3 Ways We’re Transforming Sickle Cell Care

By Ify Osunkwo, MD, MPH

As someone who spent my childhood and medical school years in Nigeria, I grew up seeing the devastating effects of sickle cell disease. One in four Africans carry the sickle cell trait, and more than half of African children with the disease will die before they reach adulthood. Sickle cell also affects about 100,000 people in the United States – mostly African Americans – but our healthcare systems struggle to deliver effective care.

That’s why my colleagues at Atrium Health Levine Cancer and I spent the past five years building one of the nation’s most innovative sickle cell programs. From haploidentical stem cell transplants to novel pain regimens that reduce the need for daily, high-dose opioids, we’re spearheading ways to improve cure rates and help more patients lead happy, productive lives. Here are three ways we’re reshaping sickle cell care:

Haploidentical Transplants for Sickle Cell Disease

Allogeneic stem cell transplants have been used to cure sickle cell disease since the 1980s, but fewer than one in five patients have an HLA-matched sibling donor. We’re participating in a multicenter, Phase II study that investigates whether haploidentical transplants, from a patient’s parent, brother, sister or other family member or close relative, are a viable alternative.

The study is open to children with sickle cell who have had a stroke or are at high risk for one, and to adults with severe disease. The preliminary results are promising: Our first two patients were stable eight months after transplant.

Improving the Transition to Adult Care

We’ve seen it far too often: Young adults with sickle cell stop going to the doctor. Then they experience severe pain and illness, and rely on emergency rooms for acute care. In fact, there’s a three-fold increase in mortality rates among young adults with sickle cell when they leave pediatric care. Levine Cancer received a $9.8 million grant from the Patient-Centered Outcomes Research Institute to test a standardized approach to transitioning young adult patients with sickle cell to adult care.

Now our team is partnering with healthcare organizations in eight states to implement this approach, which has pediatric and adult providers follow the same six-step process for all patients starting in adolescence and continuing into adulthood. We’re investigating how this impacts key metrics like quality of life, mortality rates and how often patients utilize acute care. The early results are encouraging, with all clinics showing quantifiable improvements in their processes.

Reducing Opioid Use in Sickle Cell Patients

I noticed a disturbing trend several years ago: Many sickle cell patients were ending up in the emergency room 24 to 72 hours after being discharged from the hospital. They all had symptoms of worsening generalized pain, nausea, chills and low-grade fever. I first suspected a seasonal virus – and then realized the patients were going through withdrawal from the opioid medications used to treat their acute pain in the hospital.

When a patient goes through this experience repeatedly, they require higher doses of opioid to treat their pain and end up on very high daily doses. This leads to side effects like depression, dental decay, constipation and worsening daily pain.

Instead of giving more narcotics to patients going through this cyclic withdrawal, we started offering buprenorphine to select patients. Buprenorphine is a novel pain medication that has traditionally been used to treat opioid dependency.

More than 55 patients have been successfully treated with our buprenorphine regimen and the results have been amazing. They typically spend less time in the hospital, have fewer withdrawal symptoms, and are far more likely to stop taking daily opioids. This helps many of these patients get back to holding down a job and leading a normal family life.

Setting a New Standard

We’re leading several other key projects, including a chronic red blood cell exchange transfusion program that’s reducing the risk of repeat strokes.

We also established a “STOP-LIGHT” action plan to help patients manage sickle cell pain at home, and to provide guidance to the emergency department on how best to treat these patients when they present with acute pain.

We also partner with primary care providers to manage adult patients so they receive excellent sickle cell care while getting preventative care like mammograms and colonoscopies. And this is only the beginning of our quest to advance sickle cell care in ways that improve patients’ lives here in the Southeast, and set a new standard for others nationwide.

To learn more about Atrium Health’s sickle cell program or to refer a patient, please call 980-442-4363.