scholarly journals Impact of education on the knowledge and skills of parents of children with sickle cell disease

2017 ◽  
Vol 5 (1) ◽  
pp. 209
Author(s):  
Purnima Yadav ◽  
Jayant Vagha
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parental Education ◽  
Signs And Symptoms ◽  
Poor Quality ◽  
Cell Disease ◽  
Splenic Enlargement ◽  
Post Test ◽  
Treat All ◽  
Sickle Cell Crises

Background: Sickle cell disease is a haemoglobinopathy that affects millions throughout the world. It leads to poor quality of the life and increased mortality in children and young adults. In Vidarbha, SCD is common and it is pathetic to see children in morbid state dying due to some or the other crisis. Parents must be educated about the nature of the disease, earliest signs of crises and seek help, treat all febrile illnesses promptly. We contemplated that the key to success in the management of such children is parental education and this prompted us to undertake the present study.Methods: It was interventional study, done in Pediatric SCD clinic done in 2 years. Parent’s preexisting knowledge and awareness about sickle cell disease was assessed with the help of questionnaire and OSCE. They were then educated with the help of educational module comprising of pamphlets in Marathi, pictures, and also trained in clinical examination of their child to detect pallor, fever, jaundice, respiratory distress, pulse and splenic enlargement. After intervention posttest and OSCE was conducted again.Results: The pre-existing knowledge about inheritance, signs and symptoms of sickle cell anemia was high, but parents showed lack in skills of assessment of crises. These skills improved significantly after intervention. There was statistically significant (p<0.05) difference between average pretest and post test scores of each skill tested.Conclusions: Modular teaching using OSCE helps in improving skills of parents for early detection of sickle cell crises.

scholarly journals Sickle Cell Anemia Presenting with Vaso-Occlusive Pain: Should We Screen for COVID-19?

2021 ◽  
pp. 1-4
Author(s):  
Mohammad Ali ◽  
Lina Okar ◽  
Nabil E. Omar ◽  
Jabeed Parengal ◽  
Ashraf Soliman ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Signs And Symptoms ◽  
Risk Groups ◽  
Position Statement ◽  
Interesting Case ◽  
International Federation ◽  
Cell Disease ◽  
The World

Despite the widespread of coronavirus disease-19 (CO­VID-19) infection around the world, there are very scarce reported literature about the care of patients with a known diagnosis of hemoglobin disorders such as sickle cell disease (SCD) or thalassemia and confirmed COVID-19 infection. Thalassemia International Federation issued a position statement to include patients with thalassemia and SCD among the high-risk groups of patients. Here, we present an interesting case of a 42-year-old patient know to have SCD presenting with Vaso-occlusive (VOC) pain episode in the absence of COVID-19 signs and symptoms, who tested positive for COVID-19 infection and had a smooth recovery. This case highlights the importance of screening SCD patients presenting with VOC-related events even in the absence of COVID-19 signs and symptoms.

Sickle cell crisis as evaluated from measurements of hydroxybutyrate dehydrogenase and myoglobin in plasma.

1981 ◽  
Vol 27 (2) ◽  
pp. 314-316 ◽  
Author(s):  
E F Roth ◽  
P A Bardfeld ◽  
S J Goldsmith ◽  
E Radel ◽  
J C Williams
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
High Specificity ◽  
Mean Value ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
The Mean ◽  
Low Sensitivity ◽  
Sickle Cell Crises

Abstract Data on plasma hydroxybutyrate dehydrogenase activity (I) and myoglobin concentration were used to evaluate painful sickle cell crises. I was increased during non-crisis steady state in patients with sickle cell disease as compared to normal values (232, SD 79.7 vs 85, SD 33 Sigma units/mL). During crisis, the mean value for I increased further to 379 (SD 139) Sigma units/mL. For 12 patients evaluated both during steady state and crisis, there was a mean increase in plasma I of 131% (SD 76%). Repeated determinations of I in sickle cell disease patients during several months while they were in steady state showed that baseline I varied by no more than 20% from the mean. Plasma myoglobin in patients with sickle cell disease was not above normal, but during crisis 21 of 39 patients tested had increased plasma myoglobin concentrations. Our data suggest that I may be a useful indicator of sickle cell crisis when the patient's own baseline value is available for comparison. Plasma myoglobin measurements give evidence of muscle damage during crisis with high specificity but low sensitivity.

Learning Not to Judge a Book By Its Cover: A Case Series of Malignancy Masquerading As Common Presentations of Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4872-4872 ◽  
Author(s):  
Ahmar Urooj Zaidi ◽  
Lo'Rell Martin ◽  
Michael U Callaghan ◽  
Sharada A. Sarnaik ◽  
Jeffrey W Taub
Keyword(s):  
Cancer Therapy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Hodgkin’S Lymphoma ◽  
Fetal Hemoglobin ◽  
Signs And Symptoms ◽  
Hodgkin's Lymphoma ◽  
Additional Patient ◽  
Cell Disease

Abstract Background: Sickle cell disease affects 100,000 people in the United States. Cancer does not appear to be more common in individuals with sickle cell disease however many presenting signs and symptoms of cancer overlap with signs and symptoms of sickle cell disease, complicating diagnosis. Furthermore, sickle cell disease can complicate surgical, chemo- and radio- therapies for malignancy. Methods: We reviewed the past 46 years experience of cancers in children with sickle cell at a large urban pediatric referral center. We identified 5 cases and reviewed the presentations, treatments, response to therapy, sickle cell complication and laboratory values, including transfusion needs and effect of chemotherapy on fetal hemoglobin levels. Results: We identified five cases of malignancies in children with sickle cell disease including cases of acute lymphoblastic leukemia (ALL), Hodgkin's lymphoma, Non-Hodgkin's lymphoma (NHL), Wilms tumor and renal cell carcinoma. An additional patient with sickle cell trait was diagnosed with metastatic renal medullary carcinoma. The patients were ages 6-18 years old at diagnosis and 4 were female. All had delays in diagnosis as their initial signs and symptoms overlapped with sickle cell disease (abdominal mass mimicking splenomegaly, low blood counts, fever and posterior reversible encephalopathy syndrome, hematuria). All five achieved complete remission with multi-modality therapy (excluding stem cell transplants) and are currently doing well 1- 20 years after treatment. Admissions for pain were reduced during and for some period after cancer therapy and 2 patients had marked, and in one case, prolonged elevation of fetal hemoglobin after therapy. Discussion: Children with sickle cell develop cancers that can be confused for sickle cell related complications. Cancer therapy can be well tolerated and successful in children with sickle cell. Sickle cell complications are decreased during cancer therapy likely secondary to frequent therapy related transfusion and therapy induced increases in fetal hemoglobin. Disclosures Callaghan: Biogen: Honoraria; Bayer: Honoraria; CSL Behring: Honoraria; Baxalta: Honoraria, Research Funding; Grifols: Honoraria; Roche: Honoraria, Research Funding.

Chronic tonsillitis, tonsillectomy and sickle cell crises

1987 ◽  
Vol 101 (5) ◽  
pp. 467-470 ◽  
Author(s):  
G. T. A. Ijaduola ◽  
O. O. Akinyanju
Keyword(s):  
Sickle Cell Disease ◽  
General Anaesthesia ◽  
Sickle Cell ◽  
Chronic Tonsillitis ◽  
Cell Disease ◽  
Pain Crisis ◽  
The Mean ◽  
One Year ◽  
The One ◽  
Sickle Cell Crises

AbstractA study of the effect of tonsillectomy on the frequency of sickle cell pain crises was carried out on 15 patients with sickle cell disease (Hb SS) who presented with complaints of frequent pain crises and were found to have chronic tonsillitis. They comprised 9 females and 6 males and ranged in age from 6 to 35 years, with a mean of 15 years. Tonsillectomy was performed under general anaesthesia. The mean number of pain crises in the one-year period after tonsillectomy was 1.5 and was significantly less than the mean number 4.7 in the one year preceding the operation (p<0.001). Four patients failed to show a reduced number of crises and these were those whose tonsils at operation showed no pus in their tonsillar crypts. It is thus observed that chronic tonsillitis, symptoms of which may not be volunteered at examination, is a potent inductor of sickle cell pain crisis and that tonsillectomy is an effective mode of treatment, especially when the tonsillar crypts contain pus.

scholarly journals Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course

Blood ◽  
1997 ◽  
Vol 89 (5) ◽  
pp. 1787-1792 ◽  
Author(s):  
Elliott P. Vichinsky ◽  
Lori A. Styles ◽  
Linda H. Colangelo ◽  
Elizabeth C. Wright ◽  
Oswaldo Castro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Young Children ◽  
Sickle Cell ◽  
Lower Lobe ◽  
Signs And Symptoms ◽  
Therapeutic Interventions ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Laboratory Findings ◽  
Striking Effect

Acute chest syndrome (ACS) is an important cause of morbidity and mortality in sickle cell disease (SCD). Previous studies reported conflicting pictures of ACS making therapeutic interventions difficult. The Cooperative Study of Sickle Cell Disease prospectively followed 3,751 patients enrolled from birth to 66 years of age for ACS. Data on presenting signs and symptoms, laboratory findings, and hospital course were collected. There were 1,722 ACS episodes in 939 patients. Young children (age 2 to 4 years) presented with fever and cough, a negative physical exam, and rarely had pain. Adults were often afebrile and complained of shortness of breath, chills, and severe pain. Upper lobe disease was more common in children; multilobe and lower lobe disease affected adults more often. Severe hypoxia occurred in 18% of adults tested and could not be predicted by examination or laboratory findings. Bacteremia was documented in 3.5% of episodes, but was strongly influenced by age (14% of infants and 1.8% of patients <10 years). ACS was most common in winter with children having the most striking increase. Transfusion was used less frequently, but earlier in children. Young children were hospitalized for 5.4 days versus 9 days for adults. Fifty percent of adults had a pain event in the 2 weeks preceding ACS and children were more likely to have febrile events. The death rate was four times higher in adults than in children. Fatal cases generally developed rapid pulmonary failure and one third were associated with bacteremia. Age has a striking effect on the clinical picture of ACS. In children, ACS was milder and more likely due to infection, whereas in adults ACS was severe, associated with pain and had a higher mortality rate.

scholarly journals Sickle Cell Education for Medical Professionals: A Pre and Post Test Analysis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5812-5812
Author(s):  
Diana D Simmons ◽  
Robert Lucia ◽  
Kay Linn Saving ◽  
Nicole Alwan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Test Scores ◽  
Conflicts Of Interest ◽  
Learning System ◽  
Medical Professionals ◽  
Cell Disease ◽  
Learning Module ◽  
Significant Difference ◽  
Post Test

Abstract Background and Objectives: Sickle cell disease is a severe inherited form of anemia caused by a genetic mutation. Polymerization of hemoglobin leads to a cascade of effects decreasing blood flow. This causes tissue hypoxia leading to acute and chronic damage to the organs and endothelial lining. This disease requires complex management that relies on comprehensive training and knowledge regarding the disease process. Often accurate knowledge of sickle cell disease and how to provide appropriate care in the general medical population is limited. The purpose of this project was to develop a sickle cell educational training module for medical professionals. Such a module could be used to guide the provision of accurate education regarding sickle cell disease and best practice when caring for this patient population. Methods: Goals and learning objectives were created and current medical literature about caring for sickle cell disease was reviewed. A comprehensive PowerPoint presentation was produced along with a provider tip sheet and a pre and posttest. The presentation, tip sheet, and tests were reviewed by a board certified pediatric hematologist/oncologist along with the hospital's educational review committee in the Department of Professional Regulation. Once approved, the PowerPoint, tip sheet, and tests were combined into a learning module and uploaded onto an online learning system utilized by the hospital system. The module was sent to over 2,400 outpatient providers and staff and to all inpatient staff on units where sickle cell patients stay when admitted. The module consisted of the participant completing a 10 question pretest, then reviewing the PowerPoint presentation and tip sheet. Following the review of the PowerPoint and tip sheet, the participant completed a 10 question posttest and completed an evaluation of the module. Analysis: There were 223 people who completed the Sickle Cell Disease Learning Module. A paired t-test was conducted to compare pre-test scores to post-test scores. There was a significant difference in the pre-test scores (M = 5.98, SD = 1.66) and post-test scores (M = 9.17, SD = 1.36); p = <0.0001. Conclusion: The goal of this module was to increase baseline medical knowledge of sickle cell disease. The results indicate there was statistically significant improvement in baseline knowledge, based on pre and post data (p = <0.0001). While the results indicate statistically significant increases in performance, it would be important to see if improvements are sustained over time. Reassessment of participants one year after completion of module can be beneficial to see if learned knowledge has been retained. Disclosures No relevant conflicts of interest to declare.

Sickle Cell Disease

2017 ◽  
Author(s):  
Andrew Franklin
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Complex Disease ◽  
Perioperative Period ◽  
Multiple Organ ◽  
Acute Chest Syndrome ◽  
Globin Genes ◽  
Cell Disease ◽  
Organ Systems ◽  
Sickle Cell Crises

Sickle cell disease, a hemoglobinopathy that affects multiple organ systems, is a complex disease entity that presents unique challenges during the perioperative period. The hallmark of sickle cell disease, vaso-occlusion, results from sickling of erythrocytes containing hemoglobin of abnormal conformation due to genetically mutated beta globin genes. The perioperative clinician must properly care for acute sickle cell crises including acute painful episodes and acute chest syndrome, and safely care for the sickle cell patient through the preoperative, intraoperative, and postoperative phases of surgical treatment. Both acute painful episodes and acute chest syndrome result from vaso-occlusive crises, and early stabilization of these emergencies is crucial to ensuring a positive patient outcome. The singular perioperative objective for the care of sickle cell disease patients is both simple and daunting: to achieve physiologic homeostasis in patients with preexisting multiorgan dysfunction undergoing a series of physiologic insults during and after surgery.

scholarly journals Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course

Blood ◽  
1997 ◽  
Vol 89 (5) ◽  
pp. 1787-1792 ◽  
Author(s):  
Elliott P. Vichinsky ◽  
Lori A. Styles ◽  
Linda H. Colangelo ◽  
Elizabeth C. Wright ◽  
Oswaldo Castro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Young Children ◽  
Sickle Cell ◽  
Lower Lobe ◽  
Signs And Symptoms ◽  
Therapeutic Interventions ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Laboratory Findings ◽  
Striking Effect

Abstract Acute chest syndrome (ACS) is an important cause of morbidity and mortality in sickle cell disease (SCD). Previous studies reported conflicting pictures of ACS making therapeutic interventions difficult. The Cooperative Study of Sickle Cell Disease prospectively followed 3,751 patients enrolled from birth to 66 years of age for ACS. Data on presenting signs and symptoms, laboratory findings, and hospital course were collected. There were 1,722 ACS episodes in 939 patients. Young children (age 2 to 4 years) presented with fever and cough, a negative physical exam, and rarely had pain. Adults were often afebrile and complained of shortness of breath, chills, and severe pain. Upper lobe disease was more common in children; multilobe and lower lobe disease affected adults more often. Severe hypoxia occurred in 18% of adults tested and could not be predicted by examination or laboratory findings. Bacteremia was documented in 3.5% of episodes, but was strongly influenced by age (14% of infants and 1.8% of patients <10 years). ACS was most common in winter with children having the most striking increase. Transfusion was used less frequently, but earlier in children. Young children were hospitalized for 5.4 days versus 9 days for adults. Fifty percent of adults had a pain event in the 2 weeks preceding ACS and children were more likely to have febrile events. The death rate was four times higher in adults than in children. Fatal cases generally developed rapid pulmonary failure and one third were associated with bacteremia. Age has a striking effect on the clinical picture of ACS. In children, ACS was milder and more likely due to infection, whereas in adults ACS was severe, associated with pain and had a higher mortality rate.

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