Rock-a-Bye Baby EI Services for the youngest, smallest and most vulnerable infants Children’s of Alabama Sheree York, PT,DPT,PCS Chantel Jno-Finn, PT, DPT Lauren Lovett, PT, DPT Christy Moran, OTR/L Special Guests Anna Ruth McCalley, MS,OTR/L, Mom & Frances Objectives • Recognize the eligible diagnoses for premature and medically complex infants • Recognize the risks associated with complex medical conditions • Select and complete appropriate evaluations of these infants • Provide family-centered EI services designed to support the families and promote the development and care of these infants AEIS Qualifying Medical Diagnoses/Conditions • Achondroplasia • Brain injury/degeneration • Agenesis of Corpus Callosum • Brain malformation/abnormality • Agyria (Lissencephaly syndrome (Miller-Dieker – Macroencephaly syndrome)) – Macrogyria • Albinism – Megalencephaly • Amniotic Band syndrome – Microcephaly • Anencephaly – Microgyria • Angelman’s syndrome • Cataracts • Anophthalmia • Cerebral Palsy (all types) • Apert syndrome • CHARGE syndrome • Aplasia of the brain (brain malformation/abnormality) • Chiari Malformation • Arnold-Chiari syndrome • Childhood Depression • Arthrogryposis • Childhood Disintegrative disorder • Asperger syndrome/disorder • Coloboma • Asphyxiating Thoracic Dystrophy (Jeune syndrome) • Cone Dystrophy • Attachment disorder • Connexin 26 • Auditory Neuropathy • Cornelia de Lange syndrome • Autism/Autism Spectrum disorder • Cortical vision impairment (vision loss/impairment) • Bardet-Biedl syndrome • Cri-du-Chat syndrome • Beckwith-Wiedemann Syndrome • Cytomegalovirus (CMV) • Dandy Walker syndrome/variant • De Morsier syndrome (Septo-Optic Dysplasia) AEIS Qualifying Medical Diagnoses/Conditions • Developmental Apraxia • Hydrocephaly (with or without shunt; congenital or acquired) • DeWayne Syndrome • Hypoplastic Left Heart Syndrome • DiGeorge syndrome • Incontinentia Pigmenti • Dilantin syndrome (Fetal Hydantoin syndrome) • Infantile spasms (seizure disorder) • Down Syndrome (Trisomy 21) • Intraventricular Hemorrhage (IVH) Grade III or IV • Edwards syndrome (Trisomy 18) • Klinefelter’s syndrome • Encephalomalacia • Landau-Kleffner syndrome • Encephalopathy • Lead (elevated blood levels) • Epilepsy (seizure disorder) • Leber Congenital Amaurosis (vision loss/impairment) • Esotropia • Leukodystrophy • Exotropia • Meningitis • Fetal Alcohol syndrome • Menkes syndrome • Fragile X syndrome • Mercury Poisoning • Genetic/Chromosomal malformation/abnormality (not listed) • Microphthalmia • Glaucoma • Microtia • Glue Ear • Mobius syndrome • Hearing Loss/Impairment • Mucopolysaccharidosis • Heart Disease/Defect (not listed) • Muscular Dystrophy • Hemiplegia • Myasthenia syndrome-congenital • Herpes Simplex Virus (HSV) • Myopathy • Holoprosencephaly (Arhinencephaly) • Neurological Disease/Defect (not listed) • Holt Oram syndrome • Neurofibromatosis • Hunter Syndrome • Noonan’s syndrome • Hurler Syndrome • Nystagmus • Hydranencephaly • Opitz G/BBB syndrome • Optic nerve hypoplasia or atrophy AEIS Qualifying Medical Diagnoses/Conditions • Osteogenisis Imperfecta • Seizure disorder/uncontrolled or poorly controlled seizures • Other (not listed) • Septo-Optic dysplasia (De Morsier syndrome) • Pallister-Killian syndrome • Shaken Baby syndrome • Patau syndrome (Trisomy 13) • Smith-Lemli-Opitz syndrome • Pediatric Human Immunodeficiency Virus (HIV)/Acquired • Spina Bifida (Myelomeningocele/Meningomyelocele) Immune Deficiency Syndrome (AIDS) • Spinal Muscular Atrophy (SMA) • Periventricular Leukomalacia (PVL) • Spinocerebellar Ataxia • Pervasive Developmental disorder (PDD and PDD-NOS) • Stickler syndrome • Peters Anomaly • Srabismus • Phelan McDermis syndrome • Stroke, Prenatal or Neonatal • Phiffer syndrome • Sturge Webber syndrome • Porencephalic Cyst • Tay-Sachs Disease • Prader-Willi syndrome • Traumatic Brain Injury (TBI) • Prematurity (26 weeks or less gestation and/or 1000 grams or • Traumatic Retinal Detachment less and 18 months chronological age or younger) • Treacher-Collins syndrome • Prune Belly Syndrome (Eagle –Barrett Syndrome) • Tuberous Sclerosis • Reactive Attachment disorder (RAD) • Turner syndrome • Retinoblastoma • Usher Syndrome • Retinopathy of Prematurity (ROP) • VACTERL/VATER syndrome • Rett syndrome • Ventriculomegaly • Roberts syndrome (Pseudothalidomide syndrome) • Vision Loss/Impairment • Rubella-congenital • Waardenburg-Klein Syndrome • Rubenstein-Taybi syndrome • Weaver syndrome • Schizencephaly • Williams syndrome What can we do? Vermont Oxford Network Manifesto • Family-centered • Habits in daily practice • Safe – Evidence based practice • Effective – Change – Systems thinking • Timely – Collaborative learning • Socially and • Accountability environmentally responsible • Respect for team members and family Functional Brain Plasticity • Functional Plasticity – Resilience and physiological response to stress • Promoting change while promoting stability – Stress • Positive stress can strengthen • Toxic stress weakens • Tolerable stress – Modulated by supportive caregiving Evidence: Early Experiences on Brain Development 5-20 • Safe and Secure Environments • Nutrition • Stable and Responsive Relationships • Behavior • Promoting Developmental and Family-Centered Care Physicians, CRNPs, Nursing PT, OT,SLP, RT Chaplains, Dieticians Social Services, Pharmacy, local programs Discharge planners – It Takes a Team Service Coordinators for best outcomes The Premature Infant Automatic Eligibility • Prematurity – 26 weeks or less gestation – 1000 grams or less – 18 months chronological age or younger Adjusted Age for Premature Infants • Through 18 months of life • Chronological age – weeks premature= adjusted age • Infant born at 26 weeks gestation was 14 weeks early • At 10 months: 10-3.5= 6.5 months Biological Risk Factors for Developmental Delay • Prematurity • Low birth weight • Neurological damage • Chronic illness • Genetic disease • Seizures • Malnutrition • Alcohol/drug use Outcomes in Infants Born Prematurely or Requiring NICU1 • Susceptible to respiratory tract infections and asthma • GI problems: – Reflux common – Difficulty coordinating suck-swallow-breathe – May need special formula and strategies for introducing solid food to ensure nutrition and growth • Difficulty with state control and self-regulation – Irritability or sleeping “all the time” – Easily stressed • Yawning, hiccupping, sneezing, gaze aversion Outcomes in Infants Born Prematurely or Requiring NICU1 • Severe levels of impairment are generally apparent by 2 years of age • Mild-moderate disabilities may not be apparent until preschool or school-age, even adolescence – DCD – ADHD – Cognitive delays – Behavioral/social issues Neurodevelopmental Outcomes of Prematurity1 • Premature birth is potentially disruptive to multiple aspects of neurodevelopment, especially with: – Intraventricular hemorrhage – Infection – Chronic lung disease Neurodevelopmental Outcomes of Prematurity1 • Aspects of NICU care that improve survival but may worsen long-term neurological outcomes – Mechanical ventilation – Supplemental oxygen – Bright lights – Noisy alarms – Painful procedures – Medications Risk of Neurodevelopmental Problems1 • Risk of neurodevelopmental problems proportional with gestational age and birth weight • Chance for abnormal development increases with lower gestational age and smaller birth weight Neurodevelopmental Impairments: Extremely Premature (<26 weeks gestation) • Rates high • Rates inversely related to gestational age 22-25 weeks – Death and disability at 22 weeks approaches 100% – Moderate to severe disability at 22-23 weeks >50% – Rates of disability decrease at 24-25 weeks – Rates of bilateral deafness and blindness increase with decreasing gestational age • Potential for recovery with increasing age when in good environments with access to intervention and support services 2010 Systematic Review2 • In 11 studies published after 1990 reporting the prevalence of motor impairment in children born preterm (<37 weeks gestation) – 3-4 times > general population – Mild-moderate impairment 40.5/100 – Moderate impairment 19/100 Optimizing Neurodevelopmental Outcomes after Prematurity • Best medical practices • Teaming • Developmental follow-up • Family support • Early intervention or nurse home-visits: focus on improving parenting through daily routines, relationship-based activities, reinforce comfort, create safe experiences, and address stressors Congenital Heart Disease Davis • Normal deliver at 39 WGA • Developed fever and poor feeding at day 3 and transferred from OSH for abnormal Echocardiogram • Berlin Ventricular Assistive Device (VAD) placement • Head US showed multiple focal hemmorhages • Heart transplant at 5 months requiring multiple bypass runs and Extracorporeal Membrane Oxygenation (ECMO) x2 days • Post-pump chorea • G-tube placement Commonly Seen Diagnoses • Tetrology of Fallot (TOF) • Transposition of the • Atrial Septal Defect (ASD) Great Arteries (d-TGA) • Ventricular Septal Defect • Patent Ductus (VSD) Arteriosus (PDA) • Interrupted Aortic Arch (IAA) • Cardiomyopathy • Hypoplastic Left Heart • Atrioventricular Septal Syndrome (HLHS) Defect (AV Canal) • Coarctation of the Aorta • Truncus Arteriosis CHD and the Brain • With severe defects (i.e., single ventricle, d-tga) there are often brain abnormalities before surgery – There is increasing evidence of impaired neurodevelopment in utero – Smaller head circumference and decreased brain maturation • Acquired brain injuries related to hypoxia, prolonged bypass, cardiac