Cochrane · systematic review · CD012349

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia

Geneen, LJ, Dorée, C, Estcourt, LJ · Cochrane Database of Systematic Reviews · 2023 · Cochrane Library

Cystic Fibrosis and Genetic Disorders2 versionsAMSTAR 2: not assessed
SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
42
current version
GRADE rated
25
No studies
16
Included studies
NR
No studies 16 · 38.1%Ungraded 1 · 2.4%Very low 18 · 42.9%Low 7 · 16.7%Moderate 0 · 0%High 0 · 0%

Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.

Questions and results

Show all 2 versions

Summary of findings: Comparison 1 ‐ deferiprone (DFP) versus deferoxamine (DFO)

5 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation therapy (%, SD)
Summary of findings: Comparison 1 ‐ deferiprone (DFP) vs deferoxamine (DFO)
no estimate extracted7 studies · 612 participantsVery low
Downgraded: risk of bias, imprecision, inconsistency, indirectness
Total reported SAEs (from therapy, disease, non‐adherence)
Summary of findings: Comparison 1 ‐ deferiprone (DFP) vs deferoxamine (DFO)
RR 1.43 [0.83, 2.46]1 study · 228 participantsVery low
Downgraded: risk of bias, imprecision, inconsistency, indirectness
All‐cause mortality
Summary of findings: Comparison 1 ‐ deferiprone (DFP) vs deferoxamine (DFO)
RR 0.47 [0.18, 1.21]3 studies · 376 participantsVery low
Downgraded: risk of bias, imprecision, inconsistency, indirectness
Sustained adherence
Summary of findings: Comparison 1 ‐ deferiprone (DFP) vs deferoxamine (DFO)
no estimate extractedNRNo studies
QoL (
Summary of findings: Comparison 1 ‐ deferiprone (DFP) vs deferoxamine (DFO)
no estimate extracted1 study · 1 participantsVery low
Downgraded: risk of bias, imprecision, inconsistency, indirectness

Summary of findings: Comparison 2 ‐ deferasirox (DFX) versus deferiprone (DFO)

6 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation therapy (%, SD)
Summary of findings: Comparison 2 ‐ deferasirox (DFX) vs deferiprone (DFO)
no estimate extracted3 studies · 452 participantsVery low
Downgraded: risk of bias, imprecision
SAEs Thalassaemia‐related SAEs
Summary of findings: Comparison 2 ‐ deferasirox (DFX) vs deferiprone (DFO)
RR 0.95 [0.41, 2.17]2 studies · 247 participantsVery low
Downgraded: risk of bias, imprecision
SAEs SCD‐related SAEs
Summary of findings: Comparison 2 ‐ deferasirox (DFX) vs deferiprone (DFO)
no estimate extracted1 study · 195 participantsVery low
Downgraded: risk of bias, imprecision
All‐cause mortality
Summary of findings: Comparison 2 ‐ deferasirox (DFX) vs deferiprone (DFO)
0.96 [0.06, 15.42]2 studies · 240 participantsVery low
Downgraded: risk of bias, imprecision
Sustained adherence
Summary of findings: Comparison 2 ‐ deferasirox (DFX) vs deferiprone (DFO)
no estimate extractedNRNo studies
QoL
Summary of findings: Comparison 2 ‐ deferasirox (DFX) vs deferiprone (DFO)
no estimate extractedNRNo studies

Summary of findings: Comparison 3 ‐ deferiprone (DFP) versus deferasirox (DFX)

6 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation (%, SD)
Summary of findings: Comparison 3 ‐ deferiprone (DFP) vs deferasirox (DFX)
no estimate extracted1 study · 390 participantsLow
Downgraded: risk of bias, imprecision
SAE (chelation‐related) (n/N)
Summary of findings: Comparison 3 ‐ deferiprone (DFP) vs deferasirox (DFX)
1.54 [0.44, 5.39]1 study · 390 participantsVery low
Downgraded: risk of bias, imprecision
Total SAEs
Summary of findings: Comparison 3 ‐ deferiprone (DFP) vs deferasirox (DFX)
RR 0.95 [0.46, 1.96]1 study · 390 participantsVery low
Downgraded: risk of bias, imprecision
All‐cause mortality (n/N)
Summary of findings: Comparison 3 ‐ deferiprone (DFP) vs deferasirox (DFX)
RD 01 study · 390 participantsLow
Downgraded: risk of bias, imprecision
Sustained adherence
Summary of findings: Comparison 3 ‐ deferiprone (DFP) vs deferasirox (DFX)
no estimate extractedNRNo studies
QoL
Summary of findings: Comparison 3 ‐ deferiprone (DFP) vs deferasirox (DFX)
no estimate extractedNRNo studies

Summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated tablets versus DFX dispersible tablets

5 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation therapy (%, SD)
Summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated tablets vs DFX dispersible tablets
no estimate extracted1 study · 91 participantsVery low
Downgraded: risk of bias, imprecision
Sustained adherence to iron chelation therapy (%, SD)
Summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated tablets vs DFX dispersible tablets
no estimate extracted1 study · 54 participantsVery low
Downgraded: risk of bias, imprecision
Incidence of SAEs
Summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated tablets vs DFX dispersible tablets
RR 1.22 [0.62, 2.37]1 study · 173 participantsVery low
Downgraded: risk of bias, imprecision
All‐cause mortality
Summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated tablets vs DFX dispersible tablets
7.3 [0.14, 368.15]1 study · 173 participantsVery low
Downgraded: risk of bias, imprecision
QoL
Summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated tablets vs DFX dispersible tablets
no estimate extractedNRNo studies

Summary of findings: Comparison 5 ‐ deferiprone (DFP) plus deferoxamine (DFO) versus DFP

5 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation therapy (%, SD)
Summary of findings: Comparison 5 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP
no estimate extracted4 studies · 369 participantsLow
Downgraded: risk of bias, imprecision
Incidence of SAEs
Summary of findings: Comparison 5 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP
RR 0.15 [0.01, 2.81]1 study · 213 participantsLow
Downgraded: risk of bias, imprecision
All‐cause mortality
Summary of findings: Comparison 5 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP
0.77 [0.17, 3.42]2 studies · 237 participantsVery low
Downgraded: risk of bias, imprecision
Sustained adherence
Summary of findings: Comparison 5 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP
no estimate extractedNRNo studies
QoL
Summary of findings: Comparison 5 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP
no estimate extractedNRNo studies

Summary of findings: Comparison 6 ‐ deferiprone (DFP) plus deferoxamine (DFO) versus DFO

5 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation therapy (%, SD)
Summary of findings: Comparison 6 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFO
no estimate extracted5 studies · 281 participantsLow
Downgraded: risk of bias
Incidence of SAEs
Summary of findings: Comparison 6 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFO
no estimate extracted4 studies · 180 participantsLow
Downgraded: risk of bias
All‐cause mortality
Summary of findings: Comparison 6 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFO
no estimate extractedNRNo studies
Sustained adherence
Summary of findings: Comparison 6 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFO
no estimate extractedNRNo studies
QoL
Summary of findings: Comparison 6 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFO
no estimate extractedNRNo studies

Summary of findings: Comparison 7 ‐ deferiprone (DFP) plus deferoxamine (DFO) versus DFP plus deferasirox (DFX)

5 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation therapy rates (n, N)
Summary of findings: Comparison 7 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP plus deferasirox (DFX)
RR 0.84 [0.72, 0.99]1 study · 96 participantsLow
Downgraded: risk of bias, imprecision, indirectness
Incidence of SAEs
Summary of findings: Comparison 7 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP plus deferasirox (DFX)
1 [0.06, 16.22]1 study · 96 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
All‐cause mortality ‐ at 1 year ‐ trial end
Summary of findings: Comparison 7 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP plus deferasirox (DFX)
RD 01 study · 96 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Sustained adherence
Summary of findings: Comparison 7 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP plus deferasirox (DFX)
no estimate extractedNRNo studies
QoL
Summary of findings: Comparison 7 ‐ deferiprone (DFP) plus deferoxamine (DFO) vs DFP plus deferasirox (DFX)
no estimate extracted1 study · 96 participantsUngraded

Summary of findings: Comparison 8 ‐ medication management versus standard care

5 questions
OutcomeEffectEvidenceStatus / GRADE
Adherence to iron chelation
Summary of findings: Comparison 8 ‐ medication management vs standard care
no estimate extractedNRNo studies
SAEs
Summary of findings: Comparison 8 ‐ medication management vs standard care
no estimate extractedNRNo studies
Mortality
Summary of findings: Comparison 8 ‐ medication management vs standard care
no estimate extractedNRNo studies
Sustained adherence
Summary of findings: Comparison 8 ‐ medication management vs standard care
no estimate extractedNRNo studies
QoL PedsQLTM total score
Summary of findings: Comparison 8 ‐ medication management vs standard care
no estimate extracted1 study · 48 participantsVery low
Downgraded: risk of bias, indirectness