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Title: Metabolic and psychological assessment of body functions in the course of cystic fibrosis : quality of life

Abstract:

Introduction: Cystic fibrosis is the most common genetic disease among Caucasians. Due to the systemic nature of the disease, numerous complications, and patients’ increasing life expectancy, it is important to closely monitor not only their physical but also mental health, as well as their health-related quality of life. Aim of the study: The aim of the study was to assess the metabolic and psychological function of people with cystic fibrosis and their health-related quality of life taking into account the COVID-19 pandemic. Material and methods: The study participants consisted of 81 persons aged 6 to 45 years of both sexes treated for cystic fibrosis at the Allergology and Cystic Fibrosis Clinic and the Cystic Fibrosis Clinic of the Provincial Hospital No. 2 named after St. Jadwiga the Queen in Rzeszów. The study group consisted of 48 patients with present exacerbations, while the control group consisted of 33 people in a stable condition. The study was conducted using a diagnostic survey method and analysis of selected results of patients’ functional, biochemical, somatic and general condition tests. From 2018 to 2020, a diagnostic survey was conducted using tools such as: Cystic Fibrosis Questionnaire – Revised: CFQ-R 6-11, CFQ-R 12-13, CFQ-R 14+, General Health Questionnaire (GHQ-12) and Beck Depression Scale. Other data were collected from participants’ medical records ; and gathered in a Microsoft Excel spreadsheet. The obtained data were described using descriptive statistics and subjected to statistical analysis including Spearman rank correlation analysis, Mann-Whitney U test, multivariate analysis of covariance, analysis of covariance and Pearson Chi-square test. Results: Respondents aged 6-11 years obtained the highest quality of life, especially in the physical functioning domain (Me=88.20). Among people aged 14 years and older, the quality of life was the lowest compared to the other groups. Patients during exacerbation had significantly lower quality of life in 8 of 12 domains, the largest differences were in Health Perceptions (Z=−3.045; p=0.002) and Weight (Z=−2.944; p=0.003). In the MANCOVA model, health status turned out to have a marginally significant effect on quality of life, taking into account FC% and FEV1% as covariates (Wilks λ=0.88; F=2.42; p=0.057). In the univariate analysis of covariance, only the Social Functioning domain was significantly dependent on their health status (F=8.32; p=0.005). Physical Functioning was significantly and positively correlated with all spirometry parameters (p<0.05). Depressive episodes occurred rarely in the study group, only in 18.52% (n=15) of respondents and they were mostly mild (n=12). Patients experienced a deterioration of their mental state slightly more often (38.28%; n=31), which ; were mostly mild (n=23) or moderate (n=8). The COVID-19 pandemic did not increase the frequency of depressive episodes (Δχ2=1.036, p=0.309), what is more, during this time significantly fewer people experienced a deterioration of their mental health (Δχ2=7.723, p=0.005) and obtained lower points in the General Health Questionnaire (Z=−3.044; p=0.002). The largest group of adult respondents had optimal BMI (n=26), and slightly fewer were underweight (n=21). Only 9 patients were overweight. Those who were overweight had the highest means and medians for all spirometry parameters, while underweight respondents had the lowest means and medians. Compared to the group with optimal BMI and overweight, underweight patients had significantly lower spirometry results in 5 out of 9 parameters, with the largest differences in FVC% (Z=2.812; p=0.005) and FC% (Z=2.653; p=0.008). Except for PEF%, patients’ height was significantly and negatively correlated with all spirometry parameters. Patients in stable condition presented the strongest correlations between lymphocyte count and MEF50% (rho=0.664) and FEV1% (rho=0.648), and those with exacerbations with MEF75% (rho=0.475). In terms of acid-base balance parameters among respondents in stable condition, MEF 50% presented the strongest correlation with partial pressure of oxygen (rho=0.584; p<0.05), and in those with exacerbations - base exces ; s with FC% (rho=-0.691; p<0.05). Blood iron concentration was significantly correlated with MEF 50% (rho=0.578; p<0.05) in stable patients, and with FVC% (rho=0.532; p<0.05) during exacerbations. Conclusions: 1. The quality of life of children aged 6-11 with cystic fibrosis was the best in the physical functioning and eating problems, and the worst in the domains of social functioning and digestive symptoms. Patients in this age group were generally characterized by a higher quality of life in almost every domain compared to people from the other groups. 2. The quality of life of children aged 12-13 with cystic fibrosis was the best in the domains of eating problems, social functioning, and respiratory symptoms, and the worst in the domains of social functioning, body image, and digestive symptoms. 3. The quality of life of adolescents aged 14 and older and adults with cystic fibrosis was the best in the domains of eating problems and digestive symptoms, and the worst in the domains of vitality and body image. 4. Patients with cystic fibrosis with exacerbations were characterized by a statistically significant lower quality of life than patients in a stable condition. The greatest differences were observed in the case of health perception (Z=−3.045; p=0.002) and body weight (Z=−2.944; p=0.003). 5. Patients with cystic fibrosis during the COVID-19 pandemic were characterized by ; a significantly higher quality of life in terms of treatment burden (Z=2.137; p=0.033) and social functioning (Z=2.102; p=0.036). The COVID-19 pandemic also had a significant and positive effect on the social functioning domain in the univariate analysis of covariance (F=5.89, p=0.018). 6. Patients with cystic fibrosis with a high level of physical functioning were characterized by a statistically significant better functioning of the respiratory system, especially in terms of FEV1% (rho=0.588; p<0.05). Treatment burden, body image and respiratory symptoms also had a statistically significant positive effect on all spirometry parameters except PEF%. 7. Most cystic fibrosis patients did not experience depressive episodes, and those that did occur were most often mild or moderate. 8. Most patients with CF did not experience a deterioration in their mental health, and the identified cases were mostly mild or moderate. 9. The COVID-19 pandemic did not have a statistically significant effect on the occurrence of depressive episodes in patients with cystic fibrosis (Δχ2=1.036, p=0.309). However, during the pandemic, significantly fewer patients experienced a deterioration in their mental health measured using the GHQ-12 questionnaire compared to those surveyed before the pandemic (Δχ2=7.723, p=0.005). 10. Most adult patients with cystic fibrosis had an optimal BMI, although, there wa ; s also a high number of underweight individuals. Overweight was rare in the study group, and none of the patients were obese. 11. Patients with cystic fibrosis who were underweight were characterized by poorer functioning of the respiratory system reflected in the results of spirometry compared to the other groups. The overweight patients were characterized by highest means and medians , and their group was the smallest. Underweight patients had statistically significant lower results in terms of VC% (Z=2.653; p=0.008), FVC% (Z=2.812; p=0.005), MEF% (Z=2.218; p=0.027) and MEF25% (Z=2.280; p=0.023) compared to those from the group combining people with optimal BMI and overweight. 12. Depending on the health status of patients with cystic fibrosis, individual biochemical blood parameters had a different effect on the functioning of the respiratory system. In patients in a stable condition, the number of lymphocytes, partial preassure of oxygen, oxygen saturation and iron concentration, together with their increases, had a statistically significant and positive effect on most spirometry parameters. Also, the parameters studied in patients during exacerbations, similarly to the stable condition, statistically significantly contributed to better functioning of the respiratory system. Additionally, with the increase in chloride ions concentration, spirometry results improved signific ; antly. In turn, a statistically significant adverse effect during exacerbations was observed in the number of basophils, bicarbonate concentration, standard base excess and glycosylated hemoglobin.

Place of publishing:

Kraków

Level of degree:

2 - studia doktoranckie

Degree grantor:

Rada Dyscypliny Nauki o zdrowiu

Promoter:

Bonior, Joanna ; Rachel, Marta

Date issued:

2024

Identifier:

oai:dl.cm-uj.krakow.pl:5191

Call number:

ZB-141713

Language:

pol; eng

Access rights:

tylko w bibliotece

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Jan 31, 2025

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Jan 31, 2025

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