Kim de Verdier, WBU/ICEVI Orlando 2016 Children with blindness in Sweden: characteristics of the population Preliminary data from a national register study Kim de Verdier Lic. psychologist, National Agency for Special Needs Education and Schools/Resource centre Vision Ph.D. student, Stockholm University/Department of Special Education WBU/ICEVI Orlando 2016 Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Background and aim Most Swedish students with blindness attend inclusive education Knowledge about the population is important when planning and shaping the support Reliable statistics have become increasingly difficult to retrieve The aim was to describe the population regarding specific background variables Kim de Verdier, WBU/ICEVI Orlando 2016

Ph.D. project in collaboration with research team Kim de Verdier, Lic. psychologist, Ph. D. student, National Agency for Special Needs Education and Schools, and Stockholm University Ulla Ek, Lic. psychologist, professor of special education, Stockholm University Elisabeth Fernell, MD, PhD, Gillberg Neuropsyciatry Centre, Gothenburg University Stefan Löfgren, MD, associate professor, St Erik Eye Hospital, Stockholm Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Inclusion criteria Birth years between 1988 – 2008 Pre/perinatal or early infancy blindness category 4 – 5 (WHO), i.e. total blindness or light perception Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Search procedure Data collection during 2014 – 2015 Medical, psychological and pedagogical files and records were reterieved from The National Agency’s two national resource centre units, and central archives Phase 1: Files of every child with VI born 1988 – 2008 were examnined Phase 2: An in-depth search of the files of all children who matched the inclusion criteria was performed Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Collected data Year of birth Gender Cause of blindness Gestational age Associated neurological disorders Associated neurodevelopmental impairments Cognitive level Type of school placement Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Preliminary results Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Overview n=150 (M=7 per year) Gender distribution Boys 47% (n=70) Girls 53% (n=80) 28% (n=42) were born preterm (GA <37 weeks) The majority of these were born extremely preterm (GA <28 weeks) GA M=26 (23 – 35) Birthweight M=782 g (522 – 1300) Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Causes of blindness When? Prenatal Peri- or postnatal Where? Ante-chiasmal damage Retro-chiasmal damage Kim de Verdier, WBU/ICEVI Orlando 2016

Causes of blindness represented in the studied cohort   Girls (n=80) Boys (n=70) Total (n=150) Prenatal antechiasmal causes (n=93) Optic nerve hypoplasia (ONH) 9 14 23 Leber congenital amaurosis (LCA) 13 22 Optic nerve atrophy (ONA) 8 16 Microphtalmia 5 4 Anophtalmia 6 1 7 Congenital glaucoma 2 Retinal dystrophy Coloboma Incontinentia pigmenti Norrie disease Persistent hyperplastic primary vitreous (PHPV) Sclerocornea Unspecified eye malformations 3 Prenatal retrochiasmal causes (n=10) Various cerebral malformations 10 Peri-/postnatal antechiasmal causes (n=42) Retinopathy of prematurity (ROP) 36 Retinoblastoma Congenital cataract Optic glioma Peri-/postnatal retrochiasmal causes (n=2) Cerebral damage due to early trauma or disease Cause not defined whether pre/peri- or postnatal (n=3) Unspecified eye disease (antechiasmal) Unspecified tumour (retrochiasmal) Kim de Verdier, WBU/ICEVI Orlando 2016

Causes of blindness that dominated the statistics   Girls (n=80) Boys (n=70) Total (n=150) Prenatal antechiasmal causes (n=93) Optic nerve hypoplasia (ONH) 9 14 23 Leber congenital amaurosis (LCA) 13 22 Optic nerve atrophy (ONA) 8 16 Microphtalmia 5 4 Anophtalmia 6 1 7 Congenital glaucoma 2 Retinal dystrophy Coloboma Incontinentia pigmenti Norrie disease Persistent hyperplastic primary vitreous (PHPV) Sclerocornea Unspecified eye malformations 3 Prenatal retrochiasmal causes (n=10) Various cerebral malformations 10 Peri-/postnatal antechiasmal causes (n=42) Retinopathy of prematurity (ROP) 36 Retinoblastoma Congenital cataract Optic glioma Peri-/postnatal retrochiasmal causes (n=2) Cerebral damage due to early trauma or disease Cause not defined whether pre/peri- or postnatal (n=3) Unspecified eye disease (antechiasmal) Unspecified tumour (retrochiasmal) Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Causes that dominated the statistics, with associated developmental disorders   Boys / Girls Cerebral involvement * Epilepsy Intellectual Disability Autism Spectrum Disorder Motor impairment Hearing impairment ROP n=36 (24%) 22 /14 (61% / 39%) 19 (53%) 4 (11%) 23 (64%) 21 (58%) 7 (19%) 2 (6%) ONH n=23 (15%) 14 / 9 2 (9%) 10 (43%) 16 (70%) LCA n=22 13 / 9 (59% / 41%) 5 (23%) 8 (36%) ONA n=16 (11%) 8 / 8 (50% / 50%) 14 (88%) 9 (56%) 10 (63%) 2 (13%) 3 (19%) *i.e. hydrocephalus, microcephalus, periventricular leukomalacia (PVL) and unspecified cerebral involvement. Kim de Verdier, WBU/ICEVI Orlando 2016

Cerebral involvement and additional impairmants in the population Diagnosed cerebral involvement 33% (n=50) Epilepsy 18% (n=27) Only blindness 22% (n=33) Additional impairments 72% (n=108) Intellectual disability (ID) Autism Spectrum disorder (ASD) Motor impairment Attention deficit/hyperactivity disorder (ADHD) Hearing impairment 54% (n=81) had more than one additional impairment ASD + ID ID + motor impairment Unknown 6% (n=9) Kim de Verdier, WBU/ICEVI Orlando 2016

Blindness and autism spectrum disorder - a closer look Kim de Verdier, WBU/ICEVI Orlando 2016

Previous research – prevalence of autism in children with blindness 1/3 of all children with blindness, regardless of etiology (Fraiberg, 1964; Cass et al, 1994; Hobson et al,1997) Nearly 2/3 of all prematurly born children with blindness (Ek et al, 1998) Autism in specific etiological groups ROP (Ek et al, 1998) ONH (Ek et al, 2005; Parr et al, 2010) LCA??? (Rogers et al,1989; Fazzi et al, 2007) Kim de Verdier, WBU/ICEVI Orlando 2016

Prevalence of ASD in the present study 31% (n=47) met the criteria for autism/Asperger/PDD-NOS 38% (n=57) if prominent autistic features were also included in the ASD-spectrum 63% (36/57) also had ID Dominating causes of blindness in the ASD-group: ROP 37 % (21/57) ONH/SOD 28 % (16/57) LCA 14 % (8/57) Kim de Verdier, WBU/ICEVI Orlando 2016

Prevalence of ASD in specific aetiological subgroups In the ONH-subgroup 70% had ASD In the ROP-subgroup 58% had ASD In the LCA-subgroup 36% had ASD Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 School placement 50% attended special schools All of these had additional impairments (ID, ASD, motor or hearing impairments) 39% attended inclusive educational settings Some of these had blindness only, but several had additional difficulties (mainly ASD, ADHD, motor or hearing impairments) 11% unknown school placement Kim de Verdier, WBU/ICEVI Orlando 2016

Summary and conclusions Four causes of blindness dominated, representing 65% of the studied population In the last decades isolated blindness is uncommon in children Children with blindness constitute a heterogeneous group Nearly 3/4 had multi-impairments 1/3 had ASD, even more if prominent autistic features was included Blindness in combination with additional impairments brings many challenges for the child’s development and learning (the ASD-group!) These children show up in inclusive educational settinngs as well as in specialized schools – this places great demands on the teachers’ competence Further development of support should be based on the considerable heterogeneity of the population Kim de Verdier, WBU/ICEVI Orlando 2016

Kim de Verdier, WBU/ICEVI Orlando 2016 Ongoing research Interview study focusing on children with blindness and ASD, and experiences of support to families and schools Kim de Verdier, WBU/ICEVI Orlando 2016

Thank you! Contact: kim.deverdier@spsm.se Kim de Verdier, WBU/ICEVI Orlando 2016