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  1. Article ; Online: Motivators and barriers to blood donation among potential donors of African and Caucasian ethnicity.

    Fogarty, Helen / Sardana, Muskan / Sheridan, Luke / Chieng, Phoebe / Kelly, Sarah / Ngwenya, Noel / Sheehan, Ciara / Morris, Kieran / Tuohy, Emma

    Blood transfusion = Trasfusione del sangue

    2022  Volume 21, Issue 1, Page(s) 13–23

    Abstract: Background: Minority blood donation, especially from individuals of African ethnicity, is a focus for many countries with diverse populations. As the need for antigen-negative RBC transfusions for patients with Sickle Cell Disease (SCD) continues to ... ...

    Abstract Background: Minority blood donation, especially from individuals of African ethnicity, is a focus for many countries with diverse populations. As the need for antigen-negative RBC transfusions for patients with Sickle Cell Disease (SCD) continues to grow, inclusion of more African blood donors is essential to ensure this demand is met.
    Materials and methods: This study aims to explore barriers and motivators to blood donation and awareness of SCD among potential donors of diverse ethnic backgrounds in Ireland. Following ethical approval, patients attending the National Sickle Cell Disease and Thalassemia service at St James's Hospital were invited to share an online anonymous survey within their local communities to achieve snowball-sampling.
    Results: 387 respondents completed the survey, including 311 non-donors (median age 25 years, 67% female). Ethnic backgrounds included: African or African-Irish (59%), White or Caucasian (25%), Asian (8%), Hispanic or Latino (3%), Middle Eastern (3%), Multiracial or Biracial (2%). The most commonly identified barrier overall was lack of information on blood donation. African respondents were significantly more likely to report lack of information and malaria-related barriers than Caucasians. Motivators also varied across ethnic groups, with African respondents more likely to donate to help someone within their own community or for religious motivators. Awareness of SCD was higher among African respondents.
    Discussion: While some barriers to blood donation are shared across all ethnic groups including lack of information, notable differences exist between Caucasian and African respondents. Specific actions to recruit and retain African blood donors should focus on these key areas.
    MeSH term(s) Adult ; Female ; Humans ; Male ; Anemia, Sickle Cell ; Blood Donation ; Blood Donors ; Ethnicity ; Tissue Donors ; Black People ; White People ; Motivation
    Language English
    Publishing date 2022-06-12
    Publishing country Italy
    Document type Journal Article
    ZDB-ID 2135732-8
    ISSN 2385-2070 ; 0041-1787 ; 1723-2007
    ISSN (online) 2385-2070
    ISSN 0041-1787 ; 1723-2007
    DOI 10.2450/2022.0014-22
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: VWF-ADAMTS13 axis dysfunction in children with sickle cell disease treated with hydroxycarbamide vs blood transfusion.

    Fogarty, Helen / Ahmad, Azaz / Atiq, Ferdows / Doherty, Dearbhla / Ward, Soracha / Karampini, Ellie / Rehill, Aisling / Leon, Gemma / Byrne, Ciara / Geoghegan, Rosena / Conroy, Helena / Byrne, Mary / Budde, Ulrich / Schneppenheim, Sonja / Sheehan, Ciara / Ngwenya, Noel / Baker, Ross I / Preston, Roger J S / Tuohy, Emma /
    McMahon, Corrina / O'Donnell, James S

    Blood advances

    2023  Volume 7, Issue 22, Page(s) 6974–6989

    Abstract: Previous studies have reported elevated von Willebrand factor (VWF) levels in patients with sickle cell disease (SCD) and demonstrated a key role for the VWF-ADAMTS13 axis in the pathobiology of SCD vaso-occlusion. Although blood transfusion is the gold ... ...

    Abstract Previous studies have reported elevated von Willebrand factor (VWF) levels in patients with sickle cell disease (SCD) and demonstrated a key role for the VWF-ADAMTS13 axis in the pathobiology of SCD vaso-occlusion. Although blood transfusion is the gold standard for stroke prevention in SCD, the biological mechanisms underpinning its improved efficacy compared with hydroxycarbamide are not fully understood. We hypothesized that the improved efficacy of blood transfusion might relate to differences in VWF-ADAMTS13 axis dysfunction. In total, 180 children with a confirmed diagnosis of SCD (hemoglobin SS) on hydroxycarbamide (n = 96) or blood transfusion (n = 84) were included. Despite disease-modifying treatment, plasma VWF and VWF propeptide were elevated in a significant proportion of children with SCD (33% and 47%, respectively). Crucially, all VWF parameters were significantly higher in the hydroxycarbamide compared with the blood transfusion cohort (P < .05). Additionally, increased levels of other Weibel-Palade body-stored proteins, including factor VIII (FVIII), angiopoietin-2, and osteoprotegerin were observed, indicated ongoing endothelial cell activation. Children treated with hydroxycarbamide also had higher FVIII activity and enhanced thrombin generation compared with those in the blood transfusion cohort (P < .001). Finally, hemolysis markers strongly correlated with VWF levels (P < .001) and were significantly reduced in the blood transfusion cohort (P < .001). Cumulatively, to our knowledge, our findings demonstrate for the first time that despite treatment, ongoing dysfunction of the VWF-ADAMTS13 axis is present in a significant subgroup of pediatric patients with SCD, especially those treated with hydroxycarbamide.
    MeSH term(s) Humans ; Child ; von Willebrand Factor/metabolism ; Anemia, Sickle Cell/drug therapy ; Vascular Diseases ; Hemolysis ; Hydroxyurea/therapeutic use ; Hemostatics ; Blood Transfusion ; ADAMTS13 Protein
    Chemical Substances von Willebrand Factor ; Hydroxyurea (X6Q56QN5QC) ; Hemostatics ; ADAMTS13 protein, human (EC 3.4.24.87) ; ADAMTS13 Protein (EC 3.4.24.87)
    Language English
    Publishing date 2023-09-29
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2915908-8
    ISSN 2473-9537 ; 2473-9529
    ISSN (online) 2473-9537
    ISSN 2473-9529
    DOI 10.1182/bloodadvances.2023010824
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Adherence to hydroxyurea, health-related quality of life domains and attitudes towards a smartphone app among Irish adolescents and young adults with sickle cell disease.

    Fogarty, Helen / Gaul, Alan / Syed, Saifullah / Aleksejenko, Natalija / Geoghegan, Rosena / Conroy, Helena / Crampton, Edel / Ngwenya, Noel / Tuohy, Emma / McMahon, Corrina

    Irish journal of medical science

    2021  Volume 191, Issue 2, Page(s) 809–816

    Abstract: Introduction: SCD patients experience declines in health-related quality of life (HRQOL) domains compared with healthy controls. Despite evidence supporting the benefits of hydroxyurea, medication non-adherence remains problematic, especially in ... ...

    Abstract Introduction: SCD patients experience declines in health-related quality of life (HRQOL) domains compared with healthy controls. Despite evidence supporting the benefits of hydroxyurea, medication non-adherence remains problematic, especially in adolescents and young adults (AYA). Adherence barriers include forgetfulness and lack of knowledge. Recently, increased interest in technology-based strategies to improve medication adherence has emerged. No data currently exists on hydroxyurea adherence, HRQOL or perceptions of technology-based tools in the Irish SCD population.
    Methods: In order to interrogate these domains among Irish AYA SCD patients we administered an anonymous survey at two tertiary referral centres in Dublin, Ireland, in July 2019.
    Results: Sixty-three patients participated; 63% female and 37% male, with a median and mean age of 17 and 19 years, respectively. Average monthly adherence was 76% using a visual analogue scale. Recall barriers were present in 62% while 26% omit hydroxyurea for reasons other than forgetting. Reviewing HRQOL; only 36.5% felt always physically able to engage in recreational activities, while 51% experienced disruption to school/college/work due to pain. Eighty-one percent reported that anxiety about health interferes with their lives and non-adherence correlated with worse HRQOL outcomes. Interest in a smartphone app was expressed by the majority, with daily medication reminders being the most popular feature. Sharing adherence data with doctors and discussion forums were less appealing.
    Conclusions: Representing over 10% of the Irish SCD population, our survey provides novel and valuable insights into medication adherence and HRQOL domains. Preferred app features may inform future technology-based interventions to improve medication adherence in SCD and other chronic health conditions.
    MeSH term(s) Adolescent ; Adult ; Anemia, Sickle Cell/drug therapy ; Female ; Humans ; Hydroxyurea/therapeutic use ; Male ; Medication Adherence ; Mobile Applications ; Quality of Life ; Young Adult
    Chemical Substances Hydroxyurea (X6Q56QN5QC)
    Language English
    Publishing date 2021-03-20
    Publishing country Ireland
    Document type Journal Article
    ZDB-ID 390895-1
    ISSN 1863-4362 ; 0021-1265
    ISSN (online) 1863-4362
    ISSN 0021-1265
    DOI 10.1007/s11845-021-02588-1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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