Comprehensive management reduces incidence and mortality of acute chest syndrome in patients with sickle cell disease

Basishvili, Givi; Gotesman, Joseph; Vandervoort, Kathy; Jacobs, Charleen; Vattappally, Leena; Minniti, Caterina P.

doi:10.1002/ajh.24994

Cited by 6 publications

(3 citation statements)

References 13 publications

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“…These improvements are anticipated to improve quality of life and result in greater cost-effectiveness as compared with episodic, emergency department (ED)-based care. [15][16][17][18] Patients with SCD treated at SCD centers use ED and inpatient facilities less frequently, have decreased health care costs overall, 15,16,19 and are more likely to be prescribed hydroxyurea, 20 which has been demonstrated to improve psychosocial outcomes including healthrelated quality of life. 21 Comprehensive care of SCD requires that affected individuals have a continual bidirectional relationship with their medical team.…”

Section: Introductionmentioning

confidence: 99%

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

et al. 2020

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Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

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Section: Introductionmentioning

confidence: 99%

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

et al. 2020

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“…The use of exchange transfusions could have participated to the favorable patient outcome with advantages of this procedure compared to simple transfusions, such as a large volume transfused on a short time, a reduction of inflammatory mediators, and the nonexpansion of blood volume [25]. Although less frequent than in previous publications that question whether a transfusion is needed in all cases of ACS [26,27], exchange transfusions were required in 38% and 42% for prepandemic and pandemic periods, respectively. This could indicate that improved outcome of ACS was not simply due to more transfusions, but possibly to an early and coordinated intervention and careful attention to all aspects of care.…”

Section: Discussionmentioning

confidence: 99%

Impact of COVID‐19 on incidence, clinical presentation, and prognosis of acute chest syndrome in patients with sickle cell disease

Dubois,

Virot,

Marie

et al. 2023

eJHaem

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Acute chest syndrome (ACS) is a frequent complication of sickle cell disease (SCD). Because coronavirus disease 2019 (COVID‐19) increases mortality and morbidity in many diseases, we retrospectively analyzed the impact of SARS‐CoV‐2 infection on the incidence, the clinical presentation, and the prognosis of ACS in patients with SCD by comparing ACS episode before and during COVID‐19 pandemic.Ninety‐nine episodes of ACS were registered over 24 months before pandemic versus 81 episodes over 24 months during the pandemic period. The number of ACS episodes varies among children regarding the two period of time: 26 episodes (26%) for the pre‐pandemic period versus 11 episodes (13%) for the pandemic period (p = 0.03). Comparisons between adults and children showed a higher incidence of initial VOC (45% vs. 24%; p = 0.04) in adults, and a higher incidence of initial pneumonia (35% vs. 15%; p = 0.01) and documented infection (35% vs. 7%; p < 0.001) in children. One patient died during the pandemic period but without any relationship with ACS or COVID‐19. During this pandemic period, 13 episodes of ACS (16%) were found related to coronavirus infection. These ACS episodes did not show any significant differences in terms of outcome when compared to the other ACS episodes observed during this period.Overall, coronavirus infection did not demonstrate a negative impact on incidence, clinical presentation, and outcome of ACS in patients with SCD. Early management, chronic treatment with HU, and exchange transfusions could likely explain the low morbidity and mortality rates.

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“…Proxy measures of successful TOC include patients having a successful appointment in the adult SCD program, lower ED visits and hospitalizations (compared to those not successfully transitioned), and ongoing access to hydroxyurea (or other SCD therapies). [19][20][21][22][23] However, these measures of success have not been validated, and a multicenter longitudinal registry has not been used to demonstrate that these measures translate to reduced mortality.…”

Section: Introductionmentioning

confidence: 99%

Assessing barriers and facilitators to transition in sickle cell disease care prior to implementation of a formalized program

Sheppard

Hellemann

Lebensburger

et al. 2023

Pediatric Blood & Cancer

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Over 95% of children with sickle cell disease (SCD) survive into adulthood in the United States. However, early mortality remains a problem, especially in persons between the ages of 18 and 35. One possible explanation for the increased mortality rate in young adults is difficulties in engaging in care during the transition from a heavily contiguous pediatric healthcare model to a more self‐reliant adult healthcare model. The goal of this study was to identify potential facilitators and barriers to a successful transfer in care from the pediatric to adult SCD program before the formation of a formal transition program. This is a retrospective cohort study of transition outcomes for 472 individuals with SCD (all genotypes) treated at the University of Alabama at Birmingham (UAB) sickle cell clinic (aged 18–24). The primary outcome was whether the patient continued care in (any) adult SCD program (defined as being seen at least once in an adult hematology/SCD clinic). One hundred eighty‐eight (45%) transition age patients successfully transferred to adult care. Facilitators to successful transfer in care included being treated at the same hospital for both pediatric and adult programs, having the genotype HbSS, and/or receiving an SCD‐modifying therapy at the time of transition (hydroxyurea and/or red cell transfusion therapy). Of primary interest, many of the patients who failed to transition to an adult clinic were lost to follow‐up prior to 15 years of age. Importantly, these patients who had previously been labeled as “transition failures,” were lost to follow‐up long before the transition age. Early engagement is needed for this population.

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Comprehensive management reduces incidence and mortality of acute chest syndrome in patients with sickle cell disease

Cited by 6 publications

References 13 publications

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

Impact of COVID‐19 on incidence, clinical presentation, and prognosis of acute chest syndrome in patients with sickle cell disease

Assessing barriers and facilitators to transition in sickle cell disease care prior to implementation of a formalized program

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