Nic o nas bez nas? Zaangażowanie ruchu osób z niepełnosprawnościami w monitoring wdrażania Konwencji ONZ o prawach osób niepełnosprawnych w Polsce. Studium przypadku

Article 33 of the UN Convention on the Rights of Persons with Disabilities (ratified by Poland in 2012) establishes that civil society, in particular persons with disabilities and their representative organizations, shall be involved and participate fully in the monitoring process. Their real representation and power were a subject to scrutiny by the UN Committee on the Convention on the Rights of Persons with Disabilities (CRPD), which prepared a general comment to article 33. In this paper, I analyse the monitoring of the Convention in Poland and ways in which persons with disabilities and their representative organizations have been involved in the process. In Poland the Convention is monitored mainly by experts and big NGOs, not without reasons and consequences. Persons with disabilities who are subject to discrimination and multiple exclusion, and therefore are in a particularly bad situation, are rarely involved in the monitoring and advocacy.Artykuł 33 ratyfikowanej w 2012 r. przez Polskę Konwencji ONZ o prawach osób niepełnosprawnych (KPON) wskazuje konieczność prowadzenia działań monitorujących jej wdrażanie przez społeczeństwo obywatelskie, w tym w szczególności organizacje zrzeszające osoby z niepełnosprawnościami. Faktyczna reprezentacja i wpływ środowiska osób z niepełnosprawnościami w myśl zasady „nic o nas bez nas” jest przedmiotem szczególnego zainteresowania Komitetu ONZ o prawach osób niepełnosprawnych, który przygotował komentarz generalny do art. 33 poświęcony tej sprawie. W artykule przeanalizowany został sposób prowadzenia monitoringu KPON w Polsce i włączenie w ten proces osób z różnymi niepełnosprawnościami w kontekście wymagań określonych przez Komitet ONZ. W Polsce monitoring KPON prowadzony jest w dużej mierze przez duże organizacje pozarządowe i grono ekspertów, co ma swoje konsekwencje. Z działań monitoringowych i rzeczniczych praktycznie wykluczone są grupy osób z niepełnosprawnościami narażonych na wielokrotne wykluczenie i dyskryminację, a więc w szczególnie trudnej sytuacji

The Polish policy on disability in the perspective of cruel optimism. About the promises that have not been being fulfilled

W artykule przedstawione zostało zastosowanie perspektywy teoretycznej, jaką jest okrutny optymizm (cruel optimism), do analizy sytuacji osób z niepełnosprawnościami w edukacji i na rynku pracy w Polsce. Okrutny optymizm rozumiany jest jako zespół niespełnionych obietnic oferowanych i cenionych przez współczesne społeczeństwo konsumpcyjne. Obietnice te jednocześnie są wyznacznikiem statusu, a samo ich istnienie wiąże się ze społeczną presją dążenia do ich realizacji za wszelką cenę – również za cenę degradacji jednostki. Proponowana analiza dotyczy obietnic składanych osobom z niepełnosprawnościami w ramach różnych praktyk i rozwiązań na polach edukacji zawodowej oraz działań prozatrudnieniowych, jak również wskazuje przyczyny tych niepowodzeń. Wskazane zostały również możliwości unikania (lub redukowania) efektu okrutnego optymizmu w kształtowaniu polityki publicznej wobec osób z niepełnosprawnościami. W podsumowaniu opisane zostały postulaty badawcze oraz rekomendacje związane z zastosowaniem perspektywy okrutnego optymizmu w ewaluacji programów skierowanych do osób z niepełnosprawnościami.The article presents the application of a theoretical approach which is cruel optimism to analyze the situation of people with disabilities in education and the labor market in Poland. Cruel optimism is understood as a set of unfulfilled promises offered and valued by contemporary consumer society. These promises are an indicator of status, and their very existence is associated with social pressure to strive to achieve them at all costs – also at the price of the degradation of the individual. The proposed analysis examines promises made to persons with disabilities as part of various practices and solutions in the fields of vocational education and employment-related activities, as well as indicates the reasons for their failure. Possibilities of avoiding (or reducing) the effect of cruel optimism in shaping disability policy have also been indicated. The summary contains research postulates and recommendations related to the use of cruel optimism approach in the evaluation of programs addressed to people with disabilities.Publikacja dofinansowana ze środków Polskiego Towarzystwa Socjologiczneg

Effectiveness and safety of PCSK9 inhibitor therapy in patients with familial hypercholesterolemia within a therapeutic program in Poland: Preliminary multicenter data

Background: In Poland, treatment with proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors has become available free of charge in a therapeutic program. Assessed herein, is the efficacy and safety of alirocumab and evolocumab in patients with heterozygous familial hypercholesterolemia (FH).Methods: Data of 55 adult FH patients who participated in the program were analyzed upon meeting the criteria established by the Ministry of Health (low density lipoprotein cholesterol [LDL-C] above 160 mg/dL on max. tolerated statin dose and ezetimib). The efficacy of PCSK9 inhibitors in reducing LDL-C with drug administration every 2 weeks was assessed after 3 months and 1 year of therapy. A safety profile evaluation was performed at each visit. 48 patients completed the 3-month and 21 for the 1-year observation periods (34 patients treated with alirokumab and 14 with evolocumab).Results: The mean concentration of direct-measured LDL-C decreased from the initial level of 215.1 ± 74.5 mg/dL to 75.3 ± 64.1 mg/dL, i.e., by 65 ± 14% following 3 months of treatment. This effect was stable in 1-year observation (77.7 ± 72.8 mg/dL). Adverse effects were flu-like symptoms (13.0%), injection site reactions (11.1%), fatigue (5.6%) and musculoskeletal symptoms (5.6%). Seven patients failed to complete the 3-month treatment period due to side effects or non-compliance, and 1 patient failed to complete the 1-year treatment due to myalgia.Conclusions: This study confirmed high effectiveness of PCSK9 inhibitors in reducing LDL-C levels in patients with FH. Due to restrictive inclusion criteria with LDL-C threshold level > 160 mg/dL (> 4.1 mmol/L) required for participation in the therapeutic program, a relatively small number of FH patients were eligible for treatment

The Role of Eye Gaze in Security and Privacy Applications: Survey and Future HCI Research Directions

For the past 20 years, researchers have investigated the use of eye tracking in security applications. We present a holistic view on gaze-based security applications. In particular, we canvassed the literature and classify the utility of gaze in security applications into a) authentication, b) privacy protection, and c) gaze monitoring during security critical tasks. This allows us to chart several research directions, most importantly 1) conducting field studies of implicit and explicit gaze-based authentication due to recent advances in eye tracking, 2) research on gaze-based privacy protection and gaze monitoring in security critical tasks which are under-investigated yet very promising areas, and 3) understanding the privacy implications of pervasive eye tracking. We discuss the most promising opportunities and most pressing challenges of eye tracking for security that will shape research in gaze-based security applications for the next decade

Familial hypercholesterolaemia in children and adolescents from 48 countries: a cross-sectional study

Background Approximately 450 000 children are born with familial hypercholesterolaemia worldwide every year, yet only 2·1% of adults with familial hypercholesterolaemia were diagnosed before age 18 years via current diagnostic approaches, which are derived from observations in adults. We aimed to characterise children and adolescents with heterozygous familial hypercholesterolaemia (HeFH) and understand current approaches to the identification and management of familial hypercholesterolaemia to inform future public health strategies. Methods For this cross-sectional study, we assessed children and adolescents younger than 18 years with a clinical or genetic diagnosis of HeFH at the time of entry into the Familial Hypercholesterolaemia Studies Collaboration (FHSC) registry between Oct 1, 2015, and Jan 31, 2021. Data in the registry were collected from 55 regional or national registries in 48 countries. Diagnoses relying on self-reported history of familial hypercholesterolaemia and suspected secondary hypercholesterolaemia were excluded from the registry; people with untreated LDL cholesterol (LDL-C) of at least 13·0 mmol/L were excluded from this study. Data were assessed overall and by WHO region, World Bank country income status, age, diagnostic criteria, and index-case status. The main outcome of this study was to assess current identification and management of children and adolescents with familial hypercholesterolaemia. Findings Of 63 093 individuals in the FHSC registry, 11 848 (18·8%) were children or adolescents younger than 18 years with HeFH and were included in this study; 5756 (50·2%) of 11 476 included individuals were female and 5720 (49·8%) were male. Sex data were missing for 372 (3·1%) of 11 848 individuals. Median age at registry entry was 9·6 years (IQR 5·8–13·2). 10 099 (89·9%) of 11 235 included individuals had a final genetically confirmed diagnosis of familial hypercholesterolaemia and 1136 (10·1%) had a clinical diagnosis. Genetically confirmed diagnosis data or clinical diagnosis data were missing for 613 (5·2%) of 11 848 individuals. Genetic diagnosis was more common in children and adolescents from high-income countries (9427 [92·4%] of 10 202) than in children and adolescents from non-high-income countries (199 [48·0%] of 415). 3414 (31·6%) of 10 804 children or adolescents were index cases. Familial-hypercholesterolaemia-related physical signs, cardiovascular risk factors, and cardiovascular disease were uncommon, but were more common in non-high-income countries. 7557 (72·4%) of 10 428 included children or adolescents were not taking lipid-lowering medication (LLM) and had a median LDL-C of 5·00 mmol/L (IQR 4·05–6·08). Compared with genetic diagnosis, the use of unadapted clinical criteria intended for use in adults and reliant on more extreme phenotypes could result in 50–75% of children and adolescents with familial hypercholesterolaemia not being identified. Interpretation Clinical characteristics observed in adults with familial hypercholesterolaemia are uncommon in children and adolescents with familial hypercholesterolaemia, hence detection in this age group relies on measurement of LDL-C and genetic confirmation. Where genetic testing is unavailable, increased availability and use of LDL-C measurements in the first few years of life could help reduce the current gap between prevalence and detection, enabling increased use of combination LLM to reach recommended LDL-C targets early in life. Funding Pfizer, Amgen, Merck Sharp & Dohme, Sanofi–Aventis, Daiichi Sankyo, and Regeneron

Familial hypercholesterolaemia in children and adolescents from 48 countries: a cross-sectional study

Background: Approximately 450 000 children are born with familial hypercholesterolaemia worldwide every year, yet only 2·1% of adults with familial hypercholesterolaemia were diagnosed before age 18 years via current diagnostic approaches, which are derived from observations in adults. We aimed to characterise children and adolescents with heterozygous familial hypercholesterolaemia (HeFH) and understand current approaches to the identification and management of familial hypercholesterolaemia to inform future public health strategies. Methods: For this cross-sectional study, we assessed children and adolescents younger than 18 years with a clinical or genetic diagnosis of HeFH at the time of entry into the Familial Hypercholesterolaemia Studies Collaboration (FHSC) registry between Oct 1, 2015, and Jan 31, 2021. Data in the registry were collected from 55 regional or national registries in 48 countries. Diagnoses relying on self-reported history of familial hypercholesterolaemia and suspected secondary hypercholesterolaemia were excluded from the registry; people with untreated LDL cholesterol (LDL-C) of at least 13·0 mmol/L were excluded from this study. Data were assessed overall and by WHO region, World Bank country income status, age, diagnostic criteria, and index-case status. The main outcome of this study was to assess current identification and management of children and adolescents with familial hypercholesterolaemia. Findings: Of 63 093 individuals in the FHSC registry, 11 848 (18·8%) were children or adolescents younger than 18 years with HeFH and were included in this study; 5756 (50·2%) of 11 476 included individuals were female and 5720 (49·8%) were male. Sex data were missing for 372 (3·1%) of 11 848 individuals. Median age at registry entry was 9·6 years (IQR 5·8-13·2). 10 099 (89·9%) of 11 235 included individuals had a final genetically confirmed diagnosis of familial hypercholesterolaemia and 1136 (10·1%) had a clinical diagnosis. Genetically confirmed diagnosis data or clinical diagnosis data were missing for 613 (5·2%) of 11 848 individuals. Genetic diagnosis was more common in children and adolescents from high-income countries (9427 [92·4%] of 10 202) than in children and adolescents from non-high-income countries (199 [48·0%] of 415). 3414 (31·6%) of 10 804 children or adolescents were index cases. Familial-hypercholesterolaemia-related physical signs, cardiovascular risk factors, and cardiovascular disease were uncommon, but were more common in non-high-income countries. 7557 (72·4%) of 10 428 included children or adolescents were not taking lipid-lowering medication (LLM) and had a median LDL-C of 5·00 mmol/L (IQR 4·05-6·08). Compared with genetic diagnosis, the use of unadapted clinical criteria intended for use in adults and reliant on more extreme phenotypes could result in 50-75% of children and adolescents with familial hypercholesterolaemia not being identified. Interpretation: Clinical characteristics observed in adults with familial hypercholesterolaemia are uncommon in children and adolescents with familial hypercholesterolaemia, hence detection in this age group relies on measurement of LDL-C and genetic confirmation. Where genetic testing is unavailable, increased availability and use of LDL-C measurements in the first few years of life could help reduce the current gap between prevalence and detection, enabling increased use of combination LLM to reach recommended LDL-C targets early in life

The Situation of Children with Disabilities During the Covid-19 Pandemic – an Intersectional Analysis

Głównym celem artykułu jest pokazanie specyfiki sytuacji dzieci z niepełnosprawnościami w czasie pandemii COVID-19. Na podstawie przeglądu piśmiennictwa opisano problemy, na które napotyka ta grupa, w tym związane z edukacją zdalną oraz dostępem do usług wspierających rozwój i codzienne funkcjonowanie, a także wpływem pandemii na zdrowie psychiczne. W artykule odniesiono się również do tego, czy i w jakim zakresie rząd zauważył oraz zaadresował te problemy w swoich działaniach będących odpowiedzią na wywołany pandemią kryzys, i oceniono, że niezastosowanie podejścia intersekcjonalnego sprawiło, że były one niewystarczające.The main aim of this article is to show the specificity of the situation of children with disabilities during the COVID-19 pandemic. Based on a review of the literature, the problems encountered by this group have been described, including those related to remote education and access to development and day-to-day services, as well as the impact of the pandemic on mental health. The article also addresses whether and to what extent the government identified and addressed these issues in its response to the pandemic crisis, and assessed that failure to follow an intersectional approach made them insufficient