John: Sickle cell disease is a lifelong, inherited disorder which can cause complications throughout an individual's life. In a new Cochrane Review from October 2016, Monika Asnani and colleagues from the Caribbean Institute for Health Research at the University of the West Indies in Kingston Jamaica bring together the evidence on ways to improve the knowledge of patients and their caregivers. She outlines their findings in this Evidence Pod.

Monika: Sickle cell disease can cause a huge burden on both the patient and their family, including frequent visits to healthcare facilities. The illness causes not just physical complications such as painful crises and strokes, but may have many other effects including depression, poor quality of life, coping issues and poor family relationships.
Some studies have shown that when people with chronic illness learn self-management, their clinical outcomes and quality of life improves; and they show lower dependence on healthcare services. We wanted to see if this might be the case for sickle cell disease, given that there are no reviews of which interventions improve knowledge in sickle cell disease and little is known about the impact of patient or care-giver knowledge on clinical and psychosocial outcomes in people with sickle cell disease.
We investigated the effectiveness of patient- and caregiver-centred educational interventions for changing knowledge and understanding of sickle cell disease among patients and caregivers of people with the disease. We included 12 trials with a total of more than 500 people with the disease, who ranged from 6 to 35 years of age. Several different interventions had been tested, including one lasting just one hour and others that had weekly sessions for up to eight weeks. The timing of the post-intervention assessments also varied, ranging from right after the intervention period to 12 months after completion.
Four studies reported that educational programs improved patient knowledge. Caregiver knowledge improved as well, although this finding comes from just one trial conducted in 20 families. The effect on patient knowledge was sustained at longer follow-up periods, but the effect on caregiver knowledge was not sustained. Although depression showed a statistically significant decline in intervention groups, we did not see any effects on coping, family relationships or health-related quality of life of patients. No comparative data were reported to assess whether the recognition of signs and symptoms by patients or caregivers led to better self-management or adherence to treatment.
In summary, our review identifies important positive effects of educational interventions on improving patient knowledge of sickle cell disease and reducing depression. Effects on patients’ knowledge were maintained for longer than for caregivers; but the effect on knowledge was small and it’s not clear if it offers any clinical benefit. Significant factors limiting our ability to investigate these effects are the low number of participants in the trials and high attrition rates. The shortage of trials, and of recruited patients and caregivers, also made it difficult to assess secondary outcomes; as did the moderate to high heterogeneity across the studies. We should like to see further controlled trials, with rigorous attention given to improving recruitment and retention and to decreasing bias, to better study the effects of these interventions. This would also be helped by the use of predetermined protocols, with similar measurements across multiple sites.

John: Information on all of the studies in Monika’s review and its overall findings are available in full online, at Cochrane Library dot com. You can find the current review with a simple search for ‘sickle cell and knowledge‘, and watch for updates should the extra trials be done.