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Better Health for Sufferers of Genetic Anemias May Be as Near as the Kitchen

By Amy Danzig

NHLBI's Division of Blood Diseases and Resources recently hosted the first-ever meeting of experts on the topic of nutrition and the genetic anemias. Workshops on "Nutrient Metabolism in the Genetic Anemias" and "Dietary Supplements of Potential Benefit to Patients with Sickle Cell Disease" focused on dietary supplements, particularly those that might help patients with sickle cell disease (SCD) and thalassemia. Researchers shared the status and results of current work and made recommendations to NHLBI regarding future directions. Cosponsors with DBDR were the NIH Office of Rare Diseases and the Office of Dietary Supplements.

"We have been aware of anecdotal information for many years that the deficit in growth and development seen in children with genetic anemias is at least partially due to nutritional deficiencies," said Dr. Carol Letendre, DBDR deputy director. "However, this is the first time that sufficient methods to accurately determine the nutritional deficit and to systematically approach evidence-based nutritional therapy for these patients have been presented."

Speakers pointed to exciting preliminary findings and called for further studies to address a variety of knowledge and clinical practice needs. Topics covered during the 2-day meeting included the need for better evaluation of the macro/micronutrient requirements of children with thalassemia and SCD; the role magnesium plays in reducing the number of painful days experienced by people with SCD; and the ability of an antioxidant "cocktail" of aged garlic extract, vitamin C and vitamin E to inhibit the formation of dehydrated dense cells in patients with SCD.

Several presenters gave findings from an NHLBI-funded 5-year longitudinal growth and nutritional status study of SCD being done at the Comprehensive Sickle Cell Center, Children's Hospital of Philadelphia. The study seeks to determine why participants — children with SCD, age birth to 18 years at enrollment — showed poor growth in height, weight, fat, muscle and bone mass.

One part of the study showed that resting energy expenditure (REE) during periods of usual health were higher, and activity energy expenditure significantly lower, in children wth SCD. During acute illness, REE in children with SCD was not elevated above the level measured during usual health. However, dietary intake was significantly reduced during illness. This contributed to the overall pattern of energy deficits observed in children with SCD. It also may have contributed to the observed growth failure, altered body composition and lower physical activity in these children. The researchers noted the need for further studies to determine the best approaches to treatment and prevention of undernutrition in children with SCD.

Another group involved in the Philadelphia study is looking specifically at the effects of zinc. The two-fold goal of this work is to test the feasibility of using a zinc supplement in children and adolescents with SCD and to collect data in preparation for a collaborative multicenter intervention trial.

Elsewhere, NHLBI-funded researchers at the University of Pennsylvania School of Medicine and University of Ibadan, Nigeria, are looking at SCD as a membrane disease. The investigators are testing an antioxidant cocktail of aged garlic extract, vitamin C and vitamin E. There is evidence that this combination inhibits the formation of dehydrated dense cells by protecting red cell membranes from free radical-mediated oxidative stress. A pilot clinical trial at the University of Ibadan has had promising results. The researchers note that since the cocktail consists of established dietary supplements, the therapy would be safe for patients.

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