La drépanocytose est une hémoglobinopathie chronique fréquente dont le pronostic vital s’est amélioré, au prix d’un fardeau symptomatique et psychosocial important. La douleur et les crises vaso-occlusives sont au premier plan, mais la fatigue est rapportée comme l’un des symptômes les plus fréquents et invalidants, en lien avec des troubles de l’humeur, du sommeil et une altération de la qualité de vie. Pourtant, la fatigue reste peu évaluée et largement absente des recommandations de prise en charge de la drépanocytose, en particulier en France.

Décrire l’expérience vécue de la fatigue chez des adultes vivant avec une drépanocytose, et identifier les stratégies d’adaptation utilisées au quotidien, afin d’éclairer l’adaptation d’un programme de gestion de la fatigue.

Une étude qualitative exploratoire a été menée auprès de neuf adultes (7 femmes, 2 hommes ; âge moyen : 40 ans), répartis en deux focus groups distincts : patients « experts » (n = 4) et patients « tout-venant » (n = 5).

Douze sous-thèmes, regroupés en quatre thèmes, ont été identifiés : (1) caractéristiques spécifiques de la fatigue drépanocytaire (distinction avec la « fatigue ordinaire », manifestations multidimensionnelles, répercussions plurielles) ; (2) symptomatologie associée (douleur, affectivité, somnolence) ; (3) vécu d’un handicap invisible (incompréhension, stigmatisation, dépassement de soi) ; (4) adaptations pour gérer la fatigue (stratégies d’économie d’énergie, aménagements, ajustements temporels constants).

Les participants décrivent une fatigue subjectivement distincte de la fatigue ordinaire, fortement intriquée à la douleur et aux enjeux identitaires. Ce vécu de « handicap invisible » nourrit des tensions entre désir de dépassement de soi et risque d’auto-surmenage. Ces résultats plaident pour une évaluation systématique de la fatigue dans la drépanocytose et soutiennent la pertinence d’un programme adapté aux spécificités de cette pathologie.

Sickle cell disease (SCD) is one of the most common severe monogenic disorders worldwide. Advances in neonatal screening and comprehensive care have substantially improved survival, but at the cost of physical, psychological and social burden across the life span. Beyond vaso-occlusive pain, chronic fatigue is increasingly recognised as a highly prevalent and disabling symptom, associated with depressive and anxiety symptoms, sleep disturbance and reduced health-related quality of life. Quantitative studies suggest that approximately two thirds of adults with SCD report clinically significant fatigue, with strong correlations with mood and stress. Qualitative work, mostly in English-speaking settings and among adolescents or young adults, shows that fatigue is experienced as a multidimensional, intrusive and poorly understood phenomenon. In France, research on fatigue in SCD remains scarce. A recent systematic review of fatigue measurement in SCD underlined the great heterogeneity of available tools and the absence of disease-specific validated instruments, which may contribute to the lack of clinical attention to this symptom. In contrast, several psychological self-management programmes have been developed for fatigue in other chronic conditions, such as multiple sclerosis. FACETS (Fatigue: Applying Cognitive behavioural and Energy effectiveness Techniques to lifeStyle) is a manualised group-based cognitive-behavioural and energy conservation programme that has shown sustained effects on fatigue severity and fatigue self-efficacy. No such structured intervention currently exists for adults with SCD in the French context.

This qualitative study aimed to (1) describe how adults living with SCD conceptualise and experience fatigue in their daily lives, (2) explore the emotional, interpersonal and functional consequences of this symptom, and (3) identify existing coping and energy-management strategies. These findings were intended to inform the adaptation of the FACETS programme to SCD (Drépa-FACETS), especially its psychoeducational component.

We conducted an exploratory qualitative study using focus groups. Purposive sampling was used to recruit nine adults with a confirmed diagnosis of sickle cell syndromes (SS, SC or S-β), aged 18 or older, fluent in French and not in an acute vaso-occlusive crisis at inclusion. Participants with severe psychiatric disorders or another chronic disease causing fatigue were excluded. Two distinct focus groups were organised: one with ‘expert patients’ (n = 4) involved in patient education programmes or patient organisations at a national level, and one with ‘non-expert’ patients (‘tout-venant’, n = 5) recruited from a university hospital's active file. Both groups met once for approximately two hours via a secure videoconference platform. Sessions were co-facilitated by two clinical psychologists, using a semi-structured interview guide addressing SCD symptoms, manifestations and impacts of fatigue, and strategies to manage energy in daily life. A photo-elicitation task was used to stimulate metaphors and images associated with fatigue. All sessions were audio-recorded and fully transcribed using a secure transcription software, then anonymised. Data were analysed using reflexive thematic analysis within an interpretative framework. A multidisciplinary team (psychologists, physician, psychology students) independently coded transcripts, discussed discrepancies and refined themes until consensus was achieved.

Twelve subthemes were organised into four overarching themes. (1) Specific features of SCD-related exhaustion . Participants clearly distinguished their experience from ‘normal tiredness’, describing a deep, unpredictable and sometimes sudden exhaustion that could occur ‘without any apparent trigger’, with physical, cognitive and emotional dimensions and major consequences on daily activities, social participation and identity. (2) Associated symptomatology . Exhaustion was closely intertwined with vaso-occlusive pain, mood fluctuations (irritability, sadness, guilt) and excessive sleepiness, forming a cluster of mutually reinforcing symptoms. (3) Invisible disability . Participants reported feeling misunderstood or doubted by relatives, employers and health professionals, oscillating between experiences of minimisation, stigma (being perceived as lazy or unreliable) and internalised pressure to ‘overperform’ to compensate for the invisibility of their condition. (4) Adaptation strategies and temporal adjustments . Patients described a wide range of coping strategies, from energy-saving behaviours and task reorganisation to more ambivalent forms of overinvestment and self-sacrifice. The temporal course of adaptation was marked by constant negotiations, anticipations and renunciations, within a context of highly variable and unpredictable fatigue.

This study highlights that adults with SCD conceptualise exhaustion as a distinct, multidimensional and identity-threatening experience, insufficiently recognised by health professionals and social environments. The findings converge with international literature on fatigue in SCD and other chronic conditions. while underlining specific issues related to invisibility, stigma and the tension between self-protection and self-overexertion. These elements provide concrete targets for the psychoeducational and cognitive-behavioural components of a tailored fatigue-management programme (Drépa-FACETS), including normalisation of exhaustion, pacing, self-efficacy enhancement and work on social representations of invisible disability.