Health literacy and knowledge of chronic transfusion therapy in adolescents with sickle cell disease and caregivers

Abstract Chronic monthly transfusions are a lifesaving preventative therapy for many patients with sickle cell disease; however, the burden of this therapy for patients and families is high. In the United States, there is overlap in the population affected by sickle cell disease and those with the greatest burden of social needs. Hematology providers caring for patients with SCD have an opportunity to screen for and mitigate social determinants of health, especially in those receiving chronic transfusion therapy given the frequent interactions with the healthcare system and increased demand on already potentially limited resources. Given the complexity of the treatment and medication regimens, providers caring for patients receiving chronic transfusions should implement universal strategies to minimize the impact of low health literacy, as this therapy imposes a significant demand on the health literacy skills of a family. Despite the social and literacy burden of this intervention, it is reassuring that quality of life is preserved as patients with SCD on chronic transfusion therapy often report higher health related quality of life than their peers receiving other disease modifying therapies.

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Transfusion therapy for cerebrovascular abnormalities in sickle cell disease

The Journal of Pediatrics ◽

10.1016/s0022-3476(76)80251-x ◽

1976 ◽

Vol 88 (3) ◽

pp. 382-387 ◽

Cited By ~ 98

Author(s):

Marie Olivieri Russell ◽

Herbert I. Goldberg ◽

Linda Reis ◽

Shlomo Friedman ◽

Robert Slater ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease ◽

Transfusion Therapy

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Management of Patients with Sickle Cell Disease Using Transfusion Therapy

Hematology/Oncology Clinics of North America ◽

10.1016/j.hoc.2016.01.011 ◽

2016 ◽

Vol 30 (3) ◽

pp. 591-608 ◽

Cited By ~ 51

Author(s):

Stella T. Chou ◽

Ross M. Fasano

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease ◽

Transfusion Therapy

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Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽

10.1161/str.48.suppl_1.tp402 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

J. Michael Taylor ◽

Paul Horn ◽

Heidi Sucharew ◽

Todd A Abruzzo ◽

Jane Khoury

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Care Utilization ◽

Cell Disease ◽

Common Procedure ◽

Transfusion Therapy ◽

Stroke Population ◽

Clinical Classification Software ◽

Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

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Retinal microvascular analysis using Fundus Fluorescein Angiography in Egyptian sickle cell disease patients

QJM ◽

10.1093/qjmed/hcab113.067 ◽

2021 ◽

Vol 114 (Supplement_1) ◽

Author(s):

Nayera H El Sherif ◽

Mahmoud A Kenny ◽

Waheed S Elhalfawy

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Fluorescein Angiography ◽

Sickle Cell ◽

Large Sample Size ◽

Cell Disease ◽

Fundus Fluorescein Angiography ◽

Transfusion Therapy ◽

Hydroxyurea Treatment ◽

Trans Cranial Doppler

Abstract Background Sickle cell disease can affect retina of eye via vaso-occulsive changes that occur in micro-vessels of retina which could be analysed by using Fundus Fluorescein Angiography. Aim To analyze macular microvascular alternation in patients with SCD by Fundus Fluorescein Angiography (FFA) and to assess the role of potentially contributory Clinico-pathological factors including Trans-Cranial Doppler, genotypes, hydroxyurea, transfusion therapy and finally iron overload state on the development of macular alterations. Method This was across-sectional study which included 30 Sickle cell disease patients randomly recruited from the Paediatric Haematology clinic, children Hospital, Ain Shams University, Cairo, Egypt. Complete blood count (CBC), Trans-Cranial Doppler (TCD) and Fundus Fluorescein Angiography. Results In our study, there were 30 patients with mean age (14.1± 4.02), 5 patients had abnormal/conditional Trans-Cranial, 15 patients had Vaso-occlusive crises, 11 patients were on regular simple blood transfusion; all 30 studied sickle cell disease patients had normal Fundus Fluorescein Angiography and eye examination and only one patient hadabnormal visual acuity;A 29 years oldgirl who had five attacks of cerebral strokes last year, on regular simple blood transfusion and Hydroxyurea treatment with abnormal TCD and recurrent Vaso-occlusive crises in last two years, Although her vision is hand movement yet Fundus Fluorescein Angiography was normal. Conclusion we didn’t find any Retinal microvascular alternation in our studied SCD patients using Fundus Fluorescein Angiography, we related our results to the fact that our studied SCD patients were young and all our studied patients were on hydroxyurea therapy with fair compliance, further studies using large sample size are warranted in order to illustrate the utility of Fundus Fluorescein Angiography (FFA) as a tool for better detection of sickle retinopathy.

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