scholarly journals PMD39 MEASURING HEALTH-RELATED QUALITY OF LIFE IN INDIVIDUALS WITH SICKLE CELL DISEASE UNDERGOING AUTOMATED RED BLOOD CELL EXCHANGE

2019 ◽  
Vol 22 ◽  
pp. S223
Author(s):  
K. Dierick ◽  
Y. Lee ◽  
A. Silver ◽  
N. Comasòlivas
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

scholarly journals Measuring Health-Related Quality of Life in Sickle Cell Disease Patients Undergoing Automated Red Blood Cell Exchange in the USA, France and the UK

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5892-5892
Author(s):  
Koenraad Dierick ◽  
Joseph Roig
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Blood Cell ◽  
Red Blood Cell ◽  
Blood Cells ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

Abstract Background Sickle cell disease (SCD) is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape. Additionally, affected red blood cells have a high likelihood of stacking up and causing blockages in the small blood vessels. Both mechanisms of disease may cause damage to organs requiring oxygen, which causes pain and may be deadly. SCD and its complications reduce life expectancy and the nature of its complications and treatments may cause reduced quality of life. Although a rare disease, SCD may cause significant costs to healthcare and society. Depending on the indication, either hydroxyurea or chronic RBC transfusion is employed to treat SCD patients. RBC transfusion, simple or exchange, is first-line treatment for primary and secondary prevention of stroke. When the study was performed, no published research had been completed that compared patient outcomes in terms of Health-Related Quality of Life (HRQoL) for patients treated with automated red blood cell exchange versus simple transfusion. Objectives There are multiple objectives covered within the scope of this study:To quantify HRQoL as experienced by SCD patients in aRBCx versus simple transfusion.To determine the drivers of HRQoL amongst SCD patientsTo assess whether physicians and patients have a similar view on the impact of aRBCx on HRQoL amongst SCD patients. Methodology A cross-sectional study was performed amongst 40 SCD patients, 20 from the USA, 10 from France and 10 from the UK as well as amongst 40 SCD treating physicians with experience in both simple transfusion as well as aRBCX. The physicians had the same regional distribution as the patients. Results SCD patients undergoing aRBCX reported an HRQoL that was 25% higher compared to the period where they were treated with simple transfusion (0.70 vs. 0.55; p<0.01). The main drivers of HRQoL identified were (correlation efficient): pain reduction (0.57), improved social live (0.49), autonomy in terms of all day living activities and being independent from others (0.56), feeling energetic and physical functioning (0.57) and lastly emotional worry and mental health (0.56), all with p-values < 0.01. Together these variables explain 39% of the HRQoL experienced by SCD patients (R² = 0.39, p < 0.01). 80% of the patients preferred aRBCx over simple transfusion. 87% of the participating physicians believed that switching patients from simple transfusion to automated red blood cell exchange (aRBCX) positively affected the SCD patients' quality of life. Physicians identified the following factors being responsible for the improved HRQoL in patients on aRBCX. Those with an average score of 5.5 or greater on a 7-point scale were: less iron overload, RBCX effectiveness, reduced acute complications, reduced chronic complications, and superior mechanism of action during acute situations. Conclusion Sickle cell disease patients that require chronic blood transfusion experience better health-related quality of life when they are treated with automated red blood cell exchange versus simple transfusion. This observation is supported by the opinion of their treating physicians. Disclosures Dierick: Terumo BCT: Employment. Roig:Terumo BCT: Employment.

Health-related quality of life and neuropathic pain in sickle cell disease in Jamaica

2021 ◽  
pp. 101107
Author(s):  
Rachel Bartlett ◽  
Zachary Ramsay ◽  
Amza Ali ◽  
Justin Grant ◽  
Angela Rankine-Mullings ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neuropathic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

Health Related Quality of Life of Children with Sickle Cell Anemia and Their Parents; Quantitative Study in Albaha; Saudi Arabia

2021 ◽  
pp. 9-17
Author(s):  
Turki Alzahrani ◽  
Raed Alzahrani ◽  
Amer Alzahrani ◽  
Abdullah Alzahrani ◽  
Abdu Adawi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Healthy Children ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

Aims: To gain a better understanding of the quality of life (QoL) of children and impact of this disease on parents QoL. Study Design: Cross-sectional study. Place and Duration of Study: The study was conducted in King Fahd Hospital, Albaha city, Albaha, Saudi Arabia, between March2020 and February 2021. Methodology: We included 95 responses. Two different tools were used for the purpose of this study. PedsQL™ Sickle Cell Disease Module was used to measure health-related quality of life (HRQoL) in healthy children and adolescents and those with acute and chronic health conditions. Moreover, PedsQL™ Family Information Form was completed by caregivers. Median and interquartile range were used for numerical variables since they were skewed. Bivariate analyses were carried out using non-parametrical tests and Pearson correlation. The prediction of QoL was accomplished through multivariate analysis. Results: A total of (95) responses were analyzed. Female respondents were 52.6%. The age median was 12 (IQR=10-14). Mothers represented the most frequent informant 46.3% in this current study. Significant association was found between QoL and certain independent factors, some of which is parental level of education (P< .001) and marital support (P< .001). Conclusion: Sickle cell disease (SCD) is a major condition accounts for a huge burden on variable levels. This study reported that low QoL among children affected by SCD. Higher education and current marital status of the parents were significantly associated with high QoL in SCD patients. Number of workdays affected due to child health was significantly correlated with low QoL.

scholarly journals Disease Self-Efficacy and Health-Related Quality of Life in Adolescents With Sickle Cell Disease

2020 ◽  
Vol 42 (2) ◽  
pp. 141-144 ◽  
Author(s):  
Alana Goldstein-Leever ◽  
James L. Peugh ◽  
Charles T. Quinn ◽  
Lori E. Crosby
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Self Efficacy ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

scholarly journals Health-related quality of life in patients with sickle cell disease in Saudi Arabia

2015 ◽  
Vol 13 (1) ◽  
Author(s):  
Anwar E. Ahmed ◽  
Ahmed S. Alaskar ◽  
Ahmad M. Al-Suliman ◽  
Abdul-Rahman Jazieh ◽  
Donna K. McClish ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

scholarly journals Disease Burden and Pre-Transplant Health-Related Quality of Life in Pediatric Sickle Cell Disease Patients Receiving Nonmyeloablative HLA-Identical Sibling Donor Transplantation

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4073-4073
Author(s):  
Jeremy Zack ◽  
Robert Sheppard Nickel ◽  
Allistair Abraham ◽  
Steven J. Hardy
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Disease Burden ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related ◽  
History Of

Abstract Title: Disease Burden and Pre-transplant Health-related Quality of Life in Pediatric Sickle Cell Disease Patients Receiving Nonmyeloablative HLA-identical Sibling Donor Transplantation Background: Sickle cell disease (SCD) is a hemoglobinopathy characterized by recurrent pain crises, severe organ damage, and reduced health-related quality of life (HRQL). Bone marrow transplantation (BMT) is a proven curative treatment for SCD that can improve both disease burden and HRQL. Despite the benefits, relatively few patients pursue a cure through BMT. The decision to receive a BMT is nuanced and likely to be influenced by a number of factors. One study by Roth et al. showed SCD patients interested in BMT had a higher HRQL than those uninterested in BMT, suggesting some variability between SCD patients pursuing transplant and the general SCD patient population. To date the factors that may affect pre-transplant HRQL of pediatric SCD patients remain unclear, particularly in the nonmyeloablative setting. Objective: To evaluate the impact of disease-burden, patient demographics, and hydroxyurea treatment on pre-transplant HRQL. Methods: Children and young adults with SCD set to receive a nonmyeloablative HLA-identical sibling donor BMT were administered HRQL surveys (PedsQL) in the six months leading up to the start of the conditioning regimen. Patients' disease history and demographic information were captured and analyzed for their relationship to HRQL. PedsQL parent surveys were used when patient surveys were unavailable. The mean difference in HRQL between groups were compared using an unpaired t test or linear regression. Results: 32 patients were enrolled onto this study. Six of the 32 patients HRQL were not included due to withdrawal, delay of transplant, or completion of the survey outside of window. The median age at the time of survey administration was 13.5 years (range 2-21). 57.7% of patients were male and 88% of patients had genotypes hemoglobin SS or Sβ 0thalassemia. 12% of patients had a history of overt stroke. 52% of patients were hospitalized while receiving HU treatment. 32% of patients received chronic blood transfusions leading up to transplant. The median number of hospitalizations in the two years leading up to transplant was 2 (range 0-10). 16.7% of patients met the AAPT diagnostic criteria for chronic pain. 75% of patients received HU treatment leading up to transplant. The median hemoglobin F percentage was 10.4. The median HRQL for all patients was 77.17 (range 36.9-98.91, SD +/- 15.99). Sickle cell genotype (P=0.88), history of overt stroke (P=0.79), hospitalization while receiving HU treatment (P=0.78), and the number of hospitalizations in the two years before transplant (P=0.39) were not associated with lower HRQL. History of chronic transfusion leading up to transplant approached statistical significance for lower HRQL (P=0.06). Older age (P=0.17) and female gender (P=0.89) were not associated with worse HRQL. Patients receiving HU treatment had significantly higher HRQL than patients not receiving HU (P=0.0034), with median PedsQL scores of 80.06 and 58.64, respectively. The date of survey administration was not associated with HRQL (P=0.40). The number of eligibility criteria met (P=0.63), chronic pain (P=0.17), total number of RBC transfusions (P=0.45), and pre-transplant hemoglobin (P=0.25) were not associated with HRQL. Discussion: Patient demographics and several markers of disease burden appear to have minimal impact on HRQL. Interestingly, the use of HU therapy was associated with significantly higher pre-transplant HRQL. The etiology of this finding is unclear given that disease burden was not associated with HRQL and warrants further investigation. The absence of association between disease burden and HRQL through current disease severity eligibility paradigms suggests that other factors may impact HRQL in SCD patients choosing a low toxicity nonmyeloablative BMT. Disclosures No relevant conflicts of interest to declare.

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