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Q&A With a Sickle Cell Disease Expert

Q&A With a Sickle Cell Disease Expert

This National Sickle Cell Awareness Month, we spoke with Dr. Nirmish Shah, the Director of the Sickle Cell Transition Program at Duke University. He shares some sickle cell disease basics and talks about his research aimed at helping patients manage sickle cell disease. See how he’s using technology to improve care for patients.

What is sickle cell disease?

Sickle cell disease is a lifelong disease that runs in families, affecting as many as 100,000 people in the United States. It is caused by an abnormality in hemoglobin (a protein in red blood cells that carries oxygen throughout the body), which causes the red blood cells to be sickle-shaped instead of round. People with sickle cell disease also have fewer red blood cells, and over time, blocked blood flow and lack of oxygen can cause serious health problems and organ damage.

The most common complication and cause for being admitted to the hospital for sickle cell disease is pain. Pain is caused by a decrease in blood flow to bones. Many pain crises occur quickly and without warning. Other significant complications include stroke, acute chest syndrome, and kidney problems.

What does life with sickle cell disease look like?

Everyone is different. We don’t know who will have complications from sickle cell disease, so we watch everyone closely. During childhood, which is when most people have problems, we work on preventing infections, screening for complications, and managing any problems that happen. Children and adolescents don’t often have episodes of intense pain, but the risk of pain episodes and other problems increases with age. For adults with sickle cell disease, we focus more on managing or preventing these pain episodes.

Will you tell us about your work to help people living with sickle cell disease?

As Director of Duke University’s Sickle Cell Transition Program, my team’s research focuses on treating children and adults with sickle cell disease. When I work with children, I try to teach them about their condition and how to transition successfully to living with the disease as an adult. For all of my patients, I focus on giving the medical, social, mental, and emotional support they need to be healthier. My research aims to test new medicines for sickle cell disease and use new technologies to improve care for patients.

Why is it important to you to pursue this research?

In the past, we have not had many options for treating sickle cell disease. Today, treatments can lessen complications, but they do not get rid of them completely. We are testing newer medicines that I hope can give people more options when other treatments don’t help. Also, chronic diseases such as sickle cell disease are difficult to keep an eye on and manage. I have been creating mobile applications that patients can use to record their symptoms and treatments. These apps also let health professionals remotely monitor and communicate with patients. I have also been using wearable technology (similar to a fitness tracker) to measure how well my patients are moving. This helps us better understand how pain impacts their disease. All of this research and technology is so important to helping us offer more treatments and help monitor and manage sickle cell disease.

What do you hope your work will accomplish?

We need innovative ideas to improve care for chronic diseases, such as sickle cell disease. Since most people have smartphones and access to technology, I have been working hard to use mobile apps, wearable devices, and virtual reality to improve medical care. I believe that all of this technology will soon become the norm in helping patients. 

What insights can you share with us?

First, patients are very interested in using technology, such as mobile apps, but people use technology less often when they are feeling really well and when they are feeling really bad. This is not surprising, but it is important to understand when managing patients. Second, we learned that patients who texted with their health care providers were much less likely to go to the emergency room and hospital. The patients were much better at managing their disease. Finally, we learned that we can use wearable devices to measure activity and heart rate and predict pain. The data from a wearable device could tell us how much pain a patient was experiencing with 70% accuracy. I am very excited that all of this technology is quickly developing, and I believe we will be able to use it to improve the health of people with sickle cell disease in the near future.

The statements and opinions in this blog post are those of the author and do not necessarily represent the views of the U.S. Department of Health and Human Services Office on Women's Health.