may cause allergic bronchopulmonary aspergillosis (ABPA), a complex condition that leads to worsening of airway inflammation and progressive damage and is diagnosed by specific criteria. In this ...report, we present six CF patients with respiratory deterioration that did not respond to appropriate antibiotic treatment. All had had A fumigatus in sputum cultures but did not fulfill the criteria of ABPA. Treatment with antifungal agents was followed by improvement in clinical condition. We suggest that in patients with CF, A fumigatus should be considered as a pathogen that may directly cause respiratory exacerbations. Antifungal therapy should be considered when deteriorating respiratory function is not responding to antibacterial therapy and A fumigatus is growing in sputum cultures.
The presence of co-morbidities, including underlying respiratory problems, has been identified as a risk factor for severe COVID-19 disease. Information on the clinical course of SARS-CoV-2 infection ...in children with cystic fibrosis (CF) is limited, yet vital to provide accurate advice for children with CF, their families, caregivers and clinical teams.
Cases of SARS-CoV-2 infection in children with CF aged less than 18 years were collated by the CF Registry Global Harmonization Group across 13 countries between 1 February and 7 August 2020.
Data on 105 children were collated and analysed. Median age of cases was ten years (interquartile range 6–15), 54% were male and median percentage predicted forced expiratory volume in one second was 94% (interquartile range 79–104). The majority (71%) of children were managed in the community during their COVID-19 illness. Out of 24 children admitted to hospital, six required supplementary oxygen and two non-invasive ventilation. Around half were prescribed antibiotics, five children received antiviral treatments, four azithromycin and one additional corticosteroids. Children that were hospitalised had lower lung function and reduced body mass index Z-scores. One child died six weeks after testing positive for SARS-CoV-2 following a deterioration that was not attributed to COVID-19 disease.
SARS-CoV-2 infection in children with CF is usually associated with a mild illness in those who do not have pre-existing severe lung disease.
Cystic fibrosis (CF) is a life-threatening genetic disease, affecting around 10 500 people in the UK. Precision medicines have been developed to treat specific CF-gene mutations. The newest, ...elexacaftor/tezacaftor/ivacaftor (ELEX/TEZ/IVA), has been found to be highly effective in randomised controlled trials (RCTs) and became available to a large proportion of UK CF patients in 2020. Understanding the potential health economic impacts of ELEX/TEZ/IVA is vital to planning service provision.
We combined observational UK CF Registry data with RCT results to project the impact of ELEX/TEZ/IVA on total days of intravenous (IV) antibiotic treatment at a population level. Registry data from 2015 to 2017 were used to develop prediction models for IV days over a 1-year period using several predictors, and to estimate 1-year population total IV days based on standards of care pre-ELEX/TEZ/IVA. We considered two approaches to imposing the impact of ELEX/TEZ/IVA on projected outcomes using effect estimates from RCTs: approach 1 based on effect estimates on FEV
% and approach 2 based on effect estimates on exacerbation rate.
ELEX/TEZ/IVA is expected to result in significant reductions in population-level requirements for IV antibiotics of 16.1% (~17 800 days) using approach 1 and 43.6% (~39 500 days) using approach 2. The two approaches require different assumptions. Increased understanding of the mechanisms through which ELEX/TEZ/IVA acts on these outcomes would enable further refinements to our projections.
This work contributes to increased understanding of the changing healthcare needs of people with CF and illustrates how Registry data can be used in combination with RCT evidence to estimate population-level treatment impacts.
This paper focuses on social work’s understanding of how posttraumatic counselling may help or hinder recovery from trauma. A qualitative case study was conducted using an autobiographic memoir that ...provides an in-depth personal narrative of one woman’s experience of trauma, posttraumatic stress disorder, the posttraumatic helping environment, and healing journey. Inductive thematic analysis uncovered themes that align with the existing literature. Novel or understudied aspects for consideration also emerged, including the importance of psychoeducation, behavioural activation, and secondary factors related to the posttraumatic environment that impede healing. The analysis highlighted missed opportunities to clinically address issues of identity and meaning in a spiritually sensitive manner. Although the narrator made it clear to helping professionals that she was struggling with religious beliefs and was in spiritual crisis, helping professionals seemed to eschew exploration of these concerns. Implications for clinical social work practice and future research are discussed.
Background: Patients were defined each successive month as either ‘chronic’ when more than 50% of the preceding 12 months were PA culture positive, ‘intermittent’ when ≤50% of the preceding 12 months ...were PA culture positive, ‘free of PA’, with no growth of PA for the previous 12 months, having previously been PA culture positive, or ‘never infected’, when PA had never been cultured. Methods: Cross-sectional analysis of 146 children attending the Leeds Regional Cystic Fibrosis Centre was performed to assess relationship between the new definition and clinical scores and investigations. The response variable was regressed on age and sex and the residuals analysed using the Kruskal–Wallis test. Results: The ‘chronic’ group (18% of patients) had significantly worse Shwachman–Kulczycki (SK) and Northern chest X-ray scores, and % predicted FEV1 values than the ‘free’ (28%) or ‘never’ (20%) categories (P<0.004). The ‘intermittent’ group (34%) had a significantly higher SK score than the ‘chronic’ group (P<0.0001), and a significantly lower % predicted FEV1 value than the ‘free’ or ‘never’ groups (P<0.0003). ‘Chronic’ patients were significantly associated with a positive, and ‘never’ patients with a negative, PA antibody result (P<0.001). Conclusions: The validity and importance of identifying these four subgroups is demonstrated. Previous definitions may over-estimate the prevalence of chronic infection.
Abstract Objectives Treatment regimen for families of children with cystic fibrosis (CF) is considerable, particularly when nebulised therapies for chronic Pseudomonas aeruginosa airway infection are ...prescribed. Adherence to these regimens is variable, particularly in adolescence. Previously, we reported children to be more adherent in evenings compared to mornings, suggesting an association with time-pressure. The aim of this study was to determine whether adherence would be better in adolescent patients at weekends and during school holidays when time-pressures may be less. Study design 24 patients (14 male, median range age 13.9 11.1–16.8 years) were enrolled from two regional paediatric CF centres in the United Kingdom. Data for a full scholastic year, were downloaded openly from a breath-activated data logging nebuliser (I-neb™). Adherence (% of doses taken ÷ expected number) was calculated during term-times, holidays, weekends and weekdays, for each patient. Results Large variations in adherence were seen between patients. However, adherence during term-time was significantly better than holidays ( p < 0.001). Weekday adherence was better than weekend adherence in term-time but not holidays. Interestingly, patients prescribed three daily treatments took on average 1.4 treatments/day, a similar number to those prescribed two daily treatments. Conclusion Overall adherence to inhaled therapies was reasonable, but significantly reduced during holiday periods. This suggests a need for families to have not only time, but also structure in their daily routine to maintain optimal adherence to long-term therapies. It is important for CF teams to appreciate these factors when supporting families.
The benefits of improved treatments for cystic fibrosis (CF) depend on optimal adherence, which remains problematic, particularly to aerosol therapy. In this study, we explored the process of ...adhering to aerosol therapy from the perspective of both adolescents with CF and their parents. Interviews were conducted individually with six adolescents and six parents, informed by accurate adherence data from an electronically chipped, aerosol device. Interview transcripts from audio-recordings were analyzed using grounded theory method (GTM). Major themes revealed differences in perspective between parent and adolescent, with this relationship mediating the cognitive and emotional processes that play a significant role in adherence behavior. These processes are further influenced by interactions with the aerosol therapy treatment regimen, device characteristics, and the context in which adherence is taking place. Parents and adolescents have different views of treatment and how to manage it. Both need to be addressed if optimal adherence is to be achieved.
Social capital has gained increasing attention from social workers as a way to understand the social networks and resources available to clients, especially new immigrants. Although researchers have ...developed helpful measures of social capital, a need remains to translate these tools into the native languages of immigrants, while addressing any specific cultural considerations. It would be beneficial for social workers to have access to an Arabic version of a social capital scale. This article describes the process of translation and cultural adaptation of the Onyx and Bullen social capital scale from English to Arabic. Using the responses from 326 Iraqi-Canadians, the instrument was factor analysed to verify the underlying conceptual structure. The translated instrument was found to be consistent with Onyx and Bullen's original factorial structure and to have good internal reliability with a Cronbach coefficient alpha for the overall instrument, which was comparable to the original scale.
IMPLICATIONS
Social workers' roles in Australia and Canada need to enhance culturally competent social and mental health services to immigrants and refugees.
Schools of Social Work in Australia and Canada can increase the preparation of students to work with immigrants and refugees, which can potentially help in energising the economy.
Social workers could use this scale to assess the level of social support and integration of Arabic speaking individuals, especially newcomers to Australia and Canada.
The aim of this study was to assess vitamin K status in an unselected population of children with cystic fibrosis (CF) and to investigate any vitamin K effect on bone turnover and bone mineral ...status.
Children > or =5 years of age who were attending the CF unit were invited to enter the study. Fasting blood samples were analyzed for levels of vitamin K1 and prothrombin produced in vitamin K absence; total, undercarboxylated, and carboxylated osteocalcin (OC); and bone-specific alkaline phosphatase and procollagen I carboxy-terminal propeptide (bone formation markers). Levels of N-telopeptide and free pyridinoline and deoxypyridinoline (bone breakdown products) were measured in urine samples. Bone mineral density and bone mineral content were measured at the lumbar spine and for the total body with a GE Lunar Prodigy densitometer. Statistical analyses were performed with Minitab version 9.1.
One hundred six children entered the study. Sixty-five of 93 children (70%) from whom blood samples were obtained showed suboptimal vitamin K status, on the basis of low serum vitamin K1 levels, increased prothrombin produced in vitamin K absence levels, or both abnormalities. Vitamin K1 levels showed a significant negative correlation with undercarboxylated OC levels but showed no significant correlation with any marker of bone turnover or measurement of bone mineral status. Undercarboxylated OC levels were correlated significantly with bone turnover markers, which themselves showed a significant negative correlation with measurements of bone mineral density and content. There were no significant correlations between carboxylated or undercarboxylated OC levels and bone density measurements.
Vitamin K1 deficiency is common among children with CF, and routine supplements should be considered. Through its role in the carboxylation of OC, vitamin K deficiency may be associated with an uncoupling of the balance between bone resorption and bone formation. A cause-effect relationship between vitamin K deficiency and low bone mass has not been proved.
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