What do systematic reviews say?

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

• Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis 2022 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 32 participants
  Very low

Summary of findings ‐ PEP vs PDPV

• Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  5 studies · 155 participants
  Low

Summary of findings ‐ PEP vs HFCWO

• Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  4 studies · 174 participants
  Low

Summary of findings ‐ PEP vs airway clearance techniques

• Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  95 participants
  Low

Summary of findings ‐ PEP vs oscillating PEP

• Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  7 studies · 217 participants
  Moderate

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 · 203 participants
  High

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music

The review found no eligible studies for this question.

• Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music

The review found no eligible studies for this question.

• Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Intervention not reported

It is very uncertain whether the intervention changes FEV1 % predicted (change from baseline) (very low certainty).

• Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 34 participants
  Very low

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music

The review found no eligible studies for this question.

• Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Dornase alfa vs after airway clearance techniques

It is very uncertain whether inhalation of dornase alfa before changes FEV1 (% predicted) change from baseline compared with after airway clearance techniques (very low certainty).

• Timing of dornase alfa inhalation for cystic fibrosis 2021 · Cochrane Headline outcomes only
  MD 0.83
  102 participants
  Very low

Morning vs evening inhalation of dornase alfa

Morning showed no clear difference in FEV1 (% predicted) change from baseline compared with evening inhalation of dornase alfa (low certainty).

• Timing of dornase alfa inhalation for cystic fibrosis 2021 · Cochrane Headline outcomes only
  no estimate extracted
  25 participants
  Low

High‐dose rhGH vs placebo

• Recombinant growth hormone therapy for cystic fibrosis in children and young adults 2021 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 41 participants
  Low

High‐dose rhGH vs standard‐dose rhGH

• Recombinant growth hormone therapy for cystic fibrosis in children and young adults 2021 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 42 participants
  Low

Autogenic drainage vs conventional physiotherapy

• Autogenic drainage for airway clearance in cystic fibrosis 2021 · Cochrane Headline outcomes only
  no estimate extracted
  2 studies · 54 participants
  Very low

Autogenic drainage vs intrapulmonary percussive ventilation (200 bpm)

• Autogenic drainage for airway clearance in cystic fibrosis 2021 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 4 participants
  Very low

Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)

• Autogenic drainage for airway clearance in cystic fibrosis 2021 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 4 participants
  Very low

Summary of findings: repaglinide vs insulin

Summary of findings: repaglinide showed no clear difference in FEV1 % predicted change from baseline compared with insulin (moderate certainty).

• Drug treatments for managing cystic fibrosis‐related diabetes 2020 · Cochrane Headline outcomes only
  no estimate extracted
  2 studies · 112 participants
  Moderate

Insulin vs placebo

Summary of findings: insulin showed no clear difference in FEV1 % predicted: change from baseline compared with placebo (low certainty).

• Drug treatments for managing cystic fibrosis‐related diabetes 2020 · Cochrane Headline outcomes only
  MD 1.2
  1 study · 39 participants
  Low

Summary of findings: repaglinide vs placebo

Summary of findings: repaglinide showed no clear difference in FEV1 % predicted; change from baseline compared with placebo (low certainty).

• Drug treatments for managing cystic fibrosis‐related diabetes 2020 · Cochrane Headline outcomes only
  MD 1.7
  1 study · 38 participants
  Low