SAMIR K. BALLAS, MD, FACP, FASCP, FAAPM, DABPM
Samir K. Ballas, MD received his medical degree with distinction from the American University of Beirut-Lebanon. He completed his residency training in Hematology at the Cardeza Foundation of Thomas Jefferson University in Philadelphia, Pennsylvania. He is board certified in Internal Medicine, Hematology, Blood Banking, Pain Medicine and Pain Management.
Dr. Ballas is currently Emeritus Professor of medicine and Pediatrics at Thomas Jefferson University and Honorary Staff member of the Department of Medicine/Hematology at Thomas Jefferson University Hospital. He is Former Director of the adult Sickle Cell Program of the Commonwealth of Pennsylvania for the Philadelphia Region. Dr. Ballas is also a senior member of the Cardeza Foundation for Hematological Research, Former Director of Jefferson’s Sickle Cell Center, and Former Director of the Thomas Jefferson University Hospital Blood Bank. His major research interests include 1) Red cell disorders in general and the hemoglobinopathies in particular; 2) the pathophysiology and management of sickle cell pain; 3) molecular and cellular factors that affect the phenotypic expression of sickle cell disease; and 4) preventative and curative therapy of sickle cell disease.
Dr. Ballas is a member of various prestigious professional organization and societies. He is an elected member of the Alpha Omega Alpha Honor Medical Society since 1967. He is member of the Editorial Board of the American Journal of Hematology, Hemoglobin, and Advances in Hematology. He is also the Hemoglobinopathies Editor of The Cochrane Review Database. He is Recipient of Life Time Achievement Award for Service, Research and Education for Sickle Cell Disease from Howard University on May 10, 2011. In addition, he has authored or co-authored over 700 articles and abstracts in a diverse number of publications including but not limited to the Annals of Internal medicine, American Journal of Hematology, Blood, Transfusion, British Journal of Haematology, Journal of Biological Chemistry, Human Genetics and JAMA. He has authored a book about Sickle Cell Pain published by the International Association for the Study of Pain (IASP) in 1998. The book has been sold out and a second Edition is in preparation.
Abstract: The Many Faces of Sickle Cell Pain
Sickle cell pain syndromes include a triumvirate of acute, chronic and neuropathic pain that occur sequentially or simultaneously with age. Unlike other diseases associated with pain such as osteoarthritis, rheumatoid arthritis, fibromyalgia and complex sympathetic dystrophy, sickle cell acute pain manifests itself in infancy and continues to recur throughout the life span of the affected patient. With age, acute pain retains its unpredictable relapses and spawns chronic pain as a new partner in the rhapsody of pain and suffering. Persistent chronic pain may evolve into neuropathic pain. Acute pain, however, dominates the clinical picture and requires urgent treatment with parenteral opioids in the ER and/or the hospital. . Pain that occurs between the acute episodes is usually milder and treated at home with oral analgesics and is often referred to as chronic pain. Although this classification is somewhat arbitrary, nevertheless management of sickle pain is based on these assumptions.
The painful crisis often precedes the onset of other complications of the disease such as acute chest syndrome and acute multi-organ failure. About 50% of cases of acute chest syndrome occur in patients a few days after admission to the hospital with acute painful crises. Moreover sudden death is known to occur during a painful crisis or shortly after discharge from the hospital. The frequency of acute painful crises in patients varies within and between individuals from rare occurrences during a lifetime to many times a month. About 30% of patients have rare or no pain episodes, 50% have occasional episodes and 20% have weekly or monthly episodes requiring medical attention. The frequency of pain episodes increases late in the second decade of life and decreases in frequency after the fourth decade for reasons that are not understood. Frequency of more than 3 episodes a year is associated with a reduced life expectancy. A small number of patients account for the majority of patients requiring healthcare for acute pain episodes .
Moreover, the resolution of the painful crisis is associated with rebound thrombocytosis, elevated levels of fibrinogen, orosmomucoid, RBC deformability and plasma viscosity indicating the presence of a hypercoagulable state that may cause recurrence of the crisis. About 20% of the patients who discharged from the hospital after the resolution of a crisis had recurrent crises that required treatment with parenteral opioids in the ER or hospital within one week after discharge.
Thus it seems that the prevention of the occurrence of painful crisis and the early aggressive treatment to abort a painful crisis could prevent or minimize the complications consequent to poorly managed painful crises. Approaches that may achieve this goal will be presented.