Conversations Surrounding SCD Treatment Options


Nirmish Shah, MD, shares his advice for initiating conversations with patients about treatment options and selection criteria for sickle cell disease.

Biree Andemariam, MD: I just wanted to switch to Nirmish. How do you initiate conversations with your patients about their therapeutic options? What are the options? For how long? Are they going to be on them? What are the treatment goals? What are the things you discuss?

Nirmish Shah, MD: The best time to talk about these treatments is when they don’t need the treatments. When you come into a patient’s room and say, “We’re going to start this medicine today,” they need time to digest. They need time to understand not only what the medicine does, including adverse effects and potential benefits but also how that plays into their lives. For example, we start hydroxyurea at a very early age: 9 months. But all these other therapies start a little later, and l-glutamine is at 5 years of age, voxelotor is at 4 years old, and crizanlizumab is at 16 years old. These are conversations we need to start before we even have the need for those medications. Give time for the patient to understand what this mechanism of action is, that there are other therapies out there. For many years, we’ve been emphasizing that hydroxyurea is a great drug. That’s wonderful. But we have new therapies that study these new drugs with hydroxyurea. Patients aren’t used to thinking about taking more than 1 medicine.

As Elna [Saah] brought up, it’s just like having a conversation for an acute leukemia: you need multiple therapies to control a very severe disease. I have the conversations almost every visit: “Just so you know, we have several therapies. I’m not saying you need any of them right now. But just know that 1 might be focused on pain, and 1 might be focused on anemia and hemolysis.” I’m going to keep that in my back pocket. If anything changes, I can come back to it. That way when I do have a conversation that they need a drug, I’ve already started the conversation. [They might then say] “We’ve been talking about this for a while. Tell me more about that.” Patients are really savvy these days with social media, smartphones, googling things all the time. Give them little handouts of brief information, so they can educate themselves about these new therapies. I’m very much a proponent of having conversations with the patient, their families, and their loved ones, so they all can start to think about these.

[You also asked] how long I treat patients with these new therapies. This is a semantic thing. I don’t like to say, “You’re going to be on this drug forever.” When you say forever, it rubs patients the wrong way. I say indefinitely—as long as it’s doing good, as long as you’re not having adverse effects, as long as we’re getting the benefit we hope to get. We want you to stay on these drugs as long as possible but indefinitely. I’ll always be reassessing to make sure we’re not having issues. That might be a semantic thing with how I talk to patients. It’s a minimum of 6 months with newer therapies to make sure I’m having a benefit, but they need to understand that every patient is different. If you’re benefiting from a drug, we need to keep at it. How do I get a patient to be compliant? That’s another discussion. How do I get them to be compliant and stay on therapy as I want them to be?

Matthew M. Heeney, MD: I like the idea about Nirmish saying, “Not forever.” I always say, “Until something better comes along.”

Nirmish Shah, MD: Exactly.

Transcript edited for clarity

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