Sickle cell update: Treating pain and progress toward cure

Thursday, January 23, 2020

When it comes to treating pain related to sickle cell disease, consider the underlying factors, from constipation to compression spine deformity. That’s just some of the advice from Ifeyinwa Osunkwo, MD, of Atrium Health and Levine Cancer Institute in Charlotte, N.C. She joins host David H. Henry, MD, of Pennsylvania Hospital, Philadelphia, to discuss her tips for treating pain and other complications of sickle cell disease. Dr. Osunkwo also provides an update on progress toward a cure in sickle cell disease that could be available to a large number of patients.

Plus, in Clinical Correlation, Ilana Yurkiewicz, MD, of Stanford (Calif.) University, talks about why treating patients with cancer doesn’t make her sad.

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Treating pain in sickle cell:

In sickle cell disease, patients have acute episodes of vaso-occlusive crisis, as well as chronic pain.
Consider whether the pain symptoms are an acute exacerbation of their chronic pain, an independent acute episode of pain, or chronic pain.
In her practice, Dr. Osunkwo has moved to less chronic opioid use and more adjuvant use. She says treat the pain but look for the reason underlying it. The pain could be a result of bone damage, a compression spine deformity, constipation, or other factors related to their disease or the treatment.
Consider the impact of opioid withdrawal after receiving a high dose in the hospital.

Treating acute chest syndrome:

Acute chest syndrome is usually not subtle in its presentation. It is acute and includes fever, pain, difficulty breathing or shortness of breath, hypoxia, and the patient looks sick.
Consider their last chest x-ray and look for changes. Is this a new pulmonary infiltrate?
This is a patient who should be transfused to get them out of distress.
Most of acute chest syndrome cases happen 3 days into a hospital admission.

Developments in sickle cell treatment:

Two new drugs to treat sickle cell symptoms were approved in the United States in 2019: voxelotor (Oxbryta) to increase hemoglobin and crizanlizumab-tmca (Adakveo) to reduce the frequency of vaso-occlusive crisis.
What is coming next? Researchers are working on potential cures for sickle cell that would be available to patients on a widespread basis. That includes haploidentical transplant and gene therapy.

American Society of Hematology guidelines on the treatment of sickle cell complications.

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^{David Henry, MD}

David Henry, MD, FACP, is a clinical professor of medicine at the University of Pennsylvania and vice chairman of the department of medicine at Pennsylvania Hospital in Philadelphia. He received his bachelor’s degree from Princeton University and his MD from the University of Pennsylvania, then completed his internship, residency, and fellowship at the Hospital of the University of Pennsylvania. After 2 years as an attending in the U.S. Air Force, he was drawn to practicing as a hem-onc because of the close patient contact and interaction, and his belief that, win or lose with each patient, one can always make a difference in their care and lives. Follow Dr. Henry on Twitter: @davidhenrymd. Dr. Henry reported being on the advisory board for Amgen, AMAG Pharmaceuticals, and Pharmacosmos. He reported institutional funding from the National Institutes of Health and FibroGen.