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Sickle cell pain management

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
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
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
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
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
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
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...
January 26, 2018: Journal of General Internal Medicine
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
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
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
Julian D Ford, Damion J Grasso, Sasia Jones, Teresa Works, Biree Andemariam
Almost half of sickle cell disease (SCD) patients develop chronic, debilitating physical pain with uncertain genesis for which they primarily receive opiate-based palliative treatment. Psychological trauma exposure, especially interpersonal victimization, has been linked to the perception of pain in several medical diseases, but has yet to be examined in SCD patients. This study examines self-reported chronicity of pain and use of prescribed opiates in 50 adult SCD patients with and without a history of interpersonal violence exposure...
February 1, 2017: Journal of Interpersonal Violence
Shilpa Jain, Nitya Bakshi, Lakshmanan Krishnamurti
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
Deepika S Darbari, Amanda M Brandow
Pain is a complex multidimensional experience and the most common morbidity in patients with sickle cell disease (SCD). Tools to assess pain can be of use not only to guide pain treatment but also to provide insight into underlying pain neurobiology. Mechanisms of pain in SCD are multifactorial and are not completely elucidated. Although vaso-occlusion of microcirculation by sickled red cells is believed to be the underlying mechanism of acute vaso-occlusive pain, mechanisms for chronic pain and the transition from acute to chronic pain are under investigation...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Paul Telfer, Banu Kaya
The pathophysiology, clinical presentation, and natural history of acute pain in sickle cell disease are unique and require a disease-centered approach that also applies general principles of acute and chronic pain management. The majority of acute pain episodes are managed at home without the need to access health care. The long-term consequences of poorly treated acute pain include chronic pain, adverse effects of chronic opioid usage, psychological maladjustment, poor quality of life, and excessive health care utilization...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Jeffrey A Glassberg
Pain is the leading cause of emergency department (ED) visits for individuals living with sickle cell disease (SCD). The care that is delivered in the ED is often cited by patients with SCD as the area of health care in greatest need of improvement. In 2014, the National Heart, Lung, and Blood Institute released guidelines for the care of SCD, including recommendations for the management of acute sickle cell pain in the ED. These guidelines provide a framework to understand the elements of ideal emergency sickle cell pain care; however, they do not provide guidance on barriers and facilitators to achieving these ideals in the complex system of the ED...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Joshua J Field
Chronic pain affects one-half of adults with sickle cell disease (SCD). Despite the prevalence of chronic pain, few studies have been performed to determine the best practices for this patient population. Although the pathophysiology of chronic pain in SCD may be different from other chronic pain syndromes, many of the guidelines outlined in the pain literature and elsewhere are applicable; some were consensus-adopted in the 2014 National Heart, Lung, and Blood Institute SCD Guidelines. Recommended practices, such as controlled substance agreements and monitoring of urine, may seem unnecessary or counterproductive to hematologists...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Tonya M Palermo, Joanne Dudeney, James P Santanelli, Alexie Carletti, William T Zempsky
Pain is a clinical hallmark of sickle cell disease (SCD), and is rarely optimally managed. Cognitive-behavioral therapy (CBT) for pain has been effectively delivered through the Internet in other pediatric populations. We tested feasibility and acceptability of an Internet-delivered CBT intervention in 25 adolescents with SCD (64% female, mean age=14.8 y) and their parents randomized to Internet CBT (n=15) or Internet Pain Education (n=10). Participants completed pretreatment/posttreatment measures. Eight dyads completed semistructured interviews to evaluate treatment acceptability...
March 2018: Journal of Pediatric Hematology/oncology
Kelsey Smith, Laura Reinman, Jeffrey Schatz, Carla W Roberts
Pain episodes occur for many preschoolers with sickle cell disease (SCD), but little is known about parent perceptions of managing pain episodes in young children. We surveyed parents of young children with SCD who had managed pain episodes in the past year to assess their management and satisfaction with their strategies, challenges of pain management, and interest in additional education. Parents were recruited from health maintenance visits at a SCD specialty clinic. Forty-two of 51 parents (82%) of 2- to-6-year-olds reported managing pain over the past year...
January 2018: Journal of Pediatric Oncology Nursing: Official Journal of the Association of Pediatric Oncology Nurses
Paris B Lovett, Harsh P Sule, Bernard L Lopez
Acute painful episodes are the most common reason for emergency department visits among patients with sickle cell disease (SCD). Early and aggressive pain management is a priority. Emergency providers (EPs) must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis. EPs should be aware of cognitive biases that may misdirect the diagnostic process. Administration of intravenous fluids should be used judiciously. Blood transfusion may be considered. Coordination of care with hematology is an important part of the effective emergency department and long-term management of patients with SCD...
December 2017: Hematology/oncology Clinics of North America
Alexandra M Psihogios, Lauren C Daniel, Reem Tarazi, Kim Smith-Whitley, Chavis A Patterson, Lamia P Barakat
Background: Informed by the Pediatric Self-Management Model, the present study tested relationships between parent and family functioning, sickle cell disease (SCD) self-management, and health outcomes for children with SCD. Method: 83 children with SCD and a parent completed baseline data as part of a larger investigation of a family-based, problem-solving intervention for children with SCD (M age = 8.47). Youth and parents completed a measure of child health-related quality of life (HRQOL), and parents completed measures of family efficacy, parenting stress, and SCD self-management...
September 26, 2017: Journal of Pediatric Psychology
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