Current issues in sickle cell pain and its management.

Pain is the insignia of sickle cell disease and the acute painful crisis is the number-one cause of hospital admissions. Tissue damage due to vaso-occlusion releases numerous inflammatory mediators that initiate the transmission of painful stimuli and the perception of pain. The acute sickle cell painful crisis evolves along four distinct phases coupled with changes in certain markers of the disease. Hospital readmission within 1 week occurs in about 16% of discharged patients. Failure to treat acute pain aggressively may lead to chronic pain syndrome. Management of sickle pain is primarily pharmacologic in nature, and opioids are the analgesics used most often. Cellular and molecular mechanisms of opioids explain individual differences among patients and justify the use of individualized treatment plans.

[1]  Vascular occlusion in sickle cell disease: current concepts and unanswered questions. , 1991, Blood.

[2]  S. Kaasa,et al.  Genetic variability and clinical efficacy of morphine , 2005, Acta anaesthesiologica Scandinavica.

[3]  M. Cousins.  Acute Pain and the Injury Response: Immediate and Prolonged Effects , 1989, Regional Anesthesia & Pain Medicine.

[4]  W. Weiner,et al.  Large cerebral vessel disease in sickle cell anaemia. , 1976, Journal of neurology, neurosurgery, and psychiatry.

[5]  S. Ballas,et al.  Red blood cell changes during the evolution of the sickle cell painful crisis. , 1992, Blood.

[6]  Eufemia Jacob,et al.  Are there phases to the vaso-occlusive painful episode in sickle cell disease? , 2005, Journal of pain and symptom management.

[7]  S. Ballas,et al.  Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance , 2005, American journal of hematology.

[8]  P. Lane.  Sickle cell disease. , 1996, Pediatric clinics of North America.

[9]  G. Serjeant.  The emerging understanding of sickle cell disease , 2001, British journal of haematology.

[10]  N. Mohandas,et al.  Sickle Red Cell Microrheology and Sickle Blood Rheology , 2004, Microcirculation.

[11]  T. McDonald,et al.  Post-operative pain management. , 2011, The Cochrane database of systematic reviews.

[12]  D. Weatherall,et al.  Disorders of Hemoglobin: THE MOLECULAR, CELLULAR, AND GENETIC BASIS OF HEMOGLOBIN DISORDERS , 2009 .

[13]  J. Stuart,et al.  Rheological changes in the prodromal and established phases of sickle cell vaso‐occlusive crisis , 1992, British journal of haematology.

[14]  I. Sora,et al.  The mu opiate receptor as a candidate gene for pain: polymorphisms, variations in expression, nociception, and opiate responses. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[15]  S. Mercadante,et al.  Hyperalgesia: an emerging iatrogenic syndrome. , 2003, Journal of pain and symptom management.

[16]  D. Geschwind,et al.  Association between human μ‐opioid receptor gene polymorphism, pain tolerance, and opioid addiction , 2003, American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics.

[17]  S. Kaasa,et al.  The 118 A > G polymorphism in the human µ‐opioid receptor gene may increase morphine requirements in patients with pain caused by malignant disease , 2004, Acta anaesthesiologica Scandinavica.

[18]  K. Shen,et al.  Ultra-low concentrations of naloxone selectively antagonize excitatory effects of morphine on sensory neurons, thereby increasing its antinociceptive potency and attenuating tolerance/dependence during chronic cotreatment. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[19]  A. Gitter,et al.  Ulnar nerve entrapment at the elbow: correlation of magnetic resonance imaging, clinical, electrodiagnostic, and intraoperative findings. , 1996, Neurosurgery.

[20]  G. Reed,et al.  Opioid selection during sickle cell pain crisis and its impact on the development of acute chest syndrome , 2005, Pediatric blood & cancer.

[21]  G. Lutty,et al.  Morphine Exaggerates Retinopathy in Transgenic Sickle Mice. , 2005 .

[22]  S. Ballas,et al.  The sickle cell painful crisis in adults: phases and objective signs. , 1995, Hemoglobin.

[23]  W. Winkelmüller,et al.  Long-term effects of continuous intrathecal opioid treatment in chronic pain of nonmalignant etiology. , 1996, Journal of neurosurgery.

[24]  Hilla Peretz,et al.  Ju n 20 03 Schrödinger ’ s Cat : The rules of engagement , 2003 .

[25]  J. Loeser,et al.  Bonica's Management of Pain , 2001 .

[26]  C. Pegelow,et al.  The natural history of stroke in sickle cell disease. , 1978, The American journal of medicine.

[27]  Rm,et al.  Sickle cell disease , 1996 .

[28]  R. Hebbel,et al.  Amendment history : Erratum ( September 2000 ) Hypoxia / reoxygenation causes inflammatory response in transgenic sickle mice but not in normal mice , 2018 .

[29]  Kenneth A. Holroyd,et al.  The hierarchical factor structure of the coping strategies inventory , 1989, Cognitive Therapy and Research.

[30]  M. Rao.  Acute post operative pain , 2006 .

[31]  S. Higgins,et al.  The validity of the reinstatement model of craving and relapse to drug use , 2003, Psychopharmacology.

[32]  D. Powars.  Sickle cell anemia and major organ failure. , 1990, Hemoglobin.

[33]  D. Horn,et al.  Bloodstream infections in hospitalized adults with sickle cell disease: A retrospective analysis , 2006, American journal of hematology.

[34]  B. Pace.  Renaissance of sickle cell disease research in the genome era , 2007 .

[35]  U. Alon,et al.  [Prostaglandins and the kidney]. , 1986, Harefuah.

[36]  G. Koren,et al.  Systemic Exposure to Morphine and the Risk of Acute Chest Syndrome in Sickle Cell Disease , 2003, Clinical pharmacology and therapeutics.

[37]  R. Hebbel,et al.  Morphine Induces Kidney Injury in Transgenic Sickle Cell Mice. , 2005 .

[38]  L. Benjamin,et al.  Pain in Sickle Cell Disease: A Multidimensional Construct , 2007 .

[39]  M. Rukstalis,et al.  Principles of Addiction Medicine , 1999 .

[40]  J. Zakrzewska,et al.  Wall and Melzack's textbook of pain , 2006 .

[41]  J. Beyer,et al.  A chronology of pain and comfort in children with sickle cell disease. , 1999, Archives of pediatrics & adolescent medicine.

[42]  M. Fabry,et al.  Sickle cell vaso-occlusion. , 1991, Hematology/oncology clinics of North America.

[43]  J. Haddox,et al.  Opioid pseudoaddiction — an iatrogenic syndrome , 1989, Pain.

[44]  J. Scott,et al.  The effect of magnesium on length of stay for pediatric sickle cell pain crisis. , 2004, Academic emergency medicine : official journal of the Society for Academic Emergency Medicine.

[45]  O. Platt,et al.  Sickle cell anemia as an inflammatory disease. , 2000, The Journal of clinical investigation.

[46]  Low Frequency of Meperidine-Associated Seizures in Sickle Cell Disease , 1999, Clinical pediatrics.

[47]  C. Miaskowski,et al.  Changes in intensity, location, and quality of vaso-occlusive pain in children with sickle cell disease , 2003, Pain.

[48]  R. Hebbel.  Beyond hemoglobin polymerization: the red blood cell membrane and sickle disease pathophysiology. , 1991, Blood.