Health-related Quality of Life in Infants With Sickle Cell Disease

Beverung, Lauren M.; Bemrich-Stolz, Christina J.; Torres, Sylvia

doi:10.1097/mph.0000000000000434

Cited by 8 publications

(9 citation statements)

References 19 publications

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“…When compared with an "episodic" model of care, enrollment of SCD patients in comprehensive care programs was found to reduce cost, utilization of emergency department, length of hospitalization, and rate of readmission [28,29] . Quality of life outcomes were found to be poor in hemoglobinopathy patients compared to the general population [30][31][32] . Comprehensive care of hemoglobinopathies at specialized centers improves quality of life outcomes [33] , and leads to higher patient satisfaction [34] .…”

Section: Discussionmentioning

confidence: 99%

Clinical Complications of Hemoglobinopathies in Western Saudi Arabia and the Need for Specialized Care Centers

et al. 2019

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In the absence of specialized care centers for hemoglobinopathies in Saudi Arabia, there are insufficient data on prevalence of clinical complications in this population. This is a retrospective record review about the clinical complications associated with hemoglobinopathies at King Abdulaziz University Hospital, for patients followed between January 1st 2010 through June 30th 2016. A total of 349 patients were included, with a mean age of 25.45 years, including 266 with sickle cell disease and 80 with thalassemia. Of those receiving regular transfusions, 17.5% developed alloantibodies, 16.6% tested positive for hepatitis C virus antibodies and the mean ferritin level was 2487 ng/ml. Almost half of the patients were screened for renal disease, and more than 50% were found to have proteinuria. Of 203 patients with recent echocardiograms, 15.8% had pulmonary hypertension. Thirty-one (8.9%) patients died at a mean age of 28.5 years. In conclusion, the prevalence of disease related complications is high in hemoglobinopathies. Our results reiterate the need for specialized care hemoglobinopathy centers, which can further improve patient outcomes.

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Section: Discussionmentioning

confidence: 99%

Clinical Complications of Hemoglobinopathies in Western Saudi Arabia and the Need for Specialized Care Centers

et al. 2019

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“…Sehlo and Kamfar [1] suggested significantly impaired HRQOL in all dimensions in children with SCD as compared to healthy controls. Beverung et al [21] noted poor HRQOL in infants with SCD. Menezes et al [3] reported that SCD was related to many limita- tions in different domains of HRQOL, especially school, social, emotional, and physical domains.…”

Section: Discussionmentioning

confidence: 99%

“…Many studies have detected low HRQOL among children and adolescents with SCD [ 1 – 3 , 9 , 21 – 23 ] Palermo et al . [ 10 ] found that SCD children had more restricted social, psychological, and physical well-being than healthy ones with similar socioeconomic characteristics.…”

Section: Discussionmentioning

confidence: 99%

Parent-reported sleep problems in children and adolescents with sickle cell disease: relationship to health-related quality of life

Güneş¹,

Aldemir²,

Güneş³

et al. 2021

Arch Med Sci

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IntroductionChildren with sickle cell disease (SCD) can present a variety of clinical symptoms that may affect their sleep and health-related quality of life (HRQOL). This study aims to investigate the relation between sleep problems and HRQOL in children and adolescents with SCD.Material and methodsThe sample included 86 children and adolescents in the SCD patient group and 82 healthy controls, with an age range of 8-16 years. Subjects for the study were recruited from the Sickle Cell and Thalassemia Center of Hatay State Hospital, Hatay, Turkey. The Children’s Sleep Habits Questionnaire (CSHQ) was used to evaluate sleep problems and Kinder Lebensqualitätsfragebogen: Children’s Quality of Life Questionnaire – revised (KINDL-R) was used to examine HRQOL.ResultsTotal score, bedtime resistance, and night waking subscores of CSHQ were significantly higher in children with SCD when compared to healthy children. Total score, physical well-being, emotional well-being, social, and school subscores of KINDL-R were significantly lower in the patient group. Among SCD children, total score, bedtime resistance, sleep onset delay, daytime sleepiness, and parasomnias subscores of CSHQ were negatively correlated with KINDL-R total score. In the regression model, disease severity and CSHQ total score had significant negative associations with KINDL-R total score.ConclusionsSleep problems in SCD children appear to be negatively linked with HRQOL. Disease severity and sleep problems may be predictors of overall HRQOL in children and adolescents with SCD.

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“…Sickle cell disease impacts health related quality of life (HRQL) in children and adults [32][33][34][35]. The impact is worse in females and older children [34].…”

Section: Quality Of Life and Long-term Complicationsmentioning

confidence: 99%

Stem Cell Transplantation in Patients with Sickle Cell Disease

Al-Khabori¹,

Al-Huneini²,

Rawas³

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Hematopoietic stem cell transplantation (HSCT) is currently the only established cure for sickle cell disease (SCD). Replacement of the stem cell that has the defective beta globin allele with the normal gene decreases hemoglobin S and the risk of complications of SCD. The irst case reported was a girl with acute myeloid leukemia and SCD who received HSCT and achieved long-term SCD and leukemia-free survival. Given the favorable outcomes of HSCT with thalassemia major using myeloablative preparative regimens, this approach became widely used in the initial studies of HSCT in SCD. The current standard of care is to use a myeloablative stem cell transplantation in patients with severe disease who have human leukocyte antigen-identical sibling. HSCT improves organ function, quality of life, and overall and disease-free survival. However, this is associated with high risk of gonadal dysfunction and graft versus host disease in addition to the mortality associated with the myeloablative HSCT. Reduced-intensity HSCT has also been reported with high rates of engraftment and favorable outcomes. This has been introduced to lower the gonadal dysfunction, mortality, and graft versus host disease associated with myeloablative approaches. Other approaches include HSCT using matched unrelated donors, cord blood units, and human leukocyte antigen haploidentical donors. Unfortunately, graft rejection is a common complication with these approaches. In this chapter, we review the indications of HSCT for SCD and outcomes of diferent transplant strategies including alternative donor transplant, graft rejection, and infertility after transplantation.

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Health-related Quality of Life in Infants With Sickle Cell Disease

Cited by 8 publications

References 19 publications

Clinical Complications of Hemoglobinopathies in Western Saudi Arabia and the Need for Specialized Care Centers

Clinical Complications of Hemoglobinopathies in Western Saudi Arabia and the Need for Specialized Care Centers

Parent-reported sleep problems in children and adolescents with sickle cell disease: relationship to health-related quality of life

Stem Cell Transplantation in Patients with Sickle Cell Disease

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