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Objectives: The MetabQoL 1.0 is the first disease-specific health related quality of life (HrQoL) questionnaire for patients with intoxication-type inherited metabolic disorders. Our aim was to assess the validity and reliability of the MetabQoL 1.0, and to investigate neuropsychiatric burden in our patient population. Methods: Data from 29 patients followed at a single center, aged between 8 and 18 years with the diagnosis of methylmalonic acidemia (MMA), propionic acidemia (PA) or isovaleric acidemia (IVA), and their parents were included. The Pediatric Quality of Life Inventory (PedsQoL) was used to evaluate the validity and reliability of MetabQoL 1.0.
Results: The MetabQoL 1.0 was shown to be valid and reliable (Cronbach's alpha: 0.64–0.9). Fourteen out of the 22 patients (63.6%) formally evaluated had neurological findings. Of note, 17 out of 20 patients (85%) had a psychiatric disorder when evaluated formally by a child and adolescent psychiatrist. The median mental scores of the MetabQoL 1.0 proxy report were significantly higher than those of the self report (p = 0.023). Patients with neonatal-onset disease had higher MetabQoL 1.0 proxy physical (p = 0.008), mental (p = 0.042), total scores (p = 0.022); and self report social (p = 0.007) and total scores (p = 0.043) than those with later onset disease.
Conclusions: This study continues to prove that the MetabQoL 1.0 is an effective tool to measure what matters in intoxication-type inherited metabolic disorders. Our results highlight the importance of clinical assessment complemented by patient reported outcomes which further expands the evaluation toolbox of inherited metabolic diseases.
Organic acidurias (OAs), urea-cycle disorders (UCDs), and maple syrup urine disease (MSUD) belong to the category of intoxication-type inborn errors of metabolism (IT-IEM). Liver transplantation (LTx) is increasingly utilized in IT-IEM. However, its impact has been mainly focused on clinical outcome measures and rarely on health-related quality of life (HRQoL). Aim of the study was to investigate the impact of LTx on HrQoL in IT-IEMs. This single center prospective study involved 32 patients (15 OA, 11 UCD, 6 MSUD; median age at LTx 3.0 years, range 0.8–26.0). HRQoL was assessed pre/post transplantation by PedsQL-General Module 4.0 and by MetabQoL 1.0, a specifically designed tool for IT-IEM. PedsQL highlighted significant post-LTx improvements in total and physical functioning in both patients' and parents' scores. According to age at transplantation (≤3 vs. >3 years), younger patients showed higher post-LTx scores on Physical (p = 0.03), Social (p < 0.001), and Total (p =0.007) functioning. MetabQoL confirmed significant post-LTx changes in Total and Physical functioning in both patients and parents scores (p ≤ 0.009). Differently from PedsQL, MetabQoL Mental (patients p = 0.013, parents p = 0.03) and Social scores (patients p = 0.02, parents p = 0.012) were significantly higher post-LTx. Significant improvements (p = 0.001–0.04) were also detected both in self- and proxy-reports for almost all MetabQoL subscales. This study shows the importance of assessing the impact of transplantation on HrQoL, a meaningful outcome reflecting patients' wellbeing. LTx is associated with significant improvements of HrQol in both self- and parentreports. The comparison between PedsQL-GM and MetabQoL highlighted that MetabQoL demonstrated higher sensitivity in the assessment of diseasespecific domains than the generic PedsQL tool.
Issues with professional conduct and discrimination against Lesbian, Gay, Bisexual, Transgender (LGBT+) people in health and social care, continue to exist in most EU countries and worldwide.
The project IENE9 titled: “Developing a culturally competent and compassionate LGBT+ curriculum in health and social care education” aims to enable teacher/trainers of theory and practice to enhance their skills regarding LGBT+ issues and develop teaching tools to support the inclusion of LGBT+ issues within health and social care curricula. The newly culturally competent and compassionate LGBT+ curriculum will be delivered though a Massive Open Online Course (MOOC) which is aimed at health and social care workers, professionals and learners across Europe and worldwide.
We have identified educational policies and guidelines at institutions teaching in health and social care, taken into account for developing the learning/teaching resources. The MOOC will be an innovative training model based on the Papadopoulos (2014) model for “Culturally Competent Compassion”. The module provides a logical and easy to follow structure based on its four constructs 'Culturally Aware and Compassionate Learning', 'Culturally Knowledgeable and Compassionate Learning', 'Culturally Sensitive and Compassionate Learning', 'Culturally Competent and Compassionate Learning'.
Specific training may result in better knowledge and skills of the health and social care workforce, which helps to reduce inequalities and communication with LGBT+ people, as well as diminishing the feelings of stigma or discrimination experienced.
This chapter is about school suspension through a social work lens. Young people like Martin require the collective to belong, to be a member of a group, to realise their social needs. This is the basic requirement of human mental and social stability. Suspension stands in opposition because it legitimises social exclusion and disregards the linkage between the individual and collective (Bunge 2003). This chapter advocates for a whole systems approach to tackle social problems and develop sustainable interventions that facilitate young peoples’ needs realisation at school.