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Cannabinoids are increasingly being considered for the management of various painful conditions, and could be considered as an option for treating acute pain in sickle cell disease (SCD). The objective of this study was to determine the extent of use of cannabis in the community for pain and other symptom relief, and its side effects during self-administration in patients with SCD. Patients attending Central Middlesex Hospital in London were invited to complete a structured self-administered anonymous questionnaire. Eighty-six young adults with HbSS, HbSC and HbS βthalassaemia disease (median age 30 years) participated in the study. Results showed that 31 (36%) had used cannabis in the previous 12 months to relieve symptoms associated with SCD. The main route in all but two patients was by smoking. The main reasons for use were to reduce pain in 52%, and to induce relaxation or relieve anxiety and depression in 39%. Symptoms related to sedation and mood effects were reported in 77% of patients. The majority of patients (58%) expressed their willingness to participate in studies of cannabis as a medicine. We conclude that research in the use of cannabinoids for pain relief in SCD would be both important and acceptable to adult patients. [ABSTRACT FROM AUTHOR]

This review examines the evidence for some of the common psychological complications found across the life span of patients with sickle cell disease (SCD), which are likely to be encountered by haematologists responsible for their medical management. Electronic searches of medical and psychological databases were conducted with a focus on three main areas: psychological coping, quality of life and neuropsychology. Psychological complications were identified in both children and adults with SCD, and included inappropriate pain coping strategies; reduced quality of life owing to restrictions in daily functioning, anxiety and depression; and neurocognitive impairment. There were wide variations in design and consistency of the studies, therefore, some caution needs to be observed in the findings. Moreover, interventional studies were lacking in some areas such as neuropsychology. Utilization of psychological interventions including patient education, cognitive behavioural therapy, and special educational support to help improve the quality of life of patients are recommended. [ABSTRACT FROM AUTHOR]

Summary: Ageing in sickle cell disease (SCD) is associated with a myriad of end‐organ complications, including cerebrovascular damage and cognitive impairment (CI). Although CI is very common in SCD, little is known about cognitive functioning and how it changes with age. This study examines cognitive patterns of 63 adults with SCD and 60 non‐SCD, age‐ and education‐matched controls in Ghana. Of those adults with SCD, 34 completed the neuropsychological battery at baseline and again seven years later. In cross‐sectional data, adults with SCD performed worse than controls in all cognitive test domains (p < 0.01 for all). The seven‐year follow‐up data showed that the group exhibited a significant decline in visuospatial abilities (ranging from Cohen's d = 1.40 to 2.38), and to a lesser extent, in processing speed and executive functioning. Exploratory analyses showed a significant time‐by‐education interaction, indicating that education may be protective from decline in cognitive performance. These findings have implications for clinical practice. Early neuropsychological surveillance coupled with early assessment and remedial programmes will provide avenues for enhancing the quality of life of adults living with SCD in Ghana. [ABSTRACT FROM AUTHOR]

Raised tricuspid regurgitant velocity ( TRV) occurs in approximately 30% of adults with sickle cell disease ( SCD), and has been shown to be an independent risk factor for death. TRV was assessed in 164 SCD patients who were subsequently followed up for survival. Raised pulmonary pressures were defined as a TRV jet ≥2·5 m/s on echocardiography. Elevated TRV was present in 29·1% of patients and it was associated with increased age and left atrial diameter. There were 15 deaths (9·1%) over a median of 68·1 months follow up; seven patients had increased TRV, and eight patients had a TRV<2·5 m/s. Higher TRV values were associated with a greater than 4-fold increased risk of death (Hazard Ratio: 4·48, 99% confidence interval 1·01-19·8), although we found a lower overall mortality rate than has been reported in previous studies. TRV was not an independent risk factor for death. We have confirmed the association between raised TRV and mortality in a UK SCD population whose disease severity appears to be less than that reported in previous studies. Further prospective studies are needed to more clearly characterize which patient factors modify survival in SCD patients with raised TRV. [ABSTRACT FROM AUTHOR]