer to avoid treating sickle cell patients. They can be difficult, time-consuming patients. But I encourage hematologist-oncologists to be open to connecting with a sickle cell specialist. Ideally, that sickle cell specialist would take the complicated cases — the ones where the patient is not well controlled, and the case may be very time-consuming or challenging to manage. But the majority of patients with sickle cell, particularly if we can get them into coordinated care, are going to become much less complicated to manage over time. If hematologist-oncologists could add these patients to their panels, everyone would benefit, and care would improve.

What are some barriers to increasing hydroxyurea uptake?

The reality is that there are still too many SCD patients using the emergency room as their primary source of care, which means they’re not getting the comprehensive care they should be getting. And that might include hydroxyurea, the lack of which might be setting them up for end organ damage or early mortality.

Some patients are also put off by the fact that hydroxyurea is a chemotherapy drug, even though it is safe and well-studied.

It’s also fair to note that hydroxyurea is a big reason why we need more hematologist-oncologists to be willing to treat patients with SCD. As long as patients with SCD see a hematologist once a year, it’s possible that an experienced pediatrician, internist, or family practitioner can also prescribe hydroxyurea, as long as they feel supported, especially in a care-team setting.

What are steps to take to improve care for patients with SCD?

It’s impossible to overstate the importance of coordinated care. I’m a good example of that. Even though I’m very stable on hydroxyurea, I’m still a 54-year-old Black man with SCD and am subject to higher rates of diabetes, hypertension, and renal failure than my white counterparts. In addition to problems from AVN, I also have arthritis now. I know how to function within the system to get the care I need from both my primary doctor and my hematologist, but what about others who don’t?

I would also like to call out closer adherence to the guidelines. For example, we know that penicillin prophylaxis in children protects against severe pneumococcal infections and improves outcomes, but not enough children with SCD are getting it. Similarly, transcranial Doppler ultrasound in children accurately predicts those at risk for stroke, allowing for prompt preventive treatment. 

How can hematologists improve SCD care on a systems level?

Getting involved with the SCDAA is a good option. We have an active advocacy effort, a clinical trial finder, community health worker training, and a variety of initiatives that work on large-scale change. We also work closely with the National Alliance of Sickle Cell Centers which provides consensus recommendations for infant, pediatric and adult patients, as well as acute pain crises. They also offer a variety of toolkits and resources for providers, including guidance on how to start a sickle cell center. Membership is at the institutional level, meaning that hematologists may be able to influence policy at their institution. We need more hematologists that are willing to join the NASCC and learn more about the treatment and management of SCD so that they can be a resource.

Ivy reported having paid consulting relationships with Pfizer, Novo Nordisk, and Sickle Cell Awareness Group of Ontario. He is an unpaid consultant with the Global Action Network for Sickle cell and Other Inherited Blood Disorders.

Darcy Lewis writes about clinical medicine from Chicago.