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© Borgis - New Medicine 2/2016, s. 53-58 | DOI: 10.5604/14270994.1206757
*Tímea Tóth, Erzsèbet Mák, Nóra Galló, István Szabolcs
Research on the Quality of Life of Adult Patients with Cystic Fibrosis in Hungary
Department of Dietetics and Nutrition Sciences, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary
Head of Faculty: Professor Nagy Zoltán Zsolt, MD, PhD
Introduction. Cystic fibrosis (CF) is the most common autosomal recessive metabolic disease. All symptoms associated with the gastrointestinal system have adverse effects on the nutritional status, which, in turn, is closely related to the prognosis for the patient. Insufficient energy intake results in malnutrition, leading to worse respiratory function, and the early onset of comorbidities; it is accompanied with worse quality of life and a shortened lifespan.
Aim. The aim of our research was to assess the quality of life and nutritional status of adult patients with CF in Hungary and to determine the correlations between nutritional status, lung function and the quality of life. In Hungary, we are the first to study the relationship between nutritional status and the quality of life in adult CF patients.
Material and methods. 57 patients with CF from Hungary of both sexes were studied, all of them aged over 18. An assessment of the quality of life with a questionnaire was conducted among them. Their nutritional status was measured with the bioelectrical impedance analysis. The most recently measured FEV1 (forced expiratory volume in 1 second) values for the patients were analyzed. In order to assess the quality of life, the validated Hungarian translation of the disease-specific Cystic Fibrosis Questionnaire-Revised (CFQ-R) – Teen/Adult version for ages 14 through adulthood – was used.
Results. The nutritional status is worse in female patients than in male. Undergoing lung transplantation did not have any impact on the nutritional status. However, we found significant differences in the FEV1 values between the patients who underwent transplantation and those who did not.
No strong correlation was found between any parameters of the nutritional status, FEV1 and the domains of the quality of life.
In the mean values of the quality of life domains, the highest score appeared in the eating domain, while the lowest one was found in the weight domain. We did not find any significant differences between the quality of life of male and female patients, except for the respiratory symptoms domain, where women’s scores were lower. However, this result was not confirmed by FEV1 data. Transplant patients’ quality of life was higher than non-transplant ones in almost all domains, with 6 domains where statistically significant differences were found. The transplant CF patients, however, had lower scores in the weight and body image domains. CF patients with lower BMI had lower quality of life than their peers. Muscle mass percentage did not differ between groups. FEV1 showed a statistically significant correlation with six of the CFQ-R domains.
Conclusions. Our research is the first disease-specific assessment of the quality of life among Hungarian adult patients with CF. In Hungary, we are the first to study the relationship between nutritional status and the quality of life in adult CF patients. We conclude that early dietetical interventions should be implemented to improve patients’ condition and the quality of life.

Cystic fibrosis (hereinafter CF) is the most common autosomal recessive metabolic disease. It affects multiple organs and, therefore, can present with different symptoms, such as chronic respiratory disease, gastrointestinal symptoms, as well as changes in the pancreas, liver, biliary tract or reproductive tract. The disorder is characterized by the dysfunction of exocrine glands, which produce abnormally thick and sticky mucus. The phlegm congestion causes obstructive lesions in many vital organs, especially in the lungs and in the pancreas (1). The so-called CF-related diseases, such as diabetes mellitus (cystic fibrosis related diabetes – CFRD), nasal polyposis, osteoporosis, chronic sinusitis or arthropathy, are likely to appear with ageing.
In 2012, there were 579 patients in the Hungarian registry of CF patients, including 261 women and 318 men. 248 out of these patients were adults. The proportion of adults among the patients has become significantly higher in the recent years due to improvements in therapy and easier access to transplantation. Mean age of CF patients accounted for 14.77 years of age in 2008 and rose to 16.54 years of age in 2012 (2). Due to the formerly relatively small adult population of the patients, it was not possible to conduct a proper analysis on them. Today, however, their increasing number enables to describe their problems with more accuracy.
All the symptoms related to the gastrointestinal system compromise the nutritional status. Since the nutritional status plays a significant role in the progression of cystic fibrosis, dietotherapy must be an essential part of the treatment (3, 4). Patients with good nutritional status are more likely to preserve or improve their health status, which also affects their quality of life (5). On the other hand, lower nutritional status increases the risk of death (6, 7). Therefore, watching for signs of malnutrition and promoting the development of an adequate nutritional status are crucial for optimal treatment of CF (5).
Due to the gastrointestinal symptoms, deficiencies are likely to be present in CF patients. To assess the patients’ nutritional status, several parameters can be used. Body mass index (BMI) and body fat percentage accurately depict the nutritional status. Studies have shown that, thanks to easy calculation and precise results, BMI can be applied with good effects as a help in assessing CF patients. Bioelectrical impedance analysis is commonly used for estimating body fat percentage.
The best predictor of mortality rate in CF is the decrease in the lung function, as the exacerbation of a chronic lung disease remains the leading cause of death in CF (8). There is a direct correlation between the nutritional status, energy consumption and the functional capacity of skeletal and respiratory muscles. Patients with higher body mass index often have better lung function (7). Body weight and body mass index correlate with the FEV1 value (forced expiratory volume in 1 second) (9).
In addition to physical, physiological and biochemical parameters, other factors must be taken into account when assessing the patient’s health status, such as the individual’s subjective perception of their own life, their physical and mental status, the ability to perform their everyday activities, their social perceptions, as well as the impact of all these factors on their health status. Quality of life tests can be applied specifically for this purpose.
Today there are standardised and validated methods available for the assessment of the quality of life, which include general and disease-specific questionnaires. The second ones are more sensitive and consequently, are able to detect smaller changes in the health status of a given group of patients, but they preclude the comparison of patients suffering from different diseases. The quality of life questionnaires can be further divided into index- and profile-type questionnaires. Index-type questionnaires present the quality of life as a list of scores, whereas profile-type questionnaires measure the quality of different life domains.
Among various data collecting methods, self-administered questionnaires are the most common one. They are easy to use, cost-efficient, as well as they are able to provide standardised measurements (10).
The Cystic Fibrosis Questionnaire-Revised (CFQ-R), which has a number of versions dedicated for specific age groups (children, adolescents and adults, parents), can be used specifically to measure the quality of life of patients with cystic fibrosis.
The aim of this study was to assess the quality of life in adult patients with CF in Hungary, as it had never been previously assessed. We aimed to collect the data about patients’ nutritional status, lung function, as well as to identify correlations between BMI, body fat percentage, muscle percentage, FEV1 and quality of life domains.
Material and methods
Questionnaires were used to assess the quality of life in CF patients. Participants’ nutritional parameters, including weight, height, BMI, body fat percentage and muscle percentage, were assessed. This paper analyses BMI, body fat percentage, muscle percentage, FEV1 and quality of life only. We collected surveys from 57 patients over 18 years of age.
Personal data, including anthropometric measurements and questionnaires, were collected multiple times among outpatients and inpatients in the years 2013 and 2014.
Inclusion criteria were as follows: diagnosed cystic fibrosis, age over 18 years of age, appropriate cooperative skills, willingness to fill in the questionnaire as well as to provide anthropometric data and to complete the written consent form. Inadequate cooperation was considered an exclusion criterion.
Nutritional status and lung function
The patients’ nutritional status was assessed with bioelectrical impedance devices, which enabled to obtain the patients’ body weight, body fat percentage, muscle percentage and body mass index (after entering the height of the participant).
To obtain the indicators of the lung function, we collected the most recently measured FEV1 values for each patient.
Quality of life
To assess the quality of life, we used the validated Hungarian translation of the disease specific Cystic Fibrosis Questionnaire-Revised (CFQ-R) – Teen/Adult version for ages 14 through adulthood (11, 12). This questionnaire consists of 50 separate questions, which group the aspects of the quality of life into 12 domains: physical functioning, emotional functioning, social functioning, body image, eating, treatment burden, respiratory and digestive symptoms, role fulfilment, weight, health perception and vitality. CFQ-R is a profile-type questionnaire. The scores for the subscales range from 0 to 100, where higher score indicates higher quality of life in the given domain. 54 out of 57 participants filled in the questionnaire, 41 underwent bioelectrical impedance analysis and 38 had a complete data file.
Statistical analysis
To analyse the data, we used the IBM SPSS Statistics 21.0 and Microsoft Office Excel, version 2010, software. Differences were considered statistically significant when p values were ≤ 0.05. We used Spearman’s rank correlation coefficient (r) to express the strength of correlations. Correlation coefficients were defined as weak if r < 0.3, moderate if r ranged from 0.3 to 0.7 and strong if r > 0.7 (13). We used nonparametric tests to measure the alterations of the continuous variables and the Mann-Whitney test and Kruskal-Wallis test, depending on the size of the categories, to analyse the relationship between them. In order to analyse the relationship between categorical variables, we used the chi-square test. We have also applied the nonparametric Mann-Whitney or the Kruskal-Wallis tests to calculate and measure the factors that affect the nutritional status.
The average age of the patients was 28.25 (± 8.95) years of age – 28.46 (± 9.5) for men, and 28.07 (± 8.59) for women. The 54 evaluable questionnaires revealed that there were 10 transplant patients among the respondents, evenly divided between the sexes. The majority of the non-transplant patients were female.

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otrzymano: 2016-05-04
zaakceptowano do druku: 2016-05-25

Adres do korespondencji:
*Tímea Tóth
Department of Dietetics and Nutrition Sciences Semmelweis University
17 Vas ut., H-1088, Budapest, Hungary
tel.: +36-30-214-9750
fax: +36-1-486-4830
e-mail: tothtimea@se-etk.hu

New Medicine 2/2016
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