Psychological therapies for sickle cell disease and pain.

backgroundSickle cell disease comprises a group of genetic blood disorders, and occurs when the sickle haemoglobin gene is inherited from both parents. The effects of the condition are: varying degrees of anaemia which if severe reduce the capacity for mobility; predisposition to obstruction of small blood capillaries causing pain in muscle and bone known as "crises"; damage to major organs such as the spleen, liver, kidneys, and lungs; and increased vulnerability to severe infections. There are both medical and non-medical complications, and treatment is usually symptomatic and palliative in nature. Psychological intervention for individuals with sickle cell disease seems viable in complementing current medical treatment, and studies examining their efficacy appear to have also yielded encouraging results.

objectivesTo examine the evidence that in patients with sickle cell disease, psychological treatment improves the ability to cope with the condition. SEARCH STRATEGY: The Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references from comprehensive electronic database searches. Also, hand searching relevant journals, hand searching abstract books of conference proceedings, and searches on the Internet were performed. Date of the most recent search of the Group's specialised register: January 2001. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in patients with sickle cell disease. DATA COLLECTION AND ANALYSIS: Both reviewers independently extracted data and assessed trial quality. MAIN

resultsFive studies were identified in the initial search, of which three studies, with a total of 158 patients were eligible for inclusion in the review. Published data reveal that family education and cognitive behavioural therapy can help patients cope with sickle cell disease. REVIEWER'S

conclusionsPatient education programmes improve knowledge and attitudes of patients with sickle cell disease. There is as yet however, insufficient evidence to demonstrate the role of other psychological therapies. This systematic review has clearly identified the need for well-designed, adequately powered, multicentre randomised controlled trials assessing the effectiveness of specific intervention in sickle cell disease.