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I'm Aware That I'm Rare: Liz Klings, MD (PE and Sickle Cell)

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Dr. Elizabeth Klings is an Associate Professor of Medicine and the Director of the Center for Excellence in Sickle Cell Disease at Boston University School of Medicine. Dr. Klings discusses embolism and pulmonary thromboses or blood clots in the lungs and how they relate to sickle-cell disease. Her clinical and research interests have focused on the pulmonary vascular complications of sickle cell disease, having published over 40 papers and book chapters on the subject. She led the development of the Clinical Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension in Sickle Cell Disease published in the American Journal of Respiratory and Critical Care Medicine in 2014 and is now pursuing research to try to advance our understanding of this complication of the disease. 


I’m doctor Liz Klings. I am an associate professor of medicine at Balsa University School of Medicine, and I’m director of the center for excellence in sickle-cell disease at Boston University and Boston Medical Center as well as a member of the pulmonary hypertension center.

Today we’re gonna talk a little about pulmonary embolism and pulmonary thromboses or blood clots in the lungs and how they relate to sickle-cell disease.

Sickle-cell disease patients appear to be at higher risk for having blood clots. They can have blood clots throughout different parts of their body at different stages in their lungs, and it’s thought that there are certain factors within their circulating blood as well as their platelets, which are the cells that are responsible for blood clots that may function abnormally, and this may be one of the reasons, or several of the reasons that patients are predisposed to having blood clots.

What we know is that by the time they’re 40 years of age, about 10 to 11 percent of adults with sickle-cell disease will have had a blood clot within their lungs, sometimes referred to as a pulmonary embolism, because the blood clot often embolizes or travels from someplace else in the body, but in patients with sickle-cell disease, these clots may actually just form within the blood vessels, and are just what is called thrombosis or blood clot.

It isn’t completely clear what to do in terms of treatment for patients with sickle-cell disease and a blood clot. We always treat blood clots with blood thinning medicines or anti-coagulants, and that is how we treat a blood clot in patients with sickle-cell disease, as well. The question that remains is how long somebody needs to be treated if they are diagnosed with a blood clot. Why this is important is that we do know that in patients with sickle-cell disease, once you’ve had one blood clot, you’re at higher risk than the general population for having a second one.

We in the field believe that we need to do more research to try to figure out what is the best way of preventing the second blood clot, once the first blood clot has occurred, and whether or not that will entail staying on blood-thinning medicine indefinitely. In terms of recovery, generally, the course for recovery is similar to the general population, so it may take several weeks to months, but often patients will need to stay on blood thinning or anti-coagulant medication for at least six months after their initial blood clot.

There are many patients, however, that may remain on a blood thinning or anti-coagulant medication for their entire lifetime, particularly if they have had blood clots previously or if they have another reason that may make them, in addition to their sickle-cell disease, that may make them more likely to have a blood clot.

The symptoms are actually pretty similar to what you would see in the general population so a lot of times, people who have a pulmonary embolism or a blood clot will develop shortness of breath and it will happen pretty rapidly, usually over one day or two, where it’s much more shortness of breath than they had had previously. Chest pain and shortness of breath are usually the most common reasons that people with sickle-cell disease or someone without sickle-cell disease might seek medical attention related to a blood clot in their lungs.

I think if you had sickle-cell disease and noticed all of a sudden, your breathing has gotten much worse, or you’re developing chest pain, either on the right side or the left side of your chest, that is new for you and you don’t have a reason for why that might be, this would be a reason to go to the emergency room, to try to get more of an evaluation.

One of the ways that you can diagnose a pulmonary embolism is through a test that’s called a ventilation perfusion scan or V/Q scan. In sickle-cell disease patients, sometimes using V/Q scans is a little bit difficult because there are changes that can occur, specifically in patients with sickle-cell disease, that sometimes make a V/Q scan more difficult to interpret. Because of that, we sometimes have to use a CAT or a CT angiogram to diagnose a pulmonary embolism. Many times when we use V/Q scans or ventilation perfusion scans, it’s more used to look for chronic or old blood clots within the lungs, so similar to how patients with chronic thromboembolic pulmonary hypertension [CTEPH] are diagnosed, we may use a ventilation perfusion scan.

Because this is another rare complication of a rare disease, there are still many unanswered questions about how to approach a patient with sickle-cell disease and a blood clot, and it really stressed the importance for more research efforts in this area, to try to better define what we should do for patients.

I’m Dr. Liz Klings, and I’m aware that I’m rare.

Listen to “I’m Aware That I’m Rare: the phaware® podcast” at Never miss an episode with the phaware® podcast app. Learn more about pulmonary hypertension at #phaware #phawareMD

Originally published: August 15, 2017
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