Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complications

Investigators & Affiliations:

Monika R Asnani (SCU), Kim R Quimby (CDRC), Nadia R Bennett (ERU), Damian K Francis (ERU)

Funding Obtained:


Start Date:

June 2014

End Date:

July 2015


While improved knowledge and understanding of many chronic diseases improves outcomes, there are no reviews identifying which interventions improve knowledge in Sickle Cell Disease (SCD). Additionally little is known about the impact of patient or care‐giver knowledge on clinical outcomes in people with SCD. This review will document evidence that can be used to influence practice in patient and caregiver training for improving the clinical outcomes in people living with SCD.


Randomised controlled trials (RCTs) and quasi‐RCTs. Trials in which quasi‐randomised methods are used were included if there is sufficient evidence that the treatment and control groups are similar at baseline. We included trials in any setting in which the intervention was provided for patients (aged 10 years and older), parents or adult caregivers of children with SCD of all age groups. The participants were of either sex and confirmed to have one of the SCDs (i.e. SS disease, SC disease, Sβ+ thalassaemia and Sβ0 thalassaemia) by electrophoresis and sickle solubility test, with family studies or DNA tests as appropriate. We included trials which evaluated the effectiveness of individual‐ and group‐based interventions for either the patient or caregivers. Outcomes included: Patient and caregiver understanding of SCD and related complications (e.g. the 'Sickle Cell Disease Knowledge Questionnaire' (Armstrong 1993), the 'Illness Perception Questionnaire')

  • Patient or caregiver (or both)‐reported signs and symptoms leading to self‐management
  • Utilisation of health services

A comprehensive search was conducted from the Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register using the terms: sickle cell AND (family interventions OR self‐help OR education). We also searched clinical trial registries for ongoing and completed trials maintained by the European Medicines Agency (, the US National Institute of Health ( and the WHO (

Main Results:


Expected impact:

To inform practice and interventions targeting patients and caregiver with sickle cell disease and its complications.

Next Steps / Future Plans:

Completion of data analysis, quality assessment and writing up of the review.

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