Yale New Haven Health improves adult sickle cell care: experts available for interviews

Each year 300,000 babies worldwide are born with sickle cell disease, an inherited mutation that causes typically donut-shaped and flexible red blood cells to become crescent-shaped and rigid. This deformation prevents red blood cells from efficiently ferrying oxygen throughout the body. If not treated properly, people living with sickle cell disease can suffer from anemia, infections and damage to virtually every organ in the body. Failure to give sickle cell patients competent care is the main driver of early death.

Pain crises, episodes of excruciating pain caused by blockage in blood vessels, are the hallmark of this disease. The pain cannot be measured by diagnostic tools which is one reason why the disease is complicated to manage and has been misunderstood throughout its history.

The mutation that causes the disease emerged in Africa, where it gained an evolutionary advantage by protecting some people against malaria. While most people living with sickle cell disease in the United States have some African ancestry, people with ancestry from the Mediterranean, Middle East and India, can also have sickle cell, making it one of the most common genetic blood disorders in the United States.

Racial bias, stigma around opioid use and a lack of quality sickle cell care across the United States cause many people with the disease to receive disjointed care that often results in multiple hospitalizations and heavy Emergency Room use.

According to data from a study published in PLOS One by physicians at Yale New Haven Health, creating a program devoted to treating adults who have sickle cell disease reduced Emergency Department visits by more than 60 percent and hospitalizations by 53 percent. The key to its success? Building relationships with patients.

“Many people with sickle cell disease, once they reach adult age, do not have access to consistent care and pain management,” said Mahfuz Hoq, MD, MPH, executive director, Clinical Operations and Effectiveness, Bridgeport Hospital. “Only the person in pain can tell you the appropriate way to manage their pain. Our vision was to remove barriers to quality care and ensure people in the program work closely with one prescriber who has all of the necessary information.”

One of these barriers is that only 20 percent of family physicians say they feel comfortable treating sickle cell disease, according to a 2015 survey of more than 3,000 physicians. This leaves many people without the routine, preventive care known to reduce pain crises and makes it more likely that people will seek care in an Emergency Department where staff are sometimes ill equipped to treat sickle cell.

Over the past decade, Yale New Haven Health has grown sickle cell care from piecemeal emergent care to a comprehensive program that provides patients access to primary care, social work, psychiatry, pain management and telehealth.

The Adult Sickle Cell Program at Yale New Haven Hospital increased outpatient clinic visits by 30 percent. This increase is essential for quality sickle cell management as these appointments are where patients build trust with their providers, share concerns and refine how they manage the disease.

Additionally, detailed care plans are included in medical records so that people can get proper treatment should they need to go to the emergency room or be admitted. This communication has allowed people in a pain crisis to obtain appropriate amounts of opioids, which can be difficult given increased regulations around pain medication.

John D. Roberts, MD, medical director, Adult Sickle Cell Program, Yale New Haven Health, who was an investigator in a landmark study that demonstrated chronic pain is common in adults with sickle cell disease and promoted changes in the management of sickle cell pain throughout the nation, currently focuses on improving delivery of care for adults living with sickle cell disease.

“The program can be a major change for people who are often exhausted by navigating the obstacles to getting care,” said Dr. Roberts. “We ask people to reduce their Emergency Department use and attend more outpatient appointments. There is a lot of dialogue involved in finding a treatment plan that works within someone’s comfort level.”

Dr. Roberts explained programs addressing all the factors impacting people with sickle cell help break the cycle of improper care for a disease, which if not carefully managed, robs people of their quality of life.

“I have seen lives transform from people being in the hospital for weeks on end for pain treatment – to managing their pain and other aspects of their disease largely at home, and re-engaging with their family and friends.”