The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE

Background & Aims: Sickle cell disease (SCD has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-re...

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Main Authors: Sanaa Kamal, Moheyeldeen Mohamed Naghib, Jamaan Al Zahrani, Huda Hassan, Karim Abdel Aziz Moawado, Omar Arrahman
Format: Article
Language:English
Published: PAGEPress Publications 2021-01-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
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Online Access:http://www.mjhid.org/index.php/mjhid/article/view/4381
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author Sanaa Kamal
Moheyeldeen Mohamed Naghib
Jamaan Al Zahrani
Huda Hassan
Karim Abdel Aziz Moawado
Omar Arrahman
author_facet Sanaa Kamal
Moheyeldeen Mohamed Naghib
Jamaan Al Zahrani
Huda Hassan
Karim Abdel Aziz Moawado
Omar Arrahman
author_sort Sanaa Kamal
collection DOAJ
description Background & Aims: Sickle cell disease (SCD has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-related quality of life (HRQoL) in adult patients, Methods: In this longitudinal study, we recruited and prospectively followed 62 adults with SCD  (aged ?18 years) for a  median of 93 months. At entry and follow-up, patients provided medical and dietary history,  had a physical examination and anthropometric measurements, assessment of protein-energy intake, measurement of micronutrient levels, estimation of  SCD severity score, and determination of the HRQoL  (SF-26v2). The study outcome was a composite of hospitalization due to SCD crises or death. Results: At baseline, 42 (67.74%) patients had macro and, or micro-undernutrition (Group A), and 20 (32.26%) were well nourished. (Group B).  The   BMI and most anthropometric measurements were significantly lower in SCD patients compared to control subjects.  In several SCD patients, undernutrition resulted from reduced food intake and reduced absorption due to concomitant gastrointestinal disorders. Seventy percent of  SCD patients had vitamin D,  vitamin B12, and zinc deficiencies. Under-nourished patients had significantly higher SCD severity scores, frequent SCD related hospitalizations, higher mortality rates, and reduced HRQoL compared to well-nourished patients.  Protein-energy and micronutrient deficiencies were independent predictors of severe SCD and mortality. Correction of undernutrition and hydroxyurea therapy improved SCD severity scores and HRQoL. Conclusion: Patients with sickle cell disease have various degrees of macro and micro deficiencies, which increase SCD severity and hospitalizations and reduce the health-related quality of life. Early diagnosis and prompt correction of macro and micronutrient deficiencies need to be incorporated in the standard of care of SCD patients to improve the disease outcomes.
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spelling doaj-art-f1fea909f20249cb8e964728f07197462025-08-20T02:58:44ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062021-01-0113110.4084/mjhid.2021.007The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASESanaa Kamal0Moheyeldeen Mohamed Naghib1Jamaan Al Zahrani2Huda Hassan3Karim Abdel Aziz Moawado4Omar Arrahman5Prince Sattam Bin Abdul Aziz University College of Medicine, Saudi Arabia / Ain Shams University, Cairo, EgyptPrince Sattam Bin Abdul Aziz University College of Medicine, Saudi Arabia.Prince Sattam Bin Abdul Aziz University College of Medicine, Saudi Arabia Prince Sattam Bin Abdul Aziz University College of Medicine, Saudi Arabia School of Biological Sciences, UCI, California, United States of America King Khaled Hospital, Kharj, Saudi ArabiaBackground & Aims: Sickle cell disease (SCD has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-related quality of life (HRQoL) in adult patients, Methods: In this longitudinal study, we recruited and prospectively followed 62 adults with SCD  (aged ?18 years) for a  median of 93 months. At entry and follow-up, patients provided medical and dietary history,  had a physical examination and anthropometric measurements, assessment of protein-energy intake, measurement of micronutrient levels, estimation of  SCD severity score, and determination of the HRQoL  (SF-26v2). The study outcome was a composite of hospitalization due to SCD crises or death. Results: At baseline, 42 (67.74%) patients had macro and, or micro-undernutrition (Group A), and 20 (32.26%) were well nourished. (Group B).  The   BMI and most anthropometric measurements were significantly lower in SCD patients compared to control subjects.  In several SCD patients, undernutrition resulted from reduced food intake and reduced absorption due to concomitant gastrointestinal disorders. Seventy percent of  SCD patients had vitamin D,  vitamin B12, and zinc deficiencies. Under-nourished patients had significantly higher SCD severity scores, frequent SCD related hospitalizations, higher mortality rates, and reduced HRQoL compared to well-nourished patients.  Protein-energy and micronutrient deficiencies were independent predictors of severe SCD and mortality. Correction of undernutrition and hydroxyurea therapy improved SCD severity scores and HRQoL. Conclusion: Patients with sickle cell disease have various degrees of macro and micro deficiencies, which increase SCD severity and hospitalizations and reduce the health-related quality of life. Early diagnosis and prompt correction of macro and micronutrient deficiencies need to be incorporated in the standard of care of SCD patients to improve the disease outcomes.http://www.mjhid.org/index.php/mjhid/article/view/4381Sickle cell disease, macronutrient, micronutrient, health-related quality of life, vitamin D, zinc
spellingShingle Sanaa Kamal
Moheyeldeen Mohamed Naghib
Jamaan Al Zahrani
Huda Hassan
Karim Abdel Aziz Moawado
Omar Arrahman
The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE
Mediterranean Journal of Hematology and Infectious Diseases
Sickle cell disease, macronutrient, micronutrient, health-related quality of life, vitamin D, zinc
title The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE
title_full The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE
title_fullStr The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE
title_full_unstemmed The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE
title_short The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE
title_sort influence of nutrition on disease severity and health related quality of life in adults with sickle cell disease
topic Sickle cell disease, macronutrient, micronutrient, health-related quality of life, vitamin D, zinc
url http://www.mjhid.org/index.php/mjhid/article/view/4381
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