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Sickle cell pain management

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https://www.readbyqxmd.com/read/28512745/implementation-of-multi-disciplinary-care-reduces-maternal-mortality-in-women-with-sickle-cell-disease-living-in-low-resource-setting
#1
Eugenia Vicky Asare, Edeghonghon Olayemi, Theodore Boafor, Yvonne Dei-Adomakoh, Enoch Mensah, Harriet Ghansah, Yvonne Osei-Bonsu, Selina Crabbe, Latif Musah, Charles Hayfron-Benjamin, Brittany Covert, Adetola A Kassim, Andra James, Mark Rodeghier, Michael R DeBaun, Samuel A Oppong
Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic...
May 16, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28507969/core-decompression-with-synthetic-grafting-as-a-joint-preservation-strategy-in-humeral-avascular-necrosis-due-to-sickle-cell-anemia-a-case-report
#2
Andrew M Steffensmeier, Karen Kirkham, John M Wiemann
INTRODUCTION: Avascular necrosis (AVN) of the femoral or humeral heads in patients with sickle cell anemia is a common and painful condition. There is currently no gold standard treatment protocol for this condition. Typically, the pain is managed with narcotics and activity restriction until there has been collapse of the subchondral bone with a degree of arthrosis sufficient to warrant total joint arthroplasty. This method entails prolonged pain for the patient and decreases the ability to function occupationally and recreationally...
November 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28506281/a-telephonic-mindfulness-based-intervention-for-persons-with-sickle-cell-disease-study-protocol-for-a-randomized-controlled-trial
#3
Hants Williams, Susan Silva, Leigh Ann Simmons, Paula Tanabe
BACKGROUND: One of the most difficult symptoms for persons with sickle cell disease (SCD) to manage is chronic pain. Chronic pain impacts approximately one-third of persons with SCD and is associated with increased pain intensity, pain behavior, and frequency and duration of hospital visits. A promising category of nonpharmacological interventions for managing both physical and affective components of pain are mindfulness-based interventions (MBIs). METHODS/DESIGN: The primary aim of this study is to conduct a randomized controlled study to evaluate the acceptability and feasibility, as well as to determine the preliminary efficacy, of a telephonic MBI for adults with SCD who have chronic pain...
May 15, 2017: Trials
https://www.readbyqxmd.com/read/28486096/key-pharmacogenomic-considerations-for-sickle-cell-disease-patients
#4
Alexandra Kolliopoulou, Apostolos Stratopoulos, Stavroula Siamoglou, Argyro Sgourou, Adamantia Papachatzopoulou, Theodora Katsila, George P Patrinos
Sickle cell disease (SCD), although a monogenic disease, exhibits a complex clinical phenotype that hampers optimum patient stratification and disease management, especially on hydroxyurea treatment. Moreover, theranostics, the combination of diagnostics to individualize and optimize therapeutic interventions, has not been firmly on the forefront of SCD research and clinical management to date. We suggest that if tailor-made theranostics in SCD is envisaged, pharmacogenomics is anticipated to be the way forward...
May 9, 2017: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/28484512/developing-new-pharmacotherapeutic-approaches-to-treating-sickle-cell-disease
#5
Marilyn J Telen
Survival for patients with SCD has been prolonged by improvements in supportive care, including vaccinations, antibiotic prophylaxis, and overall medical management, including tra nsfusion. However, there remains only one approved, partially effective drug for sickle cell disease-hydroxyurea (hydroxycarbamide). The world desperately needs better ways of both treating and preventing the recurrent painful vaso-occlusive episodes pathognomonic of sickle cell disease as well as the end-organ damage that still leads inexorably to severely shortened life expectancies throughout the world...
February 2017: ISBT Science Series
https://www.readbyqxmd.com/read/28470719/from-total-blood-exchange-to-erythrocytapheresis-and-back-to-treat-complications-of-sickle-cell-disease
#6
Samir K Ballas
Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient...
May 3, 2017: Transfusion
https://www.readbyqxmd.com/read/28460533/a-multidisciplinary-approach-to-impact-acute-care-utilization-in-sickle-cell-disease
#7
Rhea E Powell, Paris B Lovett, Albert Crawford, John McAna, David Axelrod, Lawrence Ward, Dianne Pulte
Sickle cell disease (SCD), an inherited red blood cell disorder, is characterized by anemia, end-organ damage, unpredictable episodes of pain, and early mortality. Emergency department (ED) visits and hospitalizations are frequent, leading to increased burden on patients and increased health care costs. This study assessed the effects of a multidisciplinary care team intervention on acute care utilization among adults with SCD. The multidisciplinary care team intervention included monthly team meetings and development of individualized care plans...
April 1, 2017: American Journal of Medical Quality: the Official Journal of the American College of Medical Quality
https://www.readbyqxmd.com/read/28455727/perioperative-considerations-for-patients-with-sickle-cell-disease-a-narrative-review
#8
Narjeet Khurmi, Andrew Gorlin, Lopa Misra
PURPOSE: Approximately 200,000 individuals worldwide are born annually with sickle cell disease (SCD). Regions with the highest rates of SCD include Africa, the Mediterranean, and Asia, where its prevalence is estimated to be 2-6% of the population. An estimated 70,000-100,000 people in the United States have SCD. Due to enhanced newborn screening, a better understanding of this disease, and more aggressive therapy, many sickle cell patients survive into their adult years and present more frequently for surgery...
April 28, 2017: Canadian Journal of Anaesthesia, Journal Canadien D'anesthésie
https://www.readbyqxmd.com/read/28450986/-major-sickle-cell-syndromes-and-infections-associated-with-this-condition-in-children-in-burkina-faso
#9
Sonia Douamba, Kisito Nagalo, Laure Tamini, Ismaël Traoré, Madibèlè Kam, Fla Kouéta, Diarra Yé
INTRODUCTION: This study aims to investigate infections in children with major sickle cell syndrome. METHODS: We conducted a monocentric descriptive retrospective hospital study in Ouagadougou, Burkina Faso, over a ten-year period. All children with major sickle cell syndrome (homozygous SS and double heterozygous SC, SDPunjab, Sβ thalassemic, SOArab and SE) hospitalized for microbiologically confirmed infections were enrolled in the study. RESULTS: One hundred and thirty-three patients met our inclusion criteria...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28432164/use-of-gabapentin-in-the-treatment-of-chronic-pain-in-an-adolescent-with-sickle-cell-disease
#10
Cátia R Correia, Ana Teresa Soares, Laura Azurara, Maria João Palaré
Vaso-occlusive crises are the most common manifestation of sickle cell disease (SCD) and the main cause of hospital admission in these patients. There is emerging evidence that vaso-occlusive pain has both nociceptive and neuropathic components. However, the treatment of SCD-related pain with neuropathic drugs has not yet been systematically studied, particularly in children. We describe a 14-year-old girl with SCD and multiple hospital admissions for pain management for severe acute vaso-occlusive pain episodes...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28405543/imaging-of-renal-medullary-carcinoma
#11
REVIEW
Federico Greco, Eliodoro Faiella, Domiziana Santucci, Carlo Augusto Mallio, Marco Nezzo, Carlo Cosimo Quattrocchi, Bruno Beomonte Zobel, Rosario Francesco Grasso
Renal medullary carcinoma (RMC) is a rare, highly aggressive tumor recognized as an independent pathological entity. African-descent adolescents and young adults with sickle cell hemoglobinopathy are the most affected groups. This rare subtype of renal cell carcinoma has its own morphogenetic and pathological characteristics. The major clinical manifestations include gross hematuria, abdominal or flank pain, and weight loss. The prognosis is very poor, with 95% of cases diagnosed at an advanced stage of the disease...
2017: Journal of Kidney Cancer and VHL
https://www.readbyqxmd.com/read/28375958/evidence-based-practice-standard-care-for-acute-pain-management-in-adults-with-sickle-cell-disease-in-an-urgent-care-center
#12
Sunghee Kim, Ron Brathwaite, Ook Kim
BACKGROUND: Vaso-occlusive episodes (VOEs) with sickle cell disease (SCD) require opioid treatment. Despite evidence to support rapid pain management within 30 minutes, care for these patients does not consistently meet this benchmark. This quality improvement study sought to decrease the first analgesic administration time, increase patient satisfaction, and expedite patient flow. METHODS: A prospective pre-/postevaluation design was used to evaluate outcomes with patients 18 years or older with VOEs in an urgent care (UC) center after implementation of evidence-based practice standard care (EBPSC)...
April 2017: Quality Management in Health Care
https://www.readbyqxmd.com/read/28353009/acute-kidney-injury-in-children-with-sickle-cell-disease-compounding-a-chronic-problem
#13
EDITORIAL
Cherry Mammen, Mei Lin Bissonnette, Douglas G Matsell
In an article recently published in Pediatric Nephrology, Baddam and colleagues discuss the relatively underreported clinical problem of repeated episodes of acute kidney injury (AKI) in children with sickle cell disease (SCD). Their report is a cautionary note about the importance of repeated kidney injury on the background of underlying chronic kidney injury and its potential implications on long-term kidney outcome. In children and adults with SCD, this includes the effects of repeated vaso-occlusive crises and the management of these painful episodes with non-steroidal anti-inflammatory drugs...
March 28, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28341086/recurrent-priapism-gone-wrong-st-elevation-myocardial-infarction-and-cardiogenic-shock-after-penile-corporal-phenylephrine-irrigation
#14
S Tyler Constantine, Anand Gopalsami, Gregg Helland
BACKGROUND: Recurrent priapism secondary to sickle cell trait in an African-American male has been reported in the literature. A common treatment for these low-flow priapism cases is aspiration and injection of the corpus cavernosum with a sympathomimetic agent. We report a rare complication not described previously in the literature of ST-elevation myocardial infarction (STEMI) and cardiogenic shock in a 29-year-old male with sickle cell trait undergoing a routine detumescence procedure...
March 21, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28334590/a-prospective-emergency-department-quality-improvement-project-to-improve-the-treatment-of-vaso-occlusive-crisis-in-sickle-cell-disease-lessons-learned
#15
Paula Tanabe, Caroline E Freiermuth, David M Cline, Susan Silva
BACKGROUND: Guidelines recommend rapid, aggressive management of vaso-occlusive crisis (VOC) for patients with sickle cell disease (SCD). A large prospective research and quality improvement (QI) project was conducted to measure changes in clinical outcomes in two EDs-academic medical centers with emergency medicine residency programs and Level 1 trauma centers-during a 2.5-year time period (October 2011-March 2014). METHODS: A QI team used a Plan-Do-Study-Act approach to modify and implement changes to opioid analgesic protocols for the emergency department (ED) treatment of VOC...
March 2017: Joint Commission Journal on Quality and Patient Safety
https://www.readbyqxmd.com/read/28279592/older-red-cell-units-are-associated-with-an-increased-incidence-of-infection-in-chronically-transfused-adults-with-sickle-cell-disease
#16
Matthew S Karafin, Erica Carpenter, Amy Pan, Pippa Simpson, Joshua J Field
BACKGROUND: In adults with sickle cell disease (SCD), the effects of the red cell storage lesion are not well defined. The objectives of this study were to: (1) describe the distribution of storage ages provided to adults with SCD, and (2) evaluate clinical outcomes associated with storage age. PATIENTS AND METHODS: We performed a retrospective cohort study of adults with SCD managed with prophylactic simple transfusion regimens. Units were universally pre-storage leukocyte reduced and CEK-matched...
February 9, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28192225/a-randomized-trial-of-yoga-for-children-hospitalized-with-sickle-cell-vaso-occlusive-crisis
#17
Karen Moody, Bess Abrahams, Rebecca Baker, Ruth Santizo, Deepa Manwani, Veronica Carullo, Doris Eugenio, Aaron Carroll
CONTEXT: Sickle cell disease (SCD) vaso-occlusive crisis (VOC) remains an important cause of acute pain in pediatrics and the most common SCD complication. Pain management recommendations in SCD include nonpharmacological interventions. Yoga is one nonpharmacological intervention that has been shown to reduce pain in some populations, however, evidence is lacking in children with VOC. OBJECTIVES: The primary objective of this study was to compare the effect of yoga versus an attention control on pain in children with VOC...
February 9, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28159390/sickle-cell-disease
#18
REVIEW
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
January 31, 2017: Lancet
https://www.readbyqxmd.com/read/28143586/socio-demographic-characteristics-and-psychosocial-consequences-of-sickle-cell-disease-the-case-of-patients-in-a-public-hospital-in-ghana
#19
Vincent A Adzika, Franklin N Glozah, Desmond Ayim-Aboagye, Collins S K Ahorlu
BACKGROUND: Sickle cell disease (SCD) is of major public health concern globally, with majority of patients living in Africa. Despite its relevance, there is a dearth of research to determine the socio-demographic distribution and psychosocial impact of SCD in Ghana. The objective of this study was to examine the socio-demographic distribution and psychosocial consequences of SCD among patients in Ghana and to assess their quality of life and coping mechanisms. METHODS: A cross-sectional research design was used that involved the completion of questionnaires on socio-demographic characteristics, quality of life, coping mechanisms, anxiety and depression...
January 31, 2017: Journal of Health, Population, and Nutrition
https://www.readbyqxmd.com/read/28133359/myonecrosis-in-sickle-cell-anemia-case-study
#20
Lalita Prabha Turaga, Prajwal Boddu, Steve Kipferl, Anupam Basu, Martin Yorath
BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. CASE REPORT We present a case study of a patient in sickle cell crisis with an episode of acute pain and swelling to the intrinsic muscles of the foot as a prominent feature of the crises. Although muscle biopsy is considered the gold standard for the diagnosis of myositis or myonecrosis, a low intensity signal on T1 and high intensity signal on T2 at the affected muscle belly can be as conclusive as imaging studies...
January 30, 2017: American Journal of Case Reports
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