What do systematic reviews say?

Alglucosidase alfa plus albuterol vs alglucosidase alfa plus placebo

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 5 participants
  Very low

Alglucosidase alfa vs placebo

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 90 participants
  Moderate

VAL‐1221 vs alglucosidase alfa

The review found no eligible studies for this question.

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Cipaglucosidase plus miglustat vs alglucosidase alfa plus placebo

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 133 participants
  Moderate

Cipaglucosidase plus miglustat vs alglucosidase alfa plus placebo

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 121 participants
  Low

Avalglucosidase alfa vs alglucosidase alfa

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 100 participants
  Moderate

Alglucosidase alfa plus clenbuterol vs alglucosidase alfa plus placebo

• Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 11 participants
  Very low

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 ‐ 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

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

Intervention not reported

It is very uncertain whether the intervention changes FVC % 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

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

Digital technology (TIM) vs standard treatment (TBM)

Digital technology (TIM) showed no clear difference in FEV1 (mean change from baseline, % predicted) compared with standard treatment (TBM) (low certainty).

• Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  MD 1
  1 study · 20 participants
  Low

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

Digital technology (web‐based platform CFHealthHub) showed no clear difference in FEV1: mean FEV1 % predicted at end of study compared with usual care (low certainty).

• Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  MD 3.7
  1 study · 556 participants
  Low

Psychological interventions vs usual care

Summary of findings: psychological interventions probably leads to higher adherence to inhaled therapies: % of prescribed inhaled therapies… compared with usual care (moderate certainty).

• Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  MD 9.5 [8.6, 10.4]
  1 study · 588 participants
  Moderate

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 · 125 participants
  Very low

Psychological interventions vs usual care

Summary of findings: psychological interventions showed no clear difference in treatment‐related adverse events Depression PHQ‐8 (Kroenke 2009) compared with usual care (low certainty).

• Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 534 participants
  Low