Moving from Awareness to Action after Sickle Cell Disease Diagnosis

Karen L. Proudford, Ph.D. is the President of Williams E. Proudford Sickle Cell Fund, Inc., and daughter to William E. Proudford, who lived with Sickle Cell Disease. To mark World Sickle Cell Awareness Day, Global Genes asked Dr. Proudford to share the legacy of her father’s life with sickle cell disease, and the work that Williams E. Proudford Sickle Cell Fund, Inc. does in the community.

This August will mark 20 years since my father’s passing. Devoted family man, colleague, and friend. Soft-spoken and stoic. Eager to give and reluctant to complain. Focused, determined, and loyal. Dedicated to education and learning. These are words and phrases I still use to describe William E. Proudford. Despite a number of chronic physical challenges, including sickle cell disease, he had a quiet, unassuming, steady and principled manner that made him the bedrock of our family.

With his spirit as a guide and with an understanding of the unique challenges sickle cell “warriors” encounter in getting treatment, my family started an effort to raise awareness about this devastating disease. After establishing the William E. Proudford Sickle Cell Fund, Inc. as a nonprofit organization, we donated start-up funds and immediately began forming
partnerships with area organizations to work towards a shared vision: a world without sickle cell disease.

The United Nations designated June 19th as World Sickle Cell Awareness Day to increase public’s knowledge about and understanding of sickle cell disease. As June 19th approaches, I find myself reflecting on the years since we formed a non-profit, and most recently, since we joined with others to launch Shine the Light on Sickle Cell.

Understanding Sickle Cell Disease

Sickle cell disease is the most common genetic blood disease in the U.S. An estimated 100,000 Americans have a form of the disease, and approximately 8-10% of African Americans carry the sickle cell trait. Normal red blood cells are shaped like doughnuts, and they flow throughout the body to deliver much-needed oxygen. Sickled red blood cells, however, become misshapen, like a crescent moon or a tool used to cut wheat. As these irregular cells circulate, they can cause pain so debilitating that it is called a “pain crisis,” damage to vital organs, and anemia. Most of us will never feel as if we are being hit by a baseball bat, or as if nails are being driven into our joints, or as if glass is running through our veins. Yet, that is how people with sickle cell, including some of our youngest warriors, have described sickle cell pain.

Sickle cell disease affects many nationalities; however, it disproportionately affects people of African descent. Unfortunately, individuals with sickle cell often report being misdiagnosed, mistreated, and dismissed when they go to the hospital in unbearable pain. It is unsettling and troubling to think that a person’s call for help would be answered with disdain, contempt and suspicion. Yet, again, that is how people with sickle cell, including some of our youngest warriors, have described their experiences at health facilities. It is critically important that individuals with sickle cell are heard, respected, and given evidence-based treatments delivered with compassion and care.

The Power of Collaboration

As our nonprofit continued to grow, the power in uniting voices became clear. That’s why we welcomed a chance to help foster connections among nonprofit sickle cell organizations and to work closely with medical providers across the Northeast U.S. SiNERGe, the Sickle Cell Improvement in the Northeast Region through education, is a HRSA-funded collaborative led by medical providers at Johns Hopkins and Thomas Jefferson. As the community-based organization (CBO) arm of the program, our aim is to gather momentum in the fight against sickle cell disease. Our vision is longer, healthier lives for sickle cell warriors who courageously fight this disease. We are inspired by the optimism, tenacity, and determination of warriors to live, create, and pursue their personal and professional dreams – despite the daunting challenges they face.

It has been a privilege for us to work with a talented, passionate, committed group of CBO leaders – many of whom have a personal connection to sickle cell, just as I do – to bring about change. One of our foundational initiatives is Shine the Light on Sickle Cell. Now in its sixth year, Shine the Light on Sickle Cell engages communities in our region to increase awareness of sickle cell disease, build support networks, and to advocate for access to high-quality care and a universal cure. Shine the Light on Sickle Cell includes all kinds of gatherings, activities, and events, such as blood drives, civic proclamations, social media posts, candlelight ceremonies, movie nights, conferences, awareness walks, webinars and more. One of the most visible Shine the Light on Sickle Cell activities is the illumination of buildings, structures, and landmarks in red. The red symbolizes the color of blood cells, and it also captures the intensity, sense of urgency, and drive that sickle cell advocates bring to this work.

Spreading the Red

We are encouraged and inspired by the way in which Shine the Light has become a connecting thread that links the efforts of individuals, groups, and communities. Participation has expanded well beyond our region, to activities around the country and the world. This year, we are thrilled to have the support and partnership of fraternal organizations, global healthcare companies, an NFL team, and the Building Owners Management Association in Philadelphia.

Over the next two weeks, structures and buildings will illuminate in red to show their support for sickle cell warriors, their loved ones, and their caregivers, including:

* The Hospital of the University of Pennsylvania, and Boathouse Row in Philadelphia
* Novo Nordisk U.S. headquarters in Plainsboro Township, New Jersey
* Baltimore Ravens’ M&T Bank Stadium and the Johns Hopkins University Dome in Baltimore
* Boston City Hall and Vertex Pharmaceuticals headquarters
* Rhode Island’s State House in Providence
* University of Maryland’s Capital Region Medical Center, Bowie Health Center, and
* Laurel Medical Center in the Washington, D.C. metropolitan area

This is a testament to the incredible impact we can have when we unite for a common cause.

A Call to Action

On World Sickle Cell Awareness Day, we invite you to Shine the Light on Sickle Cell.

Wear red, host or attend a gathering, or post on social media using the hashtag #ShinetheLightonSickleCell2024. By joining the conversation, you will play a part in moving us closer to a world without sickle cell disease. And that would make my father smile.

On behalf of my family, and our Board, thank you.

Karen L. Proudford, Ph.D.
President, Williams E. Proudford Sickle Cell Fund, Inc.
SiNERGe CBO Lead