Fall 2000
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Life expectancy in persons with
severe developmental disability
by Edward Hoffman, MD FAAP, Kansas City, MO
"My patient with multiple disabilities died recently and
I am trying to find out what is the life expectancy in a child with
multiple severe disabilities. The patient referred to me was male
age 21 years. He had been born very premature, experienced chronic
lung disease, and developed spastic quadriplegia, severe cognitive
impairment, and oral/pharyngeal dysphagia. He had epilepsy, controlled
by phenobarbital. He died suddenly overnight and vomitus was found
on the side of his face." (DBP Discussion Group, June 6, 2000)
The Problem
What developmental diagnostic category best predicts mortality? What
functional abilities most affect mortality risk? What are typical causes
of death? Some literature does exist that addresses life expectancy
in persons with severe disabilities. We now know more about the problem
than just the average age of death for level of abnormality.
The Evidence
Eyman et al (1990) reported on 99,543 persons with developmental
disabilities who obtained services from the California Department of
Developmental Services between March 1989 and October 1997. Data was
based on Client Development Evaluation Reports (CDER). All had cognitive
impairment,. Mental retardation (MR) was the handicapping condition
of focus. Functional abilities that related statistically to mortality
were degree of ambulation, mobility, toileting skills, eating skills,
and tube feeding. “Immobile” persons could not crawl, creep, or scoot
in prone. Persons were “unable to feed” if they needed to be fed completely
by others.
If a person had severe or profound mental retardation, was immobile,
not toilet trained, and was tube fed, then life expectancy was, on average,
only 4 or 5 years more than the current age for those persons between
the ages of 1 and 24 years at the start of the study. Mobility, or immobility,
was the best single predictor of mortality risk. If others could feed
a person with immobility then life expectancy doubled. If some functional
mobility was also present, then life expectancy doubled again. Finally
using the above categories of handicap and function, the addition of
presence of seizures or of cerebral palsy (CP) did not alter the predictions
of mortality.
Hutton et al (1994) evaluated 1251 cases of cerebral palsy
born between 1966 and 1984 in the Mersey region of England, using hospital
and community records. Functional abilities evaluated were ambulatory
ability, manual dexterity, and mental ability. If severe mobility impairment
(required a wheelchair and assistance for propulsion), severe manual
dexterity impairment (inability to feed and dress without assistance),
and severe mental impairment (Intelligence Quotient <50) were all
present, then average life expectancy was 17-20 years. Again impairment
of mobility was the best single predictor. Severity of mental impairment
was least predictive of these three functional classifications.
Strauss and Shavelle (1999) reported on the life expectancy
of adults (persons age 15 years or over) with a diagnosis of cerebral
palsy. The population, again, was from the California Department of
Developmental Services consisting of 182,623 persons with developmental
disabilities who received services from January 1980 to December 1995.
Eight functional abilities were evaluated; rolling/sitting, hand use,
ambulation, feeding, severity of CP, severity of MR, type and location
of CP, presence of epilepsy, and speech and communication. Life expectancy
beyond age 15 years was only 7.8 more years for persons both unable
to lift his head and tube fed. If one was able to be fed by others but
still unable to lift one's head, then life expectancy was 11.3 years
beyond 15 years of age.
Strauss et al (1997) using the same data set as their report on life
expectancy of adults with cerebral palsy, and the UCP Research Foundation
(1995), using data from death certificates provided by the National
Center for Health Statistics/USPHS for the year 1987, addressed the
cause of death in persons with cerebral palsy. This is of particular
interest since the assumption generally is that severe motor impairment
resulting in poor mobility and poor feeding skills also place one at
much higher risk for death from respiratory compromise and aspiration.
Indeed, respiratory causes underlie 15 to 45 per cent of deaths, a wide
range due to the imprecise nature of data collection. Another large
category was circulatory system or heart disease.
Summary
Anticipating the death of a person with severe functional impairments
is imprecise. There are several controversies, such as the effects of
institutional or community placement or of when and how tube feeding
is used (Blacher 1998; Strauss 1997). A family member's ability to cope
with the death of a loved one who has severe developmental disability,
family financial planning, medical-legal malpractice awards, and state
and federal disability budgets are all affected by these uncertain estimates.
Functional motor impairment so severe that head control and rolling
or scooting mobility and oral feeding ability all are poor appears to
result in drastically reduced life expectancy. Conversely, persons with
developmental disability of cognitive or neuromuscular function who
have some functional mobility or ambulation and have some functional
oral feeding skills seem to have only mildly or moderately reduced life
expectancies, depending in part on the degree of functional impairment.
Function, rather than diagnostic category, seems to be most predictive
of early mortality.
References
Blacher J. Much ado about mortality: Debating the wrong question. Ment
Retard 1998;36:412-415
Eyman RK, Grossman HJ, Chaney RH, Call TL. The life expectancy of profoundly
handicapped people with mental retardation. N Engl J Med 1990;323:584-589
Hutton JL, Cooke T, Pharoah POD. Life expectancy in children with cerebral
palsy. BMJ 1994;309:431-435
Strauss D, Cable W, Shavelle R. Causes of excess mortality in cerebral
palsy. Dev Med Child Neurol 1999;41:580-585
Strauss D, Kastner T, Ashwal S, White J. Tube feeding and mortality
in children with severe disabilities and mental retardation. Pediatrics
1997;99:358-362
Strauss D, Shavelle R. Life expectancy of adults with cerebral palsy.
Dev Med Child Neurol 1998;40:369-375
UCP Research Foundation. Causes of death of persons with disabilities
due to cerebral palsy. UCPnet (http://www.ucp.org) 1995.
Dr. Hoffman is the Director of the neonatal follow-up clinics at
Children’s Mercy Hospital in Kansas City, Missouri, and a member of
the Section on Developmental and Behavioral Pediatrics
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