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

Main Article Content

Sanaa Kamal
Moheyeldeen Mohamed Naghib
Jamaan Al Zahrani
Huda Hassan
Karim Abdel Aziz Moawado
Omar Arrahman

Keywords

Sickle cell disease, macronutrient, micronutrient, health-related quality of life, vitamin D, zinc

Abstract

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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References

1. Piel FB, Steinberg MH, Rees DC. Sickle Cell Disease. N Engl J Med 2017 April 376;16: 1561-1573
https://doi.org/10.1056/NEJMra1510865 PMid:28423290
2. Modell B, Darlison M Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 2008; 86: 480-487 https://doi.org/10.2471/BLT.06.036673 PMid:18568278 PMCid:PMC2647473
3. Macharia AW, Mochamah G, Uygoga S, Ndia CM et al. The clinical epidemiology of sickle cell anemia In Africa. Am J Hematol. 2018 Mar; 93(3): 363-370. https://doi.org/10.1002/ajh.24986 PMid:29168218 PMCid:PMC6175377
4. Hassell KL. Population estimates of sickle cell disease in the US. Am J Prev Med. 2010;38(4 suppl):S512-S521
https://doi.org/10.1016/j.amepre.2009.12.022 PMid:20331952
5. Piel FB, Talem AJ, Huang Z, Gupta S, Williams TN, Weatherall DJ. Global migration and the changing distribution of sickle haemoglobin: a quantitative study of temporal trends between 1960 and 2000. Lancet Glob Health. 2014 Feb; 2(2): e80-e89. https://doi.org/10.1016/S2214-109X(13)70150-5
6. Mohieldin Elsayid, Mohammed Jahman Al-Shehri, Yasser Abdullah Alkulaibi, Abdullah Alanazi, Shoeb Qureshi. Frequency distribution of sickle cell anemia, sickle cell trait and sickle/beta-thalassemia among anemic patients in Saudi Arabia. J Nat Sci Biol Med. 2015 Aug; 6(Suppl 1): S85-S88. https://doi.org/10.4103/0976-9668.166093 PMid:26604627 PMCid: PMC4630771
7. Jastaniah W. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med. 2011 May-Jun; 31(3): 289-293.
https://doi.org/10.5144/0256-4947.2011.289 PMid:21623060 PMCid:PMC3119971
8. Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019 Jan 24;14:263-292
https://doi.org/10.1146/annurev-pathmechdis-012418-012838 PMid:30332562 PMCid:PMC70535589.
9. Manwani D, Frenette PS. Vaso-occlusion in sickle cell disease: Pathophysiology and novel targeted therapies. Blood 2013 Dec 5;122(24):3892-8. https://doi.org/10.1182/blood-2013-05-498311 PMid:24052549 PMCid:PMC3854110
10. Quinn CT. Clinical severity in sickle cell disease: The challenges of definition and prognostication. Exp Biol Med. 2016 Apr; 241(7):679-88 https://doi.org/10.1177/1535370216640385 PMid:27013545 PMCid:PMC4871738
11. Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA acute care utilization and re-hospitalizations for sickle cell disease. JAMA, 2010; 303: 1288-1294. https://doi.org/10.1001/jama.2010.378 PMid:20371788
12. Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, Wethers DL, Smith J, Kinney TR. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 2000; 342: 83-9.
https://doi.org/10.1056/NEJM200001133420203 PMid:10631276
13. Sebastiani P, Nolan VG, Baldwin CT, Abad-Grau MM, Wang L, Adewoye AH, Lillian C McMahon, Farrer L, Taylor J, Kato GJ, Gladwin MT, Steinberg MH. A network model to predict the risk of death in sickle cell disease. Blood. 2007 Oct 1;110(7):2727-35. https://doi.org/10.1182/blood-2007-04-084921 PMid:17600133 PMCid:PMC1988954
14. Burke L, Hobart JC, Fox C, Lehrer-Graiwer,J, Bridges K, Kraus M, Rames F. The 10-Item sickle cell disease severity measure (SCDSM-10): A novel measure of daily SCD symptom severity developed to assess benefit of GBT440, an experimental HbS polymerization inhibitor. Blood 2016; 128 (22): 4760.m https://doi.org/10.1182/blood.V128.22.4760.4760
15. Cameron BF, Christian E, Lobel JS, Gaston MH. Evaluation of clinical severity in sickle cell disease. J Natl Med Assoc 1983 May;75(5):483-7.13
16. Day SW. Development and evaluation of a sickle cell assessment instrument. Pediatr Nurs 2004 Nov;30 (6):451- 8.
17. Adekile A, Kutlar F, McKie K, Addington A, Elam D, Holley L, Clair B, Kutlar A. The influence of uridine diphosphate glucuronosyl transferase 1A promoter polymorphisms, beta-globin gene haplotype, co-inherited alpha-thalassemia trait and Hb F on steady-state serum bilirubin levels in sickle cell anemia. Eur J Haematol 2005; 75: 150-5.
https://doi.org/10.1111/j.1600-0609.2005.00477.x PMid:16004608
18. Miller ST, Milton J, Steinberg MH. G6PD deficiency and stroke in the CSSCD. Am J Hematol 2011; 86: 331-331.
https://doi.org/10.1002/ajh.21958 PMid:21328436
19. Piel FB, Tewari S, Brousse V, Analitis A, Font A, Menzel S, Chakravorty S, Thein SL, Inusa B, Telfer P, de Montalembert M, Fuller GW, Katsouyanni K, Rees DC. Associations between environmental factors and hospital admissions for sickle cell disease. Haematologica. 2017 Apr;102(4):666-675. https://doi.org/10.3324/haematol.2016.154245
PMid:27909222 PMCid:PMC5395107
20. Mekontso Dessap A, Contou D, Dandine-Roulland C, Hemery F, Habibi A, Charles-Nelson A, Galacteros F, Brun-Buisson C, Maitre B, Katsahian S. Environmental influences on daily emergency admissions in sickle-cell disease patients. Medicine (Baltimore). 2014 Dec;93(29):e280. https://doi.org/10.1097/MD.0000000000000280
PMid:25546672 PMCid:PMC4602624
21. Cano N, Melchior JC. Malnutrition in chronic diseases. Rev Prat. 2003;53(3):268-73.
22. Von Ruesten A, Feller S, Bergmann MM, Boeing H. Diet and risk of chronic diseases: results from the first 8 years of follow-up in the EPIC-Potsdam study. European Journal of Clinical Nutrition 2013; 67: 412-419.
https://doi.org/10.1038/ejcn.2013.7 PMid:23388667
23. Saunders J. Malnutrition: causes and consequences. Clin Med (Lond). 2010 Dec; 10(6): 624-627.
https://doi.org/10.7861/clinmedicine.10-6-624 PMid:21413492 PMCid:PMC4951875
24. Bourke CD, Berkley JA, Prendergast AJ. Immune dysfunction as a cause and consequence of malnutrition. Trends Immunol. 2016 Jun; 37(6): 386-398. https://doi.org/10.1016/j.it.2016.04.003 PMid:27237815 PMCid:PMC4889773
25. Foucher J, Chanteloup E, Vergniol J, Castéra L, Bail B, Adhoute X, et al. Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study. Gut 2006;55(3):403?8. https://doi.org/10.1136/gut.2005.069153
PMid:16020491 PMCid:PMC1856085
26. Subjective global assessment form (Internet) available at: https://www.rand.org/health-care/surveys_tools/mos/36-item-short-form.html . Accessed on: 14/2/2020..
27. World Health Organization.. WHO Global Database on Body Mass Index. WHO-BMI 2018 [Internet]. Retrieved from http://apps.who.int/bmi/index.jsp on 16/1/2020
28. Jackson A S, Pollock, M. Practical assessment of body composition. Physician Sport Med. 1985; 13: 76-90.
https://doi.org/10.1080/00913847.1985.11708790 PMid:27463295
29. World Health Organization. Use and interpretation of anthropometric indicators of nutritional status. Bull World Health Organ 1986; 64, 929-941.
30. Nevitt SJ, Jones AP, Howard J. Hydroxyurea (hydroxycarbamide) for sickle cell disease. . Cochrane Database Syst Rev. 2017 Apr 20;4(4):CD002202 https://doi.org/10.1002/14651858.CD002202.pub2
PMid:28426137 PMCid:PMC6478259
31. The 36-Item Short Form Survey (SF-36) (Internet) available at: https://www.physio-pedia.com/36-Item_Short_Form_Survey_(SF-36)
32. Coons S, Abdulmohsin SA, Draugalis JR, Hays RD. Reliability of an Arabic Version of the RAND-36 Health Survey and Its Equivalence to the US-English Version. Medical Care, 1998; 36, (3): 428-43 https://doi.org/10.1097/00005650-199803000-00018 PMid:9520966
33. Hankins JS, Estepp JH, Hodges JR, Villavicencio MA, Robison LL, Weiss MJ, Kang G, Schreiber JE, Porter JS, Kaste SC, Saving KL, Bryant PC, Deyo JE, Nottage KA, King AA, Brandow AM, Lebensburger JD, Adesina O, Chou ST, Zemel BS, Smeltzer MP, Wang WC, Gurney JG. Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood. Pediatr Blood Cancer. 2018 Sep;65(9):e27228 https://doi.org/10.1002/pbc.27228 PMid:29797644
34. Oyedeji C, Strouse JJ, Crawford RD, Garrett ME, Ashley-Koch AE, Telen MJ. A multi-institutional comparison of younger and older adults with sickle cell disease. Am J Hematol. 2019 Apr;94(4):E115-E117.
https://doi.org/10.1002/ajh.25405 PMid:30663090 PMCid:PMC6449149
35. Ruiz AJ, Buitrago G, Rodríguez N, Gómez G, Sulo S, Gómez C, Partridge J, Misas J, Dennis R, Alba MJ, Chaves-Santiago W, Araque Clinical and economic outcomes associated with malnutrition in hospitalized patients. J Clin Nutr. 2019 Jun;38(3):1310-1316. https://doi.org/10.1016/j.clnu.2018.05.016 PMid:29891224
36. Lebwohl B., Sanders D.S., Green P.H.R. Coeliac Disease. Lancet. 2018;391:70-81 https://doi.org/10.1016/S0140-6736(17)31796-8
37. Pulley J, Todd A, Flatley C, Begun J. Malnutrition and quality of life among adult inflammatory bowel disease patients. Journal Gastentol Hepatol (JGH). 2020; 4 (3): 454-460 https://doi.org/10.1002/jgh3.12278
PMid:32514453 PMCid:PMC7273715
38. Huisman EJ, Trip EJ, Siersema PD, van Hoek B, van Erpecum KJ. Protein-energy malnutrition predicts complications in liver cirrhosis. Eur J Gastroenterol Hepatol 2011;23:982-989 https://doi.org/10.1097/MEG.0b013e32834aa4bb
PMid:21971339
39. Saeed A, Assir Ai, Assiri H, Ullah A, Rashid M. Celiac disease in Saudi children: Evaluation of clinical features and diagnosis. 2017 Saudi Med J; 38(9): 895-899. https://doi.org/10.15537/smj.2017.9.20808
PMid:28889146 PMCid:PMC5654022
40. Fadda A, Peedikayil MC, Kagevi I, Al Kahtani K, Ben Mousa A, Al Ashgar HI, Al Sohaiban Hi, Al Quaiz M, Abdulla A, Khan MQ, Helmy A. Inflammatory bowel disease in Saudi Arabia: a hospital-based clinical study of 312 patients. 2012 Ann Saudi Med; 32(3): 276-282. https://doi.org/10.5144/0256-4947.2012.276 PMid:22588439 PMCid:PMC6081028
41. Lee MT, Licursi M, McMahon DJ. Vitamin D deficiency and acute vaso-occlusive complications in children with sickle cell disease. Pediatr Blood Cancer; 2015;62(4):643-7. https://doi.org/10.1002/pbc.25399
PMid:25641631
42. Ajayi OI, Bwayo-Weaver S, Chirla S, Serlemitsos-Day M, Daniel M, Nouraie M, Edwards K, Castro O, Lombardo F, Gordeuk VR Cobalamin status in sickle cell disease. .Int J Lab Hematol. 2013 Feb;35(1):31-7.
https://doi.org/10.1111/j.1751-553X.2012.01457.x PMid:22830455 PMCid:PMC3484229
43. Antwi-Boasiako C, Dankwah GB, Aryee R, Hayfron-Benjamin C, Doku A, N'guessan BB, Asiedu-Gyekye IJ, Campbell AD. Serum iron levels and copper-to-zinc ratio in sickle cell disease. Medicina 2019 ; 55(5): 180.
https://doi.org/10.3390/medicina55050180 PMid:31117252 PMCid:PMC6572688
44. Martyres DJ, Vijenthira A , Barrowman N, Harris-Janz S, Chretien C, Klaassen RJ. Nutrient insufficiencies/deficiencies in children with sickle cell disease and its association with increased disease severity. Pediatr Blood Cancer, 2016;63(6):1060-4. https://doi.org/10.1002/pbc.25940 PMid:26855061
45. Mandese V, Marotti F, Bedetti L, Bigi E, Palazzi G, Iughetti L. Effects of nutritional intake on disease severity in children with sickle cell disease. Nutr J. 2016 Apr 30;15(1):46. https://doi.org/10.1186/s12937-016-0159-8
PMid:27130184 PMCid:PMC4851811