A quality-of-life measure for adults with primary ciliary dyskinesia: QOL–PCD

Lucas, Jane S.; Behan, Laura; Galvin, Audrey Dunn; Alpern, Adrianne N.; Morris, Anjana M.; Carroll, Mary; Knowles, Michael R.; Leigh, Margaret W.; Quittner, Alexandra L.

doi:10.1183/09031936.00216214

Cited by 69 publications

(96 citation statements)

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“…Lucas et al (2015) state that in fact, lot of stated facts influence the health status and life of a human being already in a young age. We do not realize consequences of a current hurried time, until the health problems emerge.…”

Section: Introductionmentioning

confidence: 99%

“…Fyodorov & Erlikh (2016) define the quality of life as a complete satisfaction with a life and a complete feeling of an individual well-being, of a mental harmony and a life contentment. Lucas et al (2015) also state that a synthesizing term of the life quality implies to social, economic and individual mental well-being indicators. It represents a summary of the key social life features, individuals, units of society and includes socio-political, economic, legislative, ecologicenvironmental, mental and medical-health aspects.…”

Section: Introductionmentioning

confidence: 99%

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2017

JPES

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A listed pilot survey presents results from a health and a healthy lifestyle field. Its aim was to establish a level of selected lifestyle and health determinants of adolescent students from Secondary School of Medicine in Banská Bystrica. A research group consisted of female students, ranging from the first to the fourth grade of a stated school. A total number was 421 female students and their average age was 16.74 years.We used the second field of a standardized questionnaire CINDI "health services and health status", which was created by World Health Organization (WHO). The research affirmed a presence of a musculoskeletal system weakening, especially back pain, during the last year. Statistically significant differences among groups were not detected. A low effect of effect size was detected, which means that result was not influenced by statistics possibilities (p>0.05; Chí = 2.171; w = 0.144).A presence of problems was much more diverse, during the last month, within which we could observe some connection between the particular problems. The students stated that a headache caused them most of the problems. It can be linked to a sleeplessness and a back, a scruff and a shoulders pain. The sleeplessness is to a large extent caused by a stress, which occurred in 83.4 % from all cases (p<0.01; Chí = 6.653; w = 0.253). Despite this unfavourable ascertainment, 75.9 % of the adolescent female students from the first and 73.2 % from the second group evaluate their health status to be positive (p<0.05; Chi = 9.516; φ c = 0.304). However, it clashes with a previous ascertainment because almost 80 % of all respondents have 3 and more ascertained health problems within the last month.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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2017

JPES

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“…Moreover, there are different domains for various symptoms at different age groups. The total numbers of items in the questionnaires are 37 in the questionnaire for children, 43 in the one for adolescents, 49 in the one for adults, 41 in the parents' questionnaire [5][6][7]. Content validity of this questionnaire has been shown in English-speaking populations [6].…”

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Primary Ciliary Dyskinesia: Ready for Quality of Life Assessment

Yılmaz

Akgün

2017

Turk Thorac J

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Primary ciliary dyskinesia (PCD) is a chronic, inherited disease characterized by ciliary dysfunction leading to defects in mucociliary clearance, thus causing upper and lower respiratory problems such as sinusitis and bronchiectasis as well as infertility. Diagnostic workup includes a combination of different techniques such as nasal nitric oxide measurement, high-speed video microscopy analysis, electron microscopy, and genetic analysis because there is no single gold standard due to the genetic and phenotypic heterogeneity of the disease [1,2].Due to the chronic nature of the disease, difficulty in expectorating respiratory secretions, chronic presence of symptoms such as productive cough, and requirement of daily treatment, this disease poses a burden on the patients and their families. PCD negatively impacts quality of life (QOL) because of disease complications as well as treatment burden. Moreover, treatment adherence decreases with time in patients, which might further deteriorate prognosis [3].World Health Organization defines "health" as a complete state of physical, mental, and social well-being; thus, patientreported outcomes, especially health-related QOL (HRQOL), are important components of health and need to be assessed as part of a routine care in chronic diseases such as PCD [4].In the previous issue of the Turkish Thoracic Journal, Emiralioglu et al. described the translation procedure of PCD-specific HRQOL. Forward translation procedures followed international standards, where two independent and blinded translators fluent in English and Turkish participated. Backward translation into English was performed by a third independent translator. The final Turkish translations of the questionnaires were applied to five subjects from each age group: adult, adolescent, and pediatric PCD patients as well as five parents. After the subjects completed the questionnaire, a cognitive debriefing session was performed, where each item of the questionnaire was discussed by the subjects. Finally, all these information were used to achieve a final Turkish translation of the PCD-specific HRQOL that is ready for validation and reliability studies [5].The PCD-QOL questionnaire developed by Lucas et al. has different domains for different age groups: pediatric, adolescent, and adult patients as well as parents. These domains mainly evaluate physical, emotional, and social aspects of PCD related to QOL. Moreover, there are different domains for various symptoms at different age groups. The total numbers of items in the questionnaires are 37 in the questionnaire for children, 43 in the one for adolescents, 49 in the one for adults, 41 in the parents' questionnaire [5][6][7]. Content validity of this questionnaire has been shown in English-speaking populations [6]. However, translations of QOL measures require cultural adaptation besides linguistic translation, and cognitive debriefing is one of the most important steps of this adaptation.Assessment of QOL is an essential part of follow-up of children with chronic dis...

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“…At present, it is known that the quality of life scales, which are gradually gaining importance, are used as essential tools for assessing patients in clinical research studies as well as in the daily follow-up of chronic disease patients [9][10][11]. …”

Section: Introductionmentioning

confidence: 99%

“…Although high-resolution computed tomography of the lungs is a useful technique in the monitoring of patients, it is impossible to perform tomography for all patients at certain intervals in practice [5][6][7][8]. Therefore, a quality of life scale is needed to evaluate the effects of PCD on patients [9].At present, it is known that the quality of life scales, which are gradually gaining importance, are used as essential tools for assessing patients in clinical research studies as well as in the daily follow-up of chronic disease patients [9][10][11]. …”

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confidence: 99%

Quality of Life Questionnaire for Turkish Patients with Primary Ciliary Dyskinesia

2017

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OBJECTIVES:Primary ciliary dyskinesia (PCD) is a major cause of progressive lung disease, and physiological measures do not reflect the impact of the disease on patients' daily symptoms or physical and social functions. We need valid and reliable health-related qualityof-life (HRQOL) measures in PCD to assess the symptoms and daily functions from the patient's perspective. Our aim was to develop a Turkish translation of PCD-specific HRQOL questionnairre to be used as outcomes in clinical trials. MATERIAL AND METHODS:This study was conducted at the Division of Pediatric Pulmonology, Hacettepe University Faculty of Medicine and the Division of Pediatric Pulmonology, Marmara University Faculty of Medicine. Forward and back translations were performed by three different translators. We recruited participants with PCD from different age groups of both sexes, with an aim to represent a wide spectrum of disease severity and performed the prototype of the translation in these participants. RESULTS:Five participants from each age group [children (6-12 years), teenagers (13-17 years), adults (18+ years) and parents of children aged from 6 to 12 years] responded to the HRQOL questionnaire. Content analysis of the questions included the following domains depending on age: Respiratory Symptoms, Physical Functioning, Emotional Functioning, Treatment Burden, Ears and Hearing, Sinus Symptoms, Social Functioning, Role Functioning, Vitality, Health Perceptions, School Functioning, Eating and Weight. After the participants have completed the questionnaire, a cognitive debriefing interview was conducted with them, and the results of the interviews were used to form a final version of PCD-specific HRQOL, ready for formal validation. CONCLUSION:A Turkish translation of PCD-specific HRQOL questionnaire was developed to meet the standards set by international guidelines. This questionnaire is expected to be useful as end points in clinical trials for monitoring health outcomes and for improving clinical decisions. KEYWORDS:Primary ciliary dyskinesia, quality of life questionnaire, forward translation INTRODUCTION Primary ciliary dyskinesia (PCD) is a clinical disorder characterized by chronic lower and upper respiratory tract infections associated with impaired ciliary motility. It has clinical and genetic heterogeneity, and it is mostly inherited as an autosomal recessive disease. Its incidence has been reported to be 1 in 4,000-40,000 live births in various studies conducted to determine its frequency in different societies [1,2]. In our country, where the rate of consanguineous marriage is high, the incidence of PCD is also estimated to be high; however, its exact incidence is unknown. An abnormal ciliary structure and function are found in PCD. Recurrent pulmonary infections due to mucociliary clearance dysfunction in the respiratory system; lower and upper respiratory tract diseases such as bronchiectasis, sinusitis, rhinitis, and decreased hearing; infertility due to sperm immotility in men; and decreased fertility and ectopic pr...

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A quality-of-life measure for adults with primary ciliary dyskinesia: QOL–PCD

Cited by 69 publications

References 26 publications

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Primary Ciliary Dyskinesia: Ready for Quality of Life Assessment

Quality of Life Questionnaire for Turkish Patients with Primary Ciliary Dyskinesia

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