A Quiet Disparity: Brandon’s Story
Brandon is the four-year-old nephew of Daileann Hemmings, RN, BSN, MSN, and Connecticut Health Leadership Fellow Class of 2012. He wants you to know that he is going to a new school this year, and he likes to make “Spiderman fingers” when having his picture taken.
He also has Sickle Cell Disease (SCD), an inherited blood disorder that can cause a shortened life expectancy, extreme pain, even organ death. And if you look at Brandon, you’ll know why Daileann dedicated herself to changing how the health care system manages the disease.
Daileann is a certified intermediate community sickle cell counselor, and Vice President of the Board of Directors for Citizens for Quality Sickle Cell Care, an organization in Connecticut whose mission is “To ensure the availability and accessibility of quality and comprehensive medical care and support services for children and adults in Northern Connecticut with Sickle Cell Disease and other related disorders.”
More than 100,000 Americans are living with SCD, and more than two million Americans carry the trait. Carrying the trait does not make you symptomatic. Where having the trait becomes a concern is when planning a family. If both you and your partner have the sickle cell trait, you have a 25 percent chance with each pregnancy of having a child with SCD. Families can prevent children being born with the disease as long as they are having conversations and getting screened for the trait.
The disparity in SCD is two-fold: historically, it has been believed that SCD only affect people of African descent, marginalizing the disease. The truth is that people of all races can carry the trait, which many people don’t realize, and they don’t undergo screening when deciding to have children.
The second piece of the disparity is that the needs of SCD patients are not always well-understood by providers. SCD changes the shape of the red blood cell from a from a flexible, circular shape into a hard crescent shape – a sickle. The cells can spontaneously become stuck in the joints and cause an unbearable pain crisis, requiring emergency room care. Providers, however, often treat these patients as if they were medication-seeking, and don’t give them the pain medication they need. An untreated crisis can lead to organ failure and death.
In Connecticut, the good news is that systems changes are happening at the hospital level. At Connecticut Children’s Medical Center, University of Connecticut Farmington Hospital, and Yale University, a patient that is identified as having SCD receives immediate care. Daileann herself educates providers so that they understand the needs of SCD patients in crisis.
As Daileann says in the video, awareness of the disease and its complexities is critical, both for prevention and to ensure the appropriate administration of care.
Does someone you know and love have SCD? What other systems changes could we be making in order to create better care, and ultimately, a cure for the disease?