'It's a traumatic illness, traumatic to witness': a qualitative study of the experiences of bereaved family caregivers of patients with cutaneous T-cell lymphoma.

BACKGROUND: Cutaneous T-cell lymphomas (CTCL) are rare cancers, which can be difficult to diagnose, are incurable and adversely affect quality of life, particularly in advanced disease. Families often provide care, but little is known about their experiences or needs while caring for their relative with advanced disease or in bereavement. OBJECTIVES: To explore the experiences of bereaved family caregivers of patients with CTCL. METHODS: Single, semi-structured qualitative interviews were conducted with bereaved family caregivers of patients with CTCL recruited via a supra-regional CTCL clinic. Transcribed interviews were analysed thematically, focusing on advanced disease, the approach of death and bereavement. RESULTS: Fifteen carers of 11 deceased patients participated. Experiences clustered under four themes: (1) complexity of care and medical intervention; (2) caregiver roles in advanced CTCL; (3) person-centred vs. organization-centred care in advanced CTCL and (4) knowing and not knowing: reflections on dying, death and bereavement. Caregivers often had vivid recollections of the challenges of caring for their relative with advanced CTCL and some took on quasi-professional roles as a result. Advanced disease made high demands on both organizational flexibility and family resources. For many caregivers, seeing disease progression was a prolonged and profoundly traumatic experience. The extent to which they were prepared for their relative's death and supported in bereavement was highly variable. Sub-themes within each theme provide more detail about caregiver experiences. CONCLUSIONS: Family caregivers should be considered part of the wider healthcare team, acknowledging their multiple roles and the challenges they encounter in looking after their relative with CTCL as the disease progresses. Their experiences highlight the importance of organizational flexibility and of good communication between healthcare providers in advanced CTCL.

'We had to change to single beds because I itch in the night': a qualitative study of the experiences, attitudes and approaches to coping of patients with cutaneous T-cell lymphoma.

BACKGROUND: Primary cutaneous T-cell lymphoma (CTCL) is a rare but prevalent condition which can have a significant impact on many aspects of quality of life. However, there is little evidence of patients' lived experience of CTCL. OBJECTIVES: To understand in greater depth patients' experiences of living and coping with CTCL, and to inform the development of models of care for this population in line with U.K. METHODS: Semi-structured interviews were conducted with a purposive sample of patients with CTCL who attended an inner-city teaching hospital. Participants were purposively selected according to their disease stage, age, sex and ethnicity. RESULTS: Nineteen patients with CTCL (stages IB-IVB), aged between 41 and 83 years, participated in a single interview. This included 10 men; 15 people identified themselves as white British. Interviews lasted a median of 55 (range 28-170) min. Two main themes emerged: issues regarding diagnosis, particularly a perceived delay in diagnosis, and the impact of CTCL (subthemes related to physical well-being, practical concerns and psychological and social well-being and coping). CONCLUSIONS: Findings from this study illuminate the diverse effects of CTCL on patients' lives. The universal experience of delays in diagnosis was striking and a concern to patients. The disease, particularly its physical symptoms, had a significant impact on patients' lives, including employment, leisure and relationships. Despite the symptom burden and its impact, participants described effective coping strategies such as drawing on social support, maintaining normal lives and becoming well informed about CTCL. Proactive holistic assessment and management of the range of patient concerns is needed in providing care for patients with CTCL and their family and friends.

'We're all carrying a burden that we're not sharing': a qualitative study of the impact of cutaneous T-cell lymphoma on the family.

BACKGROUND: Cutaneous T-cell lymphoma (CTCL) is a rare, progressive cancer that can be life limiting and highly disfiguring. Patients with CTCL experience poor quality of life; however, there is little published about the experiences of their families. OBJECTIVES: To describe the impact of CTCL on family members and how they cope and adjust, to inform support services. METHODS: Semistructured qualitative interviews were conducted with adult informal caregivers of patients with CTCL recruited via a supraregional CTCL clinic. Interviews explored the history of each patient's illness, the impact of CTCL on the patient and the family, and views about family support. Data were analysed thematically using the Family Adjustment and Adaptation Response model as an interpretative framework. RESULTS: Fourteen caregivers were interviewed (11 spouses, one friend, two daughters; 10 women, four men; all white British; aged 39-85 years). Three key themes emerged: (i) demands of CTCL (the disease, caregiving, financial impact, physical and emotional intimacy); (ii) family capabilities (family support, information, healthcare provider support, other coping strategies); and (iii) adjustment and adaptation (acceptance, changes in patient-caregiver relationship and family dynamics). CTCL was central in many aspects of caregivers' lives, particularly relationships, communication and intimacy. CONCLUSIONS: Our findings demonstrate the multiple demands that CTCL places on caregivers, the capabilities and resources they draw upon to cope, and the significant impact of CTCL on the family. To support families and patients, easily accessible services are needed that include the family in the unit of care, provide support and information, and understand the process of family adjustment and adaptation.

What are the supportive and palliative care needs of patients with cutaneous T-cell lymphoma and their caregivers? A systematic review of the evidence.

BACKGROUND: Primary cutaneous T-cell lymphoma (CTCL) is progressive, can cause significant symptoms, and impacts on quality of life. Therefore supportive and palliative care might have a role in the care of patients and families. OBJECTIVES: To identify and appraise the available peer-reviewed evidence on the supportive and palliative care needs of patients and their caregivers. METHODS: A systematic review of the literature was conducted. Extracted data from eligible papers were collated in themes relating to supportive and palliative care needs and outcomes for patients, informal caregivers, health professionals and reported service models. RESULTS: Eighteen retained papers reported a symptom or quality-of-life measure. Five reported only these measures, 13 reported outcomes in relation to an intervention. Systemic therapy targeted at disease remission was the most commonly reported intervention (12/13). No quality-of-life tool was consistently used. Pruritus was frequently reported as an outcome (n = 9) often using the visual analogue scale, VAS itch. Psychosocial, spiritual and caregiver needs were reported infrequently or not at all. CONCLUSIONS: No measure is routinely used to measure supportive and palliative care outcomes in CTCL. Physical needs, particularly pruritus, were reported commonly; however, qualitative evidence of experience is limited. Caregivers' needs are rarely explored. To compare outcomes from clinical studies, a single multidimensional tool used in routine practice would be useful. Further work is needed to explore a model of service that meets the specific physical, psychosocial and spiritual needs of this group of patients and their carers.

Neuropsychometric outcome predictors for adults with maple syrup urine disease.

We describe 14 adult MSUD patients (3 with intermediate MSUD) with varied clinical and neuropsychometric outcomes. Age at diagnosis appears to be a predictor of IQ even in adults, while long-term blood leucine does not correlate with IQ.