Am J Otolaryngol. 2023 May 4;44(4):103912. doi: 10.1016/j.amjoto.2023.103912. Online ahead of print.

ABSTRACT

OBJECTIVES: The main aim of this study is to analyze the possible differences between clinical, demographic or genetic characteristics, in Cystic Fibrosis (CF) patients with chronic rhinosinusitis (CRS) with different phenotype. The secondary objective is to describe the possible benefit of surgery with Centripetal Endoscopic Sinus Surgery (CESS).

METHODS: The study includes 56 who performed CT scan of the paranasal sinuses. They were divided in 3 group according to phenotype: CRS without Nasal Polyps (NP); CRS with NP; CRS complicated with Mucocele. The clinical symptoms, age, gender, genotype, microbial colonization and pulmonary disease stage were collected and analyzed to assess possible statistically significant differences. Regarding the 7 patients who performed CESS surgery, the number of hospitalizations, intravenous (iv) antibiotic courses, respiratory exacerbations, the FEV1, the Lund-Mackay Score (LMS) and the SNOT 22 were evaluated before and 1 year after surgery.

RESULTS: No statistically significant differences regarding clinical symptoms between the 3 groups were identified (p > 0.05). Furthermore, there were no differences in age, gender, genotype, microbial colonization and pulmonary disease stage (p > 0.05). Regarding the patients who performed CESS, no significative difference in FEV1 progression was found. A reduction in hospitalization, pulmonary exacerbation and in the number of iv antibiotic courses resulted statistically significant different (p = 0.004; <0.001 and <0.001 respectively). A significant improvement in SNOT-22 and LMS (p < 0.001) was obtained.

CONCLUSION: Radiological monitoring of the rhinosinus disease is necessary regardless of the clinical expression of the disease. The presence of CRS with NP complicated by mucocele is frequent and independent of the patient's age and clinical manifestations. An extensive surgical approach could represent the gold standard for patients with CF in consideration of the potential important advantages to perform a total toilet of all the sinuses and nasal cavities and at the same time eliminating a potential microbiological reservoir.

PMID:37167857 | DOI:10.1016/j.amjoto.2023.103912

BMC Med Res Methodol. 2023 May 11;23(1):114. doi: 10.1186/s12874-023-01942-4.

ABSTRACT

BACKGROUND: Clinical outcomes are normally captured less frequently than data from remote technologies, leaving a disparity in volumes of data from these different sources. To align these data, flexible polynomial regression was investigated to estimate personalised trends for a continuous outcome over time.

METHODS: Using electronic health records, flexible polynomial regression models inclusive of a 1st up to a 4th order were calculated to predict forced expiratory volume in 1 s (FEV1) over time in children with cystic fibrosis. The model with the lowest AIC for each individual was selected as the best fit. The optimal parameters for using flexible polynomials were investigated by comparing the measured FEV1 values to the values given by the individualised polynomial.

RESULTS: There were 8,549 FEV1 measurements from 267 individuals. For individuals with > 15 measurements (n = 178), the polynomial predictions worked well; however, with < 15 measurements (n = 89), the polynomial models were conditional on the number of measurements and time between measurements. The method was validated using BMI in the same population of children.

CONCLUSION: Flexible polynomials can be used to extrapolate clinical outcome measures at frequent time intervals to align with daily data captured through remote technologies.

PMID:37170205 | DOI:10.1186/s12874-023-01942-4

Stat Med. 2023 May 11. doi: 10.1002/sim.9758. Online ahead of print.

ABSTRACT

Joint modeling has been a useful strategy for incorporating latent associations between different types of outcomes simultaneously, often focusing on a longitudinal continuous outcome characterized by an LME submodel and a terminal event subject to a Cox proportional hazard or parametric survival submodel. Applications to hierarchical longitudinal studies have been less frequent, particularly with respect to a binary process, which is commonly specified by a GLMM. Furthermore, many of the joint model developments have not allowed for investigations of nested effects, such as those arising from multicenter studies. To fill this gap, we propose a multilevel joint model that encompasses the LME submodel and GLMM through a Bayesian approach. Motivated by the need for timely detection of pulmonary exacerbation and characterization of irregularly observed lung function measurements in people living with cystic fibrosis (CF) receiving care across multiple centers, we apply the model to the data arising from US CF Foundation Patient Registry. In parallel, we examine the extent of bias induced by a non-hierarchical model. Our simulation study and application results show that incorporating the center effect along with individual stochastic variation over time within the LME submodel improves model estimation and prediction. Given that the center effect is evident in lung function observed in the CF population, accounting for center-specific power parameters by incorporating the symmetric power exponential power (spep) link function in the GLMM can facilitate more accurate conclusions in clinical studies.

PMID:37170074 | DOI:10.1002/sim.9758

Sci Rep. 2023 May 11;13(1):7695. doi: 10.1038/s41598-023-34043-9.

ABSTRACT

The rheology of sputum is viewed as a powerful emerging biophysical marker for monitoring muco-obstructive pulmonary diseases such as cystic fibrosis (CF) and non-CF bronchiectasis (NCFB). However, there is no unified practice to process sputa from collection to analysis, which can lead to highly variable, and sometimes inconsistent results. The main objective of this study is to bring light into the handling of sputum samples to establish a standardised and robust protocol before rheological measurements. Sputum collected from 22 CF and 10 NCFB adults, was divided into control (vortexed and fresh: non-heated and non-frozen) and three treated conditions (either non-vortexed, heated or frozen). In addition, 6 CF expectorations were used to study the dynamics of ageing over 24 h. Sputum's mechanical properties were measured with a rotational rheometer to obtain their properties at rest, elastic ([Formula: see text]) and viscous moduli ([Formula: see text]), and at the onset of flow, critical deformation ([Formula: see text]) and critical stress ([Formula: see text]). We demonstrate that heating sputum is completely destructive while freezing sputa at [Formula: see text] has no discernible effect on their rheology. We also show that the variability of rheological measurements largely resulted from the sample's macroscopic heterogeneity, and can be greatly reduced by non-destructive vortex homogenisation. Finally, we observed contrasted ageing effects as a fonction of purulence: while the viscoelasticity of purulent samples reduced by half within 6 h after collection, semi-purulent samples did not evolve. These results guide towards a robust unified protocol for simple sputum handling in rheometry. We therefore suggest to vortex and snap freeze sputum samples immediately after collection when direct testing is not possible.

PMID:37169792 | DOI:10.1038/s41598-023-34043-9

J Cyst Fibros. 2023 May 9:S1569-1993(23)00131-5. doi: 10.1016/j.jcf.2023.05.001. Online ahead of print.

ABSTRACT

Elexacator/tezacaftor/ivacaftor (ETI) has improved cystic fibrosis (CF) outcomes. A reduction in use of maintenance medication after its initiation has been reported. Seventy-one adult people with CF (PwCF) who are followed in three CF centers and completed one year of treatment with ETI were included in this study. Their use of inhaled dornase-α, colistin, tobramycin, aztreonam and levofloxacin during this period was compared with the corresponding use during one year without ETI, using the Medication Possession Ratio (MPR). MPR was significantly decreased after ETI initiation for dornase-α (67±35% vs 48±40%, p<0.001) and for all four inhaled antibiotics together (62±33% vs 41±37%, p<0.001). The findings of this multi-center, retrospective, study suggest that the initiation of ETI significantly leads to decrease in use of standard inhaled medication in PwCF. The significance of this finding in the course of the disease is yet to be investigated by larger prospective clinical trials.

PMID:37169616 | DOI:10.1016/j.jcf.2023.05.001

JPGN Rep. 2021 Nov 29;3(1):e142. doi: 10.1097/PG9.0000000000000142. eCollection 2022 Feb.

ABSTRACT

Pancreatic insufficiency (PI) is found in 85% of individuals with cystic fibrosis (CF). Of the remaining who are pancreatic sufficient (PS), there is potential for developing pancreatitis, and is described in ~20% of PS individuals. We report a case of a 17.5-year-old female presenting with acute recurrent pancreatitis (ARP) and PS, later diagnosed with CF. This is the first reported case of ARP in an individual with a F508d/A613T genotype. To date, there are only 6 other individuals with this genotype, and the mechanisms of it causing ARP and no overt respiratory symptoms of CF are unclear. Her diagnosis occurred 10 years after her initial presentation of pancreatitis, highlighting the importance of screening for CFTR mutations in the workup for ARP with no clear etiology.

PMID:37168745 | PMC:PMC10158408 | DOI:10.1097/PG9.0000000000000142

JPGN Rep. 2021 Dec 10;3(1):e157. doi: 10.1097/PG9.0000000000000157. eCollection 2022 Feb.

ABSTRACT

Supplemental Digital Content is available in the text.

PMID:37168743 | PMC:PMC10158304 | DOI:10.1097/PG9.0000000000000157

Am J Respir Crit Care Med. 2023 May 11. doi: 10.1164/rccm.202305-0785ED. Online ahead of print.

NO ABSTRACT

PMID:37167625 | DOI:10.1164/rccm.202305-0785ED

Chron Respir Dis. 2023 Jan-Dec;20:14799731231174542. doi: 10.1177/14799731231174542.

ABSTRACT

INTRODUCTION: Glycated hemoglobin can interfere with oxygen delivery and CO2 removal during exercise. Additionally, pancreatic insufficiency increases oxidative stress and exacerbates exercise intolerance in people with cystic fibrosis (PwCF). This investigation sought to test the hypotheses that elevated Hemoglobin A1c (HbA1c) can negatively affect exercise parameters in PwCF and that reductions in oxidative stress can improve tissue oxygenation in individuals with elevated HbA1c.

METHODS: Twenty four PwCF were divided into two groups; normal HbA1c <5.7% (N-HbA1c) and elevated HbA1c >5.7% (E-HbA1c). A maximal exercise test was conducted to obtain peak oxygen uptake (VO2peak), VO2 at ventilatory threshold (VT), ventilatory parameters (VE/VCO2 slope and end-tidal CO2 (petCO2)). Near-Infrared Spectroscopy (NIRS) was used to assess muscle oxygenated/deoxygenated hemoglobin during exercise. A subset of individuals with E-HbA1cwere given an antioxidant cocktail (AOC) for 4 weeks to determine the effects on tissue oxygenation during exercise.

RESULTS: A negative relationship between HbA1c and VO2peak at VT was observed (r = -0.511; p = 0.018). In addition, a positive relationship between HbA1c and VE/VCO2 slope (r = 0.587;p = 0.005) and a negative relationship between HbA1c and petCO2 at maximal exercise (r = -0.472;p = 0.031) was observed. N-HbA1c had greater VO2peak (p = 0.021), VO2 at VT (p = 0.004), petCO2 (p = 0.002), and lower VE/VCO2 slope (p = 0.004) compared with E-HbA1c. Muscle deoxygenated hemoglobin at VT was higher in N-HbA1c vs. E-HbA1c and 4 weeks of AOC improved skeletal muscle utilization of oxygen.

CONCLUSION: Findings demonstrate that glycated hemoglobin may lead to tissue oxygenation impairment and ventilation inefficiency during exercise in PwCF. In addition, antioxidant supplementation may lead to improved tissue oxygenation during exercise.

PMID:37166356 | DOI:10.1177/14799731231174542

J Sports Med Phys Fitness. 2023 May 11. doi: 10.23736/S0022-4707.23.14937-1. Online ahead of print.

ABSTRACT

BACKGROUND: Blisters are a common running injury and are known to limit runners' performance. There have been many studies on the subject with contrasting results. It would therefore be useful to describe more clearly blister epidemiology, blister prevention methods, and risk factors of blister development.

METHODS: This study is a retrospective anonymous, post-race survey. Runners were contacted by email after races in France during the summer and autumn of 2021 and asked to fill-in an online survey about their experience with blisters and running experiences.

RESULTS: Five hundred and thirty-three runners participated, of whom were 468 (88%) men and 47 women (12%), mean age 42±9.75. Sixty-one percent (N.=329) of runners applied blister prevention methods before the start of the race and 29% (N.=155) reported blisters at the end of the race. Most commonly used blisters prevention methods were: anti-friction cream 79% (N.=260), "anti-blister socks" 33% (N.=107), paper tape 13% (N.=44), and topical lemon application 11% (N.=36). Having a history of blisters in the past is strongly associated with blisters onset OR=15.950 (9.135-29.640; P<0.0001). Distances ran between 40 to 74 km appeared to be the less likely to cause blisters OR 0.188 (0.045-0.729; P=0.019). None of the studied blister prevention methods seemed to match the protective effect of running shorter distances.

CONCLUSIONS: Having a history of previous blisters is a major risk factor for blister occurrence, while running shorter distances seems protective.

PMID:37166255 | DOI:10.23736/S0022-4707.23.14937-1

Disaster Med Public Health Prep. 2023 May 11;17:e392. doi: 10.1017/dmp.2023.54.

ABSTRACT

A mix of guidance and mandated regulations during the coronavirus disease (COVID-19) pandemic served to reduce the number of social contacts, to ensure distancing in public spaces, and to maintain the isolation of infected individuals. Individual variation in compliance to social distancing in Germany, relating to age, gender, or the presence of pre-existing health conditions, was examined using results from a total of 39 375 respondents to a web-based behavioral survey.Older people and females were more willing to engage in social distancing. Those with chronic conditions showed overall higher levels of compliance, but those with cystic fibrosis, human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS), and epilepsy showed less adherence to general social distancing measures but were significantly more likely to isolate in their homes. Behavioral differences partly lie in the nature of each condition, especially with those conditions likely to be exacerbated by COVID-19. Compliance differences for age and gender are largely in line with previous studies.

PMID:37165783 | DOI:10.1017/dmp.2023.54

Sci Rep. 2023 May 10;13(1):7604. doi: 10.1038/s41598-023-34440-0.

ABSTRACT

F508del, the most frequent mutation in cystic fibrosis (CF), impairs the stability and folding of the CFTR chloride channel, thus resulting in intracellular retention and CFTR degradation. The F508del defect can be targeted with pharmacological correctors, such as VX-809 and VX-445, that stabilize CFTR and improve its trafficking to plasma membrane. Using a functional test to evaluate a panel of chemical compounds, we have identified tricyclic pyrrolo-quinolines as novel F508del correctors with high efficacy on primary airway epithelial cells from CF patients. The most effective compound, PP028, showed synergy when combined with VX-809 and VX-661 but not with VX-445. By testing the ability of correctors to stabilize CFTR fragments of different length, we found that VX-809 is effective on the amino-terminal portion of the protein that includes the first membrane-spanning domain (amino acids 1-387). Instead, PP028 and VX-445 only show a stabilizing effect when the second membrane-spanning domain is included (amino acids 1-1181). Our results indicate that tricyclic pyrrolo-quinolines are a novel class of CFTR correctors that, similarly to VX-445, interact with CFTR at a site different from that of VX-809. Tricyclic pirrolo-quinolines may represent novel CFTR correctors suitable for combinatorial pharmacological treatments to treat the basic defect in CF.

PMID:37165082 | DOI:10.1038/s41598-023-34440-0

Gene Ther. 2023 May 10. doi: 10.1038/s41434-023-00403-3. Online ahead of print.

ABSTRACT

Lentiviral vectors are attractive delivery vehicles for cystic fibrosis gene therapy owing to their low immunogenicity and ability to integrate into the host cell genome, thereby producing long-term, stable gene expression. Nonetheless, repeat dosing may be required to increase initial expression levels, and/or boost levels when they wane. The primary aim of this study was to determine if repeat dosing of a VSV-G pseudotyped LV vector delivered into mouse lungs is more effective than a single dose. C57Bl/6 mouse lungs were conditioned with lysophosphatidylcholine, followed one-hour later by a LV vector carrying the luciferase reporter gene, using six different short-term (≤1 wk) and long-term (>1 wk) dosing schedules. Luciferase expression was quantified using bioluminescence imaging over 12 months. Most dosing schedules produced detectable bioluminescence over the 12-month period, but the shorter intervals (≤1 wk) produced higher levels of flux than the longest interval (five doses at least 1-month apart). Ex vivo lung analysis at 12 months showed that the estimated mean flux for the group that received two doses 1-week apart was significantly greater than the single dose group and the two groups that received doses over a period greater than 1-week. These results suggest that early consecutive multiple doses are more effective at improving gene expression in mouse lungs at 12 months, than longer repeat dosing intervals.

PMID:37165031 | DOI:10.1038/s41434-023-00403-3

J Cyst Fibros. 2023 May 8:S1569-1993(23)00129-7. doi: 10.1016/j.jcf.2023.04.023. Online ahead of print.

ABSTRACT

INTRODUCTION: Elevated liver function tests (LFTs) are reported in individuals with cystic fibrosis (CF) starting elexacaftor/tezacaftor/ivacaftor (ETI). We report our experience with ETI in CF liver transplant patients.

METHOD: All CF liver transplant patients under the care of the Leeds CF team were commenced on ETI. Liver biopsies were performed when ALT >3 times upper limit of normal with or without bilirubin elevation. Treatment was guided by transplant hepatology and CF teams. Clinical data including lung function, LFTs and tacrolimus levels were collected.

RESULTS: Four patients (3 male, 1 female) on tacrolimus were commenced on ETI. Median time post liver transplantation was 6.5 years. Three patients underwent liver biopsy. One biopsy was abnormal with immune-mediated liver injury, which responded to increased immunosuppression. Management of tacrolimus levels proved straightforward.

CONCLUSION: ETI therapy in CF post liver transplant recipients was encouraging. Normal liver biopsy provides re-assurance to continue treatment despite elevated LFTs.

PMID:37164896 | DOI:10.1016/j.jcf.2023.04.023

N Engl J Med. 2023 May 11;388(19):1755-1766. doi: 10.1056/NEJMoa2212895.

ABSTRACT

BACKGROUND: No approved treatment for peanut allergy exists for children younger than 4 years of age, and the efficacy and safety of epicutaneous immunotherapy with a peanut patch in toddlers with peanut allergy are unknown.

METHODS: We conducted this phase 3, multicenter, double-blind, randomized, placebo-controlled trial involving children 1 to 3 years of age with peanut allergy confirmed by a double-blind, placebo-controlled food challenge. Patients who had an eliciting dose (the dose necessary to elicit an allergic reaction) of 300 mg or less of peanut protein were assigned in a 2:1 ratio to receive epicutaneous immunotherapy delivered by means of a peanut patch (intervention group) or to receive placebo administered daily for 12 months. The primary end point was a treatment response as measured by the eliciting dose of peanut protein at 12 months. Safety was assessed according to the occurrence of adverse events during the use of the peanut patch or placebo.

RESULTS: Of the 362 patients who underwent randomization, 84.8% completed the trial. The primary efficacy end point result was observed in 67.0% of children in the intervention group as compared with 33.5% of those in the placebo group (risk difference, 33.4 percentage points; 95% confidence interval, 22.4 to 44.5; P<0.001). Adverse events that occurred during the use of the intervention or placebo, irrespective of relatedness, were observed in 100% of the patients in the intervention group and 99.2% in the placebo group. Serious adverse events occurred in 8.6% of the patients in the intervention group and 2.5% of those in the placebo group; anaphylaxis occurred in 7.8% and 3.4%, respectively. Serious treatment-related adverse events occurred in 0.4% of patients in the intervention group and none in the placebo group. Treatment-related anaphylaxis occurred in 1.6% in the intervention group and none in the placebo group.

CONCLUSIONS: In this trial involving children 1 to 3 years of age with peanut allergy, epicutaneous immunotherapy for 12 months was superior to placebo in desensitizing children to peanuts and increasing the peanut dose that triggered allergic symptoms. (Funded by DBV Technologies; EPITOPE ClinicalTrials.gov number, NCT03211247.).

PMID:37163622 | DOI:10.1056/NEJMoa2212895

bioRxiv. 2023 Apr 28:2023.04.26.538445. doi: 10.1101/2023.04.26.538445. Preprint.

ABSTRACT

Pseudomonas aeruginosa is a prevalent pathogen in cystic fibrosis (CF) lungs which displays strong resistance to various antibiotic classes, contributing to antimicrobial resistance (AMR). P aeruginosa populations in CF lungs exhibit considerable genetic and phenotypic diversity, raising questions about their susceptibility to non-traditional antimicrobials, such as bacteriocins. R-pyocins, bacteriocins produced by P. aeruginosa , are highly potent, non-replicating phage tail-like protein complexes with a narrow killing spectrum. The diversity of P. aeruginosa variants within CF lung infections may lead to varying susceptibility to R-pyocins due to changes in the lipopolysaccharide (LPS) structure, which acts as the R-pyocin receptor. However, the extent of susceptibility to the five known R-pyocin subtypes (R1-R5) remains unknown, especially considering the diverse P. aeruginosa populations in CF lungs. Additionally, the connection between LPS phenotype and R-pyocin susceptibility is not well understood. We tested 139 P. aeruginosa variants from 17 sputum samples of seven CF patients for R2-pyocin susceptibility and analyzed their LPS phenotypes. Our findings revealed that approximately 83% of sputum samples contained diverse P. aeruginosa populations without R2-pyocin resistant variants, while all samples had some susceptible variants. Moreover, there was no clear correlation between LPS phenotypes and R-pyocin susceptibility. The absence of a clear correlation between LPS phenotypes and R-pyocin susceptibility suggests that LPS packing density may significantly influence R-pyocin susceptibility among CF variants. Our research supports the potential use of R-pyocins as therapeutic agents, as numerous infectious CF variants appear to be susceptible to R2-pyocins, even within diverse P. aeruginosa populations.

IMPORTANCE: Cystic fibrosis (CF) patients often experience chronic, debilitating lung infections caused by antibiotic-resistant Pseudomonas aeruginosa . The genetic and phenotypic diversity of P. aeruginosa populations in CF lungs raises questions about their susceptibility to non-traditional antimicrobials, like bacteriocins. In this study, we focused on R-pyocins, a type of bacteriocin with high potency and a narrow killing spectrum. Our findings indicate that a large number of P. aeruginosa variants are susceptible to R2-pyocins, even within diverse bacterial populations, supporting their potential use as therapeutic agents. The absence of a clear correlation between lipopolysaccharide (LPS) phenotypes and R-pyocin susceptibility suggests that LPS packing density may play a significant role in R-pyocin susceptibility among CF variants. Understanding the relationship between LPS phenotypes and R-pyocin susceptibility is crucial for developing future effective treatments for these chronic infections.

PMID:37163048 | PMC:PMC10168318 | DOI:10.1101/2023.04.26.538445

BMC Palliat Care. 2023 May 10;22(1):56. doi: 10.1186/s12904-023-01178-5.

NO ABSTRACT

PMID:37161423 | DOI:10.1186/s12904-023-01178-5

BMJ Open Respir Res. 2023 May;10(1):e001360. doi: 10.1136/bmjresp-2022-001360.

ABSTRACT

INTRODUCTION: Cystic fibrosis (CF) is a multisystem condition that is complicated by recurrent pulmonary infections requiring aggressive antibiotic treatment. This predisposes the patient to complications such as sensorineural hearing loss, renal impairment, hypersensitivity and the development of antibiotic resistance. Pseudomonas aeruginosa is one of the more common organisms which cause recurrent infections and result in greater morbidity and mortality in people living with CF. Bacteriophages have been identified as a potential alternative or adjunct to antibiotics. We hypothesise that bacteriophage therapy is a safe and well-tolerated treatment in children with CF infected with P. aeruginosa infection in their airways.

METHODS: This single-arm, open-labelled, non-randomised trial will run for a maximum period of 36 months with up to 10 participants. Adolescents (≥12 years and <18 years of age) who continue to shed P.aeruginosa (within 3 months of enrolment) despite undergoing eradication therapy previously, will be considered for this trial. Non-genetically modified bacteriophages that have demonstrated obligate lytic activity against each of the study participants' P. aeruginosa strains will be selected and prepared according to a combination of established protocols (isolation, purification, sterility testing and packaging) to achieve close to good manufacturing practice recommendations. The selected bacteriophage will be administered endo-bronchially first under direct vision, followed by two times a day nebulisation for 7 days in addition to standard CF treatment (intravenous antibiotics, physiotherapy to be completed as inpatient for 10-14 days). Safety and tolerability will be defined as the absence of (1) fever above 38.5°C occurring within 1 hour of the administration of the nebulised bacteriophage, (2) a 10% decline in spirometry (forced expiratory volume in 1 s %) measured preadministration and postadministration of the first dose of nebulised bacteriophage. Clinical reviews including repeat sputum cultures and spirometry will be performed at 3, 6, 9 and 12 months following bacteriophage treatment.

ETHICS AND DISSEMINATION: Our clinical trial is conducted in accordance with (1) good clinical practice, (2) Australian legislation, (3) National Health and Medical Research Council guidelines for the ethical conduct of research.

TRIAL REGISTRATION NUMBER: Australia and New Zealand Clinical Trial Registry (ACTRN12622000767707).

PMID:37160359 | DOI:10.1136/bmjresp-2022-001360

Am J Respir Crit Care Med. 2023 May 9. doi: 10.1164/rccm.202303-0380LE. Online ahead of print.

NO ABSTRACT

PMID:37159937 | DOI:10.1164/rccm.202303-0380LE

Cureus. 2023 Apr 6;15(4):e37218. doi: 10.7759/cureus.37218. eCollection 2023 Apr.

ABSTRACT

Cystic fibrosis (CF) is a chronic disorder that begins at an early age, so it is crucial to be aware of the physical and emotional burden placed on individuals suffering from it and their families. It significantly impacts an individual's life; therefore, it is essential to acknowledge the effects of the disease on physical and mental health. Our systematic review aims to highlight the areas of life affected by cystic fibrosis and evaluate various non-medical treatment options that may support the mental health of CF patients. We selected PubMed, Google Scholar, and MEDLINE (Medical Literature Analysis and Retrieval System Online) as our databases. We initially found 146,095 articles and narrowed the number of articles down using filters, exclusion and inclusion criteria, and various combinations of Medical Subheadings (MeSH) and key terms. We decided to use a final count of nine articles for our systematic review. The studies we included highlighted the negative impact of cystic fibrosis on mental health, like depression and anxiety, as well as on sleep, physical health, and overall quality of life. Several non-medical interventions, such as logotherapy, psychological interventions, complementary and alternative medicine, and many more, have been shown to enhance the mental health of many participants. Studies suggested that such therapy options may greatly benefit individuals with cystic fibrosis and their current treatment plan. This review indicates that non-medical therapy options can enhance the mental health of individuals suffering from cystic fibrosis and that it is crucial to bring more attention to preventing and treating mental health issues in cystic fibrosis patients. However, as current data is limited, more research with a larger number of participants over an extended period of time is necessary to better evaluate the efficacy of non-medical interventions on mental health.

PMID:37159780 | PMC:PMC10163933 | DOI:10.7759/cureus.37218