Health-related Quality of Life in Cutaneous Lymphomas: Past, Present and Future.

Previous studies have reported that primary cutaneous lymphomas profoundly influence patients' health-related quality of life (HRQoL). However, assessment of this psycho-social concept is not common in routine patient care unless required within clinical trials. The aim of this review is to provide a comprehensive overview of HRQoL measures and outcomes in cutaneous lym-phomas in order to inform clinicians. Advanced-stage cutaneous lymphomas were found to be associated with worse HRQoL than early-stage disease. Specifically, progression of the disease, age, sex, psychosocial issues, educational level and therapy were related to the extent of impairment of HRQoL. Treatment response was linked to improved HRQoL, but notably ameliorated HRQoL scores were also reported despite objective disease response. However, the variety of instruments applied to measure HRQoL in cutaneous lymphomas makes it difficult to compare data directly. In conclusion, speciality-specific HRQoL instruments were superior to generic ones, which probably failed to recognize small, but relevant, changes, demonstrating the need for a disease-specific tool.

'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.

Patients' Illness Perception as a Tool to Improve Individual Disease Management in Primary Cutaneous Lymphomas.

The Revised Illness Perception Questionnaire (IPQ-R) has been shown to assess illness perception reproducibly in primary cutaneous T-cell lymphomas (CTCL). Illness perception reflects patients' individual concepts of understanding and interpretation of the disease, influencing illness behaviour and health-related quality of life (HRQOL). This study investigated the clinical relevance of the relationships between illness perception, illness behaviour, and HRQOL in CTCL and cutaneous B-cell lymphomas (CBCL). A total of 92 patients completed the IPQ-R, the Scale for the Assessment of Illness Behavior (SAIB), and a skin-specific HRQOL tool (Skindex-29). Data on illness behaviour were not evidently related to illness perception, whereas illness perception was significantly associated with HRQOL. Both, IPQ-R and HRQOL results correlated with disease entity, stage, and socio-demographics. Only IPQ-R results provided practical information on patients' needs to train personal coping strategies. IPQ-R assessment in CTCL and CBCL might be a useful instrument to improve individual disease management.

Illness Perception in Primary Cutaneous T-cell Lymphomas: What Patients Believe About Their Disease.

There is currently no information available on illness perception in primary cutaneous T-cell lymphomas (CTCL). The aim of this study was therefore to gather initial information on disease understanding and interpretation in patients with CTCL. Consecutive patients from a hospital-based primary cutaneous lymphoma ward completed the Revised Illness Perception Questionnaire (IPQ-R) on 2 consecutive visits. A total of 24 patients with different variants of CTCL were included in the study. Patients experienced their condition as being long-lasting, but not fundamentally affecting their lives. Patients had poor belief in personal control, but strong belief in treatment control. They did not show a good understanding of their disease, and had a moderately negative emotional response to their illness. In conclusion, the IPQ-R provides a feasible and reproducible tool for measurement and better understanding of illness perception in patients with CTCL. Knowledge of patients' attitudes towards their disease should enable optimization of the patient-physician relationship and patient care.

'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.

Cognition/Psychological Burden and Resilience in Cutaneous T-Cell Lymphoma and Psoriasis Patients: Real-Life Data and Implications for the Treatment.

Background: Psoriasis and cutaneous T-cell lymphoma (CTCL) expose patients to chronic inflammation as well as physical and psychological disabilities, but the impact of such alterations on cognitive function is unknown. Objective: This study is aimed at determining if CTCL and psoriasis impact cognitive functioning in relation to psychological and health-related quality of life (HR-QOL) status. Methods: A cross-sectional study was performed in an outpatient dermatology clinic of a university teaching hospital. Thirty-nine subjects with CTCL (N = 20) or psoriasis (N = 19) who met eligibility criteria were included. The cognitive domains of memory, attention and processing speed, and executive function were assessed with standard neuropsychological tests. Subjects were assessed for depression, anxiety, and HR-QOL (using the SKINDEX-29 questionnaire). Results: Study participants were CTCL and psoriasis subjects; cognitive impairment was found in the domain of memory in 17.9% subjects with CTCL or psoriasis. Lower scores on executive function tests were predicted by higher (worse HR-QOL) SKINDEX-29 functioning scores (p = 0.01). A higher estimated baseline intellectual functioning predicted lower scores (better HR-QOL) on the symptoms and functioning domains of SKINDEX-29 (p = 0.01 and 0.02, respectively) and a statistical trend (p = 0.07) for the emotion domain. Memory and acute anxiety were adversely impacted by shorter disease duration (p = 0.01 for both). Conclusions: Memory impairment may be associated comorbidity in CTCL and psoriasis. Subjects with stronger cognitive resources appear to cope better with health-related quality of life (HR-QOL) challenges.

Patient-reported quality of life in patients with relapsed/refractory cutaneous T-cell lymphoma: Results from the randomised phase III ALCANZA study.

BACKGROUND: Brentuximab vedotin was approved for adult patients with CD30-expressing cutaneous T-cell lymphoma treated with prior systemic therapy based on improved response rates and progression-free survival with brentuximab vedotin (1.8 mg/kg once every 3 weeks; ≤16 cycles) versus physician's choice (methotrexate/bexarotene; ≤48 weeks) in the phase III ALCANZA study. Quality of life (QoL) in ALCANZA patients was also examined. METHODS: QoL measures in ALCANZA were based on the Skindex-29, Functional Assessment of Cancer Therapy-General (FACT-G) and European QoL 5-dimension (EQ-5D) questionnaires. RESULTS: Mean maximum reduction from the baseline Skindex-29 symptom domain score (key secondary end-point) was greater with brentuximab vedotin than physician's choice (-27.96 versus -8.62); the difference, -18.9 (95% confidence interval -26.6, -11.2; adjusted p < 0.001), exceeded the study-defined minimally important difference (9.0-12.3). Mean changes from baseline to end-of-treatment visit total FACT-G scores were similar with brentuximab vedotin and physician's choice (0.15 versus -2.29). EQ-5D changes were also comparable between arms. Among brentuximab vedotin-treated patients with peripheral neuropathy (PN), mean maximum reduction in Skindex-29 symptom domain was -35.54 versus -11.11 in patients without PN. PN had no meaningful effect on FACT-G and EQ-5D QoL scores. CONCLUSIONS: In summary, brentuximab vedotin produced superior reductions in symptom burden compared with physician's choice, without adversely impacting QoL. QoL was unaffected by the presence of PN in brentuximab vedotin-treated patients. CLINICAL TRIAL REGISTRATION: NCT01578499.

Cutaneous T-cell lymphoma: overview and nursing perspectives.

Cutaneous T-cell lymphoma (CTCL) is an uncommon and complex malignancy of the immune system with a wide range of clinical presentations primarily involving the skin. An extensive menu of skin-directed and/or systemic treatment options exists. Best practices in management involve multidisciplinary collaboration. Nursing care for patients who have CTCL is a critical component in the successful management of the disease and requires special attention to the patient's physical, emotional, and spiritual needs. Nurses can make a significant impact by being accessible, offering emotional support, demonstrating advocacy, and providing ongoing education for the patient and family.

Health-related quality-of-life assessment in patients with cutaneous T-cell lymphoma.

OBJECTIVE: To measure and evaluate the health-related quality of life (HRQOL) in patients with cutaneous T-cell lymphoma (CTCL), a visible cutaneous malignancy that may have a profound effect on patients' lives. DESIGN: Monocenter, cross-sectional study. SETTING: The Skin Oncology Program, Department of Dermatology, and the Photopheresis Unit of Boston Medical Center. PATIENTS: A total of 22 adult patients with confirmed CTCL. MAIN OUTCOME MEASURES: (1) Evaluation of general oncologic and skin disease-specific HRQOL using, respectively, the Functional Assessment of Cancer Therapy-General (FACT-G) and Skindex-29 profiles; (2) assessment of HRQOL association with disease stage (early stage, IA-IIA; late stage, IIB-IVB). RESULTS: Patients with more advanced CTCL stages reported more effects on general health (FACT-G), particularly in the physical, emotional, and functional domains (P < .05). Patients with early-stage CTCL reported better skin-specific HRQOL overall (Skindex-29; P = .002) and for each specific domain than did patients with late-stage disease. The Skindex-29 scales had high internal consistency, and the confirmatory factor structure was similar to that of previous studies. CONCLUSIONS: The HRQOL of patients with CTCL can be evaluated using the Skindex-29 and FACT-G instruments. Patients with more advanced stages of CTCL had lower HRQOL scores.

Prognosis, clinical outcomes and quality of life issues in cutaneous T-cell lymphoma.

Although the need for a specific QOL index for MF/SS has been recognized, little work has been done in this area. QOL instruments should address general health concepts, as well as specific issues that are relevant to patients who have MF/SS; a combination of two instruments may be appropriate. Confirming the validity, reliability, and responsiveness to change of a combination of QOL instruments, such as SKINDEX-29 and FACT-G, in patients who have MF/SS will be relevant. For patients who have MF/SS, focusing our efforts on improving the disease and their QOL should translate into improvements that are meaningful to them. Thus, our goal should be to incorporate the findings of QOL research into practice and use the results to guide our practices, as they relate to therapy. Some important considerations are: We must be explicit about what will be done with the information that is obtained about an individual's QOL. We should consider what additional knowledge QOL measures provide that we could not otherwise have. We should be ready for unexpected results, as exemplified by instances where aggressive therapy could result in improved QOL. Although challenges exist in moving forward QOL research in CTCL, the words of wisdom of Tannock bring the concept of QOL into perspective: "When cure remains elusive, it is time to start treating the patient, not just the tumor." A final consideration is that we should not forget the dimension of spirituality, which is especially relevant to patients who have advanced MF/SS. Spirituality is characterized by the capacity to seek purpose and meaning, to have faith, to love, to forgive, and to see beyond current circumstances. Spirituality enables a person to rise above suffering. Unfortunately, research on spirituality is scarce, in part, because of the difficulty in assessing spiritual suffering. Nevertheless, physicians who are caring for patients who have advanced MF/SS should acknowledge the spiritual dimension as an integral component of the dying process.

Total skin electron beam therapy and cutaneous T-cell lymphoma: a clinical guide for patients and staff.

Total skin electron beam therapy (TSEBT) is a technically complicated treatment used in the management of mycosis fungoides (MF). A variety of technical and clinically oriented issues relating to TSEBT and its effects for patients with MF are reviewed.

Prevalence and severity of pruritus and quality of life in patients with cutaneous T-cell lymphoma.

CONTEXT: Cutaneous T-cell lymphoma (CTCL), although rare, is associated with a significant symptom burden. Pruritus appears to be one of the most prominent and disturbing symptoms. OBJECTIVES: To describe the prevalence and severity of pruritus and quality of life (QOL) in patients with CTCL. METHODS: Patients with CTCL able to complete two questionnaires were invited to complete a visual analogue scale for itch (VAS(itch)) and the Skindex-29. Prevalence of pruritus, mean score, and SD were estimated for the VAS(itch) and Skindex-29, and the Pearson's correlation coefficient was calculated to evaluate the relationship between severity of pruritus and QOL. RESULTS: One hundred patients were recruited (mean [SD] age 57.9 [12.9] years, range 30-86 years). Eighty-eight percent reported pruritus in the preceding four weeks, 46% indicating that it was often or always a problem. The mean (SD) of VAS(itch) (n=92) was 3.2 (3.2), range zero to 10. The mean (SD) total Skindex-29 score was 43.3 (27.7). More advanced disease stage was associated with poorer QOL. The Skindex-29 correlated strongly with the VAS(itch) (Pearson's correlation coefficient=0.72, P<0.001). CONCLUSION: All aspects of QOL are affected in CTCL. Pruritus is a common and troublesome symptom. A more advanced disease stage and more severe pruritus symptoms were associated with poorer QOL in this study.

Significant impact of cutaneous T-cell lymphoma on patients' quality of life: results of a 2005 National Cutaneous Lymphoma Foundation Survey.

BACKGROUND: Cutaneous T-cell lymphoma (CTCL) can have a profound impact on a patient's health-related quality of life; however, little is known about its actual impact. The authors evaluated patients' perspectives on the impact of CTCL on physical functioning, lifestyle, emotional well being, and satisfaction with treatment. METHODS: A 4-page, self-administered questionnaire was mailed and made available online in March 2005 to the entire United States membership of the Mycosis Fungoides Foundation (n = 930 members). Outcome measures were patients' perspectives on the psychosocial impact of CTCL and the management of their disease. RESULTS: The response rate was 68%, and 93.6% of respondents were white. The majority of respondents had mycosis fungoides (89%). Respondents were bothered by skin redness (94%) and by the extent of symptoms that affected their choice of clothing (63%). For most patients, the disease had a functional impact, rendering them tired or affecting their sleep. Health distress was reported by almost all respondents, with 94% reporting that they worried about the seriousness of their disease and 80% worrying about dying from the disease. Sixty-two percent of respondents reported that their disease made them feel unattractive, 85% reported that their treatment made their disease seem more manageable, but 61% reported that they felt burdened financially by their disease. CONCLUSIONS: The high response rate and patients' responses to the survey provided compelling evidence that patients believed CTCL had a profound and severe impact on their functioning, emotional, and social well being. A striking health distress was prevalent in almost all respondents. Although the majority of patients reported that treatments made their disease more manageable, a significant proportion reported that they felt burdened financially by their disease.