What do systematic reviews say?

Summary of findings ‐ triple therapy: VX‐659‐tezacaftor‐ivacaftor/deutivacaftor vs control

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor/deutivacaftor vs control

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ monotherapy: lumacaftor vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: VX‐440‐tezacaftor‐ivacaftor vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: VX‐152‐tezacaftor‐ivacaftor vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Ataluren vs placebo

• Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 232 participants
  High

Digital technology (web‐based platform CFHealthHub) vs usual care

Digital technology (web‐based platform CFHealthHub) probably leads to lower number of pulmonary exacerbations compared with usual care (moderate certainty).

• Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  rate ratio 0.92
  1 study · 607 participants
  Moderate

Digital technology (TIM) vs standard treatment (TBM)

The review found no eligible studies for this question.

• Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Psychological interventions vs active comparators

• Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 128 participants
  Very low

Psychological interventions vs usual care

• Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 607 participants
  Moderate

Summary of findings for nasal irrigation with topical steroids plus sinus surgery vs nasal irrigation with topical steroids alone

• Surgery for nasal polyposis in cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 28 participants
  Very low

Wearable fitness tracker plus text message for personalized feedback and goal setting vs wearable fitness tracker alone

The review found no eligible studies for this question.

• Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Web‐based application to record, monitor and set goals on physical activity plus usual care vs usual care

• Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  Low

Web‐based vs face‐to‐face exercise delivery

The review found no eligible studies for this question.

• Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Exercise prescription plus the use of a wearable fitness tracker vs exercise

The review found no eligible studies for this question.

• Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Digital technology vs usual care

Digital technology probably leads to higher time to next pulmonary exacerbation compared with usual care (moderate certainty).

• Digital technology for early identification of exacerbations in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  HR 1.45
  2 studies · 153 participants
  Moderate

Digital technology vs usual care

Digital technology showed no clear difference in number of pulmonary exacerbations: number of participants… compared with usual care (moderate certainty).

• Digital technology for early identification of exacerbations in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  RR 1.07 [0.92, 1.25]
  1 study · 267 participants
  Moderate

Summary of findings: colistimethate dry powder for inhalation (Colobreathe®) vs tobramycin

Summary of findings: colistimethate dry powder for inhalation… showed no clear difference in pulmonary exacerbations: number of pulmonary exacerbations compared with tobramycin (moderate certainty).

• Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis 2022 · Cochrane Headline outcomes only
  RR 1.19 [0.86, 1.64]
  1 study · 374 participants
  Moderate

Summary of findings: inhaled TOBI® (IV preparation) vs tobramycin

The review found no eligible studies for this question.

• Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis 2022 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found