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Sickle cell pain management

Shannon Kelly, Keith Quirolo, Anne Marsh, Lynne Neumayr, Alicia Garcia, Brian Custer
BACKGROUND: Chronic red blood cell (RBC) transfusion therapy (CTT) is an integral component of the management of severe sickle cell disease (SCD) and can prevent complications, such as stroke. RBC units can be administered via simple transfusion or exchange transfusion, and erythrocytapheresis (automated RBC exchange transfusion [aRBX]), is increasingly used for CTT. Comparisons of simple and aRBX transfusions are limited, and the current scope of aRBX use is not known. STUDY DESIGN AND METHODS: We administered a survey to define current transfusion practices for CTT and performed a review of the erythrocytapheresis literature...
October 19, 2016: Transfusion
Jin Han, Shubha Bhat, Michel Gowhari, Victor R Gordeuk, Santosh L Saraf
Ambulatory care clinical pharmacy services have expanded beyond primary care settings, but literature supporting the benefits of clinical pharmacy involvement with patients who have rare diseases such as sickle cell disease (SCD) is lacking. Hydroxyurea is the only agent approved by the U.S. Food and Drug Administration for the treatment of SCD; full benefit in controlling pain episodes and other complications is achieved through monitored escalation to a maximum tolerated dose. The primary objective of this analysis was to evaluate the impact of a newly implemented clinical pharmacy service on the management of patients with SCD...
September 16, 2016: Pharmacotherapy
Khuthala Mnika, Gift D Pule, Collet Dandara, Ambroise Wonkam
Sickle cell disease (SCD) is a blood disease caused by a single nucleotide substitution (T > A) in the beta globin gene on chromosome 11. The single point mutation (Glu6Val) promotes polymerization of hemoglobin S (HbS) and causes sickling of erythrocytes. Vaso-occlusive painful crises are associated with recurrent and long-term use of analgesics/opioids and hydroxyurea (HU) by people living with SCD. The present analysis offers a state-of-the-art expert review of the effectiveness of pharmacogenomics/genetics of pain management in SCD, with specific focus on HU and opioids...
October 2016: Omics: a Journal of Integrative Biology
Daphnée Michelet, Juliette Andreu-Gallien, Alia Skhiri, Arnaud Bonnard, Yves Nivoche, Souhayl Dahmani
BACKGROUND AND AIMS: Laparoscopic pediatric surgery allows a rapid postoperative rehabilitation and hospital discharge. However, the optimal postoperative pain management preserving advantages of this surgical technique remains to be determined. This study aimed to identify factors affecting the postoperative recovery of bowel function after laparoscopic surgery in children. MATERIAL AND METHODS: A retrospective analysis of factors affecting recovery of bowel function in children and infants undergoing laparoscopic surgery between January 1, 2009 and September 30, 2009, was performed...
July 2016: Journal of Anaesthesiology, Clinical Pharmacology
Carly S Gardner, Daniel T Boll, Priya Bhosale, Tracy A Jaffe
Sickle cell disease (SCD) is the most prevalent hemoglobinopathy. Survival in patients with SCD has improved over the past few decades. These patients experience a lifetime of repeated acute pain crises, which are thought to result from sickling and microvascular occlusions; acute abdominal pain is common. Moreover, repeated crises often lead to organ dysfunction, such as asplenia, hepatic failure, and renal failure. The spleen, liver, biliary system, kidneys, and gastrointestinal tract can all be affected...
September 7, 2016: Abdominal Radiology
Zan A Naseer, Malick Bachabi, Lynne C Jones, Robert S Sterling, Harpal S Khanuja
Osteonecrosis is one of the most devastating musculoskeletal manifestations of sickle cell disease and most commonly affects the femoral head. Although the exact pathophysiology of this condition in patients with sickle cell disease is unknown, it is suggested that red cell sickling and repetitive vaso-occlusion may be associated with tissue hypoxia, inflammation, and subsequent bone necrosis and collapse. If left untreated, osteonecrosis can be extremely debilitating and may lead to severe pain, loss of function, and degenerative joint changes...
September 2016: Southern Medical Journal
Christian T Evensen, Marsha J Treadwell, San Keller, Roger Levine, Kathryn L Hassell, Ellen M Werner, Wally R Smith
Documented deficiencies in adult sickle cell disease (SCD) care include poor access to knowledgeable providers and inadequate treatment in emergency departments (EDs).The aim of this study was to create patient-reported outcome measures of the quality of ambulatory and ED care for adults with SCD.We developed and pilot tested SCD quality of care questions consistent with Consumer Assessments of Healthcare Providers and Systems surveys. We applied psychometric methods to develop scores and evaluate reliability and validity...
August 2016: Medicine (Baltimore)
Erica Simon, Brit Long, Alex Koyfman
BACKGROUND: Sickle cell disease (SCD) affects approximately 100,000 individuals in the United States. Due to alterations in the structural conformation of hemoglobin molecules under deoxygenated conditions, patients with SCD are predisposed to numerous sequelae, many of which require acute intervention. OBJECTIVE: Our aim was to provide emergency physicians with an evidence-based update regarding the diagnosis and management of SCD complications. DISCUSSION: SCD patients experience significant morbidity and mortality secondary to cerebrovascular accident, acute chest syndrome, acute vaso-occlusive pain crises, SCD-related multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration, priapism, and renal disease...
October 2016: Journal of Emergency Medicine
Jianxun Lei, Barbara Benson, Huy Tran, Solomon F Ofori-Acquah, Kalpna Gupta
Pain is a hallmark feature of sickle cell anemia (SCA) but management of chronic as well as acute pain remains a major challenge. Mouse models of SCA are essential to examine the mechanisms of pain and develop novel therapeutics. To facilitate this effort, we compared humanized homozygous BERK and Townes sickle mice for the effect of gender and age on pain behaviors. Similar to previously characterized BERK sickle mice, Townes sickle mice show more mechanical, thermal, and deep tissue hyperalgesia with increasing age...
2016: PloS One
B Randall Brenn, Dinesh K Choudhry, Karen Sacks, Sandra Como-Fluehr, Robert Strain
OBJECTIVE: Despite increased focus on pediatric pain, uncontrolled pain is still a problem for hospitalized pediatric inpatients. A program was designed to find patients with uncontrolled pain and develop a framework to oversee their pain management. This report details the development of a pain stewardship program with data from the first year of its activity. METHODS: Hospitalized inpatients in a tertiary care pediatric center in the mid-Atlantic region were included in the study...
September 2016: Hospital Pediatrics
Jin Han, Santosh L Saraf, Xu Zhang, Michel Gowhari, Robert E Molokie, Johara Hassan, Chaher Alhandalous, Shivi Jain, Jewel Younge, Taimur Abbasi, Roberto F Machado, Victor R Gordeuk
Pain, the hallmark complication of sickle cell disease (SCD), is largely managed with opioid analgesics in the United States, but comprehensive data regarding the long-term use of opioids in this patient population is lacking. The pain medication prescription records from a cohort of 203 SCD patients were analyzed. Twenty-five percent were not prescribed opioid medications while 47% took only short-acting opioids, 1% took only long-acting opioids, and 27% took a combination of short-acting and long-acting opioids...
July 28, 2016: American Journal of Hematology
Xinxian Zhang, Chenglong Li, Qiancheng Li
Sickle cell disease is the result of altered genetic make up due to hereditary encounter and its form as homozygous sickle cell anemia is the most common and severe. The disease is characterized by chronic anemia, recurrent pain crises and vascular occlusion. Neurologically, there is a high incidence of stroke in childhood, as well as cognitive dysfunction. Newborn screening programmes and preventative treatments have allowed a much longer lifespan. However, recently, neurological research has shifted to characterizing more subtle aspects of brain development and functioning that may be critically important to the individual's quality of life...
August 2016: Experimental and Therapeutic Medicine
Jennifer M Allen, Danielle M Graef, Jennifer H Ehrentraut, Brooklee L Tynes, Valerie M Crabtree
BACKGROUND: Sleep disruption is a common comorbidity of pediatric pain. Consequences of pain and disrupted sleep, evidence for the pain-sleep relation, and how aspects of illness, treatment, and pharmacological pain management may contribute to or exacerbate these issues are presented. AIMS: This conceptual review explored the relation between pain and sleep in children diagnosed with chronic medical or developmental conditions. The goal of this review is to expand upon the literature by examining common themes in sleep disturbances associated with painful conditions across multiple pediatric illnesses...
July 15, 2016: CNS Neuroscience & Therapeutics
Pablo Bartolucci, Anoosha Habibi, Mehdi Khellaf, Françoise Roudot-Thoraval, Giovanna Melica, Anne-Sophie Lascaux, Stéphane Moutereau, Sylvain Loric, Orianne Wagner-Ballon, Jugurtha Berkenou, Aline Santin, Marc Michel, Bertrand Renaud, Yves Lévy, Frédéric Galactéros, Bertrand Godeau
BACKGROUND: Vaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cause of patients' Emergency-Room admissions and hospitalizations. Acute chest syndrome (ACS), a life-threatening complication, can occur during VOC, be fatal and prolong hospitalization. No predictive factor identifies VOC patients who will develop secondary ACS. METHODS: This prospective, monocenter, observational study on SS/S-β0thalassemia SCD adults aimed to identify parameters predicting ACS at Emergency-Department arrival...
August 2016: EBioMedicine
Michael R DeBaun, Robert C Strunk
Acute chest syndrome is a frequent cause of acute lung disease in children with sickle-cell disease. Asthma is common in children with sickle-cell disease and is associated with increased incidence of vaso-occlusive pain events, acute chest syndrome episodes, and earlier death. Risk factors for asthma exacerbation and an acute chest syndrome episode are similar, and both can present with shortness of breath, chest pain, cough, and wheezing. Despite overlapping risk factors and symptoms, an acute exacerbation of asthma or an episode of acute chest syndrome are two distinct entities that need disease-specific management strategies...
June 18, 2016: Lancet
Anoosha Habibi, Armand Mekontso-Dessap, Constance Guillaud, Marc Michel, Keyvan Razazi, Mehdi Khellaf, Btissam Chami, Dora Bachir, Claire Rieux, Giovanna Melica, Bertrand Godeau, Frédéric Galacteros, Pablo Bartolucci, France Pirenne
Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle-cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3-22] days after the triggering transfusion (TT). The most frequent DHTR-related clinical manifestation was dark urine/hemoglobinuria (94%). Most patients (89%) had a painful vaso-occlusive crisis and 50% developed a secondary acute chest syndrome (ACS)...
October 2016: American Journal of Hematology
Lewis L Hsu, Nancy S Green, E Donnell Ivy, Cindy E Neunert, Arlene Smaldone, Shirley Johnson, Sheila Castillo, Amparo Castillo, Trevor Thompson, Kisha Hampton, John J Strouse, Rosalyn Stewart, TaLana Hughes, Sonja Banks, Kim Smith-Whitley, Allison King, Mary Brown, Kwaku Ohene-Frempong, Wally R Smith, Molly Martin
Community health workers are increasingly recognized as useful for improving health care and health outcomes for a variety of chronic conditions. Community health workers can provide social support, navigation of health systems and resources, and lay counseling. Social and cultural alignment of community health workers with the population they serve is an important aspect of community health worker intervention. Although community health worker interventions have been shown to improve patient-centered outcomes in underserved communities, these interventions have not been evaluated with sickle cell disease...
July 2016: American Journal of Preventive Medicine
Coretta M Jenerette, Cheryl A Brewer, Susan Silva, Paula Tanabe
Sickle cell disease (SCD) is a genetic disease associated with both chronic pain and acute painful events referred to as vaso-occlusive crises. Individuals with SCD suffer from a multitude of medical complications in addition to pain. Patients often are stigmatized as "drug-seeking" and receive inadequate pain management. The purpose of this study was to compare clinicians' SCD knowledge and attitudes toward patients with SCD before attending a 2-day conference on SCD (T1) with knowledge and attitudes immediately postconference (T2) and 2 months postconference (T3)...
June 2016: Pain Management Nursing: Official Journal of the American Society of Pain Management Nurses
M Vinícius Ribeiro E Silva, F Tavares Mendonça, R Garcia Dusi
OBJECTIVE: The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. CASE: A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions...
May 27, 2016: Revista Española de Anestesiología y Reanimación
Miriam O Ezenwa, Yingwei Yao, Robert E Molokie, Zaijie Jim Wang, Molly W Mandernach, Marie L Suarez, Diana J Wilkie
To evaluate the pain coping strategies of patients with sickle cell disease (SCD) who experience healthcare injustice from either physicians or nurses during medical visits for pain management. It is unknown how patients' coping with pain relates to their experiences of healthcare injustice from physicians or nurses. This descriptive comparative study included adult outpatients with SCD who completed the PAINReportIt(®), Healthcare Justice Questionnaire(©), and Coping Strategies Questionnaire-SCD. Data were analyzed using independent t tests...
May 23, 2016: Journal of Immigrant and Minority Health
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