Measuring functional outcomes after prematurity: Developmental impact of very low birth weight and extremely low birth weight status on childhood disability

MEASURING FUNCTIONAL OUTCOMES AFTERPREMATURITY: DEVELOPMENTAL IMPACT OF VERY

LOW BIRTH WEIGHT AND EXTREMELYLOW BIRTH WEIGHT STATUSON CHILDHOOD DISABILITY

Michael E. Msall* and Michelle R. TremontChild Development Center, Hasbro Children’s and Rhode Island Hospitals

Brown Medical School, Providence, Rhode Island

Our purpose was to describe functional outcomes in essential activ-ities in preschool, school-age, and adolescent children who were born very(�32 weeks gestation) and extremely (�28 weeks gestation) prematurely.Very low birth weight (VLBW; 1000–1499 g), or extremely low birth weight(ELBW;�1000 g) populations are the focus of our analysis. We describemodels of disablement and enablement for specifying the complexity ofchildhood outcomes using a framework of pathophysiology, impairment,functional limitation and functional strengths, disability in social roles andsocial participation, societal limitations and environmental facilitators. Rep-resentative early childhood, preschool, school-age, and adolescent studieswere examined in terms of describing children’s functional strengths andchallenges after VLBW and ELBW survival. In early childhood, disability wasassessed by diagnosing neurosensory impairments and delays on develop-mental testing. Instruments for measuring functional status in essentialactivities of self-care, mobility, communication and learning are described.Rates of neurosensory disability in the first three years among recent ELBWsurvivors ranged from 9–26% for cerebral palsy, 1–15% for blindness,0–9% for deafness, and 6–42% for evolving cognitive disability (MDI�70). Rates of preschool functional limitation were 5–27% motor, 5–30%self-care, and 5–22% communicative. Rates of school-age functional edu-cational disabilities exceeded 50%. Rates of adolescent activity limitationwere 13–32% and vocational limitations were 27–71%. By examining thefunctional strengths and challenges of children with major neurodevelop-mental impairments after very or extremely preterm birth, we can examinecausal pathways that lessen the risk of severe functional disability. Amongchildren with mild to moderate disability, we can enhance functional out-comes, optimize community participation, and provide quality family sup-ports. In order to assess the changing outcomes of this vulnerable popula-tion of survivors, combinations of clinical and survey based methodologiesare required. © 2002 Wiley-Liss, Inc.MRDD Research Reviews 2002;8:258–272.

Key Words: very low birth weight; extremely low birth weight; neurode-velopmental impairments; functional outcomes; childhood disability; cere-bral palsy; mental retardation; blindness; deafness; survey methods

INTRODUCTION

In the 1990s, over 90% of very low birth weight and verypreterm (VLBW; i.e., 1000–1499 g, 28–32 weeks gestation)infants survived and over 80% of extremely low birth weight

and extremely preterm (ELBW; i.e., �1000 g, �28 weeks

gestation) infants survived [Rennie, 1996; Lorenz et al., 1998;Stevenson et al., 1998; Lemons et al., 2001]. Lorenz highlightedthat amongst inborn United States cohorts, survival at 24 weekswas 33–57% and at 25 weeks was 60–75% [Lorenz, 2000]. Inaddition, more than 50% survival is currently anticipated forinfants at 24–25 weeks gestation [El-Metawally et al., 2000].Neonatal advances contributing to this unprecedented survivalhave included regionalization of neonatal transport with empha-sis on maternal transport, the use of maternal corticosteroids,aggressive delivery room management, surfactant replacementtherapies, use of indomethacin for prophylaxis of Patent DuctusArteriosis (PDA), prevention of severe intraventricular hemor-rhage (IVH), and improved ventilatory, nutrition, and infectiousdisease management practices. Very low birth weight, extremelylow birth weight, and micropremature infants (i.e., birth weights�750 g) are at a great risk for major neurodevelopmentalimpairments defined as cerebral palsy, blindness, deafness, andsevere cognitive developmental disabilities (Bayley MDI or In-telligence Quotient/Standard Score i.e., IQ/SS �50–55) [Vohrand Msall, 1997; Hack and Fanaroff, 1999]. In addition, highrates of disorders of communication, perception, attention, cog-nition (IQ/SS 55-69 and IQ/SS 70–84), and learning disordersincrease the risk for grade repetition, special education resources,and academic underachievement [McCormick et al., 1992; Kle-banov et al., 1994; Bennett and Scott, 1997; Leonard andPiecuch, 1997; Bregman, 1998; Taylor et al., 1998; Buck et al.,2000].

Grant sponsors: Maternal Child Health Bureau and NICHD/NCMRR; Grant num-bers: 5T77 MC00016-05 and UO1 HD 37614.*Correspondence to: Michael E. Msall, Director, Child Development Center, APC-6,Rhode Island Hospital, 593 Eddy Street, Providence, RI 02903.E-mail: [email protected] 1 July 2002; Accepted 9 September 2002Published online in Wiley InterScience (www.interscience.wiley.com).DOI: 10.1002/mrdd.10046

MENTAL RETARDATION AND DEVELOPMENTAL DISABILITIESRESEARCH REVIEWS 8: 258–272 (2002)

© 2002 Wiley-Liss, Inc.

On a population basis, there hasbeen a lack of uniformity in describingthe range of disability outcomes amongsuccessive cohorts of very low and ex-tremely low birth weight survivors ex-amining major developmental periods,and impact on medical, developmental,educational, and family support services[McCormick, 1989; Msall et al., 1998].Once these core functional skills are mas-tered, additional tools are needed to assessacademic skills in reading, mathematics,and written language as well as the neu-ropsychological skills used in every daylife for problem solving, judgment,memory and social interaction. In ado-lescents, measures are required for assess-ing neuropsychological and behavioralcompetencies, community participation,instrumental activities of daily living, andvocational skills.

The purpose of our review is toexamine the functional outcomes broadlydefined of childhood survivors of verylow and extremely low birth weight sta-tus. We will focus on categorization ofneurodevelopmental impairments in thefirst three years of life as proxies of motorand developmental limitations, func-tional outcomes in self-care, mobility,and communication at the transition be-tween preschool and elementary school,including kindergarten readiness, aca-demic achievement, behavioral status andeducational supports in middle child-hood, and adolescent activity limitations,risk behaviors, and vocational skills. Bybeginning to assess disability outcomesfrom this framework, we can moreclosely examine the pathways of chil-dren’s competencies and challenges inbasic and advanced functional skills acrosskey developmental stages prior to adult-hood.

MODELS FOR MEASURING THECOMPLEXITY OF OUTCOMES

One model for understandingfunctional outcomes after new biomedi-cal technologies has been modified fromSpilker (Fig. 1) [Spilker, 1996]. Spilker’smodel was initially used for assessing out-comes of clinical trials in adults. In thecontext of an extremely preterm infant,medical management, developmental in-terventions, and family supports arebrought to bear on the child and simul-taneously interact with the family’s val-ues, beliefs, and judgments. Outcomemeasurements are multidimensional andinclude measures of health, growth, andneurological status, developmental, func-tional, and behavioral competencies, ed-

Table 1. Health, Education, and Social Role Measuresof Child Well Being

Health, Growth, & NeurosensoryStatus

Growth, health status ratings, neurosensory status(microcephaly, hydrocephaly, cerebral palsy, sei-zures, hearing, vision), asthma, use of health ser-vices

Developmental, Functional& Behavioral Status

Postural Control Head and trunk control, sitting and standing balanceSelf-mobility Rolling, scooting, creeping, crawling, cruising,

walkingHand control Reach, attain, transfer, manipulateCommunication Gestures, words, phrases, sentences, points to body

parts and pictures, understands requestsCognition/Neuropsychological Problem solving, learning, attention, organization,

information processing, memoryBehavioral Regulation Joint attention, play, sharing, sleepAdaptive Self-care activities of daily living (feeding, dressing,

toileting) and social skillsEducational, Vocational

& Independent Living statusKeeping up with curricula, not repeating a grade,

not in special education placement. Post highschool education completed

Academic Reading, mathematics, written languageExtracurricular Sports, music, art, hobbiesVocational Paper route, baby-sit, lifeguard, summer jobsInstrumental Activities of Daily Living Household chores, using a telephone, cooking, man-

aging money, shopping for groceries, taking medi-cations as prescribed

Social Roles & Interactions Driving, higher education, working, dating, marry-ing, parenting

Friendships, overnight with friends, invitation tobirthdays

Community Participation Scouts, 4H, church, summer camp

259MRDD RESEARCH REVIEWS ● FUNCTIONAL OUTCOMES AFTER PREMATURITY ● MSALL & TREMONT

Table 2. Models of Enablement and Disablement After Extreme Prematurity

2 Year Old WithDiplegic CerebralPalsy

5.5 Year Old WithSevere Retinopathyof Prematurity

8 Year Old WithReading and Atten-tional Difficulties

11 Year Old WithAcademic Under-achievement

14 Year Old WithLearning Disabilities

Impairment Cystic periven-tricular leu-komalacia in600 grams 24week gestation,tight hamstringsand Achillestendons

900 grams with 26week gestationalchronic lung dis-ease, thresholdretinopathy ofprematurity re-quiring laser sur-gery, vision 20/50with glasses

1000 grams, grade 1intraventricularhemorrhage,asthma, phono-logical processing,and attention def-icits

900 grams small forgestational age

900 grams withlearning disability

FunctionalStrengths

Sits and combatcrawls. Finger

Walks, talks in sen-tences, is toilet

Excellent runningspeed for soccer.

Many awards inscouts. Excellent

On swim team. Ex-cellent computer

feeds, removessocks. Has 50word vocabu-lary and 2word phrases.

trained. Playsboard games.

Considered aleader by neigh-borhood peers.

music ability onflute.

skills.

FunctionalLimitations

Inability to crawl,cruise, or walk.

Clumsy, difficultywith fasteners.Speech not un-derstood by kin-dergarten teacher.

Reading at pre-primer level. Un-able to understandmath word prob-lems, impulsive,and distractible.

Reads at 3rd gradelevel. Math at 4thgrade level. Diffi-culty writing bookreports. Too smalland uncoordinatedfor competitivesports.

Difficulty with writ-ing reports in so-cial studies andlanguage arts. Aca-demic skills at 7thgrade level (2 lev-els behind).

Disability (Ac-tivity)

Early interventionservices includ-ing physicaland occupa-tional

Occupational ther-apy and speechtherapy supportsin modified kin-dergarten class.

Behavioral manage-ment strategies.Tutoring

Modified curriculum.Reading resource.After school tutor.

Resource Room.Talking books forhistory and Ameri-can Literature.

therapy. Bilat-eral ankle footorthotics, sup-plemental secu-rity income.

Societal Limi-tations (Par-ticipation)

Difficulty access-ing qualityearly childhoodday care.

Creative academicoptions for en-hancing reading,and developmen-tal maturity insmall groups.

Did not attend HeadStart or full daykindergarten.Limited educa-tional supports inurban school.

Ensure appropriatesupports for ac-quiring informa-tion and problemsolving.

High school curricu-lum that enhanceshigher educationand non manualjob opportunities.

260 MRDD RESEARCH REVIEWS ● FUNCTIONAL OUTCOMES AFTER PREMATURITY ● MSALL & TREMONT

ucational, vocational and independentliving skills, and social interactions,friendships, and community participa-tion. Table 1 highlights some of thecomponents of health, educational andsocial role domains of child well beingand builds on work of McCormick[1989], Johnson [1997], Vohr and Msall[1997], Hack and Fanaroff [1999], andHogan and Park [2000] to have system-atic multidimensional components for as-sessing these outcomes longitudinally.Implicit in this measurement strategy is toholistically and comprehensively describethe child’s physical, developmental, be-havioral, and educational strengths andchallenges, as well as his or her familyecology and how these strengths andchallenges impact on family life and com-munity participation. Kleigman [1995],Bennett and Scott [1997], McCormick[1997], Hack et al. [2000a], Hamvas[2000], Saigal [2000], Jobe [2001], andZhang and Schmidt [2001] have also pro-moted the viewpoint that an expandedframework for outcome assessment needsto occur after new neonatal technologiesas well as discussing goals and long termendpoints, especially at the margins ofviability. An important area to explicitlymeasure is functional status.

FRAMEWORK OF FUNCTIONALASSESSMENT

In order to understand developmen-tal aspects of functional status amongVLBW and ELBW survivors, the WorldHealth Organization (WHO) and the Na-tional Center on Medical RehabilitationResearch (NCMRR) models of disabilityand enablement [Pharoah, 1990; NationalAdvisory Board, 1993] will be used as aframework. These models have been usedby the Institute of Medicine to describe thecomplexity of disability and its impact onadult populations, and the American Acad-

emy of Cerebral Palsy and DevelopmentalMedicine (AACPDM) Treatment Out-comes Committee in systematic reviewsfor children with cerebral palsy [Pope andTarlov, 1991; Butler et al., 1999; Gray andHendershot, 2000]. There are five dimen-sions: 1) pathophysiology (molecular orcellular events), 2) impairment (disturbanceat an organ level), 3) functional limitationsof personal activities (self-care, mobility,communication, social interaction), 4) dis-ability in social roles (play, school, andwork), and 5) societal limitations. Morerecently, the World Health Organization(WHO-2) has also added functionalstrengths, social role skills, and environ-mental facilitators to its framework(ICIDH-2, 1999; Gray and Hendershot,2000]. Table 2 illustrates this model withseveral examples: a 2-year-old boy withdiplegic cerebral palsy, a 5.5- year-old girlwith severe retinopathy of prematurity, an8-year-old boy with reading and attentionchallenges, an 11- year-old girl with aca-demic underachievement, and a 14-year-old boy with learning disabilities.

Many studies use discriminative in-struments to compare a child’s perfor-mance with representative normativesamples that are periodically updated.This can be illustrated by using the Pea-body Developmental Motor Scales-2ndEdition (PDMS-2) [Folio and Fewel,2000] to describe a child’s gross and finemotor skills at age 25 months. The childwas at a 13-month level in locomotionand at an 18-month level in stationaryskills. In object manipulation, grasping,and visual motor integration, he per-formed at a 12-month age level. His grossmotor quotient was 64, his fine motorquotient was 62 and his total motor quo-tient was 62 (average�100�15). Whenthese same abilities were scored on thePDMS-1, his gross motor quotient was54 and his fine motor quotient was 54.

Thus, depending on the standardizationfor comparison, he could be moderatelydisabled (DQ 55–69) or severely disabled(DQ �50–55). Overall, these scores donot tell us if Johnny walks, undresses,feeds himself with utensils, communi-cates by pointing or using words, or fol-lows verbal requests. Similarly, a childmay have a moderate to severe sensori-neural hearing loss and with amplifica-tion, talk in clear sentences and knownumbers, letters, and colors at kindergar-ten entry. A 10-year-old child may haveshunted hydrocephalus, could have a fullscale WISC–3 IQ of 90, and be academ-ically challenged in all academic areas be-cause of learning disabilities. A childcould have cerebral palsy with minimalgait differences, but have major chal-lenges in attention and handwriting. Ateenager could have a Stanford Binet IVIQ of 65 and a Vineland Adaptive Be-havior Scale of 75 and be able to mowthe lawn and help his father on weekendsas a stock boy in the family grocery store.Thus, in order to understand the impactof neurological or developmental impair-ments, functional assessment is requiredin order to understand the child’s degreeof challenges and degree of strengths.

ADAPTIVE AND FUNCTIONALSKILL MEASURES FORCHILDREN

A variety of adaptive and func-tional instruments are available to healthprofessionals [Lollar et al., 2000; Sattler2002]. We have previously reviewed theuse of these instruments in children withcerebral palsy, with genetic disabilities,and in preschool years [Msall et al., 1997;Msall and Tremont, 1999; 2000].

A few of these instruments (VABS,WeeFIM, PEDI, Battelle, SIB-R, andABAS) deserve comment. The VinelandAdaptive Behavior Scales (VABS) mea-sures adaptive behavior in children andadults [Sparrow et al., 1984]. Communi-cation (receptive, expressive, written),daily living skills (personal self-care, do-mestic, communication), socialization(interpersonal skills, play, learning andcoping), and motor skills (gross and fine),constitute the domains. The survey formtakes approximately 30–45 minutes tocomplete. The Classroom Edition form isdesigned for children 3–12 years andtakes teachers approximately 20 minutesto complete. High concurrent validityhas been reported between the commu-nication domain and tests of intelligenceand academic achievement [Sattler,2002]. The VABS has been used in pre-school and school age children with de-

Fig. 1. Multidimensional outcomes after very low birth weight status


velopmental disabilities and autistic spec-trum disorders as well as in severalneonatal follow-up studies.

The Battelle Developmental In-ventory (BDI) was designed to be a dis-criminative measure of children’s perfor-mance in motor, communication(receptive and expressive), personal-so-cial (peer and adult interaction, coping),cognitive, and adaptive (e.g., attending,eating, dressing, toileting) skills [New-borg et al., 1984]. The BDI was alsoexplicitly developed to be useful to earlyintervention and preschool professionalsand thus potentially could provide infor-mation on developmental skills and adap-tive change rates among VLBW andELBW survivors in the preschool years.Scoring strategies for accommodatingchildren with disabilities have been in-cluded. A short form screening test(BDIST) containing approximately 25%of items from the full battery has excel-lent psychometric properties and requires20 minutes to administer.

The Pediatric Functional Indepen-dence Measure (WeeFIM) instrument as-sesses a child’s performance in self-care,mobility, and learning (i.e., communica-tion and social cognition) [Msall et al.,1993b, 1994a; b]. The WeeFIM was de-signed to be a minimally essential trans-disciplinary assessment that would allowfor enhanced descriptions of functionalperformance in key daily activities and beuseful in health, developmental, educa-tional, home and community settings.The WeeFIM involves 18 items that de-scribe seven levels of independence basedon explicit criteria [WeeFIM SystemGuide, 1998]. The normative sample in-volved over 400 children without dis-ability from one geographic region anddemonstrated robust correlation betweenage and total scores especially for childrenages 2–5 years [Msall, 1994a]. Initial con-struct validity involved over 700 childrenwith motor, developmental, genetic,neurological, or neonatal impairments[Msall, 1994b]. Test-retest and interraterreliability are excellent. Equivalence reli-ability has been demonstrated for obtain-ing information face to face or over thetelephone thus facilitating tracking ofoutcomes over time and using surveymethodologies [Ottenbacher et al.,1996]. Excellent concurrent validity hasbeen demonstrated in children ages 1–7years with motor, communicative andcognitive impairments [Ottenbacher etal., 1996] with the Vineland AdaptiveBehavior Scales, Pediatric Evaluation ofDisability Inventory, Battelle Develop-mental Screening Inventory, and the

Amount of Assistance Questionnaire.The WeeFIM has demonstrated respon-siveness to change in children with cere-bral palsy in early intervention, childrenages 1–7 years with motor, communica-tive, developmental, or genetic impair-ments in early childhood programs and inchildren ages 7–16 years receiving ortho-pedic interventions for cerebral palsy[Msall et al., 1997; McAuliffe et al., 1998;Azuala et al., 2000; Ottenbacher et al.,2000; Msall et al., 2001]. The WeeFIMhas been used as part of outcomes assess-ment in surfactant clinical trials, aftercryosurgery for retinopathy of prematu-rity, in children with congenital heartdisease, in children with genetic disabil-ities, in children with traumatic injury,and in Chinese and Japanese children[Msall et al., 1993b; Liu et al., 1998; Tsujiet al., 1999; Msall and Tremont, 2000;Limperopoulous et al., 2001; Aitkin etal., 2002; Wong et al., 2002]. TheWeeFIM takes approximately 20 minutesto administer.

The Pediatric Evaluation of Dis-ability Inventory (PEDI) includes 73Self-Care, 59 Mobility and 65 SocialFunction items [Haley et al., 1992]. Self-care includes feeding, grooming, dress-ing, and toileting. Mobility includeschanging positions, indoor and outdoorlocomotion and negotiating stairs. Socialfunction includes understanding words,comprehending sentences, functional useof communication and expressive com-munication, problem solving, social in-teraction, peer interaction, play, self-in-formation, time orientation, chores, self-protection, and community function.The normative sample was geographi-cally and ethnically diverse. The PEDIcaregiver assistance and modificationscale includes 20 items: 8 self-care, 7 mo-bility, and 5 social interaction items.Modification Scale scoring includeschild, rehabilitation, and extensive/com-plex ratings. Scoring is ordinal and mul-tilevel from 5 (independent) to 0 (totaldependence) and has been validated byRasch analysis [Haley et al., 1993]. ThePEDI caregiver assistance categories di-rectly overlap with the WeeFIM items.Msall et al. [2001] have demonstratedrobust criterion validity between theWeeFIM and PEDI for total scores anddomain scores. The PEDI has been usedin children with traumatic brain injury,in children with cerebral palsy includingthose undergoing rhizotomies to reducespasticity, and in children with rehabili-tational needs. [Coster and Haley, 1992;Haley et al., 1993; Nordmark et al.,2000]. A Spanish version is available

[Gannotti and Cruz, 2001]. The PEDIfunctional inventory takes approximately30–45 minutes to administer. The care-giver assistance and modification compo-nent of the PEDI takes approximately 15minutes to complete.

The Scales of Independent Behav-ior-Revised (SIB-R) measures adaptivebehavior skills in gross and fine motor,social interaction, language comprehen-sion and personal living skills (self-careand domestic skills) and community liv-ing skills [Bruininks et al., 1996]. Thecommunity living skills include time andpunctuality, money and value workskills, and home/community orientation.The Short Form contains 40 items andtakes approximately 15 minutes to ad-minister [Sattler, 2002]. The Early De-velopment Scale contains 40 items andcan be used for children with develop-mental levels less than 8 years.

The Adaptive Behavior AssessmentScale (ABAS) measures adaptive behaviorin children ages 5–21 years by teacherand parent report [Harrison and Oak-land, 2000]. The Parent Form involves232 items and include communication,community use, functional academics,home/school living, health and safety,leisure, self-care, self-direction, social,and work. Though the manual states thata literacy developmental age of 5th gradelevel, the parent form requires a range of4th to 9th grade literacy skills [Sattler,2002]. The scale has excellent test-retestand interrater reliability. Criterion valid-ity is excellent with the VABS and goodfor academic achievement.

EARLY CHILDHOODDEVELOPMENTALCOMPETENCIES AT AGES 18MONTHS TO 3 YEARS

In early childhood, outcome stud-ies have commonly used neurologic, sen-sory, and developmental assessments todescribe children as intact, at-risk, orchallenged. These studies have com-monly used diagnosis of neurodevelop-mental impairments (cerebral palsy,blindness, deafness) and cognitive im-pairments (SS �70) as proxies for motor,visual, auditory, and developmentalfunctional disability. We have previouslyreviewed several of these instruments(Vohr and Msall, 1997). Table 3 summa-rizes several studies of relatively large co-horts across diverse geographic regionsusing neurodevelopmental impairmentsof cerebral palsy, blindness, deafness, andcognitive disabilities as measures of func-tional disability.


Hack et al. [2000b] investigated theneurosensory and developmental out-comes of ELBW infants born between1992 and 1995. Social risks included mi-nority status, single parenthood and pa-rental education less than high school.Overall, 48% had a neurodevelopmentalimpairment, the most common of whichwas a cognitive developmental disability.Moderate motor disability; i.e., diplegicor hemiplegic cerebral palsy occurred in10%, and severe motor disability; i.e.,triplegic or quadriplegic cerebral palsyoccurred in 5%. Predictors of cognitivedevelopmental disability were male gen-der, social risk, and chronic lung disease(CLD). Predictors of neurosensory disor-ders were a severely abnormal cranial ul-trasound and CLD. There was no asso-ciation between birth weight and eitherneurosensory impairment or cognitivedisability within this ELBW cohort.

Hack and Faranoff [1999] also ex-amined early childhood outcomes in 131micropremature children of 500–749gram birth weight status born between1982 and 1994. Thirty-eight percent hada neurodevelopmental impairment withcognitive developmental disability oc-curring in 1 in 6 of survivors. Though62% were free of any major disability at20 months, there was concern that ratesof neurodevelopmental impairments insuccessive cohorts were increasing.

Piecuch et al. [1997a] examined 86of 94 survivors of 24–26 week gestationwho were born without congenital mal-

formations. Cognitive developmentaldisability at 2 1/2 years occurred in ap-proximately 1 in 4 survivors. Survivalwithout chronic lung disease or severeretinopathy of prematurity increasedfrom 35% at 24 weeks to 78% at 26weeks [Kilpatrick et al., 1997]. Parenchy-mal brain injury (severe intraventricularhemorrhage or cystic periventricular leu-komalaica) occurred in 11% of thoseborn at 24 weeks, 23% in those born at25 weeks, and 8.9% in those born at 26weeks and predicted neuromotor out-come [Piecuch et al., 1997a]. Predictorsof cognitive developmental disabilitywere chronic lung disease, parenchymalbrain injury, maternal substance abuse,and high social risk. Overall, 28% of thebabies born at 24 weeks, 47% of thoseborn at 25 weeks, and 71% of those bornglobally at 26 weeks had (globally normaldevelopmental performance on the Bay-ley scales (SS � 70).

Piecuch et al. [1997b] also exam-ined 442 ELBW (�1000 g) infantsborn between 1979 and 1991 at ap-proximately 55 (�33) months. Socialrisk included low maternal education,poverty, and substance misuse. Sixty-one percent of all children evaluatedhad normal neurosensory, neurologic,and cognitive outcomes. Approxi-mately 1 in 7 survivors had major cog-nitive developmental disabilities. Sig-nificant predictors of poor outcomeincluded parenchymal brain injury(IVH3/4-PVL), chronic lung disease

and high social risk. There was no as-sociation between birth weight andoutcome in this cohort. In the surfac-tant era, significantly increased survivaldid not increase the rates of cerebralpalsy which ranged from 8–10%. A keycomponent of this study was to alsoexamine outcomes as if all “lost to fol-low-up” had abnormal outcomes: If allthose lost to follow up were classified asabnormal, rates of major neurodevel-opmental disability would increasefrom 39% to 46%. If all lost to fol-low-up were reported as normal, ratesof major neurodevelopmental disabilitywould decrease to 34%. Tin and Fritz[1998] have demonstrated that thosechildren who are hardest to track havehigher rates of disability. Therefore, inexamining cohort outcomes, Piecuch’sapproach of counting all lost to fol-low-up as potentially disabled is notunreasonable and adds important per-spectives for understanding the cumu-lative impact of social risks.

In Victoria, Australia, 3 cohorts ofELBW (500–999 g) were followed toage 2 years [Doyle, 1997]. In the1979–80 cohort, 25% survived, from1985-87, 38% survived, and from 1991–92, 56% survived. Cerebral palsy waspresent in 13.5%, 6.6%, and 9.3% of co-horts, respectively. Similarly, deafnessoccurred in 3.4%, 0.5% and 0.8%. Severecognitive disability (SS �55) was presentin 7.9%, 6.2%, and 5.9%. Some cognitivedisability (SS �70) occurred in 6.7%,

Table 3. Major Neurodevelopmental Outcomes in Early Childhood

Author Year N Followup Cerebral Palsy Blind DeafCognitive Disability,i.e., MDI �70

Hack, Cleveland 1992–95 �1000 g 221 20 months 15% 1% 9% 42%1982–94 500–749 g 131 20 months 12.2% 4.6% 7.6% 16.8%

Piecuch, SanFrancisco

1979–91 �1000 g 442 55 months 11% 1.1% 0.2% 14%

1990–94 24–26weeks

86 32 months 14% 1.2% 0% 23%

Vohr, USA 12Center

1998 �1000 g 1151 18 months 17% 1.9% 3.5% 37%

Wood, UK &Ireland

1995 22–25 weeks 283 36 months 16% 2% 2% 30%

Schmidt, Inter-national

1996–98 500–999 g 574 18 months 12% 1.7% 2% 26%

Doyle, Australia 1991–92 �1000 g 237 24 months 9.3% 2.1% 0.8% 12.2%1979–91 �1000 g 542 24 months 8.9% 3.7% 1.1% 11.9%

Elbourne,France &Greece

1990–91 �33weeks with 65%�1500 g

204 24 months 4% 1% 0.5% 5.7%

Emsley, UK 1984–94 23–25weeks

64 �24 months 19% 13% 5% 14%

Synnes, BritishColumbia

1983–89 23–25weeks

139 18 months 26.4% 14.7% 3.1% 15.5%


4.3% and 6.3%. Severe disability de-creased from 12.2% to 6.8% and normalearly childhood developmental status in-creased from 53% to 71% over this timeframe. In contrast, Emsley and colleagues[1998] found deteriorating outcomes andSynnes and colleagues [1994] found nochange in ELBW cohorts over time.

The Victoria group subsequentlycompared 237 ELBW from the 1991–92cohort with 242 NBW controls. 56.1%of ELBW and 81.8% of the controls werefree from disability. Moderate to severedisability was present in 18.8% of ELBWand 3.4% of the controls. Children of500–749 g weight group had higher ratesof sensorineural disability as compared tothe 750–999 gram birth weight group.

Vohr et al. [2000] examined neu-rodevelopmental and functional out-comes of extremely low birth weight in-fants born between 1993 and 1994 inNICHD’s 12 center Neonatal Network.The mean birth weight of survivors was769 grams. Cognitive developmental dis-abilities occurred in more than one-third(37%) of survivors and cerebral palsy inone-sixth. Despite these major disabili-ties, ninety three percent achieved sittingbalance, 83% walked, and 86% fed them-selves. This study indicated that basicpostural skills and hand skills werepresent in the overwhelming majority ofsurvivors and overall prognosis for inde-pendent ambulation and basic self-careexceeded 85%. Factors associated withthe inability to walk at 18 months wereparenchymal brain injury, postnatal ste-roids, and chronic lung disease. Factorsassociated with inability to feed self at 18months were parenchymal brain injuryand chronic lung disease.

Wood et al. examined the neuro-logic and developmental outcome of alarge cohort of survivors of extreme pre-mature birth (�26 weeks gestation,�800g) born in the United Kingdomand Ireland in 1995 [Wood et al., 2000].In this study as in many European andAustralian studies, developmental cogni-tive disability was rated as mild (standardscore [SS] 70–84), moderate (SS 55–69)and severe (SS �55). Criteria for severelimitations were functionally explicit andincluded the inability to walk or sit, seeor hear, or use hands to feed self using theOxford guidelines [Johnson 1994, 1997].At 30 months corrected age, 19% of chil-dren had a severe and 11% had a mod-erate cognitive disability, and 24% had aneuromotor disability distributed as 10%diplegia, 4.3% quadriplegia, 1.8% hemi-plegia, and 8.2% other. Fifty percent ofchildren at age 30 months were free of

any disability, 26% had some disability,and 24% had severe disability. In func-tional outcomes, 10% were unable towalk, 3% were unable to sit, 4% wereunable to use hands to feed, 2% wereseverely visually limited, 2% were unableto hear, and 6% were not able to com-municate by speech. Severe overall dis-ability was present in 54% of childrenwith cerebral palsy with 92% of quadri-plegics, 44% of diplegics, and 20% ofhemiplegics having severe disability.Thus, classification of cerebral palsy bynumber of limbs involved is a method forunderstanding the severity of functionaldisability [Msall et al., 1997; Msall andTremont, 2000]. However, newer meth-odologies such as the Gross Motor Func-tional Classification System (GMFCS)may allow for more uniform severitystandards across studies (Rosenbaum, un-published observations).

Schmidt and colleagues [2001], ex-amined the long-term outcome ofELBW infants who were enrolled in themulti-center prophylactic indomethacinrandomized clinical trial to prevent intra-ventricular hemorrhage. Primary out-come measures included death, cerebralpalsy, cognitive disability (MDI �70),deafness or blindness at 18 months (cor-rected age). Indomethacin reduced therate of neonates with patent ductus arte-riosus and severe periventricular-intra-ventricular hemorrhage. However ratesof cerebral palsy, cognitive disability, andblindness were not different betweengroups. Overall, almost 1 in 4 of survi-vors had cognitive developmental dis-ability and approximately 1 in 8 had ce-rebral palsy. Schmidt questioned ifendpoints chosen for many neonatal ran-domized control trials were sensitive todetecting impact on disability outcomes.

Elbourne et al. [2001] evaluatedthe impact of ethamsylate on the preven-tion of IVH and subsequent death ordisability on VLBW neonates �33weeks. Neuromotor disability at age 2years was classified as moderate if thechild was unable to sit, needed help towalk, or had difficulty using his hands.Cognitive developmental delay was clas-sified as mild if there was less than a 25%delay (i.e., 6 months delay in a 2 yearold), moderate if the delay was between25-50% and severe if the child had morethan a 50% delay (i.e., �12 month delayin a 2 year old). Severe functional disabil-ity at age 2 years included the inability tosit, see, use hands or developmentallyperform skills typical of a 1-year old.Severe functional disability was present in8.3%. The risk of death or severe disabil-

ity was 13.3% in those with intraventric-ular hemorrhage (IVH) compared to10.4% of those without IVH but werenot statistically significant.

Thus, these studies highlight thecomplexity of short-term outcomes andhigh rates of developmental, motor, sen-sory, and learning challenges. In contrastto birth weight, parenchymal brain in-jury, chronic lung disease, severe reti-nopathy of prematurity and sepsis are de-terminants of major neurodevelopmentalimpairments. However, despite success-ful efforts at decreasing proximal markersof brain injury such as intraventricularhemorrhage, lower rates of motor, sen-sory or developmental disabilities did notsubsequently occur. A broader conceptu-alization of disability beyond categoricalneurosensory impairment is required andcan be facilitated by reviewing studieswhere measures took place for explicitlydescribing functional status and educa-tional performance.

PRESCHOOL ELBW STUDIESEXPLICITLY MEASURINGFUNCTION

Saigal et al. [1990] examined 84 of90 long-term ELBW survivors from anoriginal Canadian cohort of 184 infantsborn in 1980-82 and used the VinelandAdaptive Behavior Scales (VABS) tomeasure functional limitations. At age 5.5years, 9.5% had cerebral palsy, 4.8% wereblind and 20% had mental retardation.Overall, 23% had complex neurosensoryor developmental disabilities. Overall,8.3% of the children had a Vinelandadaptive behavior composite score morethan 2 standard deviations below themean. Four point eight percent of chil-dren had communicative, 5.9% self-care(daily living skills), 4.8% social, and15.5% motor functional limitations.

Msall et al. [1993a;c] used both theWeeFIM and VABS to demonstrate thatthe overwhelming majority of ELBWchildren had success in mastering basicmobility, self-care, and communicativefunctional skills prior to kindergarten.Among a cohort of 149 extremely pre-term (�28 weeks gestation) survivors,over 95% were able to walk, maintaindaytime continency and perform basicself-care skills. Neurodevelopmental im-pairments were present in 21% and in-cluded 5% with cerebral palsy, 10% withmental retardation, 1% with blindness,and 5% with multiple impairments (com-binations of cerebral palsy, blindness, ormental retardation). Of this group, 87%were able to walk one block, 84% wereable to talk in sentences, and 81% could


maintain continency and perform self-care tasks. This study demonstrated thatfor the entire cohort, severe basic func-tional limitations were rare and that themost common functional disability wasof mild to moderate degree. The majorpredictors of functional limitations werethe presence of parenchymal brain injury,severe retinopathy of prematurity(ROP), and cognitive developmentaldisability.

Palta et al. [2000] examined func-tional outcomes using the PEDI at age 5in 425 VLBW survivors participating inthe Midwest Chronic Lung Disease Mul-ticenter Project . Twelve-point -six per-cent of children had cerebral palsy. Ofthose without cerebral palsy, 5.2% hadself-care, 21% had motor, and 7.6% hadsocial-communicative functional limita-tions of severe degree (i.e. more than 2SD below the mean on the PEDI). Ofthe children with cerebral palsy, 57% hadself-care, 89% had mobility, and 32% hadsocial communication functional limita-tions of severe degree. Severity of intra-ventricular hemorrhage, severity of bron-chopulmonary dysplasia, socioeconomicstatus, and family status were correlatedwith PEDI Functional domains. Thisstudy highlights that both biomedical andsocial variables contribute to functionalstatus.

The Cryo-ROP CooperativeGroup evaluated the functional status atage 5.5 years of very low birth weightsurvivors �1250 grams [Msall et al.,2000]. The Extended Natural HistoryCohort followed 1199 survivors of�1251 grams birth weight from fivecenters and included children withoutretinopathy of prematurity (ROP) aswell as children with all degrees of se-verity of ROP. The Threshold Ran-domized Cohort (ThRC) followed255 infants �1251 grams from 23 cen-ters who developed threshold ROP(i.e., severe ROP) and consented tocryotherapy (CRYO) in order to pre-vent retinal detachment and blindness.At 5.5 years, both cohorts had ophthal-mic and Teller visual acuity testing aswell WeeFIM functional status evalua-tions.

Eighty-seven percent of childrenhad globally normal functional skills (i.e.scores were within 2 standard deviationsof non-disabled peers using Version 5.0of the WeeFIM Manual) [1998]. AsROP severity increased, rates of severefunctional disability (WeeFIM scoresmore than 4 standard deviations belowthe mean) increased from 3.7% amongthose with no ROP, 11.4% in prethresh-

old ROP to 26% of those with severe(i.e., threshold) ROP. Among the chil-dren with favorable visual status, 25.4%had a self-care disability, 5.2% had a mo-tor disability, 4.5% had a continency dis-ability and 22.4% had a communicative-social cognitive disability. In contrast,among the children with severe ROPand subsequent unfavorable visual status,self-care disability occurred in 76.8%,motor disability in 42.7%, continencydisability in 50%, and communicative-social cognitive disability in 65.9%. Betteroverall functional status at age 5.5 years wasassociated with favorable visual status, fa-vorable two-year neurological score, ab-

sence of severe ROP, having private healthinsurance, and black race. This study dem-onstrated that both biomedical, develop-mental, and socio-environmental factorscontributed to kindergarten functionalcompetencies. In addition, explicit mea-sures of functional skills in conjunctionwith assessments of health, neurological,sensory, and developmental competenciesenhanced the understanding of pathways ofrisks and resiliencies among children whocould be categorized as the tiniest, mostmedically frail, and often the sickest neo-nates amongst the population of VLBWand ELBW infants.

KINDERGARTEN READINESSAS A KEY DEVELOPMENTALSTAGE IN CHILDHOODFUNCTIONING

An important dimension of assess-ing the interaction of biological and so-cial factors on developmental processes isthe concept of kindergarten readiness[Vohr and Msall, 1997; Msall et al., 1998]and subsequently the amount of educa-tional supports required by a child tohave classroom success. Kindergartenreadiness is a holistic multidimensionalconstruct that includes dimensions ofphysical health and well being, develop-mental competencies, approaches tolearning, social-emotional maturity, andprevious socialization experiences [Kaganet al., 1995]. Measures include coordina-tion and visual-perceptual skills, languagecomprehension and expression, conceptsof shape, color and directionality, andability to follow-directions and partici-pate appropriately in groups. After con-sensus building will all stakeholders, thismodel was applied in the Early Child-hood Longitudinal Study of Kindergar-ten (ECLS-K) students in 1998–99 andinvolved 17,223 children. This multidi-mensional model includes both readychildren and ready schools and will beuseful in examining developmental aca-demic trajectories through age 10 years.

Msall et al. [1992] examined 149 of153 children born between 23 and 28weeks gestation. Fifty percent of the chil-dren were able to enter kindergartenwith their peers. Neurological resultsshowed 9% had a severe educational dis-ability (i.e., cerebral palsy, IQ �55,blind, or some combination). The mostcommon educational outcomes were re-quiring some educational supports for 3or more delays greater than 1 standarddeviation below the mean in verbal, per-ceptual, quantitative, memory and motorMcCarthy Scales. The most commonimpairments for these delays werespeech-language, attention deficit, andlearning disabilities.

Resnick et al. [1999] linked birthrecords to educational supports at kinder-garten entry. Moderate low birth weightstatus (1500–2499 g), and 3 categories ofvery low birth weight status (1000–1499g, 750–999 g, 450–749 g) were used inaddition to maternal and sociodemo-graphic information available on birthcertificates. Nine hundred ninety sevenchildren were ELBW, 2163 wereVLBW, and 211,663 were moderate lowbirth weight. Thus, 7.3% of this popula-tion had any degree of low birth weightstatus. Twenty six point six percent of

Explicit measures offunctional skills inconjunction with

assessments of health,neurological, sensory,

and developmentalcompetencies enhancedour understanding ofpathways of risks and

resiliencies amongchildren who could be

categorized as the tiniest,most medically frail, andoften the sickest neonatesamongst the populationof VLBW and ELBW

infants.


micropremature, 20.7% of ELBW,11.7% of VLBW, and 4.5% of moderateLBW children received special educationservices compared to 1.8% of normalbirth weight children. Severe disability(cerebral palsy, sensory impairment, IQ�50), were present in 18% of micropre-mature, 11.8% of ELBW, 6.5% ofVLBW, and 7.1% of moderate LBWcompared to 0.5% of normal birthweight children. Overall, 35.8% of mi-cropremaure, 43.7% of ELBW, 52.4% ofVLBW, 61.3% of moderate LBW and70.8% of normal birth weight childrenwere ready for kindergarten. High ratesof academic problems requiring resourcesupports occurred in 34% to 38% of anyLBW child compared to 27% of normalbirth weight children. Perinatal factors(e.g., birth weight �1000 g) had thegreatest effect on severe educational dis-ability while sociodemographic factors(poverty, male gender, low maternal ed-ucation and non-white race) significantlypredicted mild educational disability.Risks for mild mental retardation were22 fold for micropremature, 15 fold forELBW, and 6 fold for VLBW comparedto normal birth weight children. Rates oflearning disability were 14 fold for mi-cropremature, 8 fold for ELBW, 4–5fold for VLBW compared to normalbirth weight. Poverty doubled the risksfor EMR or learning disabilities. Thus,the combination of biological risk andsocial disadvantage significantly increasedthe impact of VLBW and ELBW onkindergarten readiness.

EDUCATIONAL DISABILITYAND FUNCTIONAL OUTCOMESIN SCHOOL-AGE CHILDREN

Rosenbaum et al. [1995] extendedSaigal’s 5 year outcomes to 8 years withadditional extremely low birth weightsurvivors born between 1977 and 1980.Overall, 28% of the children had majorneurodevelopmental impairments, 17%had cerebral palsy, 11% had mental retar-dation, and 12% had impaired vision.Among children without a major neuro-developmental impairment, 12% hadcommunicative limitations, 13% had self-care limitations, 12% had socializationlimitations, and 16% had motor func-tional limitations using the VABS.Among children with a major neurode-velopmental impairment, 35% had com-municative limitations, 41% had lowscores in daily living skills, 35% had so-cialization limitations, and 50% had mo-tor functional limitations.

Hack et al. [1994] examined 68children weighing less than 750 grams at

age 6.7 years in Cleveland using an ex-tensive neuropsychological battery andthe VABS. These children’s performancewas compared to 65 children weighingbetween 750–1499 grams and 61 termcontrol children. Significant functionalstrengths defined as total VABS scoresgreater than 70, occurred in 74%, 86%,and 98% of these groups respectively.

McCormick et al. [1992] examinedfunctional limitations using NationalHealth Interview Survey Data in schoolage children by degrees of low birthweight. Competencies in performingdaily living skills increased as birth weightincreased. Similarly, percentages of cog-nitive strengths increased with increasingbirth weights. Behavior problems ex-ceeded 20% and were relatively consis-tent across the birth weight categories.This study demonstrated the value of sur-vey methodologies in order to under-stand complex relations between birthweight status, educational and functionaloutcomes and behavioral competencies.More recently, Hogan and Msall [2002]have proposed key measures of healthstatus, developmental status, educationalstatus and supports, and functional statusfor use in population studies. These arelisted in Table 4 and can help bridgesome of the categorization of clinicalmeasures in Table 1 and measures used insurvey studies.

ACADEMIC COMPETENCIESAND SCHOOL CHALLENGES

Hack et al. [1994] examined edu-cational achievement at 8 years in 68children weighing less than 750 grams atbirth compared with 65 children 750 to1499 grams and 61 children who wereterm and �2500 grams. The rates ofcompetitive academic achievement were73% for children �750 grams, 91% forchildren 750–1499 grams, and 98% forterm children. Social disadvantage wassignificantly correlated with poorer aca-demic achievement scores.

Hille et al. [1994] assessed 813 verypremature (�32 weeks gestation) andVLBW infants at age 9 years in the Col-laborative Project on Preterm and Smallfor Gestational Age (POPS) Infants fromthe Netherlands. Nineteen percent werein special education, 32% were belowgrade level, and 38% had special assis-tance as compared to the general Dutchpopulation where 1% are in special edu-cation classroom, 6% receive specialschool services, and 14% are below gradelevel. These authors found the majorpredictor of special education placementat 9 years were social disadvantage, male

gender, and kindergarten readiness diffi-culties; i.e., severe developmental delay,speech impairment, or attention deficitdisorder at age 5 years. These VLBWsurvivors were often held back in kinder-garten.

Buck et al. examined 8–10 yearoutcomes of 108 extremely low birthweight survivors who were free of cere-bral palsy, blindness, mental retardation,and deafness, compared to full term con-trols [Buck et al., 2000]. Among ex-tremely low birth weight survivors, 33%had repeated a grade, 20% were in specialeducation class placements, 47% receivedschool-based rehabilitation therapies, and16% received tutoring or counseling ser-vices. Among term children, 18% hadrepeated a grade, 5% were in special ed-ucation class placements, 18% receivedschool rehabilitation therapies and 6% re-ceived special education resources. Ex-treme prematurity increased the risk ofspecial education placement and graderepetition 3 fold and school based re-sources almost 5 fold.

In a 1986 follow-up of a New Zea-land cohort, 77 extremely low birthweight children and 221 very low birthweight children were compared to 1,000normal birth weight controls. [Hor-wood, et al. 1998]. There was a greaterthan threefold risk for poor reading, poormath skills, behavior challenges (i.e., at-tention deficit hyperactivity disorder), orspecial education placement for all chil-dren with a birthweight �1500 grams.

Whitfield and colleagues [1997]examined 8-year outcomes of 115 mi-cropremies (�800 g) born between1974–85 in Vancouver and 50 termcontrols. Though 86% of the micropre-mature infants were free of cerebral palsy,developmental disability, and severe sen-sory disorders, 74% had academic diffi-culties, 33–59% had behavioral chal-lenges, 13% had repeated a grade, 15%received special education supports, and2% were in special education classrooms.Among term controls, 18% had academicchallenges, and 1–8% had behavior chal-lenges. Thus, in school and behaviorfunctioning, academic performance andcommunity participation challenges oc-cur throughout middle childhood forELBW survivors.

Gross et al. [2001] examinedschool performance in 118 10-year-oldsurvivors of extremely premature birthborn in 1985 to 1986. He compared theELBW group to a term control groupwith respect to cognitive, learning, andbehavior measures. Neurological out-comes showed 9% of the cohort had a


major neurosensory impairment, 18%had repeated a grade, and 14% were re-ceiving special education classroomplacement. Among term children, 100%were free of a major neurodevelopmentaldisability. Overall, 41% of the extremelypremature children were at grade levelcompared to 70% of term controls. Pa-renchymal brain injury (i.e., grade 3–4IVH/PVL), but not birth weight werehighly predictive of poor school out-comes. Social indicators that were asso-ciated with poor school outcome in-cluded single parents, parents with aneducation less than high school, and sta-bility of geographic residence. In bothterm and extremely preterm children,optimal school outcome was significantlyassociated with social capital and familystability.

Strategies in mid-childhood forchild adaptation include behavior com-petencies and family function. Hille et al.

[2001] examined the behavioral outcomeusing the Child Behavior Checklist ofELBW children ages 8 – 10 years fromfour different countries; Netherlands,Germany, Canada, and the USA. Chil-dren born with ELBW status irrespectiveof culture had more attention, social andlearning difficulties than higher birthweight controls. However, the relationof behavior to consequences (friendships,group activities, school extracurricular)which can be obtained via teacher reporton the Child Behavior Checklist werenot reported.

Taylor et al. [2001] examined thelong-term outcomes of the families ofchildren born with ELBW, VLBW, andterm control. Outcome measures in-cluded the Parenting Stress Index, theImpact on the Family Scale, and a FamilyBurden Interview. Behavioral-develop-mental functional problems were quanti-fied as a T-score greater than 63 on the

Child Behavior Checklist (total problemscore) and a standard score less than 70 onthe Vineland Adaptive Behavior Scales.Sixty one percent of �750 gram chil-dren, 51% of those weighing 751–1499grams and 32% of full term controls en-dorsed high levels of child related stress.Parent reports of developmental andlearning problems and emotional and be-havioral problems were associated withhigher rates of stress in �750 gram chil-dren compared to higher birth weightcohorts. Adverse family outcomes oc-curred in 51% of children who weighedless than 750 grams at birth, 36% of 750–1499 grams, and 27% of term controls.The impact of neonatal medical risk onthese outcomes was mediated by familysocial capital. Children’s functional dis-ability (neurosensory, developmental,and behavioral) occurred in 53% of �750gram children, 22% of those weighing751–1499 grams, and 8% of the term

Table 4. Impairment Checklist, Activity Limitations

Does child have any of these diagnoses? (Yes/No)Asthma, Autism, Blindness, Cerebral Palsy, Deafness, Mental Retardation, Muscular Dystrophy, Down Syndrome, Spina Bifida, Diabetes, LearningDisability, Cystic Fibrosis, Attention Deficit Disorder, Hyperactivity Disorder, Sickle Cell Anemia, Special Problem, Arthritis, Heart Disease requiringsurgery, Alcohol Disorder, HIV/AIDS, Substance Disorder, Epilepsy, Malnutrition

Does child have: (Yes/No)Condition that limits play activities, behavioral or emotional condition, difficulty completing activities of daily living, vision difficulty, hearing diffi-culty, communication difficulty, developmental delay?

How would you rate your child’sPhysical health, mental health, developmental status, overall health compared to 12 months ago (5 � excellent, 4 � very good, 3 � good, 2 � fair, 1 �poor). Use of physical therapy, occupational therapy, speech therapy, tutoring, and counseling service?

List performance in the following areas: Math, Reading, Handwriting, Sports/Gym (1 � far below grade level; 2 � somewhat below grade level; 3 � at gradelevel; 4 � somewhat above grade level; 5 � far above grade level)

Does your child receive special educational services? (Yes/No)If yes, check all that apply: special educational class, resource room, both

Can your child work independently and complete assignments with minimal help? (Yes/No)

Has your child ever repeated a grade? (Yes/No)

Describe your child’s abilities in the following areas: Unsupervised play, Making friends with other children, Practical judgement, Following directionsand completing chores, Running during a game, Participating in group activities such as scouts, church, choir, band, 4H (1 � significantly behind otherchildren; 2 � somewhat behind other children; 3 � about the same as other children; 4 � slightly ahead of other children; 5 � considerably ahead of other children)

Does your child need more help than other children his/her age or have difficulties in completing the following activities due to an impairment or con-dition? (Yes/No) Eating, Grasping objects, Dressing, Reaching overhead, Bathing, Lifting, Toileting, Bending, Walking, Stooping, Climbing stairs,Standing indefinitely

Describe your child’s vision:Blindness Difficulty seeing even with glassesSees adequately with glasses No correction necessary

Describe your child’s hearing ability:Deafness Difficulty hearing even with hearing aidHears adequately with hearing aid No hearing problem

Describe your child’s ability to communicate (Indicate verbal or nonverbal )Unable to communicate in words Unable to communicate in sentencesDifficulties being understood No problem communicating


controls. The child’s functional disabilityhad the highest contribution to familyburden (i.e., stress imposed on familyroutines).

ADOLESCENT OUTCOMESSaigal et al. [2000a] followed a co-

hort of ELBW survivors born between1977 and 1982 in Ontario. Adolescentoutcome measures included intelligenceand academic achievement, grade repeti-tions and special education services, doc-umentation of neurosensory impairments(NSIs). The Health Utilities Index-2(HUI-2) was used to describe child’s or-dinal ratings of performance across majorlife categories. In the ELBW group, 28%were reported to have an NSI comparedto 1% of the controls. Thirty-eight per-cent of teens who weighed �750 grams,18% of those who weighed 750–1000grams and 2.5% of term controls hadreading scores more than 2 standard de-viations below the mean. Forty-eightpercent of �750 gram, 24% of 750–1000gram and 8% of term control hadWISC-R full scale IQ’s �70. MoreELBW children repeated a grade (25%vs. 6%) and required special educationalservices (50% vs. 10%). Seventy-two per-cent of children �750 grams reportedmore school difficulties compared to 53%of the 750–1500 gram cohort versus 13%of controls. Ninety-four percent of thecontrols and 43% of the low birthweightgroups were in regular classrooms. Ado-lescents rated themselves quite high onthe HUI-2 [Saigal et al., 2000b].

Using the same cohort, Saigal et al.[2001] examined the growth and currenthealth status of the same ELBW adoles-cents compared to teen controls. TheELBW teens were significantly smaller inheight, weight, and head circumferencecompared to term controls. Past healthproblems, including seizures, asthma, re-current bronchitis/pneumonia, and sur-gical procedures were more prevalent inthe ELBW group than the controls. Inaddition, 35% of the ELBW’s had 3 ormore health problems compared to “7%”of controls. Eighty-one percent of theELBW group had some functional limi-tations compared to 42% of the controls.Parents reported a greater percentage ofthe ELBW cohort had difficulty seeing,more emotional problems, clumsiness,developmental delays, learning disabili-ties, and hyperactivity than the controlgroup. Utilization of health care also wasgreater for the ELBW teens. They sawtheir pediatrician more, as well as oph-thalmologists, ear/nose/throat doctors,

occupational therapists, and speech ther-apists. This study reflects the long termvalue of systematically assessing health,developmental, biopsychosocial and re-source utilization in neonatal outcomesstudies.

The Victorian Infant CollaborativeStudy group assessed at 14 years theirELBW cohort of Australian childrenborn between 1979–1980 [Doyle, 2001].Neurosensory impairments were presentin 21% of the adolescents of which 10%had cerebral palsy, 6% had bilateral blind-ness, 5% required hearing aides, and 46%were slow learners (IQ 70–84). AmongELBW survivors, 14% were classified asseverely disabled, 15% as moderate, and25% as mild compared to normal birthweight teenagers where 2% had a severeand 14% had a mild functional disability.Eighty-eight percent of the children withmoderate to severe disability status at age2 had moderate to severe disability at age14 years. Of the 52 children withoutdisability at age 5, 24% had some disabil-ity at age 12. Of the 26 children withmild to moderate peer disability at age 5years, 62% had mild to moderate disabil-ity at age 14 and 38% became non-dis-abled. Of the children with normal tomild peer disability status at age 8, 96%had normal to mild disability status at age14 years. Of the 50 children withoutdisability at age 8 years, 20% subse-quently developed disability. Rates of nodisability compared to peers were 40% atage 2, 51% at age 5, 44% at age 8 and 49%at age 14. When using psychometricnorms, rates of disability at age 14 yearswere underestimated at 5 years especiallywith respect to school achievement andsocial participation such as maintainingfriendships.

Roth et al. [2001] examined theadolescent outcome of very preterm ne-onates (�33 weeks gestation) with re-spect to the predictive power of the1-year neurodevelopmental examina-tion. The one year assessment consistedof an extended neurological examinationand the Griffiths Developmental Test.The assessment at 14-years included aneurological exam, information abouteducational status, tests of reading andintelligence, and ratings of behavioralcompetencies. One-year exams revealedthat 80% of very preterm infants wereclassified as normal, 11% had non-dis-abling impairments, 9% had disablingimpairments, and 22% had educationallimitations. There was a significant rela-tionship between neurodevelopmentalstatus at one-year and the need for extraeducational provisions (extra help,

teacher dissatisfied, grade repetition, andspecial school). There was a trend of bothreading development and behavior scoresbeing associated with neurodevelopmen-tal status, but that did not reach clinicalsignificance and thus were not accuratelypredicted by assessments at age 1 year.

Hack et al. [2000a] examined thehealth status, functional status, and specialhealth care needs of teens born weighingless than 750 grams. Fifty-nine infantsborn in the Cleveland area weighing lessthan 750 grams between 1982 and 1986were matched to 54 infants weighing be-tween 750 and 1499 grams and termcontrols. Measures included the Familyand Health Questionnaire, the Report ofthe Child’s Educational Progress, and theQuestionnaire for Identifying ChildrenWith Chronic Conditions (QuICCC)[Stein et al., 2000]. Children bornweighing less than 750 grams, whencompared to their higher birth weightpeers and term controls had significantlymore functional limitations. The �750gram children also needed significantlymore physical or occupational therapy,special class instruction, special arrange-ments in school, and an individualizededucation plan (IEP) compared to thehigher birth weight and term controls.Similarly, the smallest birth weight co-hort had higher occurrences of chronichealth conditions (e.g., asthma, vision,ADHD) than the normal birth weightgroup but there were no differences be-tween the groups for medical usage. Thisstudy demonstrates that children weigh-ing less than 750 grams at birth, and eventhe slightly heavier cohort of 750–1500gram children have a greater need forservices and are more reliant on othersfor daily functioning at home and schoolthus reflecting their difficulty maintain-ing educational and community roles.Hack et al. [2002] reported 20 year fol-low-up of 242 VLBW survivors with 233normal birth weight (NBW) peers. Sev-enty-four percent of VLBW adults and83% of NBW adults graduated from highschool. Among VLBW adults, 30% wereenrolled in post-secondary educationwhile 53% of NBW were enrolled incollege. In terms of risk taking behaviors,alcohol use and illicit drug use occurredsignificantly less often in VLBW adultswithout neurosensory impairment com-pared to full term controls (OR 0.6, p.007). Rates of teen motherhood or fa-therhood were significantly less inVLBW as was delayed first intercourse.Fifty-one percent of VLBW adults hadan average IQ (i.e., IQ � 85) comparedto 67% of term adults. Academic


achievement scores in reading and math-ematics for both VLBW men andwomen were significantly lower com-pared to term adults. Though both theIQ scores and achievement tests differ-ence were less than .3Z, the overall im-pact on higher status adult roles was moresubstantial for men than women. Sixteenpercent of VLBW men attended fouryear college compared to 44% NBWmen. For women, 33% of VLBW survi-vors attended a four year college com-pared to 38% of NBW women. Thisstudy demonstrates the critical impor-tance of going beyond IQ and achieve-ment test scores and examining both risktaking behaviors and vocational training.The limitation of this study is that mea-sures of positive young adult activities(volunteering, contributing to costs ofcollege, special talents, and interests)were not measured.

In an extension of the Hille et al.1994 study, Walther et al. [2000] com-pleted a 14-year longitudinal follow-upof 1138 infants born either less than �32weeks gestation or weighing less than1500 grams in 1983 in Holland. Childrenwere assessed at 2 years, 5 years and be-tween 9–14 years (see previous section-Hille in School Age). The final cohortconsisted of 1010 infants born with agestational age �32 weeks and 1097born weighing less than 1500 grams. Thechild’s pediatrician conducted a two-yearneurological assessment and results re-vealed that 6.3% of VLBW had severefunctional disability, 12% had a mildfunctional disability and 78.7% were freeof disability. The five-year assessment re-vealed that 6.9% had a major functionaldisability, 8.4% had a mild functional dis-ability and 13.2% were disabled in roleperformance.

Severe disabilities were found in10.1%, and mild disability in 16.6% Fi-nally, at the 14-year evaluation, severedisabilities were reported in 6.3% andmild disability in 15.9%. More than halfof the teen VLBW children had disordersof learning, attention, and social-emo-tional skills. Twenty seven percent re-ceived full time special education servicescompared to 7% of Dutch peers. Thirty-four percent had normal HUI scores,36% minor problem, and 30% had majorchallenges. Overall, 50% of the VLBWcohort were predicted to have the pre-cursors of becoming fully independentadults. These authors found that bothneurodevelopmental impairment at age 2and school failure in kindergarten areboth predictors of later problems amongVLBW survivors including those that re-

sult in decreased adult recreational andcommunity participation options.

Richard Strauss [2000] examineda Small for Gestational Age (SGA) co-hort in the United Kingdom BritishBirth Cohort Study at age 26 years.Children were born between April 5 toApril 11, 1970. This population cohortwas evaluated at ages 5, 10, 16, 26years. Potential participants were sent aquestionnaire by mail that requestedinformation on years of education, oc-cupation, income, marital status, levelof disability, and self-reported growthstatus. A total of 7470 adults responded(52.6% of potential participants), 6981NBW and 489 SGA. At age 26 years,adults who were SGA had similar yearsof education, employment, marriageand satisfaction with life compared tonormal birth weight peers. Adults whowere SGA were significantly less likelyto have professional or managerial jobs.SGA adults also were shorter and had asmaller head circumference than theirNBW peers. Head size and SGA weresignificant predictors of having a pro-fessional job. Seventeen percent ofNBW with a normal head size endedup with a professional career comparedto 3% of the SGA cohort with a smallhead size (p�.01). This study illus-trated the value of key health and de-velopmental measures in early child-hood and their relationship to adultadjustment. The Strauss study also il-lustrates the value of descriptive out-comes that are critical in categorizingadult adjustment. A similar analysiscould take place for outcomes of chil-dren with moderate, very low, and ex-tremely low birth weight.

Quality of life was assessed inyoung adults born of VLBW status be-tween 1980 and 1982 in Denmark[Dinesen and Greisen, 2001]. Threeweight groups were assessed; VLBW(�1500 g), LBW (1500–2300 g), and aNBW (�2500 g) group. Subjects whoreported having a handicap or chronichealth problem (H/CHP) were put intoa separate group for analysis at the 18-year follow-up. The final follow-up sam-ple included 79 VLBW, 110 LBW, 69NBW, and 22 with chronic health orhandicapping problems. Quality of lifewas assessed by a questionnaire with thetheory that four Maslovian needs deter-mine quality of life: 1) elementary bio-logical need, 2) need for warm humanrelations, 3) need for meaningful occu-pations, and 4) need for diverse and ex-citing experiences. They examined theseneeds in terms of both objective and sub-

jective aspects. Objective quality of life inthe VLBW group was lower than theNBW group. Also, both objective andsubjective quality of life was lower in theH/CHP group than non-disabledVLBW group. However, there was nodifference in the subjective rating ofquality of life between the groups. Thisstudy demonstrated that although societymay view people with disabilities as hav-ing a lower quality of life, the individualsthemselves do not view themselves ashaving a lower quality of life. Also, thisstudy demonstrated the value of usingtelephone interviews and survey methodsand systematically measuring health re-lated quality of life.

IMPLICATIONSThere are several key lessons to be

learned from the use of functional assess-ment as a component of outcomes eval-uation. First, it is important to focus onthe whole child and focus on the child’sstrengths as well as challenges. This im-plies that if a child had diplegic or hemi-plegic cerebral palsy, one can focus onboth their developmental and functionalcompetencies and their challenges inschool and the community. Second,functional assessment allows the profes-sional to focus on adaptive skills as theyare used in the community and how thechild is independent or struggling in ev-ery day activities. In early childhood,functional assessment includes celebrat-ing sitting balance, transferring ability,and pincer use, gesture language and jar-goning as these are all developmental in-dicator of progression of mobility, self-care, and communicative skills. Inpreschool years, it is important to observeactivities of self mobility, walking, andclimbing stairs but also perceptual motorskills as they impact on feeding, dressing,drawing, and conceptual skills in prag-matic language, social interactions duringplay and social learning. In middle child-hood, functional observations go beyondindependence in mobility, basic self-care,and communication to learning ingroups, becoming literate, carrying outmathematics operations, learning andproducing written language, maintainingfriendships, participating in sports, andbeing a member of a social group(church, shouts, band, choir). In adoles-cents, observations include instrumentalactivities of daily living, learning to drive,dating, and academic competencies thatwill determine higher educational andvocational choices. In adults, by under-standing precursors of independent liv-


ing, higher education achievement, andjob success, we can build on the traditionof Hess by marveling at the complexityof growth and resiliencies among chil-dren born very early and with very lowbirth weight [Hess, 1953]. Our task is notonly to apply the best of translationalscience to the optimization of survivalwithout disabling impairment but to en-sure that development, educational, andvocational interventions optimize learn-ing, community participation, and familyunderstanding. Measuring these out-comes and the factors contributing tothem is the challenge of future long termstudies.

ACKNOWLEDGEMENTSThis manuscript is dedicated to

Drs. William Oh and Betty Vohr incelebration of 28 years of commitmentto regionalization of neonatal care, ev-idence based neonatal intensive careunits, comprehensive developmentalfollow-up and family supports and ad-vocacy. Mary Fournier provided tech-nical support for motor assessments andKathleen Schlenz encouraged ongoingapplication of functional measures.Lewis Rubin and Dennis Hogan pro-vided valuable feedback. Judy daSilvaprovided valuable assistance withmanuscript preparation. f

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Measuring functional outcomes after prematurity: Developmental impact of very low birth weight and extremely low birth weight status on childhood disability