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Hack M, Taylor HG, Drotar D, Schluchter M, Cartar L, Andreias L, Wilson-Costello D, Klein N. Chronic Conditions, Functional Limitations, and Special Health Care Needs of School-aged Children Born With Extremely Low-Birth-Weight in the 1990s. JAMA. 2005;294(3):318–325. doi:10.1001/jama.294.3.318
Context Information on the school-age functioning and special health care needs
of extremely low-birth-weight (ELBW, <1000 g) children is necessary to
plan for medical and educational services.
Objective To examine neurosensory, developmental, and medical conditions together
with the associated functional limitations and special health care needs of
ELBW children compared with normal-birth-weight (NBW) term-born children (controls).
Design, Setting, and Participants A follow-up study at age 8 years of a cohort of 219 ELBW children born
1992 to 1995 (92% of survivors) and 176 NBW controls of similar sociodemographic
status conducted in Cleveland, Ohio.
Main Outcome Measures Parent Questionnaire for Identifying Children with Chronic Conditions
of 12 months or more and categorization of specific medical diagnoses and
developmental disabilities based on examination of the children.
Results In logistic regression analyses adjusting for sociodemographic status
and sex, ELBW children had significantly more chronic conditions than NBW
controls, including functional limitations (64% vs 20%, respectively; odds
ratio [OR], 8.1; 95% confidence interval [CI], 5.0-13.1; P<.001), compensatory dependency needs (48% vs 23%, respectively;
OR, 3.0; 95% CI, 1.9-4.7; P<.001), and services
above those routinely required by children (65% vs 27%, respectively; OR,
5.4; 95% CI, 3.4-8.5; P<.001). These differences
remained significant when the 36 ELBW children with neurosensory impairments
were excluded. Specific diagnoses and disabilities for ELBW vs NBW children
included cerebral palsy (14% vs 0%, respectively; P<.001),
asthma (21% vs 9%; OR, 3.0; 95% CI, 1.6-5.6; P = .001),
vision of less than 20/200 (10% vs 3%; OR, 3.1; 95% CI, 1.2-7.8; P = .02), low IQ of less than 85 (38% vs 14%; OR, 4.5; 95%
CI, 2.7-7.7; P<.001), limited academic skills
(37% vs 15%; OR, 4.2; 95% CI, 2.5-7.3; P<.001),
poor motor skills (47% vs 10%; OR, 7.8; 95% CI, 4.5-13.6; P<.001), and poor adaptive functioning (69% vs 34%; OR, 6.5; 95%
CI, 4.0-10.6; P<.001).
Conclusion The ELBW survivors in school at age 8 years who were born in the 1990s
have considerable long-term health and educational needs.
Advances in perinatal care in the 1990s, which included surfactant therapy
and increased use of antenatal steroids, resulted in dramatic increases in
the survival of extremely low-birth-weight (ELBW, <1000 g) infants.1 This has been accompanied by an increase in the rates
of neonatal complications and early childhood neurodevelopmental problems.1-4 However,
there is little information on how these children function at school age when
neurological, cognitive, and health status has to a large extent stabilized.
With the exception of an abstract from Sweden,5 current
information on the school-age outcomes of ELBW children is restricted to neurobehavioral
and developmental disability.6,7
Information on the overall functioning and special health care needs
of recent surviving ELBW children is needed to plan for the medical and educational
services that they will require at school age. We therefore sought to undertake
a comprehensive examination of health outcomes at age 8 years in a cohort
of ELBW infants born 1992 through 1995. Outcomes included functional limitations
and special health care needs together with the more traditional measures
of neurological and developmental status.
The study group included survivors of the cohort of 344 ELBW children
admitted to the neonatal intensive care unit at Rainbow Babies and Children’s
Hospital, Cleveland, Ohio, during 1992 through 1995. Their outcomes at 20
months corrected age have been previously reported.2 Thirteen
children (10 with major malformations, 2 with AIDS, and 1 with tuberous sclerosis)
were excluded, as their poor health or development may be due to their primary
disease rather than preterm birth or ELBW. Of the remaining 331 children,
238 (72%) survived to age 8 years, of whom 219 (92%) were followed up. Of
the 19 children not examined at 8 years, 8 parents refused to participate,
4 lived out of state, and 7 were lost to follow-up. The study children did
not differ significantly from these 19 children in sociodemographic factors,
birth data, or neonatal complications.
A normal-birth-weight (NBW) child born at term gestation (>36 weeks)
by parent report was recruited at age 8 years from the same school as the
ELBW child by randomly selecting a NBW child from a list of children in the
school who were within 3 months of age and of the same race and sex as the
ELBW child. The Individual with Disabilities Education Act mandates inclusion
of all children in regular schools. Of the 219 ELBW children observed at age
8 years, matches were recruited for 176 children (80%). Reasons for not finding
matches include refusal of the school principal to participate (18 children),
inability to match (6 children, including 1 child who was home-schooled),
and repeated failure to appear for scheduled appointments (19 children). One
child with neurofibromatosis with cerebral involvement was excluded.
The 43 ELBW children who could not be matched compared with those with
matches had higher rates of cerebral palsy (12 [28%] vs 19 [11%], P = .007). The children’s mothers were more often white
(28 [65%] vs 57 [32%], P<.001) and fewer had less
than a high school education (1 [2%] vs 25 [14%], P = .03).
Parent Questionnaires. Questionnaires were
administered to the parent or primary caregiver, usually the mother, and included
sociodemographic information, the Vineland Adaptive Behavior Scales of social
functioning,8 and the Questionnaire for Identifying
Children with Chronic Conditions (QUICCC).9 Race/ethnicity
was considered as a social construct and was self-identified from the list
of racial/ethnic categories used for federal reporting.
The QUICCC incorporates the consequences of chronic health conditions
lasting 12 months or more. It has 39 question sequences divided into 3 domains:
functional limitations, which has 16 items concerning physical, cognitive,
emotional, or social development; dependence on compensatory aids, which has
12 items, including medications, special diet, assistive devices, and personal
assistance; and need for services above those routinely required by children,
which has 11 items, including medical, psychological, or educational services,
and special treatments and accommodations at home or school. The interviewer
asks the parent if his/her child experiences any of the 39 specific health-related
consequences of chronic conditions. If the parent answers “yes,”
he/she is then asked 2 follow-up questions regarding the presence and duration
of the condition.
To qualify as having a chronic condition, the child must have at least
1 of the 39 consequences of a chronic condition, and each must be attributable
to a medical, behavioral, or other condition lasting 12 months or more.10 The QUICCC has good test-retest reliability and validity.9 Following administration of the QUICCC, the research
assistant asked the parent to further describe the reported conditions and
Child Assessment. The child underwent a complete
physical and neurological examination, performed by 1 of 2 pediatricians.
Hearing was measured with pure-tone audiometry screening. Deafness was defined
as the need for hearing aids. Mild hearing loss was defined as unilateral
or bilateral hearing loss of more than 25 dB in at least 2 frequencies. Visual
acuity was tested with Snellen’s letters. Psychometric testing was performed
by 1 of 3 trained research assistants who were blinded to whether the child
was ELBW or NBW. The Kaufman Assessment Battery for Children was used as a
measure of cognitive function11 and includes
4 subtests: hand movements, triangles, word order, and matrix analogies. These
form a mental processing composite score that has proved sensitive to the
consequences of prematurity.7,12
Academic achievement was measured with the skills cluster of the Woodcock-Johnson
Tests of Achievement III that included the letter/word identification, dictation,
and applied problems subtests.13 Motor skills
were measured with the Short Form of the Bruininks-Oseretsky Test of Motor
Proficiency.14 All tests were scored according
to the child’s postnatal age. Impairments were defined in terms of standard
scores on the psychometric measures that were either 1 or 2 SDs below the
normative mean, corresponding to mild and severe impairments, respectively.
One blind child was not tested and a score of 40 (3 SDs below the mean) was
assigned to 9 additional children who were untestable (7 who had cerebral
palsy and 2 with severe retardation/autistic-type behavior).
The study protocol was approved by the institutional review board of
University Hospitals of Cleveland, Ohio, and written informed consent was
obtained from parents.
Univariate comparisons between the ELBW and NBW groups were made using t tests for continuous variables. χ2 Test
or logistic regression was used to examine group differences in dichotomous
outcomes. Because of known effects of sociodemographic status and sex on outcomes,
we controlled for these factors in all analyses. As a measure of socioeconomic
status, we formed a composite of the sample z scores
of maternal education and median family income, according to the 2000 Census
tract of the family’s neighborhood. Preliminary analyses indicated that
these 2 factors, although correlated, were independently associated with outcomes
and that other factors, including marital status and neighborhood poverty
levels, did not add to their validity. In separate analyses, we compared the
outcomes of the ELBW and NBW children who were free of neurosensory impairments.
We also examined the outcomes of singleton births after excluding multiple
births. SPSS version 12.1 (SPSS Inc, Chicago, Ill) was used for all analyses
and P<.05 was considered statistically significant.
The ELBW and NBW children did not differ significantly with regard to
their mother’s age, marital status, education, race, median family income,
or mean percentage of families living below the poverty level of the neighborhood
in which the family resided (Table 1).
Twenty ELBW children (9%) had been adopted vs 4 controls (2%) (P = .005). The ELBW children had a mean (SD) birth weight
of 810 (124) g and mean (SD) gestational age of 26.4 (2) weeks. Seventy-three
(33%) of their mothers received antenatal steroid therapy. Postnatal steroid
therapy was administered to 129 children (59%) to treat or prevent chronic
lung disease and 93 (43%) were oxygen dependent at 36-weeks corrected age.
Fifty-one children (23%) had a severely abnormal cerebral ultrasound with
either grade III or IV periventricular hemorrhage, periventricular leucomalacia,
and/or ventricular dilatation at discharge. Eighteen children (8%) had severe
(stage >3) retinopathy of prematurity.
The ELBW children were studied at a significantly younger postnatal
age than the NBW children (mean [SD], 8.7 [0.6] vs 9.2 [0.8] years; P<.001), because NBW children were only recruited after
the ELBW child’s school had been verified so that matching could occur.
The psychometric tests were, however, standardized for age and functioning,
and special health care needs would not be expected to change in relation
to this small age difference.
Thirty-six ELBW children (16%) had neurosensory impairments, including
cerebral palsy, deafness, and blindness, compared with none of the NBW children
(Table 2). There were significant differences
in mild hearing loss and uncorrected vision of less than 20/200, even when
comparisons were restricted to neurosensory intact ELBW children. Asthma requiring
therapy was reported in 21% ELBW vs 9% NBW children. Twenty-one ELBW children
(10%) had both asthma and cerebral palsy. Rates of other medical conditions,
including allergic disorders (13% in both), did not differ between groups.
The mean (SD) mental processing composite score was 87.7 (18) for the
total group of ELBW children and 91.4 (15) for the neurosensory intact subgroup
compared with 99.8 (15) for the NBW children (P<.001
for both comparisons). The ELBW children differed significantly from the NBW
group in rates of suboptimal intelligence, academic achievement, motor skills,
and adaptive functioning. These differences remained significant after excluding
ELBW children who had major neurosensory deficits. Sixty-three ELBW (29%)
and 14 NBW (8%) children were receiving supplemental security income for identified
Functional Limitations. The ELBW children had
significantly higher rates of functional limitations compared with the NBW
group (Table 3). These limitations included
delay in growth or development, mental or emotional delay, need to reduce
or inability to participate in physical activities, difficulty seeing, hearing,
speaking, or communicating, and inability to play or socialize with others.
With the exception of limitations in physical activities, these differences
remained significant when ELBW children with neurosensory impairments were
excluded. Severe functional limitations, such as difficulty with feeding,
dressing, washing, and using the toilet, were mainly restricted to children
with neurosensory impairments. Overall, 64% of the ELBW children, 57% of the
neurosensory intact subgroup, and 20% of the NBW children had 1 or more functional
limitations. For those children with functional limitations, the mean number
per child was 3.8 (SD, 3.5; range, 1-16) for the total ELBW group; 2.6 (SD,
2.0; range, 1-12) for the neurosensory intact subgroup; and 1.8 (SD, 1.1;
range, 1-5) for the NBW group (P = .001
for total ELBW vs NBW and P = .02 for intact
ELBW vs NBW).
A variety of symptoms or diagnoses contributed to the functional disabilities.
For example, 41 parents reported that their ELBW child needed to reduce time
and effort in activities. Fourteen reported asthma as the reason, 8 reported
cerebral palsy, 10 reported that the child tired easily, 2 reported poor coordination,
3 reported attention-deficit/hyperactivity disorder, and 4 reported other
conditions, including epilepsy, cold sensitivity, deformed fingers, and overweight.
Compensatory Dependence Needs. Significantly
more ELBW than NBW children took a regular prescribed medication (Table 4). The need for help or special equipment
for walking, feeding, dressing, washing, and toileting was restricted mainly
to ELBW children who were neurosensory impaired. Overall, 48% of ELBW children,
40% of the neurosensory intact ELBW subgroup, and 23% of NBW children had
1 or more compensatory dependent needs. For those children with compensatory
needs, the mean number in these groups was 2.1 (SD, 2.0; range, 1- 9); 1.4
(SD, 1.0; range, 1-7); and 1.2 (SD, 0.5; range, 1-3) (P<.001 for total ELBW vs NBW and P = .33
for intact ELBW vs NBW).
Medication usage, the most common compensatory need, was reported for
21% of ELBW children with asthma vs 6% of NBW children with asthma; 7% vs
3% of those with attention-deficit/hyperactivity disorder; 7% vs 9% of those
with allergic conditions; 3% vs 1% of those with constipation; and 2% vs 1%
of those with epilepsy. Fifteen ELBW children with cerebral palsy received
either botulinum toxin or baclofen medication, 14 required a wheelchair, 2
a walker, and 3 were tube fed.
Services Needed Above Routine. Children who
were ELBW had a significantly greater need for services above routine than
children who were NBW (Table 5). These
services included visiting a physician regularly for a chronic condition,
nursing care/medical procedures, occupational or physical therapy, special
school arrangements, or an individualized education program. With the exception
of nursing care/medical procedures, these differences remained significant
when neurosensory abnormal children were excluded. Overall, 65% of the ELBW
group, 58% of the neurosensory intact ELBW subgroup, and 27% of the NBW group
received 1 or more services above routine. For those children requiring services,
the mean number per child was 3.2 (SD, 1.8; range, 1-7) for the ELBW group;
2.7 (SD, 1.6; range, 1-7) for the neurosensory intact ELBW subgroup; and 2.0
(SD, 1.2; range, 1-6) for the NBW group (P<.001
for total ELBW vs NBW and P = .007 for
intact ELBW vs NBW).
Of the ELBW children who visited a physician regularly for a chronic
condition, 20% saw their pediatrician, 11% an ophthalmologist, 7% an orthopedist,
6% a neurologist, 3% a pulmonologist, 3% an audiologist, and 21% other specialists.
Many children saw multiple specialists. Sixteen percent received physical
therapy, 21% occupational therapy, and 22% speech therapy. Nursing care included
aerosol treatments for asthma (7 children) and tube feeding or other help
related to cerebral palsy (8 children). The majority of hospitalizations were
for pulmonary problems or surgery for cerebral palsy.
Rates of Conditions in Multiple Domains. Overall,
76% of the ELBW children, 72% of the neurosensory intact subgroup, and 42%
of the NBW children were identified with a chronic condition in 1 of 3 domains
of the QUICCC. Thirty-eight percent of ELBW children, 28% of the neurosensory
intact subgroup, and 6% of NBW children were identified in all 3 domains,
and 27%, 15%, and 30%, respectively, in 2 domains.
When we compared the outcomes of singleton ELBW children with the NBW
controls, after excluding multiple births, the results were similar to those
of the total ELBW cohort with the exception that the difference in vision
of less than 20/200 was no longer significant (20 [7%] ELBW children vs 6
[3%] NBW children, P = .19). However, the
overall rates of functional limitations, compensatory dependence, and services
above routine did not change. The results also did not change when the analyses
were restricted to the 176 ELBW children who had controls.
This is the first report to our knowledge of the school-age outcomes
of ELBW children born in the 1990s in the United States. Our results reveal
that ELBW children have extremely high rates of chronic conditions compared
with NBW children. These conditions include asthma, cerebral palsy, and visual
disability, as well as poorer cognitive ability, academic achievement, motor
skills, and social adaptive functioning. These differences are evident even
in ELBW children who do not have major neurosensory impairments and manifest
in a higher overall frequency of functional limitations and need for compensatory
aids and services above those routinely required by children in general.
The studies of school-age outcomes of ELBW children born in the 1990s
pertain to neurobehavioral and developmental disability but do not examine
other aspects of health.6,7 The
study by Marlow et al7 studied 6-year-old children
born at less than 26 weeks’ gestation in England, while the study by
Anderson et al6 studied 8-year-old children
born weighing less than 1000 g or less than 28 weeks’ gestation in Australia.
Similar to our findings, both studies reported highly significant differences
between their study populations and NBW controls. Compared with our results,
fewer Australian infants, whose birth weight group was similar to our group,
survived (53% vs 72%, respectively); and fewer children had neurosensory impairments
(12% vs 16%, respectively) or subnormal IQ (5% vs 15%, respectively).15 The study by Lorenz et al16 similarly
found that the higher survival rate of infants born at less than 26 weeks’
gestation and who were treated in New Jersey in the United States was associated
with higher rates of disabling cerebral palsy compared with similar infants
treated in the Netherlands. We postulate that the differences in outcomes
between our ELBW cohort and the Australian cohort are related to their lower
survival rate and sociodemographic differences.
We presented our results from both a categorical and noncategorical
perspective to provide a comprehensive overview of the clinical, educational,
and health care implications of the chronic sequelae experienced by ELBW children.
Our results indicate that, in addition to the more commonly described neurodevelopmental
problems, asthma plays a major role in determining the functional limitations
and special health care needs of ELBW children. The use of a noncategorical
approach (independent of diagnoses) is especially important for examining
the outcomes of survivors of neonatal intensive care as it provides an assessment
of the impact of the multiple chronic sequelae of preterm birth on functioning
and special health care needs.10,17 Children
identified in the QUICCC fit the definition of children with special health
care needs (have, or are at risk for having, a physical, developmental, behavioral,
or emotional condition, and require health or related services of a type or
amount beyond that required by children generally). This definition is used
for the identification and planning of federal aid and services for children.18 The QUICCC also encompasses most of the elements
of the World Health Organization’s International Classification of Functioning
and Disability, which includes limitations in body/structure, personal activity,
participation in society, and environmental facilitation.19-21
The functional limitations and special health care needs in our current
ELBW cohort are comparable with those results for 11-year-old children born
in the mid-1980s who weighed less than 750 g at birth and had odds ratios
(ORs) of 4.7 (95% confidence interval [CI], 2.0-11.0) for mental and emotional
delay and 9.5 (95% CI, 2.1-43.6) for special arrangements in school.22 Our results are also comparable with those of 11-year-old
Swedish children who were born less than 26 weeks’ gestation and had
ORs of 5.1 (95% CI, 1.8-14.7) for mental or emotional delay and 6.2 (95% CI,
1.3-30.6) for attending special schools.5 However,
the 42% rate of chronic conditions in our NBW group is higher than the 15%
to 30% rate reported in other studies using the QUICCC,9,23,24 its
shortened version,25 or the Children with Special
Health Care Needs screener.23,26,27 In
these studies, higher rates of chronic conditions were reported among poor
families, Medicaid recipients, and school-age children, findings which could
partly explain the relatively high rates of chronic conditions in our NBW
relationship that developed between the parent and research assistant during
the 2- to 3-hour visit for our study might also have encouraged parents to
report more conditions than during the 7-minute telephone administration of
the QUICCC or 1-minute Children with Special Health Care Needs screener interview.9,23,24,28 Another
explanation for the high rate of chronic conditions is that we considered
the full range of consequences of chronic conditions included in the QUICCC,
which may represent conditions in the borderline or “gray zone,”
such as special diet, allergy medication, or a past life-threatening reaction.9,28,29 Newacheck et al30 in 1986 postulated that most of the increase in reported
rates of disability of children in the United States could be attributed to
a shift in parental perception of the health of their children. Rates of disability
have continued to increase in the United States,31 as
have general pediatric and subspecialty visits32 and
prescription drug use for children.33 All these
factors could have contributed to the high rates of chronic conditions in
our NBW group.
The ELBW children in our study represent the outcomes of an urban tertiary
perinatal center and are thus not representative of the United States as a
whole. The mean poverty level of our families was 18% vs 12% nationally,34 and 62% were black. However, because a disproportionate
number of ELBW births in the United States are poor and black, our results
provide important information for public agencies and health care insurance
plans and organizations.35
Our study, which combined the assessment of functional health status
and special health care needs with traditional measures of neurological and
developmental status, provides important research-based and policy-related
information needed for the planning and provision of services for the increased
number of ELBW children who survive. Because survival has not changed since
the mid-1990s, our results also have relevance for current survivors.1 The majority of conditions that we described result
from periventricular brain injury, chronic lung disease, and retinopathy of
research is thus critical to prevent these complications of prematurity.
In the United States in 2002, there were 22 845 live births with
a birth weight of 500 to 999 g, of whom approximately 70% survived.35 Our findings underscore the extraordinary costs of
care that will be needed to manage the medical, educational, and other service
needs of the large proportion of these ELBW children who develop chronic conditions.39 Proactive planning for the long-term health and educational
care needs of all ELBW survivors is essential to optimally treat and possibly
improve outcomes through preventative and early intervention services.40,41 The American Academy of Pediatrics
has emphasized the importance of providing a medical home for children with
special health care needs, coordinating their care, involving family, and
assisting in navigation of the complex federal, state, and local systems that
provide services required by these children.42-44 All
of these services are highly relevant for the continuing long-term care of
ELBW children who survive as a result of neonatal intensive care.
Corresponding Author: Maureen Hack, MB,
ChB, Division of Neonatology, Suite 3100, Rainbow Babies and Childrens Hospital,
University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, OH 44106-6010
Author Contributions: Dr Hack had full access
to all of the data in the study and takes responsibility for the integrity
of the data and the accuracy of the data analysis.
Study concept and design: Hack, Taylor, Drotar,
Acquisition of data: Hack, Cartar, Wilson-Costello,
Analysis and interpretation of data: Hack,
Taylor, Drotar, Schluchter, Cartar, Andreias, Wilson-Costello.
Drafting of the manuscript: Hack.
Critical revision of the manuscript for important
intellectual content: Taylor, Drotar, Schluchter, Cartar, Andreias,
Statistical analysis: Hack, Drotar, Schluchter,
Obtained funding: Hack.
Administrative, technical, or material support:
Hack, Cartar, Klein.
Study supervision: Hack, Taylor, Wilson-Costello.
Financial Disclosures: None reported.
Funding/Support: This study was supported by
grants RO1 HD39756 and MO1 RR00080 from the General Clinical Research of the
National Institutes of Health (NIH).
Role of the Sponsor: Following approval and
funding of the grant proposal by the NIH, the authors received no further
input from the NIH concerning the design, conduct of the study, collection,
management, analysis, and interpretation of the data, or preparation, review,
or approval of the manuscript.
Acknowledgment: We thank Miriam Curran, the
project coordinator, who interviewed the parents and administered some of
the questionnaires to the parents; Terry Reid, Jennifer Eppich, and Mary Morrow,
all research assistants, who tested the children and interviewed and administered
some of the questionnaires to the children and their parents; Nori Mercuri-Minich,
who assisted in data analysis; and Alpher Torres, who provided clerical assistance.
They were all employees of Case Western Reserve University and, with the exception
of Nori Mercuri-Minich and Alpher Torres who were paid by the department,
were all paid for their work/effort by the NIH grant.