Iron status in a population with mild hemophilia A: associations and impacts

Kawaja, Marc Colin (2006) Iron status in a population with mild hemophilia A: associations and impacts. Masters thesis, Memorial University of Newfoundland.

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Abstract

Iron status, its associations and its impact on quality of life, was investigated in a cohort of patients residing in Newfoundland & Labrador. The population included individuals who were either affected with mild hemophilia A or were carriers of the Val2016Ala allele mutation that underlies the disease. The clinical impact of mild hemophilia A on serum ferritin, menstrual blood losses, quality of life and Body Mass Indices (BMIs) were investigated as part of a 2-year cross-sectional cohort study. The methodology included serum ferritin concentrations, complete blood counts (CBCs), the pictorial blood loss assessment chart (PBAC), bleeding histories, MOS short-form survey (SF-36) and BMIs. Iron depletion was prevalent in women (62% with ferritin levels <50 μg/L, 33% <20 μg/L), particularly in those who were menstruating (80% <50 μg/L, 47% <20 μg/L). Women in general self-reported a lower mean General Health Scale score (63.9, 59.9-67.9 vs. 70.6, 69.5-71.7) and a higher mean Role Emotional Scale score (89.3, 85.8-92.8 vs. 79.5, 77.7-81.3) than norms for the general U.S. female population. Men with a history of severe bleeding had significantly lower ferritin levels than men without a history of severe bleeding (123.4 μg/L, 63.6-186.3 vs. 189.8 μg/L, 105.9-301.5; p <0.05). Mutation status did not influence iron status in either sex or menstrual blood loss in women as measured by the PBAC. Serum ferritin level was not associated with PBAC score, but was related to women's BMI (r = 0.235, p <0.01). Neither the means of the eight SF-36 domains, health transition scale, nor the two component summary measures were significantly lower for mildly iron-deficient or iron-deficient women. The SF-36 is a general measure of various quality of life domains and may not have been sensitive enough to measure the effects iron deficiency could potentially have on women's cognition and fatigue. A study using measures more sensitive to these effects would better investigate the impact of iron depletion and iron deficiency. Further research is also required to determine whether the low ferritin levels observed in women could possibly be a result of inadequate dietary intake of iron or insufficient iron absorption.

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