Assessment of patients' baseline cystic fibrosis knowledge levels following translation and adaptation of the CF R.I.S.E translation program into Turkish

Abstract

Background

Cystic fibrosis (CF) patients have a limited life expectancy, but significant medical advances now highlight the need for successful transition programs from pediatric to adult care. The goal of this project was to introduce the transition program CF R.I.S.E (Responsibility. Independence. Self‐care. Education.) to a CF center with limited resources at Marmara University (MU).

Methods

The program was adapted and translated into Turkish with the CF Foundation's permission. A team of experts collaborated to develop educational materials for patients and families. After translation and implementation of the CF S.O.B.E program, 11 Knowledge Assessment Questionnaire (KQA) tests were administered online to the patients aged between 16 and 25 years to assess the lack of patient knowledge.

Results

The CF R.I.S.E program was successfully implemented within 6 months. A pilot study showed positive feedback from randomly selected patients, indicating the program's effectiveness and understandability. The mean age of the patients was 19.4 ± 2.9 years, and 42 (52%) were female. The mean forced expiratory volume (FEV1pp) was 76.3 ± 23.2. Fourteen (17.3%) and 4 (4.9%) of the patients colonized with Pseudomonas aeruginosa and Methicillin‐resistant Staphylococcus aureus (MRSA), respectively. Fifteen (18.5%) were on modulator therapy. Eleven Knowledge Assessment Questionnaires (KAQ) surveys were administered to 81 patients. The percentage of correct responses to the KAQs ranged from 47.9% to 68.3%.

Conclusion

MU CF Center in Turkey implemented the CF S.O.B.E (Responsibility, Self‐care, Independence, and Education in Turkish) program. The center aims to make the program a regular practice and expand collaboration with adult clinics. Future studies will assess its long‐term impact and applicability in different health settings. The final goal is to disseminate the program's resources and promote structured transition practices across the country.

1. INTRODUCTION

Cystic Fibrosis (CF) is a genetic disorder with an incidence rate of 1/3000–1/6000 live births. ¹ , ² , ³ In the 1930s, the life expectancy of people with CF (pwCF) was limited to just a few years; however, medical advancements have increased life expectancy, with modulatory drugs targeting specific gene defects being effective in the majority of the CF population. ⁴ Currently, more than half of the CF population in Europe, Canada, and North America is over 18 years old; a significant increase is expected in the number of pwCF transitioning from pediatric to adult care. ⁵ , ⁶ The transition period can be stressful for young adults with chronic diseases and result in poor health outcomes. ⁷

A structured transition program can help prepare pwCF and their caregivers, leading to higher patient satisfaction, lower anxiety, and greater self‐esteem. ⁸ , ⁹ When developing a successful framework for transitioning healthcare services for pwCF, it is important to consider strategies that will help prepare pwCF and their caregivers, as well as tools that can track and measure transition progress at various stages. ¹⁰

A comprehensive transition program should involve a well‐coordinated approach that includes a clinical summary with contributions from all members of the pediatric multidisciplinary team (nurses, doctors, dieticians, psychologists, social workers, and physiotherapists), an opportunity to meet the adult care team at the adult CF center, and ensuring timely access to an adult care provider. ¹¹ , ¹² In CF transfer programs, the involvement of social workers is paramount for seamless transition from pediatric to adult care. These professionals form a multidisciplinary transition team, with social workers often serving as the “transition coordinators” for individual patients. ¹² , ¹³ As the number of adults with CF increases, comprehensive transition programs and clinics are becoming more common in many countries. ¹⁴ , ¹⁵ , ¹⁶

CF R.I.S.E. (Responsibility, Independence, Self‐Care, Education) is a planned, structured transition program created to improve quality of life, maximize independence, and minimize interruptions in care as patients transition from pediatrics to adult subspecialists. ¹² , ¹⁶ This program has been successfully implemented in the United States since 2015, providing tools and resources to help patients and caregivers understand the disease and develop skills to manage it independently. CF R.I.S.E. aims to provide a gradual and purposeful transition of responsibility over time from support persons to patients while facilitating communication among pediatric and adult care teams, patients, and caregivers. ¹⁷ The program has the potential to address deficits in transition and has been positively evaluated by CF healthcare providers during its implementation period. ¹⁰ , ¹²

Although the number of adult patients has been increasing steadily over the years, the number of adults with CF in Turkey is relatively lower than that in Europe and North America. ¹⁸ Being the largest center in Turkey, there are 424 individuals with CF in Marmara CF center and only 103 (24.3%) are adults. As there was no structured transition program in our center, we decided to implement the CF R.I.S.E program by making the necessary translations and adaptations during the CF Foundation (CFF) Virtual Improvement Program‐F7 (VIP‐F7) training program. We named our transition program CF S.O.B.E, which consists of the first letters of responsibility, self‐care, independence, and education in Turkish.

Our aim was to convey our practical experience in the context of adapting and implementing the CF R.I.S.E program within a CF center with limited resources. We also aimed to provide an overview of the fundamental attributes and cognitive appraisal levels of our patient cohort with the ultimate goal of identifying areas where educational interventions may be required. To our knowledge, this is the first implementation of the CF R.I.S.E. program outside the United States and in language other than English.

2. MATERIALS AND METHODS

2.1. Setting and design

This is a quality improvement (QI) project created within the VIP‐7 program in March 2022 at the Marmara University (MU) Faculty of Medicine Selim Coremen CF Center. The study was approved by the Ethics Review Board of the participating institutes of the Ethics Committee of Marmara University. (Project No: 07.10.2022.1359). pwCF between the ages of 16–25 who were followed up in our center, resided in Istanbul, and could regularly come to CF clinics, were selected for the implementation of CF R.I.S.E. Permission was obtained from the CFF for the translation and use of CF R.I.S.E. materials. Written consent was obtained from all the participants and their families.

2.2. MU CF team

The MU CF team consisted of five pediatric pulmonologists, six pediatric pulmonology fellows, two CF nurses, one dietitian, one physiotherapist, and one patient representative from the Turkish Cystic Fibrosis Association (KIFDER). As part of the CFF QI project, all transition team members regularly attend weekly QI meetings with two QI coaches from October 2021 to October 2022. A social worker and psychologist are not part of the CF team.

2.3. Step 1: Translation and adaptation

2.3.1. CF R.I.S.E program

The CF R.I.S.E. program includes 13 Knowledge Assessment Questionnaires (KAQ) and six responsibility checklist (RCL) forms. ¹⁹ The purpose of the KAQ is to evaluate an individual's basic knowledge of CF. It covered topics related to lung and liver health, infection prevention methods, pancreatic insufficiency, nutritional principles, endocrinological problems, school, work, financial, and social security. These assessments have gradually been introduced. Each module is completed independently by the pwCF without parental or team assistance, to accurately assess areas for knowledge improvement. Once completed, the responsible CF care team member devised a plan to address the identified knowledge gaps. After providing the necessary training, the modules are reassessed in subsequent visits or in 6–12 months to measure progress. ¹⁹

The CF R.I.S.E program covers six responsibility checklists: (1) Working with the CF care team and other healthcare professionals; (2) Responsibility of CF treatments; (3) Living with CF; (4) CF transition; (5) School and Career Plan; and (6) Finance and Insurance. These were designed to facilitate the acquisition of age‐related self‐care skills. Although KAQs are applied only to patients, RCL forms are collaborative assessments involving a designated support individual and CF healthcare team to assess and monitor the patient's current level of responsibility. In the RCL forms, patients/parents were required to respond to each question using a Likert‐type scale ranging from 1 to 5. The results of each question are compared between the parent and pwCF, and if there is inconsistency, the individual's level of responsibility for that question is reevaluated. ¹⁹

A target responsibility is identified for the next meeting between the transition clinic (TC) representative and pwCF based on the RCL results. The assigned tasks are recorded in a progress report, signed by both parties, and a copy of the progress report is given to the pwCF. The assigned responsibility is determined based on the individual's needs and level, and the interaction between the TC representative and pwCF is contractual in nature. The progress of patients is reassessed 1 year later with renewed RCLs. ¹⁹

2.3.2. Translation of CF R.I.S.E materials in Turkish

1. Translation and adaptation: All materials of the CF R.I.S.E program, which are intended for individuals with CF aged 16–25, were translated into Turkish by the MU team. Necessary changes were made to the health and finance, business, and college program contents according to the conditions in our country.

2. The translated materials were evaluated for comprehensibility and content by senior team members and a team of volunteers from KIFDER, including a pwCF, a Turkish grammar teacher, and two other members of KIFDER. The materials were designed to be understandable at the third or fifth‐grade level. All translated and newly created materials were shared with the CFF.

Since the College‐Business Life and Finance‐Social Security KAQs in CF R.I.S.E contain questions about the educational and social security system in the United States, we have created new KAQs suitable for the conditions of our country and the rights of the disabled. The two KAQs of Lifestyle and Mental Health were combined into a new KAQ called “Lifestyle and Mental Health” (Table 1).

Table 1.

Comparison of CF R.I.S.E and CF S.O.B.E knowledge assessment questionnaires.

CF‐R.I.S.E	Number of questions	CF‐ S.O.B. E	Number of questions
1‐ Lung Health & Airway Clearance	16	1‐ Lung Health & Airway Clearance	16
2‐Pancreatic Insufficiency & Nutrition	16	2‐ Pancreatic Insufficiency& Nutrition	20
3‐CF Liver Disease	6	3‐ CF Liver Disease	5
4‐ CF‐Related Diabetes	14	4‐ CF‐Related Diabetes	14
4‐ General CF Health	10	5‐ General CF Health	9
6‐Screening & Prevention	7	6‐ Screening & Prevention	7
7‐Equipment Maintenance & Infection Control	9	7‐ Equipment Maintenance & Infection Control	9
8‐Female Sexual Health	9	8‐ Female Sexual Health	9
9‐ Male Sexual Health	7	9‐ Male Sexual Health	5
10‐ Lifestyle	17	10‐ Lifestyle and Emotional Health	17
11‐ Emotional Health	17
12‐Insurance & Financial	17	11‐ School & Work & Insurance	12
13‐ College & Work	17

Note: Table 1 provides a comparative presentation of the headlines of KQAs encompassed within the CF R.I.S.E and KF SOBE programs. Instead of the University‐Business Life and Finance‐Social Security KAQs in CF R.I.S.E, new KAQs were created in accordance with Turkey's conditions and disabled rights. The two KAQs on Lifestyle and Mental Health have been combined into a new KAQ called “Lifestyle and Mental.

Abbreviations: CF, cystic fibrosis; R.I.S.E., Responsibility.Independence.Self‐care.Education.; S.O.B.E., Responsibility, Self‐care, Independence, and Education in Turkish.

2.3.3. Patient education materials

Educational resources, including patient leaflets, webinars, and videos, were produced on all topics covered by the KAQ, going beyond the materials provided and translated in the original CF RISE program. As part of the CF S.O.B.E (Responsibility, Self‐care, Independence, and Education in Turkish) initiative, webinars, patient leaflets, and videos were available online through a website created specifically for this project. ²⁰

2.4. Step 2: Timeline

We planned to reassess patients' knowledge levels regarding the completed topics every 6 months via KAQs. We also planned to identify incorrect answers and focus on these subjects in their subsequent clinical appointments to provide the correct answers face‐to‐face. We created four training modules and determined the dates for online meetings every 3 months to cover the related content of these training modules. We also aimed to apply two different RCLs at each appointment and reapply them after 12 months. The timeline created within this scope is provided in detail in Figure 1.

Figure 1.

CF S.O.B.E education modules and program timeline. CF, cystic fibrosis; S.O.B.E., Responsibility, Self‐care, Independence, and Education in Turkish.

2.5. Step 3: Pilot study

A pilot study was conducted with a 16‐year‐old male and a 21‐year‐old female patient randomly selected by TC team members. During the pilot study, both patients were asked to complete 11 core KAQs and two responsibility checklists. Both patients confirmed that the materials were understandable and informative. They expressed their excitement about the implementation of such a program. The pilot visits lasted 30 and 40 min, respectively. Feedback from the CF team revealed that patients would need an extra 30–40 min in addition to their routine clinical procedures. After the pilot study, the program was introduced to all the families and patients through an online meeting.

2.6. Step 4: Assessment of initial knowledge level

The CF S.O.B.E. program was introduced to patients aged 16–25 and their families through an online meeting. Eleven KAQs were distributed via a Survey Monkey WhatsApp questionnaire to patients within a 2‐week period to gauge their baseline knowledge about CF disease.

SPSS (version 22.0) was used to analyze the data. Descriptive statistics, such as counts, means, standard deviations (SDs), and interquartile ranges (IQR), were applied to provide numerical summaries based on the distribution of continuous variables. “This study evaluated the effect of liver disease, pancreatic insufficiency, and diabetes on patients' baseline knowledge levels regarding these conditions, as well as the influence of gender on sexual health knowledge. To achieve this, independent samples t tests were conducted for each specific condition and questionnaire. For example, we compared the knowledge scores of patients with and without liver disease for the liver disease questionnaire, and similarly for the other conditions. For sexual health knowledge, we compared the scores between female and male patients. A significance level of p < 0.05 was adopted as the threshold for statistical significance.”

3. RESULTS

Eleven KAQ surveys were administered to 81 patients aged between 16 and 25 years to assess the lack of patient knowledge. The response rate was 95.1%. The median age of the patients was 18.5 years (with a range of 16.9–21.2 years). Of the 81 patients, 42 (52%) were female. The median forced expiratory volume (FEV1pp) was 81.6 (with a range of 59.2–93.2). Fourteen (17.3%) patients were colonized with —Pseudomonas aeruginosa, and four (4.9%) were colonized with Methicillin‐resistant Staphylococcus aureus (MRSA). Fifteen patients (18.5%) underwent modulator therapy. Demographic and clinical features of the patients are shown in Table 2.

Table 2.

Demographic and clinical characteristics of the study population.

Characteristics of participants
People with CF	81 (100%)
Age, median (IQR)	18.5 (16.9‐21.2)
Sex, female, n (%)	42 (52.0%)
% FEV pred., median (IQR)	81.6 (59.2‐ 93.2)
BMI Z‐score, median (IQR)	−0.17 (−1.2‐0.5)
Pseudomonas aeruginosa colonization, n (%)	14 (17.2%)
MRSA colonization, n (%)	4 (4.9%)
Allergic Bronchopulmonary Aspergillosis, n (%)	4 (4.9%)
Pancreatic insufficiency, n (%)	59 (72.8%)
Liver disease, n (%)	22 (27.2%)
Diabetes mellitus, n (%)	9 (11.1%)
Noninvasive ventilation, n (%)	1 (1.2%)
Supplemental O2 requirement, n (%)	6 (7.4%)
Usage of CFTR modulator therapy	15 (18.5%)

Abbreviations: BMI, body mass index; CF, cystic fibrosis; CFTR, cystic Fibrosis transmembrane conductance regulator; FEV, forced expiratory volume; MRSA, methicillin‐resistant Staphylococcus aureus.

The highest correct answer rates were observed in the areas of “Equipment Maintenance and Infection Control” (68%), Lifestyle (65.6%), and Lung Health and Airway Clearance (64.1%). On the other hand, the lowest correct answer rates were recorded in the fields of “CF‐Related Liver Disease (CFLD)” (47.9%), “Men's Sexual Health” (53.8%), and “School&Work&Health Insurance” (53.6%) (Table 3).

Table 3.

Descriptive data for baseline knowledge assessment questionnaires.

Knowledge assessment questionnaires	Number of questions	Completed assessments (n, %)	KAQ correct answers (%)	KAQ mean correct answers (mean ± SD)	Min.	Max.
1‐Lung health and airway clearance	16	81 (100%)	64.1	10.3 ± 2.9	4	15
2‐Pancreatic insufficiency & nutrition	20	80 (99%)	62.7	12.5 ± 4.0	4	20
3‐ CF‐related liver disease	5	80 (99%)	47.9	2.4 ± 1.3	0	5
4‐ CF‐related diabetes	14	80 (99%)	54.9	7.8 ± 2.5	2	13
5‐ General CF health	9	80 (99%)	62.6	5.6 ± 2.0	0	9
6‐ Screening and prevention	7	80 (99%)	64.4	4.6 ± 1.8	0	7
7‐ Equipment maintenance and infection control	9	79 (98%)	68.3	6.3 ± 1.7	1	9
8‐ Male sexual health	5	77 (95%)	53.8	2.8 ± 1.4	0	5
9‐ Female sexual health	9	77 (95%)	59.8	5.7 ± 2.2	1	9
10‐ Lifestyle	17	78 (96%)	65.6	12.3 ± 3.4	3	17
11‐School & work & health Insurance	12	77 (95%)	53.6	6.7 ± 2.5	0	12

Abbreviations: CF, cystic fibrosis; KAQ, Knowledge Assessment Questionnaires.

No significant disparities in sexual health knowledge were observed between males and females for both questionnaires: Female Sexual Health (p = 0.33) and Male Sexual Health (p = 0.83). Moreover, the presence of CFLD did not exhibit a substantial impact on participants' knowledge levels (p = 0.62). In contrast, individuals with conditions like pancreatic insufficiency and diabetes scored higher in these KAQs than those without these conditions, as evidenced by the statistically significant p values of 0.01 and 0.002, respectively.

4. DISCUSSION

In this QI program, we aimed to report our experiences regarding the adaptation and implementation of the CF R.I.S.E program in a CF center with limited resources. The successful translation and implementation of the program were accomplished within a 6‐month period. This program was implemented through strong collaboration among pediatric pulmonologists, CF nurses, dietitians, and volunteer patient representatives without any grant and additional staff.

Formal transition processes for pwCF have been implemented worldwide since 1982, primarily in the United States and Canada. ¹⁶ , ²¹ The United Kingdom has been at the forefront of establishing a transition program in Europe, and studies conducted in several countries have reported positive outcomes regarding structured transition programs. ¹⁵ , ²² , ²³ Among these transition protocols, the CF R.I.S.E program, developed as part of QI initiatives with resources from the CFF, has been successfully implemented in the United States for many years. ¹⁷ Therefore, the CF R.I.S.E program was chosen as a global example to be implemented in our country, representing the first translation and adaptation of the CF R.I.S.E program into a language other than English.

The outcomes of the knowledge assessments for our patients indicated that their level of knowledge in the areas of lung health, airway patency, lifestyle and equipment care, and infection control measures were commendable. This success is attributed to the constant emphasis on these topics during the clinic visits. We believe that the relatively higher knowledge assessment scores regarding the equipment maintenance and infection control are related to the standard education and training materials previously provided to all patients in our center, resulting in a significant increase in the rate of correct nebulizer hygiene. ²⁴ The knowledge areas that needed to be supported by sexual health and college, work, and health insurance. Liver disease and diabetes are not typically discussed unless there is a prior condition. The prevalences of liver disease and diabetes among the participants were 27.2% and 11.1%, respectively. Consequently, we believe that the baseline knowledge of these topics among our patients was low. These topics must also be supported.

The success of establishing the CF S.O.B.E. program can be attributed to various factors. Active participation of a multidisciplinary team, expertise of subject specialists in developing educational resources, and the inclusion of patient representatives and Turkish grammar teacher played crucial roles. Additionally, regular meetings between the transition team and QI coaches from the CFF VIP‐F7 program, as well as valuable feedback from patients and families, have facilitated continuous improvements and adaptations to meet the needs of patients and the healthcare system.

The role of a program coordinator is essential for the effective implementation of transition protocols in adult clinics as well as for their ongoing management. Social workers typically serve as program coordinators in centers where the CF R.I.S.E has been implemented for years. ¹² , ¹⁶ , ²⁵ In centers where there are no social workers, such as ours, other team members may also serve as program coordinators. A senior pediatric pulmonologist served as the project coordinator. A significant proportion of our patients did not have access to expert guidance on social security and disability rights. Our study revealed that pwCF and their families lacked awareness of government regulations concerning social security and disability rights, and their knowledge of this topic was limited. We believe that having a social worker to assist with the challenges that patients face regarding social services would be beneficial.

As part of this project, collaboration was established with volunteer experts through the CF Patient and Family Association (KIFDER) to review government regulations regarding social security and disability rights and prepare written materials. A comprehensive online meeting was conducted with the entire transition team to ensure an inclusive approach and to facilitate the dissemination of information to all team members.

Collaborative consultations and joint clinics involving professionals from both pediatric and adult clinics are often preferred to direct handovers. ¹² , ²⁵

The implementation of the program at a single center may have certain limitations regarding its generalizability to other centers in Turkey. To gain a deeper understanding of the program's effectiveness, future studies should emphasize the assessment of its long‐term impact on patient outcomes, as well as its suitability for different centers and the healthcare system in our country. With this objective in mind, following the initial evaluations of the CF S.O.B.E program at our center, our aim is to distribute the resources we have to CF centers nationwide and promote the implementation of structured transition practices throughout the country.

5. CONCLUSION

The CF R.I.S.E. was successfully translated and adapted to our center. The success of the adaptation of the CF R.I.S.E program can be attributed to the participation of a multidisciplinary team, utilization of educational resources, and regular meetings for feedback and compliance. Based on the program's outcomes, the aim was to share all materials and surveys with other centers, ensure their utilization, and benefit all pwCF.

In the coming period, our center will enroll pwCF aged 16–25 years in the CF S.O.B.E program. Enrolled patients will be reassessed for their knowledge and skills after KAQs and RCLs. pwCF and their families who receive personalized education within the scope of the project are expected to increase their knowledge and skills regarding CF at the end of 1.5 years. The baseline KAQ results will guide us to determine knowledge gaps that may hinder successful transition and the need for additional coproduced educational support. Periodically, throughout the project, the individual deficiencies of the patients will be identified through interim information assessments, and individual and collective training (e.g., webinars) will be organized to overcome the information deficiencies.

It is aimed at the project to become a routine practice at our center in the coming period. In addition, within the scope of the project, joint visits with adult clinics with limited experience in CF are aimed at increasing their expertise in CF disease and active participation in transfer clinics in the project.

The next phase of our research involved integrating the adult clinic into the transition program and establishing a fully structured joint transitional outpatient clinic aimed at optimizing the transition process for patients. These clinics would be dedicated to patients who are prepared to transition from the pediatric clinic to the adult clinic at our center.

AUTHOR CONTRIBUTIONS

Merve Selcuk Balcı: Methodology; conceptualization; investigation; data curation; writing—original draft; validation; formal analysis; software; visualization. Yasemin Gökdemir: Supervision; writing—review and editing; investigation; methodology; data curation; validation; project administration. Ela Erdem Eralp: Writing—review and editing; supervision; investigation; methodology; data curation; validation. Almala Pınar Ergenekon: Writing—review and editing; methodology; data curation; supervision. Cansu Yılmaz Yegit: Methodology; data curation. Mürüvvet Yanaz: Methodology; data curation. Aynur Gulieva: Methodology; data curation. Mine Kalyoncu: Methodology; data curation; validation. Seyda Karabulut: Methodology; data curation. Neval Metin Cakar: Methodology; data curation. Burcu Uzunoglu: Data curation. Gamze Tastan: Data curation. Damla Kocaman: Data curation. Ozge Kenis Coskun: Data curation. Ilknur Gorgun: Validation. R Randall Messier: Supervision; writing—review and editing. Pamela Mertz: Writing—review and editing; supervision. Fazilet Karakoc: Writing—review and editing; resources; data curation; methodology; investigation; validation; project administration. Bülent Karadag: Validation; investigation; writing—review and editing; supervision; project administration.

CONFLICT OF INTEREST STATEMENT

The authors declare no conflict of interest.

Balcı MS, Gökdemir Y, Eralp EE, et al. Assessment of patients' baseline cystic fibrosis knowledge levels following translation and adaptation of the CF R.I.S.E translation program into Turkish. Pediatr Pulmonol. 2024;59:3483‐3490. 10.1002/ppul.27235

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.