Development a Core Set for Adults With Cystic Fibrosis Based on the ICF

Cystic Fibrosis (CF) is a chronic, progressive, and autosomal recessive disease that shortens life expectancy caused by mutations in the Cystic Fibrosis Transmembrane Regulator (CFTR ) gene. It affects more than one body system, mainly the respiratory and digestive. The quality of life of individuals with CF is adversely affected by the increasing treatment burden in addition to multi-system involvement. Disorders in CF affect the structures and functions of various systems, especially the respiratory and digestive systems. This influence causes a decrease in the activity and participation of individuals with CF. Factors such as age, gender, and coping strategies of individuals may affect the severity of these symptoms in individuals with CF. Scientific advances in biology in CF have provided therapeutic diversity, thereby improving patient care and prolonging survival. Pancreatic enzyme supplementation, airway cleansing, long-term antimicrobial therapy for respiratory tract infection, and nutritional supplements are among the current treatments. CFTR modulators, which have been developed in recent years to improve CFTR protein function, have been reported to greatly benefit individuals with CF. It can be said that the life expectancy of individuals with CF is prolonged by developing-changing treatment strategies and multidisciplinary approaches. It has been reported in international records that approximately 150,000 patients worldwide have been diagnosed with CF. The incidence of the disease is estimated to be 1/3,000 live births in Northern Europe, 1/4.000-10,000 in Latin Americans, and 1/15,000-20,000 in African-Americans. There is limited number of studies on the incidence of CF in Turkey. In the study conducted by Gurson in 1973, the incidence of CF was determined as 1/3,000. According to the annual records of the Cystic Fibrosis Society of Turkey, there are 1930 CF patients followed in various centers in 2021 in Turkey. The International Classification of Functioning, Disability, and Health (ICF) describes human functioning and states of disability and provides a framework for organizing this information. The World Health Organization approved the ICF in 2001 to classify and describe patients' functions within the specific social and environmental context in which they live.

ICF not only defines the health status of individuals but also allows health professionals to create a common language. The ICF includes five interrelated components: body functions (b), body structures (s), activity and participation (d), environmental factors (e), and personal factors. ICF, each of these components is hierarchically structured for one, followed by the second, third, and fourth levels. As you progress from the first to the fourth level, the descriptions of the categories become more detailed. Since it contains ICF 1495 categories, it is practically difficult to apply in clinical settings. Disease-specific assessment tools are required because diseases affect people in many ways. Therefore, there is a need to create disease-specific subsets of ICF categories that will make it easier to apply in the clinic. For this reason, the ICF Core Set is designed to be purposeful, and essential and can be used in daily clinical practice. ICF Core Sets are created by selecting the appropriate categories for the current disease from the ICF classification. ICF Core Sets are smaller than ICF, allowing practical evaluation based on ICF in clinical use. It evaluates the current situation of the patient quickly and practically, and improves interdisciplinary cooperation.Work is underway to develop the ICF Core Set, which is internationally recognized shortlist of the most relevant and essential ICF categories that can be used to assess and document the condition of patients living with a particular health condition. Core sets have been developed for various health conditions such as neurological, pediatric, cardiopulmonary, musculoskeletal, and cancer. Studies investigating Cystic Fibrosis from the ICF perspective are limited, but there are no studies within the scope of the core set. Our study, it was aimed to develop a core set that quickly and practically evaluates the current situation of the patient with CF in Turkey based on ICF and increases the coordination within the interdisciplinary team.

In our practice, we will follow an established, very comprehensive, scientific and evidence-based guide published by the 'ICF Research Branch', which should be followed when creating the ICF-Core Set. The research consists of 4 steps.

A 'literature review' that allows us to view primary care CF from the perspective of the investigator. In this step, studies on CF for the last 10 years will be searched from various databases (PUBMED, Scopus, Embase).

Literature Review-Inclusion Criteria

• Studies with individuals over the age of 18 with a diagnosis of Cystic Fibrosis
• Studies published in the last 10 years
• Studies in English
• Randomized controlled, clinical control study, observational studies, cross-sectional studies, qualitative studies

Literature Review - Exclusion Criteria

-Psychometric and prevention studies, phase II clinical research studies, studies with laboratory parameters only, animal experiments, letters, comments and editorials

Qualitative research that allows us to view second-line CF from the perspective of individuals diagnosed with CF. It is planned to reach adult individuals who are followed up with the diagnosis of CF in Hacettepe University Department of Chest Diseases and referred to Hacettepe University Faculty of Physical Therapy and Rehabilitation. Focus group discussions with a maximum of 7 people will be held. With the guidance of the moderator, 6 open-ended questions covering the components of the ICF will be asked.

Qualitative Research- Inclusion Criteria

• Individuals over the age of 18 with a diagnosis of CF
• Volunteering to participate in research

The 'expert survey' allows us to see tertiary CF through the eyes of healthcare professionals. At this step, 6 open-ended questions covering ICF components for individuals with CF will be asked via e-mail to healthcare professionals who treat individuals with CF or have experience in research in this field. We are planning to reach the physicians working in the field of CF in the Hacettepe University Chest Diseases Department and the people with whom these physicians are in the National Cystic Fibrosis Patient Registry and collaborate. We plan to reach physiotherapists who work with patients diagnosed with CF academically and clinically at Hacettepe University Faculty of Physical Therapy and Rehabilitation, and physiotherapists who have experience in this field in different centers. Other health professionals (dieticians, nurse) who are familiar with the problems related to CF will be reached with the cooperation of physicians and physiotherapists.

Expert Questionnaire-Inclusion Criteria

• Have at least three months of experience in treating and/or researching individuals with CF, with knowledge of CF-related problems
• Volunteering to participate in research

The fourth step is the 'consensus' that we will create the final Core Set as a result of the data we collect. A multidisciplinary team (Physician, Physiotherapist, Dietitian, Nurse) meeting will be held for the Short Core Set created in this step.