PedsQL Neurofibromatosis Module: Brazilian Portuguese Translation & Adaptation

A new study has focused on ensuring the accurate and culturally relevant use of a quality-of-life questionnaire for individuals living with neurofibromatosis type 1 (NF1) in Brazil. Researchers successfully translated and adapted the PedsQL Neurofibromatosis module 3.0, a tool designed to assess the impact of this genetic condition on patients aged 5 to 25 and their families.

Understanding Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF1) is a relatively common genetic disorder, affecting approximately 1 in 3,000 births. It’s characterized by the growth of tumors along nerves throughout the body. While not typically life-threatening, NF1 can cause a wide range of health problems, impacting physical appearance, learning abilities, and overall quality of life. Currently, there is no cure for NF1, meaning management focuses on addressing specific symptoms and complications as they arise.

The Importance of Quality-of-Life Assessment

Because the clinical presentation of NF1 varies significantly from person to person, and because there is no medical cure, understanding a patient’s quality of life is crucial for providing holistic care. Factors like pain, fatigue, social stigma, and psychological distress can significantly affect well-being. Standardized questionnaires, like the PedsQL, allow healthcare professionals to systematically measure these aspects and track changes over time, informing treatment decisions and support services.

Adapting the PedsQL for Brazilian Patients

The PedsQL (Pediatric Quality of Life Inventory) is a widely used modular system for measuring health-related quality of life in children and adolescents. It consists of core scales applicable to all pediatric populations, as well as disease-specific modules designed for conditions like NF1. However, simply translating a questionnaire isn’t enough. Cultural adaptation is essential to ensure that the questions are understood and interpreted in the same way by patients from different backgrounds.

The process undertaken by researchers at the Universidade Federal de São Paulo (UNIFESP) involved a rigorous four-phase approach. First, the original English version of the PedsQL Neurofibromatosis module 3.0 was translated into Brazilian Portuguese. This was followed by a “back-translation” – translating the Portuguese version back into English by a different translator – to identify any discrepancies in meaning. Next, a panel of 15 medical professionals, including doctors and nurses from various regions of Brazil, reviewed the translated questionnaire for clarity and relevance. Finally, the questionnaire was pre-tested with both patients (42 individuals aged 5-25) and their family members (46 individuals aged 28-54, with an average age of 40.6 years) diagnosed with NF1.

Ensuring Accuracy and Cultural Sensitivity

The researchers used established metrics, including the Content Validity Index (CVI) and Kappa statistic, to assess the quality of the translation and adaptation. The results demonstrated adequate levels of agreement among the experts, with a CVI of 0.75 and a Kappa of 0.6. The pre-testing phase revealed the need for minor adjustments to enhance understanding, particularly in the family questionnaire. For example, the word “temer” (to fear) was replaced with “tem medo” (is afraid) to improve clarity.

The Significance of This Work

This successful translation and cultural adaptation of the PedsQL Neurofibromatosis module 3.0 into Brazilian Portuguese provides a valuable tool for clinicians and researchers working with individuals with NF1 in Brazil. It allows for a standardized and culturally sensitive assessment of quality of life, which can inform personalized care plans and improve outcomes. The availability of a validated Portuguese version also facilitates participation in international research studies, contributing to a broader understanding of NF1 and its impact on patients worldwide.

According to the study, the process highlights the importance of a systematic and collaborative approach to cross-cultural adaptation of health-related quality of life instruments. The researchers emphasize that ensuring linguistic and cultural equivalence is crucial for obtaining reliable and meaningful data that can truly reflect the experiences of patients from diverse populations.

The PedsQL scales have been translated into multiple languages, but linguistic validation is a necessary step to ensure accuracy. The Mapi Research Institute, in collaboration with Dr. James W. Varni, who developed the PedsQL measurement model, oversees the translation process and maintains copyright over new translations. Funding is often required to support the linguistic validation process, and companies sponsoring translations may receive exemptions from access right costs for specific studies.