scholarly journals Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

2012 ◽  
Vol 59 (7) ◽  
pp. 1275-1279 ◽  
Author(s):  
Raffaella Colombatti ◽  
Maria Montanaro ◽  
Fabiola Guasti ◽  
Patrizia Rampazzo ◽  
Giorgio Meneghetti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Comprehensive Care ◽  
Standards Of Care ◽  
Cell Disease ◽  
Minimum Standards ◽  
High Adherence ◽  
Minimum Standards Of Care

Hospitalization Rate and Regional Differences in Comprehensive Care in Transfused Patients with Sickle Cell Disease Compared to Thalassemia: A Report from the Multi-Center Study of Iron Overload.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3189-3189
Author(s):  
Ellen B. Fung ◽  
Paul Harmatz ◽  
Meredith Milet ◽  
Samir K. Ballas ◽  
Laura DeCastro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Liver Biopsy ◽  
Iron Overload ◽  
Sickle Cell ◽  
Comprehensive Care ◽  
Organ Injury ◽  
Standards Of Care ◽  
Cell Disease ◽  
Multi Center Study ◽  
Center Study

Abstract Red blood cell transfusions are often used to prevent the complications of thalassaemia (Thal) and sickle cell disease (SCD), though chronic therapy frequently results in iron overload-related organ injury. The Multi-Center Study of Iron Overload is a prospective, natural history study conducted to compare the comorbidities of iron overload in Thal with SCD. This report examines differences in morbidity and comprehensive care by disease and geographic region. 584 subjects aged 12 years and over, were screened for inclusion from 30 clinical hematological centers in the US, Canada and the UK. Socioeconomic and clinical data from subjects with iron overload (LIC of ≥ 10 mg/g dry wt by biopsy) and either Thal (N=142, 54% Male) or Tx-SCD (N=199, 43% Male) were collected. A group of non-iron overloaded SCD (NonTx-SCD N=64; 50% Male) were also enrolled. Tx-SCD were hospitalized more frequently (4.1± 3.8 times/year) compared to Thal (1.8± 1.7) or NonTx-SCD (2.1± 1.7; p<0.001) subjects, though average length of stay was similar (Tx-SCD: 7.0± 5.3 days; Thal: 5.7± 4.6; NonTx-SCD: 5.3± 3.8, p=0.16). Within the Tx-SCD group, adult subjects were 2.4 times more likely to be hospitalized in the previous 12 months compared to pediatric subjects (p=0.004, 95%CI 1.3–4.4). Roughly half of the SCD hospitalizations were for pain, whereas subjects with Thal were hospitalized for a variety of reasons, most often secondary to infection. Subjects with Thal were more likely to have routine iron assessments (LIC by biopsy: 66% vs. 37%), and screening for iron related organ injury such as thyroid function (TSH: 85% vs. 33%) and cardiac function (ECHO: 75% vs. 54%; EKG: 60% vs. 41%) compared to Tx-SCD (all p<0.001). Subjects with Thal had 3.4 times higher odds of having a recent liver biopsy compared to Tx-SCD (p<0.001; 95%CI: 2.2–5.3). Pediatric Tx-SCD subjects were more likely to have a biopsy compared to adults (46.5 vs. 23.5; p <0.001), however this likelihood was not related to serum ferritin level, duration of transfusion or chelation. Combining Thal and Tx-SCD groups, liver biopsy was more commonly performed at international vs. U.S. centers, (p<0.001), however the size of the clinical center was unrelated. Whereas, both regional and center size differences were observed in ECHO and TSH testing (p<0.01). A total of 26 subjects have died or exited from the study due to significant medical events. The mortality among adult Tx-SCD subjects was 2.8 fold greater than that of Thal (p=0.017). These data suggest that despite significant morbidity and iron burden, Tx-SCD subjects are monitored on a less frequent basis for iron related organ damage compared to Thal subjects. Differences were also observed in standards of care by region and center size. This disparity in care requires attention and guidelines for the assessment and management of Tx-SCD subjects should be developed and supported until the effect of iron overload in this population is better understood.

A North American Experience Of Hemoglobin SC Disease, Its Complications, and Management

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2221-2221 ◽  
Author(s):  
Veronique Naessens ◽  
Richard Ward ◽  
Kevin H.M. Kuo
Keyword(s):  
Sickle Cell Disease ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Care Center ◽  
Comprehensive Care ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Baseline Hemoglobin ◽  
Hemoglobin Sc Disease

Background The phenotype of hemoglobin SC (HbSC) disease is distinct from sickle cell anemia (SCA) (HbSS and S/b0) but management of adults is mostly derived from studies of the latter group. Longitudinal observational studies on the complications and outcomes of hemoglobin SC disease are largely confined to centers outside North America. The unique ethnic composition of our cohort, consisting of mostly Western Africans and West Indians, permits further characterization of the HbSC phenotype. Objective to describe the baseline characteristics and long-term complications of a cohort of adult HbSC patients followed in a Canadian sickle cell comprehensive care center. Methods A retrospective observational cohort study was conducted on all adult patients with HbSC disease attending a sickle cell comprehensive care center in Toronto, Canada from 1994 to 2013. Baseline demographics, acute and chronic complications attributable to sickle cell disease, and laboratory data were collected. Medians were used to describe continuous variables, while percentages or ratios for categorical variables. Logistic regression was used to examine factors influencing the main clinical complications. Results 104 patients were included in the analysis, comprising of 38.5% males and 61.5% females. Median length of follow-up was 3.5 years (1 - 19) and median age at last recorded visit was 35 years (18 - 68). Median baseline hemoglobin was 119 g/L (82 - 153 g/L), hematocrit 0.340 (0.250 - 0.440), and fetal hemoglobin (HbF) fraction 1% (0 - 7.7%). Most frequent complications encountered were retinopathy (55.8%) and symptomatic avascular necrosis (27.9%), followed by painful vaso-occlusive crises requiring emergency room visit (23.1%). Presence of retinopathy was associated with higher baseline hemoglobin (OR 2.72 for every 10 g/L of hemoglobin, p = 0.037) and older age (OR 2.72 for every decade, p < 0.001). Acute chest syndrome (7.7%), priapism (7.5% of men), and renal involvement (8.2%), were less common than reported in the literature, while the rates of venous thromboembolism (8.7%), symptomatic infarctive or hemorrhagic stroke (2.9%) were slightly more common. Median right ventricular systolic pressure on 2D-transthoracic echocardiogram was 29 mmHg (17 – 43 mmHg). No patient underwent a right heart catheterization. Two patients died from septic shock, both at the age of 29. Disease-modifying therapy most often consisted of hydroxyurea (28.8%), followed by exchange transfusion (6.7%) and phlebotomies (5.8%). Hydroxyurea significantly increased the median HbF fraction (from 1% to 2.75%, p = 0.004 by related-samples Wilcoxon signed rank test). Conclusion In this large North American cohort of adult patients, we have again shown that HbSC disease is not as benign as traditionally thought. As such, patients with HbSC disease warrant similar follow-up to that provided to SCA. Retinopathy, avascular necrosis and painful vaso-occlusive crises were the most common complications in our study, albeit lower than in other reported cohorts. The frequent use of hydroxyurea in this cohort is quite unique compared to other sickle cell comprehensive care centers reported in the literature. However, therapeutic phlebotomy is less often used compared to the European experience. We also observed a lower frequency of retinopathy, avascular necrosis, painful vaso-occlusive crises, priapism and acute chest syndrome. Whether this observation is due to hydroxyurea use or to other genetic or environmental factors remains to be determined. Further studies are also required to develop a more evidence-based therapeutic strategy for this genotype of Sickle Cell Disease. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Comprehensive care for patients with sickle cell disease in Cuba

Haematologica ◽  
2008 ◽  
Vol 93 (1) ◽  
pp. e20-e20 ◽  
Author(s):  
J.D. Fernandez Aguila ◽  
M. Cabrera Zamora ◽  
O. Alvarez Fernandez ◽  
L. Prieto Jimenez ◽  
O. Mediaceja Vicente ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Comprehensive Care ◽  
Cell Disease

Adult Sickle Cell Disease Epidemiology and the Potential Role of a Multidisciplinary Comprehensive Care Center in a City with Low Prevalence

Hemoglobin ◽  
2014 ◽  
Vol 38 (5) ◽  
pp. 312-315 ◽  
Author(s):  
Andrew Binding ◽  
Karen Valentine ◽  
Man-Chiu Poon ◽  
Farzana A. Sayani
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Potential Role ◽  
Care Center ◽  
Comprehensive Care ◽  
Cell Disease ◽  
Disease Epidemiology ◽  
Low Prevalence

scholarly journals Perspectives on Building Sustainable Newborn Screening Programs for Sickle Cell Disease: Experience from Tanzania

2021 ◽  
Vol 7 (1) ◽  
pp. 12
Author(s):  
Daima Bukini ◽  
Siana Nkya ◽  
Sheryl McCurdy ◽  
Columba Mbekenga ◽  
Karim Manji ◽  
...  
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Newborn Screening ◽  
Sickle Cell ◽  
Health Care Systems ◽  
Comprehensive Care ◽  
Cell Disease ◽  
Care Services ◽  
Care Systems ◽  
Screening Services

The prevalence of sickle cell disease is high in Africa, with significant public health effects on the affected countries. Many of the countries with the highest prevalence of the disease also have poor health care systems and a high burden of infectious diseases with many other competing health care priorities. Although considerable efforts have been made to implement newborn screening for sickle cell disease programs in Africa, coverage is still low. Tanzania has one of the highest birth prevalence of children with sickle cell disease in Africa. In 2015, the country implemented a pilot project for Newborn Screening for Sickle Cell Disease to assess feasibility. Several efforts have been made afterwards to continue providing the screening services as well as related comprehensive care services. Using qualitative methods, we conducted in-depth interviews and focus group discussions with policy makers (n = 4), health care providers (n = 21) and families (n = 15) to provide an analysis of their experiences and perspectives on efforts to expand and sustain newborn screening for sickle cell disease and related comprehensive care services in the country. Thematic content analysis was used to analyze the data through the framework analysis method. The findings have demonstrated both the opportunities and areas that need addressing in the implementation and sustainability of the services in low resource settings. A key area of strengthening is full integration of the services in countries’ health care systems to facilitate the coverage, accessibility and affordability of the services. Although the coverage of newborn screening services for sickle cell disease is still low, efforts at the local level to sustain the implementation of the programs and related comprehensive care services are encouraging and can be used as a model for other programs implemented in low resources settings.

The comprehensive care of sickle cell disease

2002 ◽  
Vol 68 (3) ◽  
pp. 157-162 ◽  
Author(s):  
Iheanyi Okpala ◽  
Veronica Thomas ◽  
Neil Westerdale ◽  
Tina Jegede ◽  
Kavita Raj ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Comprehensive Care ◽  
Cell Disease

scholarly journals Initial outcomes of a comprehensive care model for patients with sickle cell disease in a tribal population in rural Western India

2016 ◽  
Vol 82 (3) ◽  
pp. 532
Author(s):  
S. Banerjee ◽  
K. Dave ◽  
G. Desai ◽  
P. Babaria ◽  
R. Gupta
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Comprehensive Care ◽  
Western India ◽  
Tribal Population ◽  
Cell Disease ◽  
Care Model ◽  
Comprehensive Care Model
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