Psychological therapies for sickle cell disease and pain

Sickle cell disease is a group of blood disorders. It can cause anaemia, which if severe can reduce mobility. It may also allow small blood vessels to become blocked causing pain in muscle and bone and it can damage major organs such as the spleen, liver, kidneys, and lungs. People with sickle cell disease are more vulnerable to severe infections. Treatment is usually in response to symptoms and designed to ease pain. Psychological treatment to help people cope with sickle cell disease might complement current medical treatment. There are four types of treatment: patient education; cognitive therapy (to do with thoughts and feelings); behavioural therapy (to do with actions); psychodynamic psychotherapy (talking to relieve emotional pain). We searched for randomised or quasi-randomised controlled trials which compared psychological treatments to each other or to no treatment in sickle cell disease. We included seven studies in the review, of which five, with 260 people, had data we could enter into the review. One study showed that cognitive behaviour therapy reduced the affective part of pain (feelings about pain), but not the sensory part (pain intensity). Another study of this therapy had inconclusive results for coping strategies and showed no difference on how different groups used the health service. A study using cognitive behavioural therapy with teenagers and their families at home did not show any difference when compared with education about sickle cell disease. One education study did not show a reduction in depression. Furthermore, one study in patient education helped improve attitudes to healthcare workers and medication use in adolescents and young adults. The authors believe that some patient education seems relevant for children, adolescents and young adults, while methods to improve the ability to cope in both children and adults are important. Nonetheless, these results may not apply to across all ages, clinical severity, types of pain (acute or chronic) that people with sickle cell disease have, or which country they live in. More research needs to be done in this area.

Authors' conclusions: 

Evidence for the efficacy of psychological therapies in sickle cell disease is currently limited. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions in sickle cell disease.

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Background: 

Sickle cell disease comprises a group of genetic blood disorders. It occurs when the sickle haemoglobin gene is inherited from both parents. The effects of the condition are: varying degrees of anaemia which, if severe, can reduce mobility; a tendency for small blood capillaries to become blocked causing pain in muscle and bone commonly 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 interventions for individuals with sickle cell disease might complement current medical treatment, and studies of their efficacy have yielded encouraging results. This is an update of a previously published Cochrane Review.

Objectives: 

To examine the evidence that psychological interventions improve the ability of people with sickle cell disease to cope with their condition.

Search strategy: 

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and the Internet, handsearches of relevant journals and abstract books of conference proceedings.

Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 17 February 2015.

Selection criteria: 

All randomised or quasi-randomised controlled trials comparing psychological interventions with no (psychological) intervention in people with sickle cell disease.

Data collection and analysis: 

Both authors independently extracted data and assessed the risk of bias of the included studies.

Main results: 

Twelve studies were identified in the searches and seven of these were eligible for inclusion in the review. Five studies, involving 260 participants, provided data for analysis. One study showed that cognitive behaviour therapy significantly reduced the affective component of pain (feelings about pain), mean difference -0.99 (95% confidence interval -1.62 to -0.36), but not the sensory component (pain intensity), mean difference 0.00 (95% confidence interval -9.39 to 9.39). One study of family psycho-education was not associated with a reduction in depression. Another study evaluating cognitive behavioural therapy had inconclusive results for the assessment of coping strategies, and showed no difference between groups assessed on health service utilisation. In addition, family home-based cognitive behavioural therapy did not show any difference compared to disease education. One study of patient education on health beliefs showed a significant improvement in attitudes towards health workers, mean difference -4.39 (95% CI -6.45 to -2.33) and medication, mean difference -1.74 (95% CI -2.98 to -0.50). Nonetheless, these results may not apply across all ages, severity of sickle cell disease, types of pain (acute or chronic), or setting.

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