Diagnosed with sickle cell disease (SCD) when he was very young, Damien Nevels grew up receiving treatment at St. Louis Children’s Hospital and feared moving over to its big neighbor, Barnes-Jewish Hospital, when he turned 18.
“Children’s is like family,” he says. “It’s like growing up in your parents’ house and not wanting to leave.”
Although he resisted making the change, Damien, now 23, is proud to be a part of Barnes-Jewish Hospital, working as its first sickle cell transition intern. In this paid, part-time role, he assists younger patients and their families as they deal with challenges of the disease and the move to adult care.
The goal is to help patients transition to adult care while managing their illness and obtaining regular medical treatment, thus reducing their reliance on emergency room care. The program also aims to educate medical staff about misperceptions they might have about SCD patients and help them overcome any unconscious biases. Thanks to loyal donors, The Foundation for Barnes-Jewish Hospital is able to fund this innovative internship.
“The sickle cell community is tight-knit and Damien is able to pass along his knowledge to patients and serve as a mentor,” says J. Evan Sadler, MD, PhD, holder of the Foundation’s Ira M. Lang Endowed Chair, director and professor of medicine, Division of Hematology at Washington University School of Medicine. “Just the fear of the unknown is an important barrier and Damien is able to help them overcome that barrier.”
As a sign of national concern about the challenges facing teen SCD patients, Victoria Faulkner, BSN, RN, hematology clinical nurse coordinator at Washington University School of Medicine, and Damien were invited to speak about the internship at the 45th Annual National Sickle Cell Disease Association of America Convention in Atlanta, Georgia, in October 2017. Also this year, Washington University School of Medicine received a six-year National Institutes of Health grant to research the best care for teens and adults with SCD.
In the United States, SCD is the most common hematological genetic disease in people of African, Middle Eastern and Mediterranean descent. SCD encompasses a group of red blood cell disorders resulting from abnormal hemoglobin, a protein that delivers oxygen in the body. The condition causes typically round red blood cells to become crescent-shaped, or sickled, and leads to chronic pain and fatigue, organ damage and reduced lifespans.
Studies on children with sickle cell disease have shown that early diagnosis and continued medical treatment can reduce symptoms, contribute to enhanced well-being and, ultimately, prolong life.
However, some young patients, who are used to the child-centered environment at a pediatric hospital, avoid getting regular care as an adult. According to a Washington University School of Medicine study of 3,200 SCD patients, emergency room visits tripled from age 15 to age 24. Damien was almost one of them.
“I waited as long as I could to transition to adult care. I was thinking ‘I’m healthy enough maybe I can wing it,” says Damien. “But my mother wouldn’t allow it.”
Those teens and young adults who do choose to “wing it” often end up seeking emergency room treatment when they suffer a pain crisis, Victoria says. Although they are experiencing excruciating pain, they may not appear to have a serious condition and end up waiting hours to be seen.
“One of the challenges is the misperception of these patients’ pain,” she says. “People have lived with it for so long they are able to function, masking their pain by using their cell phones or listening to music through headphones.”
But when they have to wait for a long period of time in the emergency room after suffering for so many hours, they often get angry and frustrated. This can affect their behavior towards medical staff, who treat them as difficult patients.
Because Damien’s internship includes shadowing and interacting directly with health care professionals, he acts as an educator, helping them to better understand what SCD patients are going through and paving the way for them to become more sympathetic.
In his mentoring role, Damien acts as a sounding board for young SCD patients, listening to concerns that they might not feel comfortable expressing to adults. He introduces them to the hematology clinic at Barnes-Jewish and gives them tips on managing pain and avoiding the triggers that cause them.
And a very big part of what he does is gives patients and their families hope.