Abstract
We present a study of hydration in ALS patients and its effects on survival. This was a multicenter study over 48 weeks in 80 ALS patients who underwent 250 individual measurements using doubly labeled water (DLW). Total body water (TBW) and water turnover (a surrogate for water intake) were 3.4% and 8.6% lower, respectively, in patients compared to age- and gender-matched healthy controls, and both significantly decreased over study duration. In 20% of patients, water turnover measured over 10 d was 2 standard deviations below the mean value in healthy controls. In a separate clinic cohort of 208 patients, water intake estimated from a de novo equation created from common clinical endpoints was a prognostic indicator of survival. Regardless of nutritional state assessed by BMI, survival was two-fold longer in the group above the median for estimated water intake, suggesting that hydration may be a more important predictor of survival than malnutrition. Risk factors for poor hydration were identified. Water intake equations recommended by US Centers for Medicare and Medicaid Services in healthy elderly were inaccurate for use in ALS patients. We developed equations to estimate TBW and water intake in ALS patients for use in clinics to accurately estimate hydration and improve clinical care.
| Original language | English |
|---|---|
| Pages (from-to) | 220-231 |
| Number of pages | 12 |
| Journal | Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration |
| Volume | 19 |
| Issue number | 3-4 |
| DOIs | |
| State | Published - Apr 3 2018 |
Bibliographical note
Publisher Copyright:© 2017 World Federation of Neurology on behalf of the Research Group on Motor Neuron Diseases.
Funding
This study was supported by the National Institute of Neurological Disorders and Stroke grant RO1 NS045087; the General Clinical Research Centers grants at Columbia University (RR00645), University of Kentucky (RR02602), Penn State University (RR10732 and CO6-RR016499), University of Utah (RR00064) and the University of Vermont (RR00109); the National ALS Association; the ALS Hope Foundation; the Cynthia Shaw Crispen Endowment; the Kevin Heidrich/Team 7 Endowment; and the Department of Neurological Sciences ALS Research Fund, University of Vermont. The authors gratefully acknowledge the enthusiastic participation of ALS patients and caregivers, and Clinical Research Center staff at the participating institutions in undertaking this study. The authors thank Robin Conwit and Janice Cordell of the National Institute of Neurological Disorders and Stroke, and the members of the Data Safety Monitoring Board for their assistance in the course of this study. MBB is a medical consultant for Accordant Health Services and on the speaker’s bureau for Grifols and Genzyme; EJK is a consultant for Neuraltus Pharmaceuticals, Inc. and CDC/ATSDR for the ALS Registry, DSMB member for Cytokinetics, Inc. and has research funding from Neuraltus Pharmaceuticals, Inc., Amylyx Pharmaceuticals, Inc. and Mallinckrodt Pharmaceuticals; HMM is on the Advisory Board of Cytokinetics, Inc., Mitsubishi-Tanabe Pharma and Sunovion Pharmaceuticals and has research funding from the Centers for Disease Control, Cytokinetics, Inc., Muscular Dystrophy Association, Tsumura & Co., Wings Over Wall Street Fund and Spastic Paraplegia Foundation; ZS is the Editor-in-Chief of Muscle & Nerve, a medical consultant for Cytokinetics, Inc. and Neuralstem, Inc. and has research funding from Biogen, Inc., Cytokinetics, Inc. and GlaxoSmithKline; RT is a medical advisory board member of the Myasthenia Gravis Foundation of America, a member of the Medical Education Council at Option Care and has research funding from Alexion Pharmaceuticals, Inc., Cytokinetics, Inc., Grifols, Inc., National ALS Association, UCB Pharma and Geisel School of Medicine at Dartmouth. CNS, DBH and DEM have no conflict of interest. The authors alone are responsible for the content and writing of this paper.
| Funders | Funder number |
|---|---|
| Accordant Health Services | |
| Advisory Board of Cytokinetics, Inc. | |
| Alexion Pharmaceuticals, Inc., Cytokinetics, Inc. | |
| Amylyx Pharmaceuticals, Inc. | |
| Biogen, Inc., Cytokinetics, Inc. | |
| Centers for Disease Control, Cytokinetics, Inc. | |
| Cynthia Shaw Crispen Endowment | |
| Biogen, Inc., Cytokinetics, Inc. | |
| Editor-in-Chief of Muscle & Nerve, a medical consultant for Cytokinetics, Inc. | |
| Kevin Heidrich/Team 7 Endowment | |
| Accreditation Council for Graduate Medical Education | |
| National ALS Association | |
| Neuralstem, Inc. | |
| Neuraltus Pharmaceuticals, Inc. | |
| Spastic Paraplegia Foundation | |
| Tsumura and Co. | |
| Institute of Neurological Disorders and Stroke National Advisory Neurological Disorders and Stroke Council | RO1 NS045087 |
| Institute of Neurological Disorders and Stroke National Advisory Neurological Disorders and Stroke Council | |
| National Center for Research Resources | M01RR000109 |
| National Center for Research Resources | |
| ALS Hope Foundation | |
| Muscular Dystrophy Association | |
| Agency for Toxic Substances and Disease Registry | |
| North Carolina GlaxoSmithKline Foundation | |
| Columbia University | RR00645 |
| Columbia University | |
| University of Kentucky | RR02602 |
| University of Kentucky | |
| University of Utah Health | RR00064 |
| University of Utah Health | |
| The Pennsylvania State University | RR10732, CO6-RR016499 |
| The Pennsylvania State University | |
| University of Vermont | RR00109 |
| University of Vermont |
Keywords
- ALS
- doubly labeled water
- hydration
- survival
- total body water
- water intake
- water turnover
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
