Sickle cell disease (SCD) is a life-limiting condition and the most common genetic disease among African Americans, affecting approximately 100,000 individuals in the United States. The pulmonary complications of sickle cell disease, which are in themselves associated with high levels of acute and chronic morbidity and early mortality, are poorly understood, understudied, and under-recognized by clinicians. Through Dr. Robyn Cohen’s collaboration with the multicenter Sleep and Asthma Cohort research group, she demonstrated that features of asthma (recurrent wheezing, airway obstruction and inflammation, and atopy) are common among children with sickle cell anemia and associated with significant morbidity. Current projects involve evaluating the impact and effectiveness of using BiPAP as supportive care for hospitalized individuals with SCD.
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Investigators
Department of Pediatrics
Department of Medicine
Research projects
SNAP: Supportive Non-invasive Ventilation for Acute Chest Syndrome Prevention in Hospitalized Children with Sickle Cell Disease, 1UL1TR001430
Publications
1: Burrowes SA, Jones KC, Williams CJ, Neri CM, Klings ES, Bellamy SL, Walkey
AJ, Drainoni ML, Cohen RT. Supportive noninvasive ventilation for acute chest
syndrome prevention in hospitalized children with sickle cell disease:
Determinants of implementation. J Hosp Med. 2025 Jul 17:10.1002/jhm.70137. doi:
10.1002/jhm.70137. Epub ahead of print. PMID: 40677003; PMCID: PMC12469202.
2: Alwang AK, Law AC, Klings ES, Cohen RT, Bosch NA. Lactated Ringer vs Normal
Saline Solution During Sickle Cell Vaso-Occlusive Episodes. JAMA Intern Med.
2024 Nov 1;184(11):1365-1372. doi: 10.1001/jamainternmed.2024.4428. PMID:
39250114; PMCID: PMC11385329.
3: Cohen RT, Burrowes SAB, Williams CJ, Neri CM, Klings ES, Jones KC, Walkey AJ,
Drainoni ML. SNAP: Supportive non-invasive ventilation for acute chest syndrome
prevention for hospitalized children with sickle cell disease: Perspectives of
patients, parents, and the healthcare team. Pediatr Blood Cancer. 2024
Sep;71(9):e31142. doi: 10.1002/pbc.31142. Epub 2024 Jun 19. PMID: 38896013;
PMCID: PMC11394287.
4: Willen SM, Cohen RT. Unmasking Acute Chest Syndrome: Understanding the Role
of Nonpharmacologic Interventions on Children with Sickle Cell Disease During
the COVID-19 Pandemic. Chest. 2024 Jan;165(1):9-11. doi:
10.1016/j.chest.2023.09.002. PMID: 38199737.
5: Desai AA, Machado RF, Cohen RT. The Cardiopulmonary Complications of Sickle
Cell Disease. Hematol Oncol Clin North Am. 2022 Dec;36(6):1217-1237. doi:
10.1016/j.hoc.2022.07.014. PMID: 36400540; PMCID: PMC10323820.
6: Cohen RT, Klings ES. Systemic Steroids and the Risk of Vasoocclusive Events
in Patients with Sickle Cell Disease. Ann Am Thorac Soc. 2023 Jan;20(1):18-20.
doi: 10.1513/AnnalsATS.202207-627PS. PMID: 36166277; PMCID: PMC9819271.
7: Guenther CS, Pae VJ, Neri CM, Barry K, Duggan MA, Cohen RT. SNAP: Supportive
noninvasive ventilation for acute chest syndrome prevention in children with
sickle cell disease. Pediatr Blood Cancer. 2021 Aug;68(8):e29136. doi:
10.1002/pbc.29136. Epub 2021 Jun 8. PMID: 34101983.
8: Gupta VK, Strykowski R, Scarpato B, Lawrence R, Khan SL, Patel J, Nouraie SM,
Cohen RT, Sloan JM, Klings ES. The effect of anticoagulant choice on venous
thromboembolism recurrence and bleeding in sickle cell disease. Am J Hematol.
2020 Oct;95(10):E279-E281. doi: 10.1002/ajh.25923. Epub 2020 Aug 18. PMID:
32619024.
9: Liem RI, Lanzkron S, D Coates T, DeCastro L, Desai AA, Ataga KI, Cohen RT,
Haynes J, Osunkwo I, Lebensburger JD, Lash JP, Wun T, Verhovsek M, Ontala E,
Blaylark R, Alahdab F, Katabi A, Mustafa RA. American Society of Hematology 2019
guidelines for sickle cell disease: cardiopulmonary and kidney disease. Blood
Adv. 2019 Dec 10;3(23):3867-3897. doi: 10.1182/bloodadvances.2019000916. Erratum
in: Blood Adv. 2023 Jul 25;7(14):3530. doi: 10.1182/bloodadvances.2023009802.
PMID: 31794601; PMCID: PMC6963257.
10: Ruhl AP, Sadreameli SC, Allen JL, Bennett DP, Campbell AD, Coates TD, Diallo
DA, Field JJ, Fiorino EK, Gladwin MT, Glassberg JA, Gordeuk VR, Graham LM,
Greenough A, Howard J, Kato GJ, Knight-Madden J, Kopp BT, Koumbourlis AC,
Lanzkron SM, Liem RI, Machado RF, Mehari A, Morris CR, Ogunlesi FO, Rosen CL,
Smith-Whitley K, Tauber D, Terry N, Thein SL, Vichinsky E, Weir NA, Cohen RT,
Klings ES. Identifying Clinical and Research Priorities in Sickle Cell Lung
Disease. An Official American Thoracic Society Workshop Report. Ann Am Thorac
Soc. 2019 Sep;16(9):e17-e32. doi: 10.1513/AnnalsATS.201906-433ST. PMID:
31469310; PMCID: PMC6812163.
11: Worsham CM, Martin ST, Nouraie SM, Cohen RT, Klings ES. Clinical and
laboratory findings associated with sleep disordered breathing in sickle cell
disease. Am J Hematol. 2017 Dec;92(12):E649-E651. doi: 10.1002/ajh.24892. Epub
2017 Oct 27. PMID: 28833466.
12: Cohen RT, Klings ES, Strunk RC. Sickle cell disease: wheeze or asthma?
Asthma Res Pract. 2015 Dec 8;1:14. doi: 10.1186/s40733-015-0014-2. PMID:
27965767; PMCID: PMC5142438.