Open Access Williams-Hooker, J Blood Disorders Transf 2013, 4:3 DOI: 10.4172/2155-9864.1000143 Open Access Volume 4 • Issue 3 • 1000143 J Blood Disorders Transf ISSN: 2155-9864 JBDT, an open access journal Evaluation of Hydration Status, Sodium and Fluid Intake in Children with Sickle Cell Anaemia Ruth Williams-Hooker*, Jane Hankins, Karen Ringwald-Smith, Michelle Stockton, Teresa A Shurley Clinical Nutrition Services, St. Jude Children’s Research Hospital, Memphis, USA *Corresponding author: Ruth Williams-Hooker, Clinical Nutrition Services, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105-3678, USA, Tel: 901-678-3108; E-mail: mrwillia@memphis.edu Received May 07, 2013; Accepted June 27, 2013; Published June 30, 2013 Citation: Williams-Hooker R (2013) Evaluation of Hydration Status, Sodium and Fluid Intake in Children with Sickle Cell Anaemia. J Blood Disorders Transf 4:143. doi:10.4172/2155-9864.1000143 Copyright: © 2013 Williams-Hooker R. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Keywords: Sickle cell anemia; Sickling Introduction In an adequately hydrated person, overall total body water (TBW) accounts for 55-65% of overall body weight [1]. Poor hydration increases the thickness or viscosity of blood. Even mild dehydration can have an impact on hem concentration [1]. In SCA, dehydration can occur for a number of reasons, including but not limited to diarrhea, severe vomiting, excessive sweating, high atmospheric humidity and temperatures, cold temperatures, or inadequate fuid intake [2]. Furthermore, hyposthenuria (inability to concentrate urine resulting in urine of low specifc gravity) is a product of tubular damage, and is present afer 6 - 12 months of age, and becomes progressively worse with age [3-5]. Hypothenuria compounds the risk for dehydration in patients with SCA. When patients with SCA take in too little fuid and too much sodium, this may force water from the red cells and dehydrate the cell, thus the concentration of sickle hemoglobin increases in the red cell. Tis increase in sickle hemoglobin can lead to pain crisis. A study conducted at St. Jude Children’s Research Hospital looked at a relationship between dietary intake of water and sodium in patients with SCA [6]. Researchers found water intake was signifcantly lower than adequate while sodium intake was signifcantly higher than sodium upper limit. An additional beneft of hydration was noted in a study by Beyer and Simmons who found when sickle cell patients increased fuid intake as a comfort measure, venous occlusive crisis (VOC) was decreased [7]. Tere is a defcit in the amount of information regarding hydration status in patients with SCA. In addition, the role of that both dietary water and sodium play in the incidence of VOC and pain crisis in children with SCA is not fully understood. To date, few studies have shown a clear relationship between fuid intake and total body water in SCA. Additional research could help determine if proper hydration may prove to be a simple treatment option, or an adjuvant of other anti- sickling therapies. Tis knowledge could help nutritionists develop diet plans that have a proper balance of all nutrients including water, which may help to minimize the risk of pain crisis. Te purpose of this pilot study was to measure the hydration status utilizing bioelectrical impedance analysis in children with SCA and to determine if sodium and fuid intake were associated with hydration status and pain crises. Methods Subjects Children with sickle cell anemia (Hgb SS or Hb Sβ0-thalassemia genotypes) being treated by a pediatric Sickle Cell Program in Memphis, TN were recruited. Te subject’s physician discussed the study with them, reviewing the pros/cons of the study. Subjects were willing to participate and signed informed consent (and assent if older than 5 years). Inclusion criteria for enrollment were; patients with SCA (HbSS or HbSβ 0 -thalassemia), between the ages of 5 to 17 years, who had the ability to lie still for up to fve minutes and the ability to fast for 12 hours prior to the measurement of total body water analysis. Subjects were excluded if were using diuretics or other medications that could alter hydration status. Subjects were asked to provide details of food and fuid intake for the 24 hours prior to the measurement of TBW, and details on pain crisis, hospitalizations or use of pain medicine 2 weeks prior and 2 weeks afer the TBW analysis. Te study was approved by the Institutional Review Board of the University of Memphis and all research was conducted at Te University of Memphis Human Performance Lab. Total body water analysis Bioelectrical Impedence (BIA) was performed on all subjects to access TBW [8]. Subjects were measured without their right shoe and sock, lying supine, with both arm 30 degrees from the body and legs not touching. Jewelry was also removed before conducting the analysis. Te right hand and foot were cleaned with alcohol before electrodes were positioned. Subjects lay still while the analyzer was turned on and the test was conducted, which took about 3 minutes. Afer completion, the electrodes were removed and discarded. Data needed to calculate TBW (i.e. age, weight, height, resistance, and reactance) was entered into RJL Systems Body Composition Program, Version 2.1. Nutrient analysis A 24 hour food and fuid recall was obtained by the study investigator to assess sodium and fuid intake [9]. Te quantity and time of day the food was consumed, and the name brand of the food if convenience Abstract Sickle cell anemia (SCA) is an inherited condition of chronic hemolytic anemia that includes several different genotypes. People with SCA are encouraged to drink extra fuids in order to prevent dehydration that may lead to red cell sickling and consequently more serious problems. Proper hydration is an important factor in maintaining homeostasis in the body; it is necessary to regulate body temperature, maintain energy levels, aid digestion and eliminate toxins. Research Article Journal of Blood Disorders & Transfusion J o u r n a l o f B l o o d D i s o r d e r s & T r a n s f u s i o n ISSN: 2155-9864