Sickle cell disease; An overview of the disease and its systemic effects

Alrayyes, Sahar; Baghdan, Danny; Haddad, Rami Y.; Compton, Anne Ashley; Mohama, Sandra; Goreishi, Reihaneh; Kawar, Nadia

doi:10.1016/j.disamonth.2017.12.003

Cited by 15 publications

(9 citation statements)

References 40 publications

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“…Sickle cell disease (SCD) is the most prevalent genetic condition worldwide with estimates of around 300 000 births of patients with SCD each year. 1,2 In the United States, ;100 000 people are living with the disease. 3 The majority of cases in the United States are among African Americans and Hispanic/ Latinx Americans, which impacts 1 of 365 births to African American parents and 1 of 16 300 births to Hispanic-American parents.…”

Section: Introductionmentioning

confidence: 99%

“…3 The majority of cases in the United States are among African Americans and Hispanic/ Latinx Americans, which impacts 1 of 365 births to African American parents and 1 of 16 300 births to Hispanic-American parents. 1,3 In 1998, the US Food and Drug Administration approved hydroxyurea for use in SCD after a pivotal clinical trial which showed that hydroxyurea reduced the frequency of pain events by .40%. 4,5 Subsequent trials continued to demonstrate the role of hydroxyurea in the reduction of pain crises 6 and mortality rates and the improvement in quality of life for people living with SCD.…”

Section: Introductionmentioning

confidence: 99%

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Intentional and unintentional nonadherence to hydroxyurea among people with sickle cell disease: a qualitative study

et al. 2020

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Hydroxyurea is an efficacious treatment for sickle cell disease (SCD), but adoption is low among individuals with SCD. The objective of this study was to examine barriers to patients’ adherence to hydroxyurea use regimens by using the intentional and unintentional medication nonadherence framework. We interviewed individuals with SCD age 15 to 49.9 years who were participants in the Sickle Cell Disease Implementation Consortium (SCDIC) Needs Assessment. The intentional and unintentional medication nonadherence framework explains barriers to using hydroxyurea and adds granularity to the understanding of medication adherence barriers unique to the SCD population. In total, 90 semi-structured interviews were completed across 5 of the 8 SCDIC sites. Among interviewed participants, 57.8% (n = 52) were currently taking hydroxyurea, 28.9% (n = 26) were former hydroxyurea users at the time of the interview, and 13.3% (n = 12) had never used hydroxyurea but were familiar with the medication. Using a constructivist grounded theory approach, we discovered important themes that contributed to nonadherence to hydroxyurea, which were categorized under unintentional (eg, Forgetfulness, External Influencers) and intentional (Negative Perceptions of Hydroxyurea, Aversion to Taking Any Medications) nonadherence types. Participants more frequently endorsed adherence barriers that fell into the unintentional nonadherence type (70%) vs intentional nonadherence type (30%). Results from this study will help SCD health care providers understand patient choices and decisions as being either unintentional or intentional, guide tailored clinical discussions regarding hydroxyurea therapy, and develop specific, more nuanced interventions to address nonadherence factors.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Intentional and unintentional nonadherence to hydroxyurea among people with sickle cell disease: a qualitative study

et al. 2020

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“…Full texts of these 50 studies were screened, and 32 studies of which were subsequently omitted because of low quality or lack of data to estimate the outcomes of interest. These excluded studies were 14 review articles [1, 3, 5, 7, 8, 11–13, 18, 24–28], ten studies had small number of patients with SCD [29–38], a study included patients with several types of chronic hemolytic anemia [39], four studies done among patients with TAC [40–43], a study analyzed all the acute admissions of patients with SCD to a district general hospital [44], a study included all patients attending some hospitals [45], and a study done among healthy blood donors [46].…”

Section: Resultsmentioning

confidence: 99%

A Meta-Analysis on the Seroprevalence of Parvovirus B19 among Patients with Sickle Cell Disease

Mohamed

Osman

et al. 2019

BioMed Research International

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Background Parvovirus B19 (B19 V) infection had been reported to be more frequent with serious clinical outcomes in patients with sickle cell disease (SCD) than in the general population. There is a wide variation in data among the existing literature regarding the seroprevalence of B19 V in patients with SCD. These data require further summary and analyses for better accuracy. This systematic review and meta-analysis was done to estimate the seroprevalence of B19 V in patients with SCD. Methods This study was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The databases of MEDLINE/PubMed, Virtual Health Library (VHL), ScienceDirect, Google Scholar, and OpenGrey were used for the systematic search. The random-effects model was used to estimate the pooled prevalence with the corresponding 95% confidence interval (CI) using OpenMeta Analyst software. Publication bias was estimated based on Begg's test, Egger's test, and examination of the funnel plot. Subgroup analyses and metaregression were used to explore the moderators of heterogeneity between studies. Results A total of 18 studies including 2890 patients were analyzed. The overall IgG seroprevalence of B19 V infection among patients with SCD was found to be 48.8% (95% CI 39.5%–58.0%). Evidence of publication bias was not detected. Evidence of acute viral infection detected by positive IgM antibodies among the screened SCD patients was found in 8.30% (95% CI 5.20%–11.4%) of them. There was a statistically signiﬁcant association between seroprevalence of B19 V and geographical areas. Conclusion There was a high prevalence of B19 V in patients with SCD. Healthcare providers need to be aware of the magnitude of B19 V infection in patients with SCD to ensure effective management. This review could provide a comprehensive view of B19 V prevalence in this susceptible population.

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“…Regular dental visits are important for controlling odontogenic complications and improve the oral health‐related quality of life of SCD patients (Alrayyes et al, 2018). Therefore, clinical case studies and review papers recommended an “aggressive” preventive strategy in SCD cases, focusing on preemptive procedures including oral hygiene instructions, periodic cleaning, application of fluoride varnish, and avoid more complicated dental treatment needs and higher chances of complications (Javed et al, 2013; Mulimani et al, 2016).…”

Section: Discussionmentioning

confidence: 99%

“…According to relevant literature, patients with SCD are most frequently admitted to hospital emergency care and require subsequent hospitalization compared to other medical conditions. The incidence of oral and dental issues in SCD is higher than that in many other disorders (Acharya, 2015; Alrayyes et al, 2018), but evidence from available clinical studies is very limited for sickle cell patients (Kawar, Alrayyes, Yang, & Aljewari, 2018; Yawn et al, 2014). Previous reports and clinical recommendations highlighted the significance of oral health maintenance by all dental care providers and the development of oral health promotion programs in the communities highly affected with SCD (Laurence et al, 2013; Mulimani et al, 2016; Yawn et al, 2014).…”

Section: Introductionmentioning

confidence: 99%

Predictors of dental complications post‐dental treatment in patients with sickle cell disease

Gusmini

Feng

et al. 2020

Clinical & Exp Dental Res

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Objective Our aim was to explore potential medical or dental indicators associated with dental complications and the utilization of emergency services in sickle cell disease (SCD), especially that clinical reports on adverse outcomes post‐dental treatment are scarce. Materials and methods A retrospective analysis of dental treatments of 47 eligible adults with confirmed SCD between May 2016 and October 2019. Logistic regression analysis was used whether clinical outcomes, course of dental treatment, and regularity of dental care are associated with dental complications after dental procedures and/or resulted in emergency care or hospital admissions. Results We identified a new, statistically significant association (p‐value = .01) between the number of prescription medications taken and complications (10%) after dental procedures. The most frequent dental procedures were tooth extractions (36%) and pain management (28%) during a non‐scheduled dental encounter (68%). The majority of cases did not participate in regular recall exams and periodical oral hygiene maintenance. Conclusions A higher number of prescription medications was associated with an increased risk of post‐dental complications in SCD patients. A thorough medical history, including a list of prescribed medications, and collaboration with the patient medical team are important to assess the risk of complications post‐dental procedures and the need for antibiotic prophylaxis according to the case complexity.

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Sickle cell disease; An overview of the disease and its systemic effects

Cited by 15 publications

References 40 publications

Intentional and unintentional nonadherence to hydroxyurea among people with sickle cell disease: a qualitative study

Intentional and unintentional nonadherence to hydroxyurea among people with sickle cell disease: a qualitative study

A Meta-Analysis on the Seroprevalence of Parvovirus B19 among Patients with Sickle Cell Disease

Predictors of dental complications post‐dental treatment in patients with sickle cell disease

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