Nutritional physiology of Sickle Cell Disease

ING Staff: Sharon Cox, Andrew Prentice, Tony Fulford

Sharon Cox has been based in Tanzania since 2007. Here within the Muhimbili Wellcome Programme and the Muhimbili Sickle Cohort (MSC) of over 2,000 patients with SCD, she leads a developing programme of work into the nutrition and physiology of SCD.

  1. The role of micronutrients, and genes affecting their metabolism, as determinants of the severity of sickle cell disease. This work is focused on iron and B-vitamin status, genetic determinants and effects on outcomes in SCD, including severity of anaemia, hospitalization, incidence of infections, cerebrovascular blood flow velocities and other intermediate endpoints. Data analysis and write up are ongoing.
  2. Nutritional status and growth in SCD. Mild to moderate stunting and underweight is common in SCD, even in high income countries. However the long term effects or relative causes of malnutrition have rarely been assessed in SCD. We are investigating in a double blind randomized controlled trial the effect of a micronutrient fortified ready-to-use supplementary food (RUSF), made in conjunction with Nutriset on growth and clinical disease symptoms in SCD.
  3. Arginine and nitric oxide in SCD. Altered amino acid profiles and low levels of arginine are associated with an increased risk of mortality in adults with SCD and in young Tanzanian patients. We are investigating the effects of arginine and citrulline supplementation in SCD within the “Vascular Function Intervention Trial”
  4. Effects of Vitamin C and iron status on haemoglobin oxygen saturations and oxygen sensing pathways. In the MSC Vitamin C deficiency is associated with an increased risk of intermittent nocturnal hypoxaemia, which is known to be associated with adverse outcomes in SCD. It is not clear if this association is causal. Oxidant stree, iron status and co-inheritance of alpha-thalassaemia may result in different properties of sickle red cells potentially altering the haemoglobin oxygen dissociation curve and/or the propensity to sickle.

 

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