Affiliations: [a] Section of Hematology, Children’s Center for Cancer and Blood Diseases, Children’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
| [b] Department of Biomedical Engineering, Viterbi School of Engineering, University of Southern California, Los Angeles, CA, USA
| [c] Department of Pediatrics, Pediatric Pain and Palliative Care Program Division of Hematology-Oncology, Department of Pediatrics, David Geffen School of Medicine at the University of California Los Angeles, Los Angeles, CA, USA
Correspondence:
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Corresponding author: Thomas D. Coates, MD, Section Head of Hematology, Childrens Center for Cancer, Blood Disease and Bone Marrow Transplantation, Children’s Hospital Los Angeles, 4650 Sunset Blvd, MS 54, Los Angeles, CA 90027, USA; Professor of Pediatrics and Pathology, University of Southern California Keck School of Medicine, Los Angeles, CA, USA. Tel.: +1 323 361 2352; E-mail: tcoates@chla.usc.edu.
Abstract: Sickle cell disease (SCD) is a genetic disorder of hemoglobin producing hemoglobin-S (HbS) and resulting in recurrent severe episodes of pain, organ damage and premature death due to vaso- occlusion. Deoxy HbS polymerizes, causing red cells to become rigid and lodge in the microvasculature if they do not escape into larger vessels before this transformation occurs. The mechanism that triggers this transition from steady state to vaso-occlusive crisis (VOC) is not known. Patients state that cold, emotional stress, and pain itself can trigger these events. In spite of the connection between these symptoms and the autonomic nervous system (ANS), and the fact that the ANS regulates regional microvascular blood flow, the role of the ANS in sickle pathophysiology has not been significantly investigated. We will briefly review the mechanism of SCD vaso-occlusion, the dysautonomia associated with SCD and sickle trait, and the role that the ANS may play in the genesis of sickle vaso-occlusive crisis.
