CF world/ general

Cystic fibrosis (CF) is a life-shortening multisystemic monogenetic disease caused by

mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It is the
most common autosomal recessive cause of early mortality in Caucasians worldwide [1,2].
Among Caucasians, CF occurs in approximately 1 in 3000 to 4000 live births and it is
estimated that 1 in 25 to 30 white persons are carriers of a pathogenic mutation of the CFTR
gene [2]. In Portugal, the annual incidence is approximately 1 in 9000 live births and, in 2017,
341 patients were listed in the European Cystic Fibrosis Society Patients Registry (ECFSPR)
[3,4]. The CFTR gene, located in chromosome 7, encodes for the CFTR protein, which
functions as an ATP-gated chloride channel and as a bicarbonate channel [5]. It regulates the
flow of ions across the apical surface of epithelial cells and it is widely distributed across
several epithelial tissues [6]. More than 2000 potentially pathogenic mutations of the CFTR
have been described [7]. The most common CF causing mutation is the f508del, a class II
mutation that is present in up to 90% of people with CF Rosa, 2022
onal status [1]. For both pulmonary and gastrointestinal manifestations, treatment is lifelong
and begins when the diagnosis is established. Regarding lung disease, treatment focuses
mainly in controlling chronic airway infection and airway clearance of secretions. For the first
aspect, routine cultures of respiratory secretions and targeted antimicrobial treatment are key.
As for the latter, treatment is based in physical therapy techniques, (e.g., postural drainage
and percussion, oscillating positive expiratory pressure, high frequency chest wall oscillation
and exercise) and inhaled therapies to thin viscous mucus, such as recombinant human DNase
(dornase alfa) and hypertonic saline [5]. Alongside conventional therapies, CFTR modulators
(e.g., lumacaftor-ivacaftor) represent an important advance in management of CF, as instead
of treating the consequences of the CFTR dysfunction, they target the production or function
of the mutant CFTR protein, with the potential of dramatically changing the natural history of
this disease Rosa, 2022
Cystic fibrosis (CF) is the commonest autosomal recessive life-limiting condition in
Caucasian populations, affecting between 70,000 [1] and 100,000 [2]people world
wide.Median age of death and median predicted survival have both increased significantly
over recent decades [3]. The CF gene is located on chromosome 7 and mutations lead to
absence or dysfunction of the cystic fibrosis transmem-brane conductance regulator (CFTR),
a protein which formsa chloride channel in cell membranes in many body sys-tems. In
respiratory epithelial cells, lack of CFTR function leads to failure of the mucociliary
escalator, stasis ofrespiratory secretions and opportunistic infection. CF is characterized by a
cycle of recurrent pulmonary infection, inflammation and tissue damage which may
ultimately lead to respiratory failure and death Nicola, 2019

CF in Oman
The estimated prevalence of CF in Oman is 10.3 per 100 000 individuals. (Sumaya, 2022)
stic fibrosis (CF) is a rare autosomal recessive disorder caused by mutations of the cystic
fibrosis transmembrane conductance regulator (CFTR) gene located on the long arm of
human chromosome 7,1,2 The CFTR gene encodes a protein in the cell membranes of the
lungs, liver, pancreas, skin sweat glands, and reproductive organs.3,4 The CFTR protein is
essential for the transportation of chloride ions and water molecules. Mutations of the CFTR
gene cause CFTR protein dysfunction, resulting in decreased secretion of chloride and
increased reabsorption of sodium and water across the epithelial cells. The consequent
accumulation of thickened, purulent, and dehydrated secretions leads to airway obstruction,
recurrent bacterial infections, and chronic airway inflammation.3,5–7 The organs and systems
most affected by CF include the respiratory tract, pancreas, gastrointestinal tract, sweat
glands, and reproductive system. This progressive and often lethal disease affects
approximately 1 in 25 000 newborns in the Caucasian populations, with over 2000 CFTR
mutations described.8,9 Of these, the p.Phe508del mutation accounts for 70–90% of CF cases
in Caucasians (Sumaya, 2022)

Oman The study conducted by Fass et al. [19] included both retrospective and prospective
analyses to estimate CF prevalence in the NAB region and in Oman as well. Retrospectively,
47 patients with CF were identified during the period of 1998 to 2012 in the NAB region. On
the other hand, a total of 204 unrelated Omani volunteers from Oman Medical Collage of
Sohar in the NAB region were recruited for survey and screening for CF-causing mutations.
Based on the number of CF cases identified over the 14-year period and the average annual
rate birth, the incidence was estimated to be 1/2410 affected newborns in the NAB region,
whereas the prevalence of the disease was estimated based on the genotype frequency
resulting in a predicted prevalence of 1/8264 was reported for Oman Samer, 2021

CF and PT
Much has been written in recent years about improving quality of care for patients with cystic
fibrosis (CF) [1–8]. Evidence points to improved patient outcomes associated with the
development of quality improvement processes in CF care sites [9,10], and benchmarking
efforts in both the United States and Germany have identified structural factors and care
processes within care sites that are associated with improved patient outcomes [4,6]. Ideally
one would like to know which therapies or practices result in better patient outcomes. Given
the increasing number of therapies for treating CF, comparative effectiveness studies looking
at real world use of multiple different therapies are needed to counter the tendency to place
every patient on every therapy proven beneficial by randomized controlled trials. However,
benchmarking and comparative effectiveness studies are influenced by the choice of outcome.
Different care sites may be identified as top performing, depending on whether one chooses
survival as the outcome or chooses to focus on pulmonary, nutritional, or quality-of-life
outcomes. Jeffrey, 2015
PA is defined as any bodily movement produced by the contraction of skeletal muscles
resulting in a substantial increase in caloric requirements over resting energy expenditure [7].
There is overwhelming evidence on the short-term health benefits (e.g. higher energy levels,
improved concentration and emotional well-being) and the long-term advantages (e.g.
primary and secondary prevention of several chronic diseases and mortality) of regular PA in
the healthy population Manon, 2022

Many studies (n= 11) report associations between self‐efficacy andhealth‐related outcomes,
reporting a positive association betweenhigh levels of self‐efficacy and better physical health
management,mental, and social health outcomes (Figure3). For example, highlevels of self‐
efficacy are positively associated with physical activityengagement, reduced anxiety and
depression symptoms, highertransition readiness, increased comfort disclosing CF
diagnosis,persistent airway clearance, and improved psychological well-being.20–
26Furthermore, one study also identifies reduced medica-tion self‐efficacy as a mediator
between higher depressive symptomsand lower medication adherence Maggi, 2023
The use of airway clearance therapies is recommended forall people with CF as this facilitates
the movement of, andexpectoration of, mucus to keep the lungs clear (ACPCF 2017;
Flume2009; IPG/CF 2009). People with CF usually start airway clearancetherapies soon after
diagnosis, and perform them at least daily forthe rest of their lives.There are a variety of
airway clearance techniques, includingconventional chest physiotherapy, positive expiratory
pressure(PEP) therapy, high-pressure PEP therapy, active cycle ofbreathing techniques,
autogenic drainage, airway oscillatingdevices (e.g., Flutter®, Cornet®, Acapella®, Quake®,
Aerobika®, andintrapulmonary percussive ventilation), external high frequencychest
compression devices (e.g., The Vest™, ThAIRapy Vest®,SmartVest®, and Hayek Oscillator),
and exercise. While these airwayclearance techniques may differ in terms of the need for
assistanceor equipment, they all have the same goal of removing mucussecretions from the
lungs. Selecting the most appropriate airwayclearance technique is influenced by age,
individual preference,adverse events, an individual's airway pathophysiology, and
cost.Descriptions of each airway clearance therapy included in thisreview are provided in the
'Types of Interventions' section below(Criteria for considering reviews for inclusion).CF is a
progressive disease and as respiratory function deteriorates,airway clearance techniques may
not be sufficient. It may beuseful to consider an adjunctive therapy, such as hypertonicsaline
or dornase alfa, or a combination of adjunctive therapies inconjunction with airway clearance
techniques (Dentice 2016; Elkins2016). However, it is beyond the scope of this overview to
evaluatethe efficacy and safety of adjunctive therapies.How the intervention might workThe
goal of airway clearance techniques is to clear the airwaysof mucus, thus helping to prevent
infection and improvelung function. Conventional chest physiotherapy uses manualtechniques
of percussion and vibration (and on occasion modifiedgravity-assisted positioning) to loosen
and move mucus throughthe airways. Active cycle of breathing techniques and
autogenicdrainage use a series of breathing manoeuvres to move mucussecretions. Techniques
such as PEP therapy, high-pressure PEPtherapy, airway oscillation devices, and external high
frequencychest compression can be used independently of an assistant orcarer, thus affording
the individual with CF independence anda more flexible approach to airway clearance
management. InPEP and high-pressure PEP therapy, the devices clear mucus bycausing
pressure to build up in the airways. High-pressure PEPtherapy is a modification of PEP which
involves the full forcedexpiration against a fixed mechanical resistance usually between40 cm
H2O to 140 cm H2O (Prasad 1993). Airway oscillatingdevices and external high frequency
chest compression devices useintra- or extra-thoracic oscillation to help loosen mucus.
Exerciseimproves physical health and strength, and exercise is proposedto improve mucus
clearance by changes to airflow and mucus.Exercise increases ventilatory demand, which is
met by increases intidal volume and respiratory flow. In CF, the increase in ventilationand
peak expiratory flow (PEF) with exercise could increase thepropulsion or mechanical
clearance of mucus Wilson, 2019
CF and PFT
CFTR 76 dysfunction causes defective mucociliary clearance of thickened mucus which
potentially 77 leads to obstruction of the airways [2]. Such obstructions create a hypoxic
environment that 78 can harbor different bacteria [4]. The predominant microorganisms
colonizing the airways of 79 CF patients are Haemophilus influenzae and Staphylococcus
aureus early in life, and 80 Pseudomonas aeruginosa (PA) later on. Besides these pathogens,
methicillin resistant 81 Staphylococcus aureus (MRSA) and Achromobacter xylosoxidans
also plays an important role 82 in more advanced lung disease. These bacteria can potentially
cause infectious exacerbations, 83 inflammation, and finally lung functional degradation with
structural abnormalities due to a 84 vicious circle of inflammation [3] In terms of lung
function, CF is considered to be a chronic progressive obstructive 86 disorder [4] with the
forced expiratory volume in one second (FEV1) being an important 87 indicator of lung
disease severity [5]. Decline in FEV1 has indeed been shown to be 88 associated with
different phenotypic characteristics of disease severity such as pancreatic 89 insufficiency,
cystic fibrosis related diabetes (CFRD), colonization with PA and MRSA [6-7]. 90 However,
limiting CF lung disease to its spirometric assessment (which can only establish 91 airway
obstruction) seems inadequate since restrictive pulmonary disorders may occur in CF 92 as
well [8]. Also, to confirm normal lung function in some adult CF patients [9], a full lung 93
function evaluation, including diffusing capacity and lung volume measurements is required.
94 The goal of the present study was to scrutinize the different pulmonary function patterns in
a 5 95 CF population from the adult CF center at University Hospital UZ Brussel. Secondly,
we 96 aimed to explore possible associations between distinct pulmonary function patterns
and 97 genotype and certain phenotypical characteristics. Stylemans, 2019

Spirometry is the most frequently used method for measuring lung function [7] and ‘is the
most valuable and reproducible lung function test used in children’ [8]. Spirometry yields a
range of measures including FEV1, forced vital capacity (FVC; the volume of air that can be
forcibly and maximally exhaled out of the lungs until no more can be expired) and forced
expiratory flow rate 25–75% (FEF25–75% the amount of air expelled from the lungs during
the middle half of the FVC test) [9]. The percentage of predicted FEV1 has been referred to as
an index of lung health Christine, 2006

Table 2 Distribution of young people with cystic fibrosis according to forced expiratory
volume in 1 second (FEV1, as % of predicted value) Christine, 2006

It has been suggested [1] that the percentage recovery in FEV1 after 1 week of hospitalisation
is the highest predictor of outcome, including duration of hospital stay, for children with
cystic fibrosis, supporting its inclusion in this study. The results of this study support the
importance of collecting FEV1 data, showing clearly that there was a significant difference in
the rate of improvement in FEV1 over the hospital stay; this was greatest in the moderately
impaired band. While this supports the efficacy of overall team management strategies, it also
implies that different outcomes may be expected for groups with different FEV1 at admission.
It may be that when FEV1 is severely impaired, there is less potential for reversibility of lung
status, and the smaller rate of improvement in subjects with normal or mild impairment of
FEV1 may occur because less actual change in FEV1 is possible. In this study, FEV1 band
had no overall effect on the rate of change in FEF25–75%. However, young people in the
severely affected band demonstrated significantly less improvement in the rate of change of
FEF25–75% than young people in either the normal or moderately affected band. This may
suggest that the reduced flow in small airways in this severely affected group was less
amenable to reversal. However, it must be remembered that this test produces more variable
results [8], which may have a greater effect on the smaller number of subjects in the severely
affected band. There was improvement in FVC during hospitalisation in all FEV1 bands,
demonstrating an element of reversibility of the obstructive component in line with findings
of other studies [1]. In this study, considering the rate of change in FVC, those in the
moderately and severely affected bands (80% of the group) improved more than those in the
normal band, which may simply reflect the amount of improvement possible Christine, 2006
Lung disease in cystic fibrosis (CF) develops from the first weeks of life, often without any
clinical signs and symptoms [1–5]. Over time, lung function deteriorates and irreversible
structural changes occur, such as bronchiectasis, cirrhosis, and pulmonary fibrosis [6,7].
Although many organs and systems are affected by CF, the course of lung disease, in most
cases, determines the length and quality of life. Thus, early assessment of respiratory system
abnormalities is essential, enabling prompt intervention and delaying the progression of
pathological changes [8]. Managing early lung disease and preventing its progression are
becoming priorities in CF care [5]. However, assessment is problematic due to certain
pulmonary function test (PFT) limitations in the paediatric population. Spirometry is the most
commonly used test for monitoring lung disease, which is characterised by relatively low
sensitivity in the early stages of CF when less advanced structural abnormalities are present.
This characteristic of spirometry is due to the pathological process mainly involving the small
airways, which are not detected by standard PFTs [9]. In addition, spirometry and body
plethysmography are technically challenging to perform correctly and require good
cooperation with patients. It is known that this is very difficult and often impossible to
achieve in the paediatric group. Katarzyna, 2023

Conflict
Risk of bias can lead to gaps in the evidence, for example when the study design of
underlying trials is inappropriate or there are major methodological limitations to the
available studies. A large proportion of the studies included in a review of positive expiratory
pressure physiotherapy for CF airway clearance were reported only in abstract form which
meant that the risk of bias across the domains was unclear. The same review found that many
of the included trials were of a cross-over design with the inherent problems of carry-over
effect. So, although there were 26 included trials, the authors were still unable to provide
goodquality evidence for clinicians and people with CF Nicola, 2018
NARELLE, 2016 Obs
. Finally, we added hospitalisations (i.e. number of hospitalisations and number of days in
hospital) to secondary outcomes as there is evidence that a higher level of physical activity is
associated with reduced hospital admission Radtke, 2022 SR
Rational
Our findings showed that the severity of pulmonary disease is associated with quality of life
and functional capacity in adult patients with CF. These associations were found for some
CFQ-R domains, even those not related to pulmonary function. It confirms the multisystem
profile of the disease and suggests that the pulmonary impairments may reflect the overall
progression and severity of the disease. Among the main pathophysiological findings in CF,
lung function severity is considered an important mortality and morbidity marker.3,18,19
Thus, a more severely impaired lung function is commonly associated with a worse
metabolic, nutritional and digestive function, which in turn, may explain the correlations
found in our study between the pulmonary function variables, CFQ-R domains and the
6MWT results. The worsening of lung function associated with reduced exercise tolerance
starts a vicious cycle where the patient abstains from physical activity, resulting in
progressive deconditioning and increased dyspnoea. Although this physical limitation
compromises daily life activities, impacting negatively on the quality of life in adults with
CF,20 we found no studies that have assessed the correlation between the functional capacity
(6MWT) and the quality of life in adults with cystic fibrosis. In children and adolescents,
Donadio et al. found an association between the physical domain of the CFQ-R and the
6MWT.21 In our study, maybe because of the small sample size, there was no association
between the CFQ scores and the 6MWT. Therefore, our results suggest that the pulmonary
function may reflect the overall disease severity better than the functional capacity, and that
the exercise performance is not necessarily the foremost factor influencing the quality of life
in such subjects. Moreover, some studies observed no differences in the CFQ-R physical
domain after a supervised exercise program.22,23 Maybe this domain does not reliably reflect
the exercise performance of such patients, which may have influenced the absence of
association between the exercise capacity and quality of life in our study. Ribeiro, 2015

On top of these benefits, research has suggested that there are specific health benefits for
PwCF. Higher PA levels have been associated with a slower lung function decline, reduced
hospitalisation, improved quality of life (QoL), nutritional status, and improved prognosis in
both children and adults with CF [12–14]. Additionally, higher PA intensities, i.e. moderate-
to-vigorous PA (MVPA) and vigorous PA (VPA), are more consistently associated with health
indicators than lower intensities, i.e. light PA (LPA). Consequently, these higher intensities
have an important long-term protective effect on health [15, 16]. In several adult populations
with respiratory illness, decreased PA was shown to be a predisposing factor of greater
incidence of cardiovascular disease, obesity, diabetes, cancer, dementia and physical disability
[17]. The World Health Organization (WHO) published new PA guidelines in 2020, with only
a limited section dedicated to populations with chronic illness in general and, to date, no CF-
specific PA guidelines exist [18, 19]. Despite the WHO guidelines and the well-known
benefits of regular PA, the majority of both healthy and clinical populations do not engage in
the recommended amounts of PA [20, 21]. However, it remains unclear whether PwCF have
different PA levels compared with healthy peers and whether PA should be targeted in
interventions in this population. Importantly, a healthy lifestyle starts to evolve in early
childhood, which highlights the need to explore the PA levels of children and adolescents with
CF as well. In the development of interventions and the setup of studies aiming to improve
PA, knowledge on the influencing factors (i.e. correlates) of PA should be integrated. PA is a
complex, multidimensional behaviour, subject to multiple internal and external factors [22].
In the healthy population, a wide range of PA correlates have been investigated, and the
social–ecological model of health behaviour is used to categorise these correlates as either
intrapersonal (e.g. cognitions/emotions), interpersonal (e.g. family and friends) or
environmental factors (e.g. neighbourhood) [23]. Despite the excessive amount of research
conducted in the healthy population, this research field is still in its infancy in the CF
population. Taking into account the limited knowledge on the PA levels of PwCF and the
associated correlates, the main objective of this systematic literature review was twofold: 1)
summarise the existing evidence on the PA levels of PwCF compared to healthy peers, both in
youths and adults separately; 2) identify general and disease-specific correlates of PA in
PwCF. Evidence gathered and summarised in this review could inform the development of
future interventions to target PA in the CF population. In general, the CF population is as
active as their healthy peers. However, in youths with CF, some tendencies towards less
intense activities were found. Regarding the correlates of PA, positive associations were
found with QoL, lung function, (maximal) exercise capacity, BMD and Qc force.
Furthermore, PA was lower on weekdays compared to weekend days and lower when
experiencing pulmonary exacerbations. Notably, the methodological quality of the included
studies was limited, particularly missing objectively measured PA data and longitudinal
studies. This restricted the possibility to draw firm conclusions. Future research should focus
on further unravelling the associations between PA and its influencing factors, as this
knowledge is crucial in the development and implementation process of interventions aiming
to stimulate PwCF to be more active (e.g. duration, content and modalities). This will be
increasingly important to prevent obesity and its associated health risks in a growing
population of PwCF on highly effective CFTR modulator therapy. Manon, 2022
Research question.
Aim
Objective
Implication
Method
Results were analyzed using SPSS version 23. Data was summarized using mean, standard
deviation, numbers, and percentages for different variables. Comparison between groups was
performed using Fisher’s exact or chi-square tests which are suitable for the categorical
variables. P values will measured Raghad, 2021

The demographic and clinical data of the studied sample (see this reference to have a look of
some criteria of demographic factors) Raghad, 2021

(Sumaya, 2022) Katarzyna, 2023 Ribeiro, 2015

Rosa, 2020 the best with tables

CFTR modulator therapies act by 2 mechanisms to enhance CFTR function. Potentiators, like
ivacaftor, increase the probability that the protein channel is open, so chloride or bicarbonate
can flow more easily through the cell membrane (Figure 2). Correctors, like lumacaftor,
tezacaftor, and elexacaftor, improve channel quantity at the cell surface by helping the protein
fold properly, enabling transport to the cell surface (Figure 2). Severe variants such as
F508del need both potentiators and correctors to improve channel quantity and function
(Figure 2). Four modulators are currently approved by US and European drug regulatory
agencies, and eligibility for each treatment depends on the specific CFTR genetic variants
present (Table 2). Ivacaftor is available as a monotherapy, and lumacaftor-ivacaftor,
tezacaftor-ivacaftor, and elexacaftortezacaftor-ivacaftor are available as combination therapies
(Table 2

Primary and secondery Outcome measure: from Wilson, 2019

Justification : . Routine monitoring throughout the year and not just during admissions is
required to overcome this issue. James, 2019
hetherincreasing PA levels can improve clinical outcomes inadults with CF warrants further
investigation NARELLE, 2016 RCT

uture research should consider larger sample sizes and the use of valid, reliable instruments to
assess and track PA levels over a longer period of time. Furthermore, consideration should be
given to telehealth interventions or alternative strategies that are effective in optimising PA in
other patient populations or should consider subgroups of CF such as division by gender, age
or severity of disease Currana, 2022

hildren and adolescents with CF have a similar MVPA and sedentary time compared to
controls. There are many options, subjective and objective, for assessing PA in this
population. Optimal tool selection should guarantee more valid results. Homero, 2020
Background:

Cystic fibrosis (CF) is a life-shortening multisystemic monogenetic disease caused by

mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It
is the most common autosomal recessive cause of early mortality in Caucasians
worldwide. The prevalence of CF varies among different populations, with estimates
ranging from 1 in 3000 to 4000 live births among Caucasians and approximately 1 in
25 to 30 white persons being carriers of a pathogenic CFTR mutation (Rosa, 2022).

In Oman, the prevalence of CF is estimated to be 10.3 per 100,000 individuals,

highlighting the global impact of this condition (Sumaya, 2022). Early diagnosis and
comprehensive management are crucial for optimizing outcomes in CF patients. The
resultant accumulation of thickened, dehydrated mucus in the airways predisposes
individuals with CF to recurrent respiratory infections and chronic inflammation,
ultimately leading to progressive lung disease and respiratory failure. Standard
treatments for CF include airway clearance techniques, antimicrobial therapy, and
CFTR modulators, which target the underlying genetic defect. (Sumaya, 2022).
Spirometry is a commonly used method for assessing lung function in CF patients,
providing measures such as forced expiratory volume in one second (FEV1) and
forced vital capacity (FVC). Decline in FEV1 is indicative of disease progression and
is associated with various phenotypic characteristics of CF severity, including
pancreatic insufficiency, cystic fibrosis-related diabetes (CFRD), and colonization
with pathogens such as Pseudomonas aeruginosa (Stylemans, 2019; Christine, 2006).
However, spirometry may have limitations in detecting early lung disease, particularly
in pediatric patients, due to its relatively low sensitivity in detecting early structural
abnormalities, especially in the small airways (Katarzyna, 2023). Physiotherapy plays
a vital role in CF management by promoting airway clearance, improving lung
function, and enhancing overall respiratory health(Wilson, 2019) (Rosa,
2022)..However, there is limited evidence on the effectiveness of physiotherapy
interventions in pediatric CF patients associated with hospitalization (Jeffrey, 2015).

Previous research has demonstrated associations between self-efficacy, physical

activity engagement, and improved health outcomes in individuals with CF (Maggi,
2023). Additionally, studies have highlighted the importance of assessing lung
function using spirometry and other pulmonary function tests (PFTs) for early
detection and monitoring of CF-related lung disease (Katarzyna, 2023). However, the
use of spirometry in pediatric CF patients presents challenges due to technical
difficulties and poor patient cooperation (Katarzyna, 2023).
Despite advances in CF management, there are still gaps in our understanding of the
ideal management strategies for pediatric CF patients, particularly in regions with
lower prevalence rates such as Oman. Nicola,2018 and Radtke, 2022 enhance on
doing further Comparative effectiveness studies to evaluate the physiotherapy
interventions and their impact on pulmonary function and other clinical outcomes in
pediatric CF with exploring the associations between physical activity, and health
outcomes in CF patients (Radtke, 2022).

At this point, there are gaps in understanding the association between the PT & PF.
Therefore, the specific question under investigation in this study is: What is the effectiveness
of physiotherapy management on pulmonary function in pediatric CF patients at SQUH? This
research aims to address this question by conducting an observational study to evaluate the
impact of physiotherapy interventions on pulmonary function in pediatric CF patients aged 6-
13 years at SQUH. Specifically, the study seeks to assess the impact of physiotherapy
interventions on pulmonary function test (PFT) results, investigate associations
between physical activity levels and hospital admissions, and contribute to improving
the quality of care and outcomes for pediatric CF patients in Oman.

The justification for this research lies in the significant burden of CF on pediatric patients and
their families. Maintaining optimal pulmonary function is essential for slowing disease
progression, reducing respiratory symptoms, and improving quality of life in pediatric CF
patients. Physiotherapy is a cornerstone of CF management, but its effectiveness in pediatric
patients needs to be further elucidated. By conducting this study, the intention is to generate
evidence by inform clinical practice and improve outcomes for pediatric CF patients at
SQUH.

The overarching aim of this research is to investigate the effectiveness of

physiotherapy management on pulmonary function in pediatric cystic fibrosis (CF)
patients at Sultan Qaboos University Hospital (SQUH). The research question driving
this study is: "What is the effectiveness of physiotherapy management on pulmonary
function in pediatric CF patients at SQUH?"

Cystic fibrosis is a debilitating genetic condition characterized by progressive lung

disease, making the optimization of pulmonary function crucial for improving
outcomes and quality of life. Despite the established role of physiotherapy in CF
management, its effectiveness in pediatric patients remains understudied, particularly
in regions with lower prevalence rates such as Oman.

The study will employ an observational design, focusing on pediatric CF patients

aged 6-13 years at SQUH. Physiotherapy interventions will be administered, and
pulmonary function tests (PFTs) will be conducted to assess lung function before and
after the interventions. Additionally, physical activity levels and other relevant factors
will be evaluated to explore their associations with pulmonary function outcomes.

Key variables include pulmonary function test results (FEV1, FVC), physical activity
levels, and other clinical indicators of CF severity. Data will be collected using
standardized protocols and analyzed using appropriate statistical methods, including
descriptive statistics, correlation analysis, and regression modeling.

Anticipated outcomes include improvements in pulmonary function following

physiotherapy interventions, as evidenced by increases in FEV1 and FVC.
Additionally, the study aims to elucidate the associations between physical activity
levels and pulmonary function outcomes in pediatric CF patients.

Overall, this research endeavors to contribute to the existing knowledge base

regarding the effectiveness of physiotherapy in managing pulmonary function in
pediatric CF patients, particularly in the context of SQUH. By providing evidence-
based insights, the study aims to inform clinical practice, enhance patient care, and
ultimately improve outcomes for pediatric CF patients in Oman.

Both in children and adults with CF, the physical exercise interventions included in
this systematic review failed to improve lung function measured by FEV1