Correlated Drug Terms (6)

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	Name (Synonyms)	Correlation
drug433	CFTR Modulators Wiki	0.71
drug1654	Nutrition Wiki	0.71
drug1292	JNJ-66525433 Wiki	0.71
drug2601	Unsupervised exercise Wiki	0.71
drug1940	Psychological and Behaviour Change Support Wiki	0.71
drug1822	Placebo Wiki	0.04

Correlated MeSH Terms (5)

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	Name (Synonyms)	Correlation
D003550	Cystic Fibrosis NIH	0.41
D005355	Fibrosis NIH	0.32
D014456	Ulcer NIH	0.29
D003092	Colitis NIH	0.29
D003093	Colitis, Ulcerative NIH	0.29

Correlated HPO Terms (2)

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	Name (Synonyms)	Correlation
HP:0002583	Colitis HPO	0.29
HP:0100279	Ulcerative colitis HPO	0.29

Primary Outcomes

Description: Changes in strength will be measured using a five repetition maximum test (5RM)

Measure: Change in Strength

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in cardiorespiratory fitness will be measured using a cardiopulmonary exercise test (CPET)

Measure: Change in Cardiorespiratory Fitness

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Secondary Outcomes

Description: Changes in FEV1 will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in Forced expiratory volume in 1 second (FEV1)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in FVC will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in Forced vital capacity (FVC)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in FEV1/FVC ratio (FEV1%) will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in FEV1/FVC ratio (FEV1%)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in FEF will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in Forced expiratory flow (FEF)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in physical activity levels will be measured using PAQ-C for children under 14 years of age and PAQ-A for adolescents over 14 years of age. Items 1 to 9 will be used in the physical activity composite score, and means will be calculated to obtain the final PAQ-C activity summary score. Items 1 to 8 will be used in the physical activity composite score, and means will be calculated to obtain the final PAQ-A activity summary score. A score of 1 indicates low physical activity, whereas a score of 5 indicates high physical activity.

Measure: Changes in Physical Activity Questionnaire (PAQ) for children and adolescents

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in quality of life will be measures with the Cystic Fibrosis-Questionnaire-Revised (CFQ-R). Scores for each health related quality of life domain are calculated; after recoding, each item is summed to generate a domain score and standardized. Scores range from 0 to 100, with higher scores indicating better health.

Measure: Change in quality of life: Cystic Fibrosis-Questionnaire-Revised (CFQ-R)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Chloride concentration in sweat (mEq/L) will be measured in the laboratory using an MK II Chloride Analyzer 926S

Measure: Sweat chloride level

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Primary Outcomes

Description: Physical function will be measured using the EORTC-QLQ-C30. Participants will choose from the choices 1 = Not at all; 2 = A little, 3 = Quite a bit, 4 = Very much

Measure: Physical Function

Time: Baseline up to 1 year post enrolment

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Secondary Outcomes

Description: Emotional function will be measured using the EORTC-QLQ-C30. Participants will choose from the choices 1 = Not at all; 2 = A little, 3 = Quite a bit, 4 = Very much

Measure: Emotional Function

Time: Baseline up to 1 year post enrolment

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Description: EQ-5D-5L is a standardised measure of health status developed by the EuroQol Group. Applicable to a wide range of health conditions and treatments, it provides a simple descriptive profile and a single index value for health status that can be used in the clinical evaluation of health care

Measure: Health related quality of life

Time: Baseline up to 1 year post enrolment

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Description: EQ-5D-5L is a standardised measure of health status developed by the EuroQol Group. Applicable to a wide range of health conditions and treatments, it provides a simple descriptive profile and a single index value for health status that can be used in the economic evaluation of health care

Measure: Health economics analysis

Time: Baseline up to 1 year post enrolment

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Description: Patients knowledge skills and confidence to manage their own health and care will be measured using the Patient Activation Measure (PAM) Agreement or disagreement with statements. Choice between disagree strongly, disagree, agree, agree strongly and not applicable

Measure: Patient activation

Time: Baseline up to 1 year post enrolment

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Description: Measured using the Godin Leisure Time Exercise Questionnaire, a 7-day physical activity recall

Measure: Self-reported physical activity

Time: Baseline up to 1 year post enrolment

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Description: In the oncology population where malnutrition prevalence is high, the Patient-Generated Subjective Global Assessment Short Form (PG-SGA SF) is a descriptive screening tool that can provide further information to assist triaging and capture acute change in nutritional state

Measure: Nutrition assessment

Time: Baseline up to 1 year post enrolment

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Description: Self-efficacy (confidence) to self-manage chronic disease (SEMCD), measured using the Lorig SEMCD scale A confidence scale that represents the patients confidence that a regular task can be achieved. 1 = Not confident at all and 10= totally confident.

Measure: Self-efficacy for self-management of chronic disease

Time: Baseline up to 1 year post enrolment

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Description: Levels of distress as measured by the Emotions Thermometers. Participants will score on a scale where 0 = None and 10 = Extreme

Measure: Distress levels

Time: Baseline up to 1 year post enrolment

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Description: The Dukes Activity Status Index is a 12 item self-report measure of functional capacity that can be used to estimate peak oxygen uptake

Measure: Functional capacity

Time: Baseline up to 1 year post enrolment

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Description: As measured by the Impact of Event Scale - a 22-item self-report measure of the imapact of a specific traumatic event against the DSM criteria for Post Traumatic Stress Disorder. 0 = Not at all and 4 = Extremely.

Measure: The impact of COVID-19 pandemic on psychological functioning

Time: Baseline up to 1 year post enrolment

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Description: Semi-structured interviews with participants and professionals to explore acceptability, usefulness and barriers to delivery/engagement

Measure: Process Evaluation

Time: Through study completion, an average of 12 months

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Other Outcomes

Description: Overall survival at 1 year following date of enrolment

Measure: Exploratory: Overall survival

Time: 1 year post enrolment

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Description: Adherence to intervention advice is defined as number of sessions the participants attends divided by the total number of sessions.

Measure: Exploratory: Adherence to the interventions/advice

Time: Baseline up to 1 year post enrolment

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