A Review of Treatment Options for the Management of Sickle Cell Disease - Episode 9

Unmet Needs in Sickle Cell Disease Management

June 21, 2021
Michael R. DeBaun, MD, MPH, Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease

Strategies to avoid complications of sickle cell disease and ensure non-dependency on opioids when treating patients for pain as a result of the disease.

Michael R. DeBaun, MD, MPH: In terms of avoiding complications, a family that is well educated about the disease, the treatments available, the preventive strategies for complications, including nonmedicinal strategies explicitly, such as mindfulness, good eating habits, exercise within moderation, and good sleep habits, they are going to do far better than the family that does not have these in their lives. Most providers are not well informed about sickle cell disease. Thus, a well-informed parent or adult with the disease who provides information in a nonthreatening fashion can improve their own health care. I would stay abreast of the research as another strategy, because treatment of sickle cell disease in 2020 is vastly different than treatment of sickle cell disease in 2010, and I would wager that curative therapy options in the next decade will be better. We will have improved outcomes with less toxicity. 

Lastly, I would consider all the FDA-approved drugs as potential options for treatment. Particularly for those individuals with hemoglobin SS and S beta-zero thalassemia, with each passing year, we learn more about the benefit of this drug. In my opinion, by far this drug should be in the stable of anyone with hemoglobin SS and S beta-zero thalassemia because the benefits far outweigh any of the toxicities or complications in taking the medication. For the other drug, I think it will take time before the research will demonstrate what the specific benefits are in decreasing the rate of CNS [central nervous system] disease, heart, lung, and kidney disease, as well as potentially priapism and other complications of the disease. But I would say stay tuned because we will learn more about how these additional drugs will prevent complications in organ function in the next decade. 

The first recognition for decreasing opioid dependency is acknowledgment that pain occurs. There should be trust between the provider and the patient, and the patient needs to develop a strategy to manage the pain that is not dependent on opioids but is dependent on first, understanding the triggers, and understanding how to use mindfulness and other strategies to decrease the severity and intensity of the pain. Then, the goal is to learn enough about their disease and enough about their treatment to use a stepwise approach to managing their disease when it comes to acute vaso-occlusive pain. That would include the use of nonsteroidal drug, the use of hydrocodone as a stepwise approach, and then eventually morphine. After the pain subsides, it’s important to stop the medications I just mentioned so the use of pain medications will not result in other complications.

Transcript Edited for Clarity

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