Browsing by Subject "sickle-cell disease"

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    Haptoglobin 1 allele predicts higher serum haptoglobin concentration and lower multiorgan failure risk in sickle cell disease
    (ASH Publications, 2022-12-22) Ruiz, Maria A.; Shah, Binal N.; Ren, Guohui; Hussain, Faiz; Njoku, Franklin; Machado, Roberto F.; Gordeuk, Victor R.; Saraf, Santosh L.; Medicine, School of Medicine
    Haptoglobin (HP) is an acute-phase protein and the main scavenger of cell-free hemoglobin. When HP is depleted, as observed in hemolytic conditions such as sickle cell disease (SCD), cell-free hemoglobin can lead to acute organ damage. The impact of the HP 1-1, 2-1, and 2-2 isoforms on HP and cell-free hemoglobin concentrations and SCD-related complications is unclear. In a longitudinal cohort of patients with SCD, the HP 1 allele was associated with higher HP and lower cell-free hemoglobin concentrations at a routine clinic visit as well as during hospitalization for a vaso-occlusive episode or acute chest syndrome. With a median follow-up of 6.8 years, acute chest syndrome occurred in 42% (n = 163) and multiorgan failure in 14% (n = 53) of 391 patients with SCD with a minimum follow-up of 6 months. The HP 1 allele was independently associated with lower risk of developing multiorgan failure during acute chest syndrome (additive model hazard ratio, 0.5; P < .001). Future studies assessing the regulation of HP concentrations and ability to bind cell-free hemoglobin according to the HP genotype may help to identify patients with SCD at high risk for multiorgan failure and to guide interventions, such as rapid initiation of exchange transfusion or HP replacement therapy.
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    What does shared decision making look like in natural settings? A mixed methods study of patient–provider conversations
    (Equinox, 2017) Lee, Joy; Callon, Wynne; Haywood, Carlton, Jr.; Lanzkron, Sophie M.; Gulbrandsen, Pål; Beach, Mary Catherine; Medicine, School of Medicine
    Objective: To understand the variability and nature of shared decision making (SDM) regarding a uniform type of serious medical decision, and to make normative judgments about how these conversations might be improved. Methods: This was a mixed-methods sub-analysis of the Improving Patient Outcomes with Respect and Trust (IMPORT) study. We used the Braddock framework to identify and describe seven elements of SDM in audio-recorded encounters regarding initiation of hydroxyurea, and used data from medical records and patient questionnaires to understand whether and how these tasks were achieved. Results: Physicians covered a spectrum of SDM behaviors: all dialogues contained discussion regarding the clinical issue and the pros and cons of treatment; the patient's understanding and role were not explicitly assessed or stated in any encounter. Yet no patient agreed to start hydroxyurea who did not already prefer it. There was no uniform approach to how physicians presented risk; many concerns expressed by patients in a pre-visit questionnaire were not discussed. Conclusion: In this analysis, patients seemed to understand their role in the decision-making process, suggesting that a patient's role may not always need to be explicitly stated. However, shared decision making might be improved with more routine assessment of patient understanding and concerns. Standardized decision aids might help fully inform patients of risks and benefits.