Inadequate Dietary Intake in Patients with Thalassemia

Abstract

Background Patients with thalassemia have low circulating levels of many nutrients, but the contribution of dietary intake has not been assessed. Objective Assess dietary intake in a large contemporary sample of patients with thalassemia. Design Prospective, longitudinal cohort study using a validated food frequency questionnaire Participants 221 patients (19.7±11.3 yrs, 106 female) categorized into three age groups: young children (3–7.9 y), older children/adolescents (8–18.9 yr), and adult (≥ 19 yr). 78.8% β-thalassemia; 90% chronically transfused. Setting 10 hematology outpatient clinics in the United States and Canada. Main outcome measures Comparison of intake with U.S. Dietary Reference Intakes, and correlation with serum 25-OH vitamin D and total body iron stores. Statistical Analyses Performed Intake was defined as inadequate if less than the estimated average requirement (EAR). Chi-square, Fisher’s exact and Student’s t-test were utilized to compare intake between age categories and logistic regression analysis to test the relationship between intake and outcomes, controlling for age, gender and race. Results Over 30% of patients consumed inadequate levels of vitamin A, D, E, K, folate, calcium, and magnesium. The only nutrients for which >90% of patients consumed adequate amounts were riboflavin, vitamin B12 and selenium. Dietary inadequacy increased with increasing age group (p<0.01) for vitamins A, C, E, B6, folate, thiamin, calcium, magnesium and zinc. Over half the sample took additional supplements of calcium and vitamin D, although circulating levels of 25-OH vitamin D remained insufficient in 61% of patients. Dietary iron intake was not related to total body iron stores. Conclusion Patients with thalassemia have reduced intake of many key nutrients. These preliminary findings of dietary inadequacy is concerning and supports the need for nutritional monitoring to determine which patients are at greatest risk for nutritional deficiency. Future research should focus on the effect of dietary quality and nutritional status on health outcomes in thalassemia.

Children with hemoglobin E/β‐thalassemia have a high risk of being vitamin D deficient even if they get abundant sun exposure: A study from thailand

“…Nevertheless, our results emphasize the importance of monitoring serum vitamin D levels in Hb E/β‐thalassemia children regardless of their clinical severity or the amount of sunlight to which they are exposed. The results of our study indicate that adequate sun exposure is insufficient for adequate vitamin D synthesis in Hb E/β‐thalassemia children and we suggest that early intervention strategies in children with vitamin D deficiency could improve bone health and reduce the risk of fractures .…”

Guidelines for the Standard Monitoring of Patients With Thalassemia

“…Patients who were able to maintain sufficient lean mass and fat stores had greater overall height and more optimal growth patterns, after correction for age, gender, ethnicity and transfusion status [36]. A more recent TCRN cross-sectional analysis suggests that nearly one-third of patients with thalassemia consumed inadequate amounts of nutrients important for optimal growth and bone health, such as calcium, vitamin D and magnesium [38]. …”

“…All patients should have a bone density assessment by dual energy x-ray absorptiometry (DXA) performed annually beginning at age 10 years to evaluate for low bone mass. Treatment options for low bone density include optimization of vitamin D and zinc levels, encouragement of weight bearing activity, and possibly a trial of bisphosphonates [34, 38, 39]. …”

Thalassemia Major and Sickle Cell Disease in Adolescents and Young Adults

“…Growth hormone secretory dysfunction has been described in thalassemia as well as diminished levels of insulin-like growth factor-1 [35,36]; these may also directly contribute to demineralization of the femoral head [37]. Additional factors that affect the tendency for osteoporosis include vitamin D deficiency and depletion of trace elements by poor nutrition combined with chelation therapy [38,39,40,41]. The best way to treat osteoporosis in thalassemics is prevention.…”