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A scoping review of transition interventions for young adults with sickle cell disease
Viola A, Porter J, Shipman J, Brooks E, Valrie C
Pediatric blood & cancer. 2021;:e29135
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Abstract
Standardized programming for individuals with sickle cell disease (SCD) transitioning from pediatric to adult-centered care does not currently exist, resulting in high rates of mortality and morbidity. This scoping review examines and evaluates the current literature on SCD transition programs and interventions. Eligible studies described an existing program for individuals with SCD aged 12-29 years preparing to transition. The Evidence Project risk-of-bias tool was used to assess article quality. We identified 30 eligible articles, of which, only two were randomized controlled trials. Many studies have incomplete reports of feasibility information, such as completion rates, patient characteristics, and attrition; all studies were limited to a single institution; and most studies were rated high for risk of bias. Progress has been made in designing and gathering initial evaluation data for SCD transition programs; however, there is a need for higher quality studies, consistent assessment, and better dissemination of programs.
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Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials
Gyamfi J, Ojo T, Epou S, Diawara A, Dike L, Adenikinju D, Enechukwu S, Vieira D, Nnodu O, Ogedegbe G, et al
PloS one. 2021;16(2):e0246700
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Editor's Choice
Abstract
BACKGROUND Despite ~90% of sickle cell disease (SCD) occurring in low-and middle-income countries (LMICs), the vast majority of people are not receiving evidence-based interventions (EBIs) to reduce SCD-related adverse outcomes and mortality, and data on implementation research outcomes (IROs) and SCD is limited. This study aims to synthesize available data on EBIs for SCD and assess IROs. METHODS We conducted a systematic review of RCTs reporting on EBIs for SCD management implemented in LMICs. We identified articles from PubMed/Medline, Global Health, PubMed Central, Embase, Web of Science medical subject heading (MeSH and Emtree) and keywords, published from inception through February 23, 2020, and conducted an updated search through December 24, 2020. We provide intervention characteristics for each study, EBI impact on SCD, and evidence of reporting on IROs. MAIN RESULTS 29 RCTs were analyzed. EBIs identified included disease modifying agents, supportive care agents/analgesics, anti-malarials, systemic treatments, patient/ provider education, and nutritional supplements. Studies using disease modifying agents, nutritional supplements, and anti-malarials reported improvements in pain crisis, hospitalization, children's growth and reduction in severity and prevalence of malaria. Two studies reported on the sustainability of supplementary arginine, citrulline, and daily chloroquine and hydroxyurea for SCD patients. Only 13 studies (44.8%) provided descriptions that captured at least three of the eight IROs. There was limited reporting of acceptability, feasibility, fidelity, cost and sustainability. CONCLUSION EBIs are effective for SCD management in LMICs; however, measurement of IROs is scarce. Future research should focus on penetration of EBIs to inform evidence-based practice and sustainability in the context of LMICs. CLINICAL TRIAL REGISTRATION This review is registered in PROSPERO #CRD42020167289.
PICO Summary
Population
Children and adults with sickle cell disease (SCD) in 14 low- and middle-income countries, (30 studies).
Intervention
Evidence-based interventions including: disease modifying agents, supportive care agents/analgesics, anti-malarials, systemic treatments (e.g., red blood cell transfusions), patient/provider education, and nutritional supplements.
Comparison
Placebo or comparator intervention
Outcome
Studies using disease modifying agents, nutritional supplements, and anti-malarials reported improvements in pain crisis, hospitalization, children's growth and reduction in severity and prevalence of malaria. Two studies reported on the sustainability of supplementary arginine, citrulline, and daily chloroquine and hydroxyurea for SCD patients. Only 13 studies (44.8%) provided descriptions that captured at least three of the eight implementation research outcomes. There was limited reporting of acceptability, feasibility, fidelity, cost and sustainability.
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Neonatal Uterine Bleedings: An Ignored Sign but a Possible Cause of Early-Onset Endometriosis - A Systematic Review
Dekker J, Hooijer I, Ket JCF, Vejnović A, Benagiano G, Brosens I, Mijatovic V
Biomedicine hub. 2021;6(1):6-16
Abstract
OBJECTIVE Based on the hypothesis that neonatal uterine bleedings (NUB), occurring mostly in the first week after birth, could represent a pathogenetic mechanism for early-onset endometriosis, this systematic review (SR) was undertaken to evaluate the prevalence and screening strategies used to assess and quantify NUB. DESIGN Both a SR and a sample literature search in PubMed and Embase were conducted to gather information on NUB prevalence and screening techniques. This was performed by an information specialist. Only full-text articles regarding the assessment of NUB in neonates in the first 2 weeks after birth were included. No limit on language or publication data was used. MATERIALS AND METHODS The SR was registered in PROSPERO (CRD42019138121). Data was first assessed for eligibility on title and abstract by 2 blinded review authors. Any disagreements were discussed with a third reviewer if necessary. Subsequently, full-text articles were read and assessed for quality using the Cochrane Collaboration Handbook. RESULTS Out of 1,988 articles in the systematic search, 10 relevant articles were selected, of which 8 were identified through the systematic search and 2 were found through other sources. The sample search of 4,445 articles did not bring up relevant articles. Results were not comparable due to the heterogeneity of screening techniques, although data showed consensus. The prevalence of visible bleeding ranged from 3.3 to 53.8% and the prevalence of occult bleeding from 25.4 to 96.7%. The occurrence was the highest between the 3rd and 7th day postpartum (PP) and the bleeding lasted for 3-4 days on average. Various screening techniques for detecting NUB were found in the literature, including the use of hemoglobin detection devices (such as Hemastix) in the vaginal vestibulum, comparison of diapers with stains of known volume, colposcopy, and ultrasonography. CONCLUSION The reported prevalence of NUB varies considerably, with a consistent occurrence between the 3rd and the 7th day PP. Literature to assess NUB is dated. The techniques are poorly described and heterogeneous. Future research should focus on prospective cohort studies in order to attempt to correlate NUB cases to (early-onset) endometriosis.
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Prevalence of neonatal hyperbilirubinaemia and its association with glucose-6-phosphate dehydrogenase deficiency and blood-type incompatibility in sub-Saharan Africa: a systematic review and meta-analysis
Aynalem YA, Mulu GB, Akalu TY, Shiferaw WS
BMJ paediatrics open. 2020;4(1):e000750
Abstract
BACKGROUND Hyperbilirubinaemia is a silent cause of newborn disease and death worldwide. However, studies of the disease in sub-Saharan Africa are highly variable with respect to its prevalence. Hence, this study aimed to estimate the overall magnitude of neonatal hyperbilirubinaemia and its association with glucose-6-phosphate dehydrogenase (G6PD) deficiency and blood-type incompatibility in sub-Saharan Africa. METHODS PubMed, Scopus, Google Scholar and the Cochrane Review were systematically searched online to retrieve hyperbilirubinaemia-related articles. All observational studies reported the prevalence of hyperbilirubinaemia in sub-Saharan Africa were included for analysis and excluded if the study failed to determine the desired outcome. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Heterogeneity across the included studies was evaluated using the inconsistency index (I(2)). Subgroup and meta- regression analysis were also done. Publication bias was examined by funnel plot and the Egger's regression test. The random-effect model was fitted to estimate the pooled prevalence of neonatal hyperbilirubinaemia. The meta-analysis was performed using the STATA V.14 software. RESULTS A total of 30 486 studies were collected from the different databases and 10 articles were included for the final analysis. The overall magnitude of neonatal hyperbilirubinaemia was 28.08% (95% CI20.23 to 35.94, I(2)=83.2) in sub-Saharan Africa. Neonates with G6PD deficiency (OR 2.42, 95% CI 1.64 to 3.56, I(2)=37%) and neonates that had a blood type that was incompatible with their mother's (OR 3.3, (95% CI 1.96 to 5.72, I(2)=84%) were more likely to develop hyperbilirubinaemia. CONCLUSION The failure to prevent and screen G6PD deficiency and blood-type incompatibility with their mother's results in high burden of neonatal hyperbilirubinaemia in sub-Saharan Africa. Therefore, early identification and care strategies should be developed to the affected neonates with G6PD deficiency and blood-type incompatibility with their mother's to address long-term medical and scholastic damages among those exposed to hyperbilirubinaemia.
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A Systematic Review of Medication Adherence Interventions in Pediatric Sickle Cell Disease
Shih S, Cohen LL
J Pediatr Psychol. 2020
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Editor's Choice
Abstract
OBJECTIVE Adherence to medication regimens is of critical importance in sickle cell disease (SCD). Most notably, data indicate that hydroxyurea, penicillin, and iron chelators increase life expectancy and decrease comorbid medical problems (e.g., strokes). However, average pediatric SCD adherence rates are only 55-74%. Studies have introduced interventions for pediatric SCD adherence, but no review has synthesized these data. METHODS We conducted a systematic review of interventions for enhancing medication adherence in pediatric SCD. There were 9 studies that met inclusion and exclusion criteria. The Pediatric Self-Management Model provided a framework for organizing the modifiable factors targeted by existing interventions. RESULTS The 9 studies had high risk of bias levels and most targeted hydroxyurea. All studies used multiple measures of adherence, the interventions were multicomponent, and most included behavioral or technological interventions. There was variability in terms of whether the intervention targeted the individual, family, community, or healthcare system. CONCLUSIONS Consistent with the broader adherence literature, targeting knowledge alone was insufficient in increasing adherence. Findings suggest that reminders and targeting self-efficacy were key to success. In addition, addressing multiple domains in an intervention yielded larger effects on adherence. Although these results are promising, this review highlights several limitations of the extant literature, including a paucity of intervention studies and several methodological weaknesses, such as small sample sizes, few randomized controlled trials, and variable measures of adherence. Recommendations for advancing scientific understanding of adherence promoting interventions in pediatric SCD are provided.
PICO Summary
Population
Paediatric patients with sickle cell disease (SCD), (9 studies, n=247).
Intervention
Systematic review on interventions for improving SCD medication regimen adherence.
Comparison
Outcome
Most studies targeted hydroxyurea followed by iron chelator, and penicillin. All studies used multiple measures of adherence, the interventions were multicomponent, and most included behavioral or technological interventions. There was variability in terms of whether the intervention targeted the individual, family, community, or healthcare system.
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Clinical Pharmacist-Provided Services In Iron-Overloaded Beta-Thalassaemia Major Children: A New Insight Into Patient Care
Bahnasawy SM, El Wakeel LM, Beblawy NE, El-Hamamsy M
Basic & Clinical Pharmacology & Toxicology. 2017;120(4):354-359
Abstract
Iron-overloaded β-thalassaemia major (BTM) children have high risk of delayed sexual/physical maturation, liver/heart diseases and reduced life expectancy. The lifelong need to use iron chelators, their unpleasant administration, side effects and lack of awareness regarding iron overload risks all hamper BTM patient compliance to iron chelators. This study evaluated the impact of clinical pharmacist-provided services on the outcome of iron-overloaded BTM children. Forty-eight BTM children were randomly assigned to either control group, who received standard medical care, or intervention group, who received standard medical care plus clinical pharmacist-provided services. Services included detection of drug-related problems (DRPs) and their management, patient education regarding disease nature and iron chelators, as well as providing patient-tailored medication charts. After six months of study implementation, there was a highly significant difference between the control and intervention groups in serum ferritin (SF) (mean: 3871 versus 2362, μg/l, p = 0.0042), patient healthcare satisfaction (median: 24.47 versus 90.29, p < 0.0001) and quality of life (QoL) (median: 49.84 versus 63.51, p = 0.0049). The intervention group showed a decline from baseline to the end of study in DRPs (64-4), the number of non-compliant patients (24-3) and mean SF levels (3949-2362 μg/l, p < 0.0001). Clinical pharmacist-provided services can positively impact the outcome of BTM children.
