Development of a Disease-Specific Health-Related Quality of Life Questionnaire (DTF-QoL) for Patients with Desmoid-Type Fibromatosis

Abstract

Sporadic desmoid-type fibromatosis (DTF) is a rare, non-metastasising soft-tissue tumour. Patients can experience a variety of disease-specific issues related to the unpredictable clinical course and aggressiveness of DTF, which negatively impacts health-related quality of life (HRQoL). These DTF-specific issues are not captured by generic HRQoL tools. A 102-item provisional DTF-specific HRQoL tool, the DTF-QoL, was previously developed. The aim of this study was to pre-test the psychometric properties of the DTF-QoL by administering it together with the EORTC Quality of Life Questionnaire Core 30 (EORTC QLQ-C30) to 236 DTF patients from the United Kingdom and the Netherlands. Construct validity and reliability were determined based on factor analysis, multi-trait scaling analysis, Cronbach’s alpha, and correlations with the EORTC QLQ-C30 scales. Ninety-six items were selected, conceptualised into three symptom scales, eleven disease-impact scales and six single items, together forming the final DTF-QoL. Scaling assumptions were fully or moderately met for ten out of fourteen scales. Cronbach’s alpha ranged from 0.551–0.908. Most scales of the DTF-QoL were weakly or moderately correlated with the EORTC QLQ-C30. The DTF-QoL is a promising tool capturing the whole spectrum of DTF-specific issues. Implementation of the DTF-QoL in research and clinical practice will help to personalise HRQoL measurement and clinical care for DTF patients.

Clinical trials in desmoid‐type fibromatosis in children and adults: A systematic review

“…For the GODDESS PRO, a pediatric validation study (NCT04195399) is currently ongoing, the developers of the DTF-QoL are considering developing an age-specific questionnaire for children with DTF. 29,30 In this way, appropriate support and analgesic treatment can be provided. 29,31 For YAs who are in an important developmental stage of their lives, age-specific support is crucial.…”

“…There are currently two validated DTF‐specific HRQoL questionnaires available, both primarily developed for adults. For the GODDESS PRO, a pediatric validation study (NCT04195399) is currently ongoing, the developers of the DTF‐QoL are considering developing an age‐specific questionnaire for children with DTF 29,30 . In this way, appropriate support and analgesic treatment can be provided 29,31 .…”

Unraveling Desmoid-Type Fibromatosis-Specific Health-Related Quality of Life: Who Is at Risk for Poor Outcomes

“…The DTF-specific HRQoL was measured by the DTF-QoL [ 19 ]. The DTF-QoL was developed according to the guidelines of the EORTC Quality of Life Group to supplement the EORTC QLQ-C30 and to assess the disease-specific issues that DTF patients experience [ 19 , 23 ]. The questionnaire consists of 96 items, which are divided into 3 symptom scales, 11 disease impact scales, and 6 single items.…”

“…These DTF-specific HRQoL issues are not captured by generic or cancer-generic HRQoL questionnaires, such as the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC QLQ-C30), which are predominantly used in DTF studies and in clinical care [ 17 ]. Therefore, a DTF-specific HRQoL questionnaire, the DTF-QoL, was recently developed by our group, which can be used to evaluate the prevalence of HRQoL issues in DTF patients [ 18 , 19 ]. Furthermore, the small number of previous studies focused on the population of DTF patients as a whole because of small sample sizes.…”

Unraveling Desmoid-Type Fibromatosis-specific Health-Related Quality of Life: Who Is at Risk for Poor Outcomes

“…The DTF-specific HRQoL was measured by the DTF-QoL [19]. The DTF-QoL was developed according to the guidelines of the EORTC Quality of Life Group to supple-ment the EORTC QLQ-C30 and to assess the disease-specific issues that DTF patients experience [19,23].…”