scholarly journals Nutrition Access in Adult Sickle Cell Patients: An Exploratory Study

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3472-3472
Author(s):  
Sarah Agamah
Keyword(s):  
Food Insecurity ◽  
Sickle Cell ◽  
Exploratory Study ◽  
Conflicts Of Interest ◽  
Small Sample Size ◽  
Well Being ◽  
Small Sample ◽  
Health Science ◽  
Dimensional Approach ◽  
Waiting Room

Background Sickle Cell Disease (SCD) is the most common lethal genetic blood disease in the world, impacting approximately 100,000 people in the US, and about 25 million people worldwide (WHO, CDC 2019). Some literature refers to the SCD patients' barriers to appropriate care as "biopsychospiritual," indicating the complexity of the disease, and hence, call for a multi-dimensional approach to care (Adegbola et al., 2012). Considering the role of energy, protein and Vitamin D deficiency in SCD patients, a multi-dimensional approach to care that includes nutritional supplementation is worth investigating (Hyacinth, Gee and Hibbert 2010). There is limited research that illuminates the relationship between nutrition, food insecurity and health outcomes of those with SCD. Patients with SCD experiencing challenges of undernutrition are more prone to growth retardation in childhood, disease exacerbation, impaired immune function, and poor ulcer healing among other complications (Mandese et al., 2015). In Chicago, Illinois on the South and West Side where many SCD patients live, it is estimated that 35.1%-57.8% of people are food insecure (Gunderson, Dewey, Crumbaugh,Kato & Engelhard, 2016). This study aims to better understand food insecurity for SCD patients at the University of Illinois Hospital and Health Science Systems (UIHHSS) in Chicago, the largest adult sickle cell center in the Midwest. Physicians are uniquely positioned to address health inequities among their patients when they practice patient centered-care (Law, Leung, Veinot, Miller & Mylopoulos, 2016). Research shows that healthcare providers who are aware of external factors such as food insecurity, can be better advocates for their patients, and help them find ways to gain access to healthy foods (Nesbitt and Palomarez, 2016). Methods An exploratory study involving data collection of patient demographics, food consumption, and ability to access food was conducted. All patients at the Sickle Cell Clinic at UIHHSS who were interested in participating received a full explanation of the purpose of the study.Twenty-seven adult patients (19 female, 8 male) were recruited in the Sickle Cell Center waiting room. Food intake by patients with SCD was documented using the validated Food Insecurity Experience Scale (FIES) from the Food and Agriculture Organization (FAO). Participants self-administered the FIES questionnaire, followed by a one-on-one discussion with the interviewer. Qualitative data gathered from the follow-up interviews was used to investigate themes and provide clarity. Demographic information was collected to supplement the screening tool, as it is not a sufficient stand alone measure. Patient zip codes were referenced with the Distressed Community Index (DCI), a comparative measure of community well-being using census bureau data from 2012-2016. Outcomes Results of the analysis indicated the nutritional status of sickle cell patients and identified barriers to food access especially economic factors. Of the twenty-seven patients interviewed, 40% screened moderate and 22% screened as severe for food insecurity. The remaining 48% screening negative for food insecurity, but 80% of all patients did not consume the daily recommended dietary allowance of vegetables and fruits, and 44% worried about having enough money for food, citing that the cost of healthy food was a barrier to eating healthy. Based on zip code, 80% of patients live in a high DCI zone. Discussion and Limitations This study utilizes convenience sampling with a small sample size, which limits external validity. Conclusions from the data should not be made, but rather used to design larger confirmatory studies. The information could aid in aligning undernourished patients with social support and provide potential insight for clinicians into the management of patients with SCD. Disclosures No relevant conflicts of interest to declare.

Comparison of Quality of Life across Adolescents with Cancer, Sickle Cell Disease and Hemophilia.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 5176-5176
Author(s):  
Mukta Sharma ◽  
Kristin Stegenga ◽  
Gerald Woods
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Small Sample Size ◽  
Well Being ◽  
Small Sample ◽  
The Other ◽  
Oncology Group ◽  
Cell Disease

Abstract The purpose of this study is to assess Quality of Life (AQoL) of adolescents with cancer and compare it to adolescents with other hematological disorders who receive medical care from the Hematology/Oncology Clinic at Children’s Mercy Hospitals and Clinics (CMHC). Children with cancer are surviving longer due to improved medical care, and cancer is recently being recognized as a chronic illness. The other two disease conditions we are comparing the QoL of adolescents with cancer are - sickle cell disease (SCD) and hemophilia. Literature shows evidence that for each disease condition, patients have lower QoL compared to their healthy counter-parts, but there is a void in area of research comparing QoL of adolescents with cancer to other blood diseases like hemophilia and SCD. This project was funded by Children’s Mercy Cancer Center Board. Methods - Subjects between ages of 11 to 17 years with diagnosis of cancer, sickle cell disease or hemophilia that are followed at CMHC were eligible to participate. We mailed out AQoL questionnaires to 100 oncology, 100 sickle cell, and 52 hemophilia adolescents who were identified from database. Data was collected using a Likert-scaled instrument with 25 questions under four major sub-domains - Physical, emotional, social and functional well being. The AQOL scores for each domain represented an average of the Likert scale items with a theoretical minimum of 0 and a theoretical maximum of 4. We defined a difference of 0.5 or greater on any subdomain as having clinical relevance. Results 41 oncology, 29 SCD and 17 hemophilia responses were received back. Demographic data were self-disclosed to describe the sample population. Overall AQoL scores ranged from 0.0 to 4.0 for Phys, 1.0 to 4.0 for Soc, 0.4 to 4.0 for Emotional, and 1.4 to 4.0 for Functional subdomain. For all four measures, the scores were skewed right (clustered towards the upper limit with a long tail extending to the lower values). Only the Physical subdomain showed a borderline effect (p=0.056) with the Hemophilia group showing a larger mean than the other two groups. The remaining subdomains did not exhibit differences large enough to be clinically or statistically significant. Although there were small difference in the Oncology group with gender on the Physical and Emotional scale, these difference were not statistical significant. The differences on the Social and Functional well being scale were very small and within the range of clinical indifference, even after allowing for sampling error. There were no statistical or clinical differences in the Sickle Cell group between boys and girls, but this may be due to the small sample size in this group. No comparison with gender was possible in the hemophilia group. There were no statistical differences in the oncology group between those on or off treatment, but this may also be due to the small sizes in these groups. Conclusion With a few exceptions, the QOL subdomains showed no statistically significant or clinically significant differences between the three disease groups or between the boys and girls within each disease groups. This may be due, in part, to the small sample size of this study. There were two trends with achieved borderline significance: hemophilia patients showed higher AQOL scores on the physical and emotional subdomains.

scholarly journals Optimal Hemoglobin Level for Pediatric Sickle Cell Patients Who Undergo Adenotonsillectomy

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4938-4938 ◽  
Author(s):  
Zhongbo Hu ◽  
Simone Megan Chang ◽  
Ramzi Younis ◽  
Ofelia A. Alvarez
Keyword(s):  
Sickle Cell ◽  
Exchange Transfusion ◽  
Conflicts Of Interest ◽  
Small Sample Size ◽  
Sleep Apnea Syndrome ◽  
Small Sample ◽  
Beta Thalassemia ◽  
Control Group ◽  
Complication Rates ◽  
Obstructive Sleep

Abstract Background:The prevalence of obstructive sleep apnea syndrome (OSA) in children with sickle cell disease (SCD) is higher than in the general pediatric population. Adenotonsillectomy involves significantly increased risks in patients with SCD because of the need for general anesthesia,which could induce a vaso-occlusive event. Various preoperative regimens have been suggested to reduce peri-operative risks for SCD patients. Objective: We retrospectively reviewed the perioperative management for pediatric SCD patients undergoing adenotonsillectomy. We sought to identify the optimal preoperative hemoglobin (Hb) level for patients to have lowest risk of post-operative complications. Methods: Children between 1 and 18 years old with SCD including HbSS, HbSC, HbS beta thalassemia (Sβ0 and Sβ+) who underwent adenotonsillectomy from 2007 to 2017 were identified at Holtz Children's Hospital. Patients without these diseases who underwent the procedures were collected as a control. The study was approved by the University of Miami Institutional Review Board (IRB). De-identified data were exported to GraphPad Prism version 5.02 to perform the statistical analyses. Means were analyzed by student t test, rates by Chi-squared test. p < 0.05 is considered statistically significant. Results: Thirty-four patients with SCD (mean 7.1 yrs) and 145 controls (mean 7.0 yrs) were identified with adenotonsillectomy (see Table 1). SCD patients had significant longer hospital stay (2.6 versus 0.9 days, p< 0.001), and higher postoperative complication rates than the control group (20.6% vs 4.0%, p= 0.02). Most of the sickle cell patients' complications were hypoxemia (5 out of 7), except one supraventricular tachycardia and one headache. Four out of 7 patients with pretransfusion (pretrx) Hb level above 10 g/dL received manual exchange transfusion to keep Hb over 10 g/dL. None had complication or PICU stay. When patients' pretrx Hb levels was 9-10 g/dL, 5/8 received transfusion without significant complications, with the goal of not increasing post transfusion Hb over 11.5 g/dL. Most patients with pretrx Hb level 7-9 g/dL received simple blood transfusion. For patients with preoperative Hb level 9-11.5 g/dL, the PICU transfer rate was significantly lower than ones with Hb <9 or >= 11.5 g/dL (11.5% vs 60%, p=0.04; see Figure 1). Complication rates were also lower in the Hb 9-11.5 g/dL group, but without statistical difference (19.2% vs 40%, p=0.3). Data is limited by small sample size in the SCD group. Conclusion(s): We concluded that for pediatric SCD patients planning for adenotonsillectomy, it is better to keep the posttransfusion Hb level at least above 9 mg/dL but less than 11.5 g/dL before procedure to avoid significant post operational complications. If pretransfusion Hb level is above 9 mg/dL, exchange transfusion by partial phlebotomy or simple transfusion (not to exceed 11.5 g/dL) may be considered. Disclosures No relevant conflicts of interest to declare.

Bereavement Support Groups: Timing of Participation and Reasons for Joining

2001 ◽  
Vol 43 (3) ◽  
pp. 247-258 ◽  
Author(s):  
Cliff Picton ◽  
Brian K. Cooper ◽  
Diana Close ◽  
Jean Tobin
Keyword(s):  
Support Groups ◽  
Emotional Support ◽  
Exploratory Study ◽  
Qualitative Data ◽  
Small Sample Size ◽  
Well Being ◽  
Small Sample ◽  
Future Research ◽  
Bereavement Support ◽  
Target Individual

This exploratory study investigated the experiences of a small group of people who had participated in professionally led bereavement support groups, with particular emphasis on perceptions of the appropriate timing for initial involvement with a group and the reasons for joining. The results support the need for timely group-based bereavement support. The proposition that bereavement support in the early weeks of bereavement is too early was voiced by some participants but strongly negated by others who found early support of major value to their well-being. Taking account of the small sample size, qualitative data suggest that the reasons for joining are similar regardless of the length of time since the death, although there is evidence that the need for emotional support is felt most acutely by those without adequate family support and who join within the first three months after the death. Findings suggest that group interventions are helpful to target individual circumstances, needs, and reactions throughout the process of mourning and offer direction for future research.

An influence of social isolation on the level of physical activity as well as on well-being and mental state of people during the coronavirus COVID-19 pandemic

2019 ◽  
Vol 129 (4) ◽  
pp. 127-131
Author(s):  
Agnieszka Parfin ◽  
Krystian Wdowiak ◽  
Marzena Furtak-Niczyporuk ◽  
Jolanta Herda
Keyword(s):  
Physical Activity ◽  
Social Isolation ◽  
Small Sample Size ◽  
Statistical Tests ◽  
Population Group ◽  
Well Being ◽  
Small Sample ◽  
Subjective Well Being ◽  
Tract Infections ◽  
The Impact

AbstractIntroduction. The COVID-19 is the name of an infectious disease caused by a new strain of coronavirus SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus 2). It was first diagnosed in December 2019 in patients in Wuhan City, Hubei Province, China. The symptoms are dominated by features of respiratory tract infections, in some patients with a very severe course leading to respiratory failure and, in extreme cases to death. Due to the spread of the infection worldwide, the WHO declared a pandemic in March 2020.Aim. An investigation of the impact of social isolation introduced due to the coronavirus pandemic on selected aspects of life. The researchers focused on observing changes in habits related to physical activity and their connections with people’s subjective well-being and emotional state.Material and methods. The study was carried out within the international project of the group „IRG on COVID and exercise”. The research tool was a standardized questionnaire.Results. Based on the data collected and the analysis of the percentage results, it can be observed that the overwhelming majority of people taking up physical activity reported a better mood during the pandemic. However, statistical tests do not confirm these relationships due to the small sample size.Conclusions. Isolation favours physical activity. Future, in-depth studies, by enlarging the population group, are necessary to confirm the above observations.

Fetal Doppler Parameters at Term in Pregnancies Affected by Gestational Diabetes: Role in the Prediction of Perinatal Outcomes

2018 ◽  
Vol 41 (06) ◽  
pp. 675-680 ◽  
Author(s):  
Alessandra Familiari ◽  
Caterina Neri ◽  
Chiara Vassallo ◽  
Giulia Di Marco ◽  
Serafina Garofalo ◽  
...  
Keyword(s):  
Gestational Diabetes ◽  
Apgar Score ◽  
Adverse Outcome ◽  
Small Sample Size ◽  
Perinatal Outcomes ◽  
Expectant Management ◽  
Well Being ◽  
Good Clinical Practice ◽  
Small Sample ◽  
Fetal Doppler

Abstract Objective The timing of delivery for women affected by gestational diabetes (GDM) is still controversial. Good clinical practice often suggests offering induction of labor at term in order to reduce the complications associated with this condition, while recent evidence supports expectant management. Fetal Doppler parameters represent a validated tool for testing fetal well-being at term and can select pregnancies that need increased surveillance. The aim of the present study was to evaluate the role of fetal Doppler parameters at term for the prediction of pregnancy outcomes in patients affected by GDM. Methods Prospective cohort study in a single center. Evaluation of umbilical artery (UA) PI, middle cerebral artery (MCA) PI, cerebroplacental ratio (CPR) and umbilical-to-cerebral ratio (UCR) at > 37 weeks of gestation in singleton, morphologically normal pregnancies affected by GDM, was performed in order to estimate the association between ultrasound measurements at term and perinatal outcome. Regression linear analysis was used to estimate the association between fetal Doppler parameters and neonatal pH, neonatal Apgar score, neonatal weight and a composite adverse outcome. The receiver operating characteristic (ROC) curve was used to estimate the possible predictive value of the above association. Results Our results on 130 women showed MCA PI to be the best predictor of perinatal outcomes in terms of low Apgar score at the 1st minute (p = 0.00), pH (p = 0.02) and composite adverse outcome (p = 0.05). UCR showed a significant correlation with neonatal pH (p = 0.02). No significant correlations for UA PI and CPR MoMs have been demonstrated in our population. However, the small sample size is a limitation of the study. Conclusion Evaluation of MCA Doppler and eventually UCR at term can be a useful tool to discriminate pregnancies affected by GDM that can benefit from IOL before 41 weeks in order to reduce complications related to this condition.

scholarly journals Italian Children’s Well-Being after Lockdown: Predictors of Psychopathological Symptoms in Times of COVID-19

2021 ◽  
Vol 18 (21) ◽  
pp. 11429
Author(s):  
Marcella Caputi ◽  
Barbara Forresi ◽  
Ludovica Giani ◽  
Giovanni Michelini ◽  
Simona Scaini
Keyword(s):  
Small Sample Size ◽  
Well Being ◽  
Small Sample ◽  
Novelty Seeking ◽  
Cross Sectional ◽  
Convenience Sample ◽  
Childhood Experiences ◽  
Adverse Experiences ◽  
Adverse Childhood ◽  
Cross Sectional Design

The first Italian lockdown imposed to fight the spread of COVID-19 caused important disruptions in families’ everyday lives. The main aim of this research was to investigate the predictors of psychopathology in children aged 5–10 years, immediately after the national 2-month lockdown. A total of 158 Italian parents (148 mothers, 10 fathers, mean age = 41 years) were recruited and asked to complete an online research concerning their 158 children (76 boys, mean age = 7.4 years). Parents completed questionnaires on parent–child conflict, resilience, temperament, behavior, and previous adverse childhood experiences. Hierarchical regressions showed that children’s psychopathology was predicted by low child resilience, high novelty seeking and harm avoidance, adverse experiences, and high flooding levels. Moreover, girls exposed to adverse experiences appeared more vulnerable to psychopathology. The recruitment of a convenience sample, the small sample size, and the cross-sectional design of our study limit the generalizability and interpretation of the present findings. Nonetheless, this research extends our knowledge of children’s functioning in such an exceptional period. Shedding light on predictors of children’s psychopathology following prolonged quarantine can indeed guide effective psychological interventions now and in future similar situations.

Extra care housing: exploring motivations, expectations and perceptions

2020 ◽  
Vol 23 (1) ◽  
pp. 15-26
Author(s):  
Michael James Buckland ◽  
Anthea Tinker
Keyword(s):  
Social Life ◽  
Housing Association ◽  
Small Sample Size ◽  
Well Being ◽  
Small Sample ◽  
Structured Interviews ◽  
Content Type ◽  
High Expectations ◽  
Health And Social Care ◽  
Extra Care Housing

Purpose The purpose of this paper is to explore and compare the motivations and expectations that older people have when choosing to move into either a private or housing association (HA) extra care housing (ECH) scheme, and any effects this had on its residents. Design/methodology/approach This qualitative study is based on findings from four HA schemes in Tower Hamlets, London, and one private scheme in Warwickshire. Eight semi-structured interviews were conducted with five women and three men of varying backgrounds, from schemes managed by different associations and companies. Interview transcripts were coded and analysed thematically. Findings All residents moved into ECH in response to deteriorating health. However, almost all residents had felt obliged to move by others, generally their children. Few residents had any expectations of ECH on arrival, but many developed high expectations of an increased sense of independence and security and of an improved social life. ECH appeared to be beneficial for residents’ health and well-being. Research limitations/implications The inability to recruit an equal number of people from HA and private scheme, alongside the small sample size, may compromise the external validity of any conclusions drawn from any comparisons. Practical implications This research identifies a lack of knowledge about ECH among the general population and offers insight into areas of poor management within ECH schemes which could be improved. Social implications Inadequacies in the ECH model could be attributed to failures in the current health and social care system. Differences between expectations and perceptions of HA vs private schemes should be acknowledged and responded to. Originality/value This is a rare example of research exploring the relationships between ECH residents’ motivations and expectations (Hillcoat-Nallétamby and Sardani, 2019), and between those in HA vs private schemes.

The Supervisor’s Perceived Role in Employee Well-Being: Results From Mayo Clinic

2018 ◽  
Vol 33 (2) ◽  
pp. 300-311 ◽  
Author(s):  
Kaisa C. Wieneke ◽  
Karen S. Schaepe ◽  
Jason S. Egginton ◽  
Sarah M. Jenkins ◽  
Nicole C. Block ◽  
...  
Keyword(s):  
Role Model ◽  
Small Sample Size ◽  
Health Care Organization ◽  
Qualitative Interviews ◽  
Well Being ◽  
Small Sample ◽  
Care Organization ◽  
Positive Role ◽  
Worksite Health ◽  
Administrative Units

Purpose: Novel approaches are needed to enhance employee well-being and perhaps supervisors can be an effective agent for worksite health promotion. The aim of this study was to examine the supervisor’s perceived needs, barriers, and role for influencing employee well-being for incorporation into program development. Design: Semistructured, qualitative interviews of supervisors. Setting: Large, integrated academic health-care organization with over 30 000 employees and 2600 supervisors having access to comprehensive well-being programs and a successful well-being champion network comprised of 600 champions. Participants: Twenty supervisors representing clinical, research, and administrative units. Methods: Semistructured, one-on-one interviews were conducted and audio recorded. Analysis included content log development and open coding by a trained analyst to reveal key themes. More formalized content coding using specialized software for qualitative analyses was also conducted. Results: Supervisor responses were wide ranging regarding their perceived and desired role in promoting workplace well-being. Barriers from the supervisor perspective included high current workload, ambivalence about promoting wellness, lack of support from leadership, lack of flexibility and control at work, and difficulty accessing on-site resources. They perceived their potential role in well-being as remaining a positive role model and encouraging their staff in wellness activities. Conclusion: Although findings are generated from a small sample size, these qualitative data provide compelling and early insights into building a workplace well-being strategy leveraging an underutilized key stakeholder, the workplace supervisor.

Periodic Evaluation Of The Clone Size Is Mandatory In PNH: Study Of The Spanish Cohort Of The International PNH Registry

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3715-3715
Author(s):  
Ana Villegas ◽  
Ana Gaya ◽  
Emilio Ojeda ◽  
Ataulfo Gonzalez ◽  
Alvaro Urbano ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Small Sample Size ◽  
Small Sample ◽  
Additional Patient ◽  
Clone Size ◽  
Hematopoietic Stem ◽  
Future Analysis ◽  
Group I ◽  
Group Ii ◽  
Pnh Clone

Abstract Background Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic, life threatening hematopoietic stem cell disorder with chronic hemolytic anemia, peripheral blood cytopenias and thrombosis Aims To observe the PNH clone and LDH evolution of the Spanish patients enrolled in the International PNH Registry, the thrombotic events and the role of eculizumab Methods We analyzed the 117 patients enrolled in the Registry until Dec. 31st 2012, classified in 3 groups: Classic/ hemolytic (group I, n 59), PNH with another bone marrow disorder (group II, n 42) and Subclinical (group III, n 14). The variables analyzed were PNH clone size, LDH levels, and incidence of thrombosis. Medians and percentages should be taken with caution due to the relatively small sample size. In addition to data collected in the Registry, additional patient information was obtained from local physicians. Results The median (range) age at presentation was 36.6 yrs. (16-83); 48 patients (41.0%) were women. Median (range) time from disease start to enrollment was 11.3 years in group I (0.1-41.2), 3.5 in II (0.1-33.8) and 3.4 (0.3-20.8) in III. A total of 49 patients (39 in group I) were started on eculizumab, 38 prior to enrollment (31 in group I) and 11 on or after enrollment; 3 were treated prior to enrollment but discontinued for different reasons (pregnancy, ending trial, access problems). Clone evolution (Table 1). In group I the median clone size remained stable during the follow-up period in the Registry; however, 4 patients in group II evolved to group I, with granulocyte clones > 50% and LDH levels >2000 U/L, while 3 initially in group I evolved to group II at 6, 12 and 18 months respectively. At enrollment 64 patients had a clone ≥30% and 31<30%; 7 patients in group II had a clone ≥30% despite hypoplasia, and they were treated with eculizumab. In groups I and II median clone size increased from Diagnosis to Enrollment in line with the physiopathology of the disease. LDH evolution (Table 2). Median LDH levels at diagnosis were higher in group I. In this group the decrease in LDH level between Diagnosis and Enrollment could be attributed to the start of treatment in 31 patients before the enrollment visit, but that hypothesis will need confirmation in future analysis. Thrombotic episodes (Table 3). Twenty six patients (22.6%) presented 52 TEs along the study period, 41 in group I and 11 in group II. Of the 26, fourteen presented 1 TE, six 2, one 3, four 2 and one 5. Twenty five patients presented 51 TEs since the moment of diagnosis while they were not being treated with eculizumab. Only one patient in the treated group presented a TE (CVA), of which he recovered well; after 30 months of the episode continues with the treatment and scores 90 in the Karnofsky index. Fifty eight percent of the patients presenting TEs were male, showing they may be more prone to TE than women. Conclusions These data show the dynamic features of the disease in some patients, which justifies the necessity of regularly monitoring the clone size LDH levels are higher in patients with classical PNH at diagnostic; the effect of the treatment in the whole cohort will require future analysis Thrombosis is highly prevalent in PNH; 22.6% of the patients in this sample had at least 1 episode along their time in the study. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Predictors of Hydroxyurea Use in Children with Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4934-4934
Author(s):  
Chisom Ifeoma Okwor ◽  
Robert J Klaassen ◽  
Mary-Ann Harrison ◽  
Ken Tang ◽  
Isabelle Laforest ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Small Sample Size ◽  
Small Sample ◽  
Care Utilization ◽  
Lower Income ◽  
Cell Disease ◽  
Hematological Indices ◽  
Hydroxyurea Treatment

Abstract Background: Since hydroxyurea emerged as an effective therapy for sickle cell disease (SCD), there have been numerous studies that have demonstrated its safety and efficacy in children and adults with SCD. In their 2014 guidelines, the NHLBI recommended that hydroxyurea treatment should be offered to all infants and children with sickle cell anemia (HbSS and HbS/beta0 thalassemia) starting at 9 months of age. However, hydroxyurea is underused among children and adolescents with SCD and to date, there have been no studies that have identified the specific determinants that may predict hydroxyurea adherence in these patients. Objectives: 1. To identify predictors of hydroxyurea adherence in children with SCD. 2. To measure the rate of hydroxyurea use among CHEO patients with SCD who were born between January 1, 2003 and December 31, 2015; and 3. To compare the rates of SCD-related complications between patients who were not prescribed hydroxyurea, patients who were adherent to hydroxyurea and patients who were not adherent to hydroxyurea Methods: We extracted medical chart data to identify patients with SCD who were born between January 1, 2003 and December 31, 2015. Patients were classified as either "Not prescribed hydroxyurea" or "Prescribed hydroxyurea" based on clinical documentation and the presence of at least one hydroxyurea outpatient prescription. For those patients who were prescribed hydroxyurea, hematological indices were collected and analyzed over time to estimate adherence to hydroxyurea. To measure the adherence of children prescribed hydroxyurea, we examined the trends in the patient's hematological indices after their first prescription of hydroxyurea. Adherence was defined as increased hematological indices (from baseline) by greater than or equal to any 2 of the following: Mean corpuscular volume (MCV) by 10 fL; Hemoglobin levels (g/L) by 10 g/L and/or %HbF (fetal hemoglobin) by 10%. We measured the frequency of disease-related complications among CHEO patients with SCD according to their use of hydroxyurea and used multivariate analyses to evaluate immigration status, newborn screening status, SCD subtype, SCD complications, income, age and sex as predictors for hydroxyurea adherence. Results: Children with HbSS were more likely to have been prescribed hydroxyurea compared to children with HbSC (87.8% vs. 9.5%). Canadian citizenship, newborn hemoglobinopathy screening and lower familial income were associated with better hydroxyurea adherence (Table 1). Although the association was not statistically significant, patients were more likely to be prescribed hydroxyurea if they were from a lower income background (61.9% for lowest and second lowest quartiles vs. 38.1% for third and highest quintiles). Patients were also more likely to adhere to hydroxyurea if they did not have private medical insurance for hydroxyurea coverage (Table 1). Finally, hydroxyurea adherence was associated with reduced rates of health care utilization and SCD-related complications (Table 2). Conclusions: In line with previous studies of hydroxyurea for the treatment of SCD, patients who were adherent to hydroxyurea had fewer complications compared to those patients who were either non-adherent to or not prescribed hydroxyurea. Similarly, patients had fewer complications after being prescribed hydroxyurea compared to before they started hydroxyurea with a reduction in the rate of ED visits, acute chest syndromes, complications, transfusions and hospitalizations. Patients from non-immigrant families, patients who were identified through newborn hemoglobinopathy screening and patients from lower income families were more likely to be adherent to hydroxyurea. Although the results of this study were limited by its small sample size, further studies will clarify these determinants of hydroxyurea adherence among SCD patients and enable clinicians to improve hydroxyurea adherence for SCD patients. Disclosures Klaassen: Shire: Consultancy; Novartis: Research Funding; Hoffman-La Roche: Consultancy; Amgen Inc.: Membership on an entity's Board of Directors or advisory committees; Octapharma AG: Consultancy, Honoraria; Agios Pharmaceuticals Inc.: Consultancy; Cangene: Research Funding.

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