File photo: Jeffrey Glassberg, MD

At the start of the COVID-19 pandemic, physicians who specialize in sickle cell disease feared that their vulnerable patients would be especially hard hit. Indeed, COVID-19 is still a serious public health threat, but the experience of patients with sickle cell disease has been surprising in many ways, according to Jeffrey Glassberg, MD, Director of the Comprehensive Program for Sickle Cell Disease at the Icahn School of Medicine at Mount Sinai. Here is what Dr. Glassberg says people should know about COVID-19—and about advances in sickle cell treatment that have made this a time of “tremendous optimism.”

What have you learned about the COVID-19 risk for people with sickle cell disease?

When COVID-19 initially became a problem for us in North America, we were very worried. This is especially true because people with sickle cell disease get something called acute chest syndrome, which is a situation where the lungs fill up with fluid and it becomes harder to breathe. Since COVID-19 is a disease where you get basically a viral pneumonia, I was very scared about what was going to happen to all the people that I take care of.

As it turned out, it was not nearly as bad as I had feared. Our patients actually wound up doing quite well. One after another was treated for a day or so, and released. So we were very relieved. And we pooled our data with other centers and found that only sickle cell patients with other serious risk factors, like major heart disease or kidney failure, did poorly with COVID-19. As the Centers for Disease Control and Prevention points out, COVID-19 is a new disease, and there is still only limited data and information about its impact and risks. But here on the ground, in clinics, this is what we have seen in recent months.

Is there an explanation for these outcomes?

We aren’t sure yet. But one thing we know about COVID-19 is that the older you are, the worse it is. And in general, our patients tend to be a little bit younger, partially because, sadly, the average lifespan for someone with sickle cell disease is probably around 50 years old. So we have a lot of young patients.

 What do you advise your sickle cell patients to do now?

COVID-19 is still an infection that you don’t want to get. However, if you have sickle cell disease, there are real dangers to not getting your medical care, and so you shouldn’t put a stop to all visits to the hospital.

People with sickle cell disease need a lot of medical care. They need to be watched closely; they need to have their labs checked very often, on very specialized medicines. If our patients with sickle cell disease are unfortunate enough to get coronavirus, it seems as if they don’t have any additional risk, or at least not much more risk than a normal young person experiences. But the risk of not getting your medicine or not getting your labs checked—that’s big. You could be on the wrong dose of medicine. So especially now that the pandemic is cooling off, and we have low rates of coronavirus in the New York region, this is a great time to come and get your medical care and catch up on things that didn’t get taken care of during the height of the pandemic.

How prevalent is sickle cell disease, and what does it do to the body?

Sickle cell disease occurs in about 100,000 Americans and about three million people worldwide. It affects people who are descended from areas of the world that have had malaria—so that can be Africa, South America, or the Middle East. It is a disorder of the blood caused by a genetic mutation. And it causes effects in every part of the body, because blood supplies every part of the body, but the most common manifestation that we see is pain. Patients will have episodes where suddenly they feel terrible pain. That is described as worse than delivering a baby, worse than having your bones broken, and you very often need to come to the hospital to be treated.

What are some of the recent big advances in sickle cell treatment?

Sickle cell disease today is in a place where we have tremendous optimism. I remember back in 2010—the 100th anniversary of the discovery of the gene that causes sickle cell—we were lamenting the fact that we had only one medicine to treat this disease, hydroxyurea. Fast forward 10 years, and we have 40 medicines that are in development, and four really good medicines that are FDA-approved. In addition to hydroxyurea, we now have L-glutamine oral powder and crizanlizumab, which reduce the number of painful crises, and voxelotor, which improves anemia in people with sickle cell disease. And then we have gene therapy, which cures sickle cell disease.  Gene therapy at this point should not be the option for everybody because you do need to get chemotherapy to get gene therapy.  But we are really at the cusp, I feel, of curing the disease.

And while we wait for this cure, we have new medicines that enable us to control the disease to a level we never have before. So this is an incredible time for the community of people with sickle cell disease. If you don’t already have a sickle cell specialist, come and see somebody who is really plugged into all of this to make sure that you are availing yourself of all these new therapies.

Any final advice for people with sickle cell during the pandemic?

Anybody can have a bad outcome with this COVID-19, even a perfectly healthy 25-year-old person. And you can spread this virus even if you feel well. So we should all be very cautious. We should continue to wear masks; we should continue to wash our hands; and we should avoid unnecessary travel and unnecessary trips to crowded places.

We have been fortunate enough through this pandemic to learn a lot about telemedicine. And so we have expanded those options, where you can see a sickle cell specialist through telemedicine wherever you are in the tristate area, and get many of your needs taken care of. When it gets to the point where you need treatment in person or lab tests, it now makes sense to come in, because hospitals have done an excellent job making it safe.