Ocular manifestations in children and adolescents with sickle cell disease attending a Pediatric Hematology Unit in Damanhur Teaching Hospital

2020 ◽  
Vol 33 (2) ◽  
pp. 88
Author(s):  
SaadS Abo-Zied ◽  
Hosam-EldinM Elgemaey ◽  
HalaM Abd-Aal
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Cell Disease ◽  
Pediatric Hematology ◽  
Ocular Manifestations

Understanding Provider Barriers to Hydroxyurea Use for Pediatric Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 255-255
Author(s):  
Suzette O. Oyeku ◽  
Nancy S. Green ◽  
Farzana Pashankar ◽  
Patricia Giardina ◽  
Craig A. Mullen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Young Children ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Phase Iii ◽  
Evidence Based ◽  
Cell Disease ◽  
Pediatric Hematology ◽  
Clinical Indications ◽  
Provider Barriers

Abstract Abstract 255 Despite proven efficacy in clinical trials, hydroxyurea (HU) has not been uniformly adopted into the care of children with sickle cell disease (SCD). In 2008, the NIH Consensus Development Conference on Hydroxyurea Treatment for Sickle Cell Disease postulated that barriers to HU use may occur at the provider level. Limited evidence exists on barriers to the use of HU, and prior studies have largely focused on use in adults. HU use is rapidly expanding to different indications, to use in patients with less common hemoglobin genotypes and to young children. Initial data from the Pediatric Hydroxyurea Phase III Trial (BABY HUG) in very young children (ages 9–18 months) is also now becoming available. To better understand current provider barriers to effective translation of efficacy trial results into “real-world” clinical care of children with SCD, we surveyed pediatric hematology providers within several regional consortiums of pediatric hematology programs in the eastern US. The objectives of our study were to: 1) describe practice patterns related to HU use among providers of children and adolescents with SCD; 2) identify provider level barriers to HU use among SCD children; and 3) solicit provider recommendations to overcome the perceived barriers. A close-ended, self-administered web-based survey was sent to 230 pediatric hematology providers in June 2010. Provider demographics, practice characteristics, clinical indications to prescribe HU, concerns related to HU use and suggestions to improve HU use were assessed by this survey. Forty-two percent (N=97) of 230 surveys were completed by hematologists (84%), nurse practitioners (12%) and physician assistants (3.7%). The number of SCD patients in provider practices ranged from 2 to 1,200 patients. 57% of respondents were female. 42% of respondents were in practice for more than 20 years. The majority (72%) of providers were white. Many providers (83%) were somewhat/very familiar with the NHLBI guidelines about HU use in SCD. Among those surveyed, the most frequent indications to start HU were: 1) history of 3 painful episodes, 2) acute chest syndrome, 3) chronic pain use requiring narcotics, 4) priapism and 5) symptomatic anemia. A majority of providers (82%) reported using HU in children ages 3–5 years of age, with 41% of providers indicated using HU in children less than 3 years of age. Fewer than half of providers (28%) prescribe HU to patients with Hgb SC or other Hgb S variants. Only 74% of providers attempted to titrate HU to maximal tolerated dose. This goal dose ranged from 20 to 40mg/kg/day among our respondents. Major provider concerns about HU in children are: 1) patient compliance with taking HU, 2) compliance with attending drug monitoring visits, 3) compliance with taking contraception, 4) effects of HU on fertility and 5) long term side effects. Almost 50% of clinicians were concerned about the age of the patient when starting HU: 48.5% of clinicians considered patients less than 1 years of age too young to start HU, while 40% of clinicians felt patients' ages 1–2 years were too young. Some providers (39%) had concerns about the efficacy of HU in patients with Hgb SC, while 24.1% were concerned about efficacy in patients with Hgb S variants. Providers' suggestions to improve HU use included: 1) developing updated evidence based practice guidelines for HU use (89%), 2) developing culturally appropriate patient educational materials about HU (84%), 3) extending FDA approval for HU to children (80%), and 4) developing a national registry of patients on HU to monitor clinical outcomes and adverse events (74%). Our survey highlights that HU use varies among pediatric providers with respect to: 1) the broader clinical indications for HU use, 2) optimal maximal tolerated dose of HU, 3) appropriate lower age limit to prescribe HU, and 4) sickle cell genotype in which to use HU. Updated national evidence- based guidelines to assist clinicians in using HU in pediatric sickle cell care are indicated given the efficacy of HU for SCD over a wide range of indications, the logistical limits and tempo of clinical studies, the paucity of other widely available treatments, and persistent barriers to HU use at the provider level. Additional studies are warranted to examine alternative indications for HU, HU use in younger ages, optimum dosing, potential impact on fertility, teratogenicity and possible carcinogenicity, and use of HU for other sickle cell genotypes. Disclosures: Off Label Use: Hydroxyurea has not been FDA approved for use in children and adolescents with sickle cell disease, the topic of the submitted abstract.

Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease

2006 ◽  
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Childhood Cancer ◽  
Children And Adolescents ◽  
Peer Relationships ◽  
Sickle Cell ◽  
Cell Disease ◽  
Life Issues ◽  
Art Research

Over recent decades, tremendous advances in the prevention, medical treatment, and quality of life issues in children and adolescents surviving cancer have spawned a host of research on pediatric psychosocial oncology. This important volume fulfills the clear need for an up-to-date, comprehensive handbook for practitioners that delineates the most recent research in the field--the first of its kind in over a decade. Over 60 renowned authors have been assembled to provide a thorough presentation of the state-of-the art research and literature, with topics including: -Neuropsychological effects of chemotherapy and radiation therapy -Bone marrow transplantation -Important issues about quality of life during and following treatment -Collaborative research among child-focused psychologists -Standards of psychological care for children and adolescents -Stress and coping in the pediatric cancer experience -The role of family and peer relationships The Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease represents both multidisciplinary and international efforts, an alliance between physicians and parents, and a combination of research and service. With a wealth of information of great interest to patients and their families, this volume will also be a welcome resource to the psychologists, psychiatrists, pediatricians, oncologists, nurses, and social workers who confront these issues as they help children and their families through the treatment, recovery, and grieving processes.

scholarly journals Predictors of musculoskeletal manifestations in paediatric patients presenting with sickle cell disease at a tertiary teaching hospital in Lusaka, Zambia

2020 ◽  
Vol 1 (6) ◽  
pp. 175-181
Author(s):  
Raymond Mpanjilwa Musowoya ◽  
Patrick Kaonga ◽  
Alick Bwanga ◽  
Catherine Chunda-Lyoka ◽  
Christopher Lavy ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Clinical Manifestations ◽  
Classification Systems ◽  
University Teaching ◽  
Cell Disease ◽  
Tertiary Teaching ◽  
Musculoskeletal Manifestations

Aims Sickle cell disease (SCD) is an autosomal recessive inherited condition that presents with a number of clinical manifestations that include musculoskeletal manifestations (MM). MM may present differently in different individuals and settings and the predictors are not well known. Herein, we aimed at determining the predictors of MM in patients with SCD at the University Teaching Hospital, Lusaka, Zambia. Methods An unmatched case-control study was conducted between January and May 2019 in children below the age of 16 years. In all, 57 cases and 114 controls were obtained by systematic sampling method. A structured questionnaire was used to collect data. The different MM were identified, staged, and classified according to the Standard Orthopaedic Classification Systems using radiological and laboratory investigations. The data was entered in Epidata version 3.1 and exported to STATA 15 for analysis. Multiple logistic regression was used to determine predictors and predictive margins were used to determine the probability of MM. Results The cases were older median age 9.5 (interquartile range (IQR) 7 to 12) years compared to controls 7 (IQR 4 to 11) years; p = 0.003. After multivariate logistic regression, increase in age (adjusted odds ratio (AOR) = 1.2, 95% confidence interval (CI) 1.04 to 1.45; p = 0.043), increase in the frequency of vaso-occlusive crisis (VOC) (AOR = 1.3, 95% CI 1.09 to 1.52; p = 0.009) and increase in percentage of haemoglobin S (HbS) (AOR = 1.18, 95% CI 1.09 to 1.29; p < 0.001) were significant predictors of MM. Predictive margins showed that for a 16-year-old the average probability of having MM would be 51 percentage points higher than that of a two-year-old. Conclusion Increase in age, frequency of VOC, and an increase in the percentage of HbS were significant predictors of MM. These predictors maybe useful to clinicians in determining children who are at risk. Cite this article: Bone Joint Open 2020;1-6:175–181.

Bioelectrical impedance analysis of the body composition of children and adolescents with sickle cell disease

2002 ◽  
Vol 140 (6) ◽  
pp. 681-687 ◽  
Author(s):  
Dorothy J. VanderJagt ◽  
Paul Harmatz ◽  
Ajovi B. Scott-Emuakpor ◽  
Elliot Vichinsky ◽  
Robert H. Glew
Keyword(s):  
Body Composition ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Bioelectrical Impedance Analysis ◽  
Bioelectrical Impedance ◽  
Impedance Analysis ◽  
The Body ◽  
Cell Disease
Sign in / Sign up

Export Citation Format

Share Document