People with sickle cell disease have compared the excruciating pain they can experience to thousands of nails being driven into their body. The inherited blood disorder affects an estimated 100,000 Americans and 8 million people worldwide, yet it is often overlooked or misunderstood. The disease gets its name from the damage it does to red blood cells, causing them to contort from the round shape of healthy cells to a rigid sickle shape that prevents the cells from properly transporting oxygen throughout the body.
In honor of September’s national Sickle Cell Awareness Month, sickle cell disease investigator Candice J. Adams-Mitchell, SLP.D., CCC-SLP, shares her insights. Adams-Mitchell is a clinical associate professor of speech, language, and hearing sciences at the University of Florida College of Public Health and Health Professions and director of the undergraduate program in communication sciences and disorders. As a researcher and the mom of Creid, a 7-year-old son with sickle cell disease, Adams-Mitchell has a unique perspective on the challenges of living with the disease, common misconceptions and the ways we can support people with sickle cell disease.
Question: What is sickle cell disease?
Answer: Sickle cell disease is a genetic disorder, mainly characterized by pain. Where blood flows, sickle cell goes. Because people with the disease have sickle cells throughout their body, it may also cause organ damage and many conditions that people don’t normally associate with the disease. For example, sickle cell disease increases the likelihood for stroke, other neurological conditions, swallowing problems, asthma and a lung-related condition known as acute chest syndrome.
Q: What are some common misconceptions about sickle cell disease?
A: Patients with sickle cell disease may be characterized as being difficult patients because of the severity of their pain and how hard it is to manage. They are high utilizers of the medical system, which may cause them to be viewed as drug seekers by health care professionals. Because of this misconception, patients may not feel comfortable seeking treatment and end up suffering at home. These are people who deserve dignity and respect and we need to do whatever we can to support them.
Another misconception is that sickle cell disease is contagious or caused by “bad blood.” Sickle cell disease is an inherited condition; you cannot catch it from someone else.
Some people believe that sickle cell disease is a death sentence. You can live and you can live well with sickle cell disease. We need to appropriately manage patients with sickle cell disease, not just from a pain perspective, but also from a whole life perspective using a more comprehensive model. To address this, we need better education for health care providers and increased research funding.
Q: How has having a son with sickle cell disease shaped your research interests?
A: As time has passed, my research has naturally evolved, shaped by Creid’s experiences, the health care challenges we encounter, and my growing understanding of what is most urgent in sickle cell care. Currently, my focus is on acute chest syndrome and pneumonia. Why are some people with sickle cell more susceptible to these complications? And why, in 2024, are individuals with sickle cell disease still dying from these conditions?
Q: What is on the horizon for sickle cell disease?
A: The Food and Drug Administration has given approval to two companies to offer gene therapy to patients with sickle cell disease. An estimated 20,000 people in the U.S. may qualify for the treatment, but it is currently very expensive, up to $3 million, and most health insurance providers have not yet decided if they will cover the costs of treatment. I do have hope that one day we’ll have a cure for sickle cell that is accessible to everyone. As I tell my son all the time, ‘There’s hope and there’s sickle cell. They both exist.’
Q: How can we support people with sickle cell disease?
A: My desire is for people to learn about sickle cell. Donate blood when you have an opportunity. Right now, we have a huge blood shortage. And for a kid like my son, that’s the difference between life and death for him. So donate blood. Go to NMDP (formerly Be the Match) and find out if you can be a donor for bone marrow transplants. We need people to step up and make sickle cell disease as popular as we’ve made other diseases and disorders.
If you’re in the Gainesville, Florida area, join us on Saturday, Sept. 21 for the Strides for Sickle Cell Walk. The event features a walk in Depot Park, along with food, health resources, vendors and community partners. Proceeds support Streetlight’s Sickle Cell Warriors, a program that supports young patients living with sickle cell disease.