Asthma in Children with Sickle Cell Disease
Main Article Content
Abstract
Sickle Cell Disease is a life-threatening hereditary blood disorder which affects millions of people worldwide. Pulmonary complications are important causes of morbidity and mortality in patients with sickle cell disease. Asthma is a recognised comorbidity of sickle cell disease and may occur in between 15 and 28% of children with sickle cell disease. It has been associated with increased episodes of acute chest syndrome and all cause mortality. Obstructive lung disease, however, is common in children with sickle cell disease, independent of an asthma diagnosis. This review explores the pathophysiology, diagnosis and therapeutic opportunities for asthma in sickle cell disease patients. The diagnostic challenges and inconsistencies in current clinical approaches are highlighted. Convergence of inflammatory pathways in sickle cell disease and asthma occurs, but there is also a heightened level of inflammation unique to sickle cell disease. Thus, wheezing may not be due to asthma but be a manifestation of sickle cell disease per se and the result of the increased pulmonary vascular volume. As a consequence, anti-asthma therapy may not be appropriate for all wheezy children with sickle cell disease and commencing treatment on the basis of a physician’s diagnosis alone is inappropriate. Data from paediatric cohorts suggest use of spirometry, aeroallergen sensitisation tests, impulse oscillometry and dedicated interdisciplinary pulmonary clinics could improve diagnosis accuracy. Corticosteroids and bronchodilators are well-established treatments for asthma; observational studies suggest they may provide benefit for some children with sickle cell disease, but therapies such as hydroxyurea may improve respiratory outcomes in others. It is, therefore, essential children are thoroughly investigated and followed-up and a personalised approach taken to their care. Prospective randomised studies are required to establish the effectiveness of asthma therapies in children with sickle cell disease.
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How to Cite
SIKDAR, Oishi; GREENOUGH, Anne.
Asthma in Children with Sickle Cell Disease.
Medical Research Archives, [S.l.], v. 11, n. 5, may 2023.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/3567>. Date accessed: 26 apr. 2024.
doi: https://doi.org/10.18103/mra.v11i5.3567.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
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4. Jain S, Bakshi N, Krishnamurti L. Acute chest syndrome in children with sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2017;30(4):191-201.
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6. Ferrante G, La Grutta S. The burden of pediatric asthma. Front Pediatr. 2018;6:186.
7. Singh AM, Busse WW. Asthma exacerbations 2: Aetiology. Thorax. 2006;61(9):809-816.
8. DeBaun MR, Strunk RC. The intersection between asthma and acute chest syndrome in children with sickle-cell anaemia. Lancet. 2016;387(10037):2545-2553.
9. Field JJ, DeBaun MR. Asthma and sickle cell disease: two distinct diseases or part of the same process? Hematol. 2009;2009(1):45-53.
10. DeBaun MR, Rodeghier M, Cohen R, et al. Factors predicting future ACS episodes in children with sickle cell anemia. Am J Hematol. 2014;89(11):E212-E217.
11. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323.
12. Huo J, Xiao H, Garg M, Shah C, Wilkie D, Mainous III A. The economic burden of sickle cell disease in the United States. Value in Health. 2018;21:S108.
13. Nurmagambetov T, Kuwahara R, Garbe P. The economic burden of asthma in the United States, 2008–2013. Ann Am Thorac Soc. 2018;15(3):348-356.
14. Samarasinghe AE, Rosch JW. Convergence of inflammatory pathways in allergic asthma and sickle cell disease. Front Immunol. 2020;10:3058.
15. Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108(9):2923-2927.
16. An P, Barron-Casella EA, Strunk RC, Hamilton RG, Casella JF, DeBaun MR. Elevation of IgE in children with sickle cell disease is associated with doctor diagnosis of asthma and increased morbidity. J Allergy Clin Immunol. 2011;127(6):1440-1446.
17. Faith A, Ganiyu A, John O, et al. Increased IgE level in Nigerian sickle cell disease children: Implication for severity of allergic reactions. J Appl Hematol. 2013;4(4):149.
18. Nandedkar SD, Feroah TR, Hutchins W, et al. Histopathology of experimentally induced asthma in a murine model of sickle cell disease. Blood. 2008;112(6):2529-2538.
19. Conran N, Saad STO, Costa FF, Ikuta T. Leukocyte numbers correlate with plasma levels of granulocyte–macrophage colony-stimulating factor in sickle cell disease. Ann Hematol. 2007;86(4):255-261.
20. Profita M, Sala A, Bonanno A, et al. Increased prostaglandin E2 concentrations and cyclooxygenase-2 expression in asthmatic subjects with sputum eosinophilia. J Allergy Clin Immunol. 2003;112(4):709-716.
21. De A, Agrawal S, Morrone K, et al. Airway inflammation and lung function in sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2019;32(3):92-102.
22. Knight-Perry J, DeBaun MR, Strunk RC, Field JJ. Leukotriene pathway in sickle cell disease: a potential target for directed therapy. Expert Rev Hematol. 2009;2(1):57-68.
23. Benson RC, Hardy KA, Morris CR. Arginase and arginine dysregulation in asthma. J Allergy. 2011;2011:1-12.
24. Morris CR. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAMA. 2005;294(1):81.
25. Lunt A, Sturrock SS, Greenough A. Asthma and the outcome of sickle cell disease. Expert Opinion Orphan Drugs. 2018;6(12):733-740.
26. Rath M, Muller I, Kropf P, Closs EI, Munder M. Metabolism via arginase or nitric oxide synthase: two competing arginine pathways in macrophages. Front Immunol. 2014;5:532.
27. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. New Engl J Med. 1986;314(25):1593-1599.
28. Marra F, Lynd L, Coombes M, et al. Does antibiotic exposure during infancy lead to development of asthma? Chest. 2006;129(3):610-618.
29. Murk W, Risnes KR, Bracken MB. Prenatal or early-life exposure to antibiotics and risk of childhood asthma: a systematic review. Pediatrics. 2011;127(6):1125-1138.
30. Ver Heul A, Planer J, Kau AL. The human microbiota and asthma. Clin Rev Allergy Immunol. 2018;57(3):350-363.
31. Zeissig S, Blumberg RS. Life at the beginning: perturbation of the microbiota by antibiotics in early life and its role in health and disease. Nat Immunol. 2014;15(4):307-310.
32. Schulfer A, Blaser MJ. Risks of antibiotic exposures early in life on the developing microbiome. PLoS Pathog. 2015;11(7):e1004903.
33. Chaturvedi S, Ghafuri DL, Glassberg J, Kassim AA, Rodeghier M, DeBaun MR. Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype. Am J Hematol. 2016;91(12):1185-1190.
34. Bendiak GN, Mateos-Corral D, Sallam A, et al. Association of wheeze with lung f function decline in children with sickle cell disease. Eur Respir J. 2017;50(5):1602433.
35. Lunt A, Desai SR, Wells AU, et al. Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease. Thorax. 2014;69(8):746-751.
36. Wedderburn CJ, Rees D, Height S, et al. Airways obstruction and pulmonary capillary blood volume in children with sickle cell disease. Pediatr Pulmonol. 2013;49(7):716-722.
37. Lunt A, McGree E, Robinson P, Rees D, Height S, Greenough A. Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease. Respir Physiol Neurobiol. 2016;222:6-10.
38. McLaren A, Klingel M, Behera S, Odame I, Kirby-Allen M, Grasemann H. Effect of hydroxyurea therapy on pulmonary function in children with sickle cell anemia. Am J Respir Crit Care Med. 2017;195(5):689-691.
39. Malinovschi A, Janson C, Borres M, Alving K. Simultaneously increased fraction of exhaled nitric oxide levels and blood eosinophil counts relate to increased asthma morbidity. J Allergy Clin Immunol. 2016;138(5):1301-1308.e2.
40. Arigliani M, Gupta A. Management of chronic respiratory complications in children and adolescents with sickle cell disease. Eur Respir Rev. 2020;29(157):200054.
41. Lunt A, Ahmed N, Rafferty GF, et al. Airway and alveolar nitric oxide production, lung function, and pulmonary blood flow in sickle cell disease. Pediatr Res. 2016;79(2):313-317.
42. Chaudry RA, Rosenthal M, Bush A, Crowley S. Reduced forced expiratory flow but not increased exhaled nitric oxide or airway responsiveness to methacholine characterises paediatric sickle cell airway disease. Thorax. 2014;69(6):580-585.
43. Cohen RT, Rodeghier M, Kirkham FJ, et al. Exhaled nitric oxide: Not associated with asthma, symptoms, or spirometry in children with sickle cell anemia. J Allergy Clin Immunol. 2016;138(5):1338-1343.e4.
44. Strunk RC, Cohen RT, Cooper BP, et al. Wheezing symptoms and parental asthma are associated with a physician diagnosis of asthma in children with sickle cell anemia. J Pediatr. 2014;164(4):821-826.e1.
45. Patel AP, Krupani S, Stark JM, et al. Validation of the breathmobile case identification survey for asthma screening in children with sickle cell disease. J Asthma. 2020;58(6):782-790.
46. Kassim AA, Payne AB, Rodeghier M, Macklin EA, Strunk RC, DeBaun MR. Low forced expiratory volume is associated with earlier death in sickle cell anemia. Blood. 2015;126(13):1544-1550.
47. Intzes S, Kalpatthi RV, Short R, Imran H. Pulmonary function abnormalities and asthma are prevalent in children with sickle cell disease and are associated with acute chest syndrome. Pediatr Hematol Oncol. 2013;30(8):726-732.
48. Duckworth L, Black LV, Ezmigna D, et al. Spirometry use in patients with sickle cell disease with and without asthma and acute chest syndrome: A multicenter study. EJHaem. 2020;1(1):239-242.
49. Arteta M, Campbell A, Nouraie M, et al. Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 2014;36(3):185-189.
50. Murray C, Foden P, Lowe L, Durrington H, Custovic A, Simpson A. Diagnosis of asthma in symptomatic children based on measures of lung function: an analysis of data from a population-based birth cohort study. Lancet Child Adolesc Health. 2017;1(2):114-123.
51. Mondal P, Yirinec A, Midya V, et al. Diagnostic value of spirometry vs impulse oscillometry: A comparative study in children with sickle cell disease. Pediatr Pulmonol. 2019;54(9):1422-1430.
52. Knight-Madden JM, Hambleton IR. Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2022;12(12):CD003733.
53. Kopel LS, Klings ES, Monuteaux MC, Gaffin JM, Heeney MM, Phipatanakul W. Bronchodilator use for acute chest syndrome among large pediatric hospitals in North America. Clin Pediatr. 2018;57(14):1630-1637.
54. Bokov P, El Jurdi H, Denjoy I, et al. Salbutamol worsens the autonomic nervous system dysfunction of children with sickle cell disease. Front Physiol. 2020;11:31.
55. Griffin TC, McIntire D, Buchanan GR. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. New Engl J Med. 1994;330(11):733-737.
56. Bernini JC, Rogers ZR, Sandler ES, Reisch JS, Quinn CT, Buchanan GR. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood. 1998;92(9):3082-3089.
57. Darbari DS, Fasano RS, Minniti CP, et al. Severe vaso-occlusive episodes associated with use of systemic corticosteroids in patients with sickle cell disease. J Natl Med Assoc. 2008;100(8):948-951.
58. Kumar R, Qureshi S, Mohanty P, Rao SP, Miller ST. A short course of prednisone in the management of acute chest syndrome of sickle cell disease. J Pediatr Hematol Oncol. 2010;32(3):e91-e94.
59. Sobota A, Graham DA, Heeney MM, Neufeld EJ. Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission. Am J Hematol. 2010;85(1):24-28.
60. Field JJ, Kassim A, Brandow A, et al. Phase 2 trial of montelukast for prevention of pain in sickle cell disease. Blood Adv. 2020;4(6):1159-1165.
2. Lubeck D, Agodoa I, Bhakta N, et al. Estimated life expectancy and income of patients with sickle cell disease compared with those without sickle cell disease. JAMA Network Open. 2019;2(11):e1915374.
3. Pauling L, Itano H, Singer SJ, Wells I. Sickle cell anemia, a molecular disease. Science. 1949;110(2865):543-548.
4. Jain S, Bakshi N, Krishnamurti L. Acute chest syndrome in children with sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2017;30(4):191-201.
5. Castro O, Brambilla D, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994;84(2):643-649.
6. Ferrante G, La Grutta S. The burden of pediatric asthma. Front Pediatr. 2018;6:186.
7. Singh AM, Busse WW. Asthma exacerbations 2: Aetiology. Thorax. 2006;61(9):809-816.
8. DeBaun MR, Strunk RC. The intersection between asthma and acute chest syndrome in children with sickle-cell anaemia. Lancet. 2016;387(10037):2545-2553.
9. Field JJ, DeBaun MR. Asthma and sickle cell disease: two distinct diseases or part of the same process? Hematol. 2009;2009(1):45-53.
10. DeBaun MR, Rodeghier M, Cohen R, et al. Factors predicting future ACS episodes in children with sickle cell anemia. Am J Hematol. 2014;89(11):E212-E217.
11. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323.
12. Huo J, Xiao H, Garg M, Shah C, Wilkie D, Mainous III A. The economic burden of sickle cell disease in the United States. Value in Health. 2018;21:S108.
13. Nurmagambetov T, Kuwahara R, Garbe P. The economic burden of asthma in the United States, 2008–2013. Ann Am Thorac Soc. 2018;15(3):348-356.
14. Samarasinghe AE, Rosch JW. Convergence of inflammatory pathways in allergic asthma and sickle cell disease. Front Immunol. 2020;10:3058.
15. Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108(9):2923-2927.
16. An P, Barron-Casella EA, Strunk RC, Hamilton RG, Casella JF, DeBaun MR. Elevation of IgE in children with sickle cell disease is associated with doctor diagnosis of asthma and increased morbidity. J Allergy Clin Immunol. 2011;127(6):1440-1446.
17. Faith A, Ganiyu A, John O, et al. Increased IgE level in Nigerian sickle cell disease children: Implication for severity of allergic reactions. J Appl Hematol. 2013;4(4):149.
18. Nandedkar SD, Feroah TR, Hutchins W, et al. Histopathology of experimentally induced asthma in a murine model of sickle cell disease. Blood. 2008;112(6):2529-2538.
19. Conran N, Saad STO, Costa FF, Ikuta T. Leukocyte numbers correlate with plasma levels of granulocyte–macrophage colony-stimulating factor in sickle cell disease. Ann Hematol. 2007;86(4):255-261.
20. Profita M, Sala A, Bonanno A, et al. Increased prostaglandin E2 concentrations and cyclooxygenase-2 expression in asthmatic subjects with sputum eosinophilia. J Allergy Clin Immunol. 2003;112(4):709-716.
21. De A, Agrawal S, Morrone K, et al. Airway inflammation and lung function in sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2019;32(3):92-102.
22. Knight-Perry J, DeBaun MR, Strunk RC, Field JJ. Leukotriene pathway in sickle cell disease: a potential target for directed therapy. Expert Rev Hematol. 2009;2(1):57-68.
23. Benson RC, Hardy KA, Morris CR. Arginase and arginine dysregulation in asthma. J Allergy. 2011;2011:1-12.
24. Morris CR. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAMA. 2005;294(1):81.
25. Lunt A, Sturrock SS, Greenough A. Asthma and the outcome of sickle cell disease. Expert Opinion Orphan Drugs. 2018;6(12):733-740.
26. Rath M, Muller I, Kropf P, Closs EI, Munder M. Metabolism via arginase or nitric oxide synthase: two competing arginine pathways in macrophages. Front Immunol. 2014;5:532.
27. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. New Engl J Med. 1986;314(25):1593-1599.
28. Marra F, Lynd L, Coombes M, et al. Does antibiotic exposure during infancy lead to development of asthma? Chest. 2006;129(3):610-618.
29. Murk W, Risnes KR, Bracken MB. Prenatal or early-life exposure to antibiotics and risk of childhood asthma: a systematic review. Pediatrics. 2011;127(6):1125-1138.
30. Ver Heul A, Planer J, Kau AL. The human microbiota and asthma. Clin Rev Allergy Immunol. 2018;57(3):350-363.
31. Zeissig S, Blumberg RS. Life at the beginning: perturbation of the microbiota by antibiotics in early life and its role in health and disease. Nat Immunol. 2014;15(4):307-310.
32. Schulfer A, Blaser MJ. Risks of antibiotic exposures early in life on the developing microbiome. PLoS Pathog. 2015;11(7):e1004903.
33. Chaturvedi S, Ghafuri DL, Glassberg J, Kassim AA, Rodeghier M, DeBaun MR. Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype. Am J Hematol. 2016;91(12):1185-1190.
34. Bendiak GN, Mateos-Corral D, Sallam A, et al. Association of wheeze with lung f function decline in children with sickle cell disease. Eur Respir J. 2017;50(5):1602433.
35. Lunt A, Desai SR, Wells AU, et al. Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease. Thorax. 2014;69(8):746-751.
36. Wedderburn CJ, Rees D, Height S, et al. Airways obstruction and pulmonary capillary blood volume in children with sickle cell disease. Pediatr Pulmonol. 2013;49(7):716-722.
37. Lunt A, McGree E, Robinson P, Rees D, Height S, Greenough A. Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease. Respir Physiol Neurobiol. 2016;222:6-10.
38. McLaren A, Klingel M, Behera S, Odame I, Kirby-Allen M, Grasemann H. Effect of hydroxyurea therapy on pulmonary function in children with sickle cell anemia. Am J Respir Crit Care Med. 2017;195(5):689-691.
39. Malinovschi A, Janson C, Borres M, Alving K. Simultaneously increased fraction of exhaled nitric oxide levels and blood eosinophil counts relate to increased asthma morbidity. J Allergy Clin Immunol. 2016;138(5):1301-1308.e2.
40. Arigliani M, Gupta A. Management of chronic respiratory complications in children and adolescents with sickle cell disease. Eur Respir Rev. 2020;29(157):200054.
41. Lunt A, Ahmed N, Rafferty GF, et al. Airway and alveolar nitric oxide production, lung function, and pulmonary blood flow in sickle cell disease. Pediatr Res. 2016;79(2):313-317.
42. Chaudry RA, Rosenthal M, Bush A, Crowley S. Reduced forced expiratory flow but not increased exhaled nitric oxide or airway responsiveness to methacholine characterises paediatric sickle cell airway disease. Thorax. 2014;69(6):580-585.
43. Cohen RT, Rodeghier M, Kirkham FJ, et al. Exhaled nitric oxide: Not associated with asthma, symptoms, or spirometry in children with sickle cell anemia. J Allergy Clin Immunol. 2016;138(5):1338-1343.e4.
44. Strunk RC, Cohen RT, Cooper BP, et al. Wheezing symptoms and parental asthma are associated with a physician diagnosis of asthma in children with sickle cell anemia. J Pediatr. 2014;164(4):821-826.e1.
45. Patel AP, Krupani S, Stark JM, et al. Validation of the breathmobile case identification survey for asthma screening in children with sickle cell disease. J Asthma. 2020;58(6):782-790.
46. Kassim AA, Payne AB, Rodeghier M, Macklin EA, Strunk RC, DeBaun MR. Low forced expiratory volume is associated with earlier death in sickle cell anemia. Blood. 2015;126(13):1544-1550.
47. Intzes S, Kalpatthi RV, Short R, Imran H. Pulmonary function abnormalities and asthma are prevalent in children with sickle cell disease and are associated with acute chest syndrome. Pediatr Hematol Oncol. 2013;30(8):726-732.
48. Duckworth L, Black LV, Ezmigna D, et al. Spirometry use in patients with sickle cell disease with and without asthma and acute chest syndrome: A multicenter study. EJHaem. 2020;1(1):239-242.
49. Arteta M, Campbell A, Nouraie M, et al. Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 2014;36(3):185-189.
50. Murray C, Foden P, Lowe L, Durrington H, Custovic A, Simpson A. Diagnosis of asthma in symptomatic children based on measures of lung function: an analysis of data from a population-based birth cohort study. Lancet Child Adolesc Health. 2017;1(2):114-123.
51. Mondal P, Yirinec A, Midya V, et al. Diagnostic value of spirometry vs impulse oscillometry: A comparative study in children with sickle cell disease. Pediatr Pulmonol. 2019;54(9):1422-1430.
52. Knight-Madden JM, Hambleton IR. Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2022;12(12):CD003733.
53. Kopel LS, Klings ES, Monuteaux MC, Gaffin JM, Heeney MM, Phipatanakul W. Bronchodilator use for acute chest syndrome among large pediatric hospitals in North America. Clin Pediatr. 2018;57(14):1630-1637.
54. Bokov P, El Jurdi H, Denjoy I, et al. Salbutamol worsens the autonomic nervous system dysfunction of children with sickle cell disease. Front Physiol. 2020;11:31.
55. Griffin TC, McIntire D, Buchanan GR. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. New Engl J Med. 1994;330(11):733-737.
56. Bernini JC, Rogers ZR, Sandler ES, Reisch JS, Quinn CT, Buchanan GR. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood. 1998;92(9):3082-3089.
57. Darbari DS, Fasano RS, Minniti CP, et al. Severe vaso-occlusive episodes associated with use of systemic corticosteroids in patients with sickle cell disease. J Natl Med Assoc. 2008;100(8):948-951.
58. Kumar R, Qureshi S, Mohanty P, Rao SP, Miller ST. A short course of prednisone in the management of acute chest syndrome of sickle cell disease. J Pediatr Hematol Oncol. 2010;32(3):e91-e94.
59. Sobota A, Graham DA, Heeney MM, Neufeld EJ. Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission. Am J Hematol. 2010;85(1):24-28.
60. Field JJ, Kassim A, Brandow A, et al. Phase 2 trial of montelukast for prevention of pain in sickle cell disease. Blood Adv. 2020;4(6):1159-1165.