Assessment of fatigue in adult patients with sickle cell disease: Use of the functional assessment of chronic illness therapy-Fatigue (FACIT-fatigue) questionnaire.

Few studies have used validated scales to assess the intensity and determinants of fatigue, a major symptom of sickle cell disease (SCD). We aimed to assess the level of basal fatigue in adult patients with SCD, using the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) questionnaire. We prospectively included 102 stable adult outpatients with SCD over 2 months, who answered the FACIT-Fatigue (ranging from 0 (worst imaginable fatigue) to 52 (no fatigue)) and reported on the intensity of fatigue and its impact on quality of life. The cut-off for significant fatigue was <34. The median [IQR] FACIT-Fatigue score was 29 [22-37], indicating moderate-to-severe fatigue. In a multivariate analysis, the FACIT-Fatigue score was significantly associated with female sex, high body mass index, high level of stress, poor sleep quality, and number of previous episodes of acute chest syndrome, but not with the genotype or the haemoglobin level. Most adult patients with SCD experience significant and sometimes intense fatigue; this is probably due to several factors, including disease activity. Fatigue should be evaluated systematically during consultations and in patient education programmes and as an end-point in therapeutic trials.

Determinants of the haemoglobin level in patients with sickle cell disease living in sub-Saharan Africa: Major impact of the country of residence and independent effects of leucocyte and platelet counts and haemolysis.

The degree of anaemia in sickle cell disease (SCD) is a well-known contributor to morbidity and mortality. We aimed to explore the factors affecting haemoglobin (Hb) level in African SCD patients, considering haemolysis biomarkers (LDH and bilirubin level, and reticulocyte count), leucocyte and platelet counts and socio-demographic characteristics (gender, age group, country of residence and BMI). The research was part of the CADRE multinational cohort and involved 3699 SCD patients living in Mali, Senegal, Ivory Coast, Democratic Republic of Congo, Gabon and Cameroon: 2936 SS/Sß0, 587 SC and 176 Sß + patients with median Hb level of 8, 11.3 and 11.2 g/dL respectively (p < 0.001). In multivariate analysis conducted in 1394 SS/Sß0 patients, living in Cameroon, female gender, lower BMI, higher haemolysis markers (especially LDH) and higher leucocyte and platelet counts were independently associated with lower Hb level (all p < 0.05). In 497 SC and 156 Sß + patients, female gender (p < 0.001), lower BMI (p < 0.05) and higher platelet counts (p < 0.001) were independently associated with lower Hb level. Anaemia in African SCD patients is not only associated with haemolysis but also with the country of residence, lower BMI and leucocyte or platelet counts which might reflect inflammation related to infectious burden in the region.

Determinants of cognitive dysfunction in adults with sickle cell-related stroke or suspected neurological morbidity.

ABSTRACT: The prognosis of sickle cell disease (SCD) in adults is determined primarily by damage to targeted organs such as the brain. Cognitive dysfunction in SCD is a common chronic neurological manifestation, but studies remain mostly descriptive in adults. The objective of this study was to better characterize the cognitive profile and the association between cognitive dysfunction and brain lesions. We included adult patients with SCD referred for a neurological assessment. An adapted battery of neuropsychological tests was used to assess cognitive deficits. Brain or arterial abnormalities were assessed using brain magnetic resonance imaging/magnetic resonance angiography and a cervical and transcranial Doppler ultrasound. The cognitive profile of 96 patients was characterized by deficits in processing speed (58%), short-term memory (34%), and working memory (24%). Brain infarcts were found in 56% of patients and intracranial vasculopathy in 49%. Twenty percent of patients had no brain abnormalities. Processing speed dysfunction was associated with territorial infarcts (odds ratio [OR], 3.1; P = .03) and education outside of France (OR, 4.7; P = .02). Short-term memory dysfunction was associated with territorial infarcts (OR, 3.4; P = .01) and a low educational level (OR, 8.2; P = .01). Working memory dysfunction was associated with a low educational level (OR, 4.3; P = .05) and vasculopathy (OR, 3.7; P = .03). Cognitive dysfunction appears to be a hallmark sign of SCD, particularly for adults with sickle cell-related stroke or suspected neurological morbidity. Assessment of such dysfunction could be used in longitudinal follow-up and clinical trials.

Psychological burden associated with incident persistent symptoms and their evolution during the COVID-19 pandemic: a prospective population-based study.

BACKGROUND: Identifying factors that predict the course of persistent symptoms that occurred during the COVID-19 pandemic is a public health issue. Modifiable factors could be targeted in therapeutic interventions. OBJECTIVE: This prospective study based on the population-based CONSTANCES cohort examined whether the psychological burden associated with incident persistent symptoms (ie, that first occurred from March 2020) would predict having ≥1 persistent symptom 6-10 months later. METHODS: A total of 8424 participants (mean age=54.6 years (SD=12.6), 57.2% women) having ≥1 incident persistent symptom at baseline (ie, between December 2020 and February 2021) were included. The psychological burden associated with these persistent symptoms was assessed with the Somatic Symptom Disorder-B Criteria Scale (SSD-12). The outcome was having ≥1 persistent symptom at follow-up. Adjusted binary logistic regression models examined the association between the SSD-12 score and the outcome. FINDINGS: At follow-up, 1124 participants (13.3%) still had ≥1 persistent symptom. The SSD-12 score at baseline was associated with persistent symptoms at follow-up in both participants with (OR (95% CI) for one IQR increase: 1.42 (1.09 to 1.84)) and without SARS-CoV-2 infection prior to baseline (1.39 (1.25 to 1.55)). Female gender, older age, poorer self-rated health and infection prior to baseline were also associated with persistent symptoms at follow-up. CONCLUSIONS: The psychological burden associated with persistent symptoms at baseline predicted the presence of ≥1 persistent symptom at follow-up regardless of infection prior to baseline. CLINICAL IMPLICATIONS: Intervention studies should test whether reducing the psychological burden associated with persistent symptoms could improve the course of these symptoms.

Management perspectives from patients with fibromyalgia experiences with the healthcare pathway: a qualitative study.

Background: Fibromyalgia is a prevalent condition affecting 1-2% of the general population and can result in significant disability. Physicians and patients frequently encounter challenges in managing this condition. Aim: The aim of this study was to explore novel management approaches through a qualitative analysis of the doctor-patient relationship. Design and setting: Telephonic interviews were conducted with fibromyalgia patients to investigate their healthcare experiences. Methods: Qualitative analysis was performed on patients' narratives using interpretative phenomenological analysis, a methodology that delves into each individual's subjectivity. Results: A total of 19 adult patients with fibromyalgia, primarily middle-aged women (84% women, mean age 49.8 years), recruited from two university centers in Paris, were included in the study. The narratives of participants revealed substantial suffering and considerable functional impairment, which is paradoxical for a condition often considered benign. They reported an ongoing sense of loss of control, exacerbated by an imbalanced patient-doctor relationship. Patients constantly feared not being heard or believed, and they frequently sought attention from their caregivers. Most participants displayed significant ambivalence toward the nature of their condition and actively sought causal links. Patients' adaptive strategies sometimes worsened their symptoms, as in the case of muscular deconditioning. The healthcare system appeared deficient in managing these patients, characterized by a lack of health professional training, frequent inappropriate responses from healthcare providers, and stigmatization of psychological conditions. Conclusion: Despite its perceived benign nature, fibromyalgia should be regarded as a severe condition due to its substantial long-term consequences. Participants reported a challenging experience with the doctor-patient relationship, marked by a strong sense of dependence and a lack of recognition. The care pathway for these patients appeared unsuitable and disorderly, potentially resulting in iatrogenic consequences. The management of patients with fibromyalgia should be enhanced and directed toward a patient-centered approach. The study provides practical recommendations regarding communication methods and patient care.