Our second case received multiple therapies including corticosteroids, intravenous immunoglobulin, cyclophosphamide, rituximab, azathioprine, hydroxychloroquine, and plasma exchange; however, she was unable to come off of ECMO. She was evaluated by the lung transplant team, but was not felt to be an appropriate lung transplant candidate due to prolonged ECMO course (39 days), deconditioned state and concern for persistent active disease. The care team and the family made the decision to discontinue ECMO contingent upon the arrival of distant family members, but prior to this, she died suddenly of cardiac arrest after 71 days on ECMO.
Lessons Learned
These experiences highlight a critical need for further development of guidelines and research in this area, particularly because the prevalence of autoimmune disease continues to rise. There is a need for transparent discussion among transplant teams, physicians and families regarding whether a patient is an organ transplant candidate as early in the course as possible.
We learned from these two cases; however, more uncertainties than answers remain when rheumatology patients with active disease are in need of organ transplant.
W. Blaine Lapin, MD, and Jennifer L. Rammel, MD, MPH, are second-year pediatric rheumatology fellows at Baylor College of Medicine, Texas Children’s Hospital, Houston.
Andrea A. Ramirez, MD, MEd, is an assistant professor of pediatrics and associate program director of the pediatric rheumatology fellowship at Baylor College of Medicine, Texas Children’s Hospital, Houston.
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