This study is a clinical trial. Convenience sampling was done based on the inclusion criteria consisting of the child's fluency in Farsi, no chronic mental disease or other physical illnesses, living with both parents, the parents being aware of the FEV1 level or having a spirometry report, and having a CD Player. The subjects were assigned to the intervention and control groups randomly, based on their medical files’ numbers. Even and odd numbers were placed in the intervention and control groups, respectively. The exclusion criteria consisted of quitting the trainings after two sessions, and the deterioration of the child’s physical condition and his/her hospitalization. The necessary number of subjects in each group was calculated to be 64 according to the sample size determination formula.
The data was collected using two questionnaires:
- The child’s demographic and clinical information questionnaire consists of 8 sections, and covers basic information (age, sex, birth order, lifestyle, number of school absences), nutrition assessment (height, weight, body mass index), systems assessment, spirometry report, etc.
- Revised Cystic Fibrosis Questionnaire
The version for the parents of 6 to 11-year-old children was completed through interviews, and the version for 11 to 13-year-old children was filled out by the child, as a self-report (Groeneveld I. SE, 2012; Henry B. AP, 2003). In order to assess health-related quality of life, the translated Farsi version of the questionnaire was received from the translator and used in the study.
A study was conducted by Ariafar (2016) in Iran to examine the face validity and the content validity of the Farsi version of this questionnaire, the results of which show that all the items of both Farsi versions (parents and children) have acceptable face and content validities. Based on this finding, for all the items, the CVR was calculated to be above 0.49; the CVI, above 0.79; and the impact score, above 1.5 (Talebi M., 2016).
2.1. Revised Cystic Fibrosis Questionnaire (the version for 11 to 13-year-old children)
The questionnaire has 35 main items, 16 of which are scored on a 4-point Likert scale including always, often, sometimes, and never. Other 19 items have true/false answers including absolutely true, somewhat true, somewhat false, and absolutely false. The obtained scores fall between 0 and 100, and higher scores indicate a better quality of life.
2.2. Revised Cystic Fibrosis Questionnaire (the version for the parents of 6 to 11-year-old children)
The parents’ report of the health-related quality of life in the children with cystic fibrosis are assessed using Parent CFQ-R, which consists of 44 items with 4 options. The scores range from 0 to 100, and a higher score indicates a better quality of life.
In the present study, in order to determine the qualitative content validity of the CFQ-R (both versions: for parents and for children), the questionnaires were distributed among 10 faculty members of the School of Nursing and Midwifery, Shahid Beheshti University of Medical Sciences. In addition, in order to examine the face validity, the scale was provided to 10 children and 10 mothers meeting the inclusion criteria. Then the internal consistency reliability and the stability reliability were measured. To examine the internal consistency, the Cronbach’s alpha was calculated to be 0.89 and 0.81, and the ICC, 0.94 and 0.91, for the Parent’s Version and the Child’s Version of CFQ-R, respectively.
In this study, after obtaining the necessary permits, offering explanations to the parents, and receiving written informed consents, the sampling was done from Oct 2012 to Feb 2021 (Fig. 1).
At first, the basic data were collected through the researcher-developed demographic and clinical information questionnaires for examining the children and CFQ-R, in both control and intervention groups, after informed consent forms were signed by the parents. The patients in the control group received routine follow-ups, i.e., monthly visits to the cystic fibrosis clinic, and taking the prescribed medications. In the intervention group, pulmonary rehabilitation was performed. The educational content of the sessions and breathing exercises were provided by the research team in the form of a booklet and based on the references of Cystic Fibrosis Foundation documents. Text reviews were done and offered in a booklet by the research team, whose validity was approved by the professors of Pediatric Nursing Department of the School of Nursing and Midwifery of Shahid Beheshti University of Medical Sciences, a pediatric gastroenterology and pulmonology specialist, a physiotherapist, and a nutritionist. Moreover, the researcher was instructed in performing physical exercises by a physiotherapist in a physiotherapy clinic. The training was recorded as videos due to the children’s lack of presence in the hospital. The physical exercises were provided as CDs, and the educational content, in the form of booklets for the parents and stories for the children. Besides, the educational videos were uploaded in the WhatsApp group. The educational content included familiarity with cystic fibrosis and pulmonary rehabilitation, chest physiotherapy through several different approaches, medications and oxygen therapy, the management and the prevention of attacks, and tips on a healthy lifestyle.
Due to COVID-19 pandemic, the intervention was done in 12 sessions (1 in-person and 11 remote sessions) during 6 weeks, in two parts, including education and exercises. At the end of Week 6, after the intervention was completed, CFQ-R was completed in both control and intervention groups. Again, 8 weeks after the intervention, the questionnaires were sent to the mothers’ WhatsApp accounts and completed by them.
At the end, in order to observe ethical considerations, the researcher provided the control group subjects with all the educational content in the form of booklets, and the physical exercises as videos in the form of CDs.
In this research, Kolmogorov–Smirnov test was used to examine the normality of the data and the student’s t-test, to compare the mean scores between the intervention and the control groups. Repeated-measures ANOVA was also applied with the aim of investigating the relationship between the qualitative variables of the research, in both control and intervention groups. The results were then analyzed using SPSS V25 considering the level of significance of 0.05.