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Sickle cell pain management

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https://www.readbyqxmd.com/read/29791238/low-dose-ketamine-infusion-for-adjunct-management-during-vaso-occlusive-episodes-in-adults-with-sickle-cell-disease-a-case-series
#1
Nicole Palm, Catherine Floroff, Tanna B Hassig, Alice Boylan, Julie Kanter
The optimal management of recurrent painful episodes in individuals living with sickle cell disease (SCD) remains unclear. Currently, the primary treatment for these episodes remains supportive, using fluids and intravenous opioid and anti-inflammatory medications. Few reports have described the use of adjunct subanesthetic doses of ketamine to opioids for treatment of refractory pain in SCD. This article reports a retrospective case series of five patients admitted to the intensive care unit (ICU) with prolonged vaso-occlusive episodes (VOEs)...
May 23, 2018: Journal of Pain & Palliative Care Pharmacotherapy
https://www.readbyqxmd.com/read/29778312/biomarker-signatures-of-sickle-cell-disease-severity
#2
Mengtian Du, Sarah Van Ness, Victor Gordeuk, Sayed M Nouraie, Sergei Nekhai, Mark Gladwin, Martin H Steinberg, Paola Sebastiani
Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster analysis to discover 17 signatures of 17 common circulating biomarkers in 2320 participants of the Cooperative Study of Sickle Cell Disease, and evaluated the association of these signatures with risk for stroke, pain, leg ulceration, acute chest syndrome, avascular necrosis, seizure, death, and trend of fetal hemoglobin and hemolysis using longitudinally collected data...
May 16, 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29755709/perioperative-management-of-sickle-cell-disease
#3
REVIEW
Kwame Ofori Adjepong, Folashade Otegbeye, Yaw Amoateng Adjepong
Over 30 million people worldwide have sickle cell disease (SCD). Emergent and non-emergent surgical procedures in SCD have been associated with relatively increased risks of peri-operative mortality, vaso-occlusive (painful) crisis, acute chest syndrome, post-operative infections, congestive heart failure, cerebrovascular accident and acute kidney injury. Pre-operative assessment must include a careful review of the patient's known crisis triggers, baseline hematologic profile, usual transfusion requirements, pre-existing organ dysfunction and opioid use...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29749702/management-of-severe-chronic-pain-with-methadone-in-pediatric-patients-with-sickle-cell-disease
#4
Zachary LeBlanc, Chris Vance, Jason Payne, Jie Zhang, Lee Hilliard, Jeffrey D Lebensburger, Thomas H Howard
Vasocclusive pain crises are common among pediatric patients with sickle cell disease (SCD). Some patients with repeated pain crises develop chronic pain. We performed a retrospective cohort study of pediatric patients with SCD with chronic pain treated with methadone. We identified a significant reduction in pain hospitalizations following methadone treatment (0.35 ± 0.19 vs. 0.19 ± 0.17 hospitalizations/month, P = 0.016). In addition, we did not observe overt organ toxicity nor symptoms of opioid withdrawal during methadone wean...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29737522/interventions-for-improving-adherence-to-iron-chelation-therapy-in-people-with-sickle-cell-disease-or-thalassaemia
#5
REVIEW
Patricia M Fortin, Sheila A Fisher, Karen V Madgwick, Marialena Trivella, Sally Hopewell, Carolyn Doree, Lise J Estcourt
BACKGROUND: Regularly transfused people with sickle cell disease (SCD) and people with thalassaemia (who are transfusion-dependent or non-transfusion-dependent) are at risk of iron overload. Iron overload can lead to iron toxicity in vulnerable organs such as the heart, liver and endocrine glands; which can be prevented and treated with iron chelating agents. The intensive demands and uncomfortable side effects of therapy can have a negative impact on daily activities and well-being, which may affect adherence...
May 8, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29729787/key-components-of-pain-management-for-children-and-adults-with-sickle-cell-disease
#6
REVIEW
Amanda M Brandow, Michael R DeBaun
Sickle cell disease pain manifests as severe acute pain episodes and a debilitating chronic pain syndrome. Acute pain episodes are the most common reason for health care use; however, acute pain episodes are also frequently managed at home. Chronic pain syndrome develops in 30% to 40% of individuals with sickle cell disease, with an increasing incidence and severity with age. We review the critical aspects of pain management that are integral to the comprehensive approach to sickle cell disease pain and are rooted in the biopsychosocial model...
June 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29693797/mobile-health-intervention-for-youth-with-sickle-cell-disease-impact-on-adherence-disease-knowledge-and-quality-of-life
#7
Lindsay M Anderson, Sarah Leonard, Jude Jonassaint, Joseph Lunyera, Melanie Bonner, Nirmish Shah
BACKGROUND: Adherence to illness self-management among youth with sickle cell disease (SCD) positively impacts health outcomes and decreases overall healthcare costs. Despite this, children with SCD face several barriers to adherence, with adherence rates that remain moderate to low. The current feasibility study examined the Intensive Training Program (ITP), a mobile health (mHealth) intervention for youth with SCD designed to promote disease knowledge, adherence, and patient-provider communication...
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29620434/transient-receptor-potential-polymorphism-and-haplotype-associate-with-crisis-pain-in-sickle-cell-disease
#8
Ellie H Jhun, Xiaoyu Hu, Nilanjana Sadhu, Yingwei Yao, Ying He, Diana J Wilkie, Robert E Molokie, Zaijie J Wang
AIM: Episodes of acute pain crisis contribute to considerable morbidity and mortality in sickle cell disease (SCD). Incomprehensive understanding of the underlying pain heterogeneity results in inadequate pain management. The transient receptor potential (TRP) family of voltage-gated ion channels acts as sensory transducers of diverse noxious stimuli. We performed an association study of polymorphisms in candidate genes TRPV1 and TRPA1 with pain in SCD patients. METHODS: Utilization rate, in other words, number of emergency department/acute care center admissions over 12 months as a result of pain crisis, served as a marker for acute pain...
April 5, 2018: Pharmacogenomics
https://www.readbyqxmd.com/read/29614627/sickle-cell-disease-classification-of-clinical-complications-and-approaches-to-preventive-and-therapeutic-management
#9
Samir K Ballas
Sickle cell disease (SCD) is an inherited disorder of hemoglobin structure that has no established cure in adult patients. Cure has been achieved in selected children with sickle cell anemia (SCA) using allogeneic bone marrow transplantation or cord blood transplantation. SCD is essentially a triumvirate of (1) pain syndromes, (2) anemia and its sequelae and (3) organ failure, including infection. Pain, however, is the hallmark of SCD and dominates its clinical picture throughout the life of the patients. The prevalence of these complications varies with age from infancy through adult life...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29578946/the-bivalent-ligand-mcc22-potently-attenuates-nociception-in-a-murine-model-of-sickle-cell-disease
#10
Giuseppe Cataldo, Mary M Lunzer, Julie K Olson, Eyup Akgün, John D Belcher, Gregory M Vercellotti, Philip S Portoghese, Donald A Simone
Sickle cell disease (SCD) is a chronic inflammatory disorder accompanied by chronic pain. In addition to ongoing pain and hyperalgesia, vaso-occlusive crises-induced pain can be chronic or episodic. Since analgesics typically used to treat pain are not very effective in SCD, opioids, including morphine, are a primary treatment for managing pain in SCD but are associated with many serious side effects, including constipation, tolerance, addiction, and respiratory depression. Thus, there is a need for the development of novel treatments for pain in SCD...
March 22, 2018: Pain
https://www.readbyqxmd.com/read/29545351/endothelin-type-a-receptors-mediate-pain-in-a-mouse-model-of-sickle-cell-disease
#11
Brianna Marie Lutz, Shaogen Wu, Xiyao Gu, Fidelis E Atianjoh, Zhen Li, Brandon M Fox, David M Pollock, Yuan-Xiang Tao
Sickle cell disease is associated with acute painful episodes and chronic intractable pain. Endothelin-1, a known pain inducer, is elevated in the blood plasma of both sickle cell patients and mouse models of sickle cell disease. We show here that the levels of endothelin-1 and its endothelin type A receptor are increased in the dorsal root ganglia of a mouse model of sickle cell disease. Pharmacologic inhibition or neuron-specific knockdown of endothelin type A receptors in primary sensory neurons of dorsal root ganglion alleviated basal and post-hypoxia evoked pain hypersensitivities in sickle cell mice...
March 15, 2018: Haematologica
https://www.readbyqxmd.com/read/29528178/iliopsoas-hematoma-in-a-patient-with-sickle-cell-disease
#12
Benjamin Zielonka, Alan R Cohen, Kim Smith-Whitley, Bhavya S Doshi
Although musculoskeletal pain in patients with sickle cell disease (SCD) is most frequently the result of vaso-occlusive episodes, clinicians often consider other etiologies including osteomyelitis, avascular necrosis, and trauma. In this study, we report the case of a young female with SCD with hip and back pain secondary to a nontraumatic iliopsoas periosteal hematoma with evidence of adjacent bone infarction. The pathophysiology, diagnostic considerations, and management of periosteal hematomas in SCD are reviewed...
March 12, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29512881/parent-pain-catastrophizing-predicts-child-depressive-symptoms-in-youth-with-sickle-cell-disease
#13
Alana Goldstein-Leever, Lindsey L Cohen, Carlton Dampier, Soumitri Sil
BACKGROUND: Youth with sickle cell disease (SCD) are at risk for recurrent pain and depressive symptoms, both of which contribute to poorer health outcomes. Furthermore, youth and family coping with child pain, including pain catastrophizing, is known to be associated with poorer psychosocial adjustment and greater functional disability among youth with SCD. In particular, child catastrophizing about pain and parent catastrophizing about their child's pain have been linked to increased pain and depressive symptoms in youth with chronic pain conditions...
March 7, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29484190/a-rare-case-of-avascular-necrosis-in-sickle-cell-trait-a-case-report
#14
William J Sanders
Background: Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29446825/phytomedicines-medicines-derived-from-plants-for-sickle-cell-disease
#15
REVIEW
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29382114/utility-of-non-invasive-monitoring-of-cardiac-output-and-cerebral-oximetry-during-pain-management-of-children-with-sickle-cell-disease-in-the-pediatric-emergency-department
#16
Pradeep Padmanabhan, Chikelue Oragwu, Bibhuti Das, John A Myers, Ashok Raj
Pain crisis in children with sickle cell disease (SCD) is typically managed with intravenous fluids and parenteral opioids in the pediatric emergency department. Electrical cardiometry (EC) can be utilized to measure cardiac output (CO) and cardiac index (CI) non-invasively. Near-infrared spectroscopy (NIRS) measuring cerebral (rCO₂) and splanchnic regional (rSO₂) mixed venous oxygenation non-invasively has been utilized for monitoring children with SCD. We studied the value and correlation of NIRS and EC in monitoring hemodynamic status in children with SCD during pain crisis...
January 29, 2018: Children
https://www.readbyqxmd.com/read/29374357/do-words-matter-stigmatizing-language-and-the-transmission-of-bias-in-the-medical-record
#17
Anna P Goddu, Katie J O'Conor, Sophie Lanzkron, Mustapha O Saheed, Somnath Saha, Monica E Peek, Carlton Haywood, Mary Catherine Beach
BACKGROUND: Clinician bias contributes to healthcare disparities, and the language used to describe a patient may reflect that bias. Although medical records are an integral method of communicating about patients, no studies have evaluated patient records as a means of transmitting bias from one clinician to another. OBJECTIVE: To assess whether stigmatizing language written in a patient medical record is associated with a subsequent physician-in-training's attitudes towards the patient and clinical decision-making...
May 2018: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/29351102/fatal-sickling-triggered-by-massive-foreign-particle-embolism-a-case-report-of-unrecognized-indwelling-venous-catheter-drug-abuse-in-sickle-cell-disease
#18
Christopher A Febres-Aldana, Lydia Hernandez Howard
Sickle cell disease (SCD) manifests itself with vaso-occlusive episodes leading to infarction. Placement of intravascular catheters provides a useful route for management of pain crises as well as other complications. However, catheter misuse is a commonly unrecognized problem, which can have lethal consequences. We present a case of fatal splenic sequestration/hyperhemolysis secondary to foreign body pulmonary and systemic embolization due to intravenous administration of hydromorphone pills in a young woman with SCD...
January 18, 2018: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/29321025/diagnostic-challenges-of-prolonged-post-treatment-clearance-of-plasmodium-nucleic-acids-in-a-pre-transplant-autosplenectomized-patient-with-sickle-cell-disease
#19
Paul M Luethy, Sean C Murphy, Annette M Seilie, Yingda L Xie, Chuen-Yen Lau, John F Tisdale, Matthew M Hsieh, Jessica L Reinhardt, Anna F Lau, Gary A Fahle
BACKGROUND: Autosplenectomy, as a result of sickle cell disease, is an important risk factor for severe malaria. While molecular methods are helpful in providing rapid and accurate infection detection and species identification, the effect of hyposplenism on result interpretation during the course of infection should be carefully considered. CASE PRESENTATION: A 32-year old autosplenectomized Nigerian male with severe sickle cell disease was referred to the National Institutes of Health for allogenic hematopoietic stem cell transplant...
January 10, 2018: Malaria Journal
https://www.readbyqxmd.com/read/29316596/van-neck-odelberg-disease-a-3-5-year-follow-up-case-report-and-systematic-review
#20
Patrick J Mixa, Frank A Segreto, Hiram Luigi-Martinez, Bassel G Diebo, Qais Naziri, Srinivas Kolla, Aditya V Maheshwari
V an Neck-Odelberg disease (VND) is a benign skeletal overgrowth of the ischiopubic synchondrosis (IPS) in prepubescent patients. There is a paucity of long-term follow-up data and reviews on management decision-making. We report on a 15-year-old female, with a history of sickle-cell disease (HbSS), presenting with unilateral groin pain. Patient's physical examination, radiographs, and a literature-review determined a diagnosis of VND. Conservative treatment was issued. Clinical symptoms resolved at three months, followed by complete lesion resolution at three years...
December 12, 2017: Surgical Technology International
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