Aging in Down Syndrome: Experiences of Families
Alison Downes, MD, Anna Esbensen, PhD, Ryan Macks, PhD, Erin Murphy, BS, Theodora Schade, BA
Leadership Education in Neurodevelopmental and related Disabilities (LEND) Program
The Division of Developmental and Behavioral Pediatrics
Cincinnati Children's Hospital Medical Center
The University of Cincinnati University Center for Excellence in Developmental Disabilities
WAISMAN CENTER
Introduction/Background
Sample
Themes Specific to Mothers
Conclusion
A highly significant change in the survival of people with Down
syndrome (DS) has occurred during the last two generations,
with life expectancy estimates increasing from 12 years in
1949 to nearly 60 years of age today (Bittles & Glasson, 2004).
Individuals with DS, as a group, have better functional abilities
and fewer behavior problems from childhood through mid-life
as compared to individuals with other causes of intellectual or
developmental disabilities (Esbensen et al., 2008). As adults
with DS age beyond midlife, there is evidence of an elevated
rate and earlier onset of the cognitive and behavioral declines
associated with dementia (Zigman et al., 2002). Research has
shown that there is a great deal of within-group heterogeneity
in aging among adults with DS. We hypothesize that some of
the heterogeneity is associated with family and environmental
factors. This study is an initial exploration of the family
environment, investigated by extracting themes from a fiveminute speech sample (FMSS).
This study builds from a study of 461 co-residing families of
adults with intellectual disability (R01 AG08768), which
included 169 families of adults with DS. The surviving adults
with DS are now ages 35-68.
Demographics of Caregiver
Impact of caregiving on caregiver: The joys, challenges and emotions experienced
by mothers caring for their aging child with DS.
“I can’t imagine my life now without him.”
Life expectancy of individuals with DS has increased dramatically with the
advancement of medical and surgical interventions. There is a great deal of
heterogeneity in this group of aging individuals, potentially related to family and
environmental factors. Using the FMSS, this study identified numerous themes from
caregiver subsets that help characterize the family environment that may contribute to
this heterogeneity in aging.
Mean Age (SD)
Sibling
(n=35)
84.3 (5.1)
59.2 (7.3)
100%
71.4%
Gender
(% female)
Dementia
(n=19)
Parent
Sibling
(n=8)
(n=11)
82.9 (4.5)
63.3 (6.6)
75%
81.8%
Demographics of Adult with DS
Mother
(n=36)
50.5 (4.6)
Sibling
(n=35)
52.2 (7.0)
Dementia
(n=19)
55.7 (4.7)
Gender
(% female)
36.1%
31.4%
47.4%
% Mild/ Moderate
Intellectual Disability
69.4%
Mean Age (SD)
To determine key themes expressed by family members
during a FMSS. The extracted themes were analyzed
separately for (1) mothers as caregivers, (2) siblings as
caregivers, and for (3) family members of individuals with DS
with signs and symptoms of dementia.
Methods
Semi-structured interviews were conducted with 75 family
members (respondents) of adults with DS (36 mothers, 4
fathers, 35 siblings) and transcribed. The family member
interviewed was identified as the primary caregiver for their
relative with DS. A qualitative analysis of a FMSS was done to
determine key themes expressed by family members.
Respondents were asked to: “Speak for 5 minutes, telling me
what kind of a person (name) is and how the two of you get
along together.” The FMSS is a brief measure of expressed
emotion and assesses the quality of the respondent-relative’s
home environment (Magaña et al, 1986).
Themes were identified from the FMSS by three independent
reviewers using consensus coding and then evaluated for
reliability.
Reliability Coding
92% (one error of commission)
100% (one error of commission)
90%
Societal stigmatization: The stereotypes and assumptions about individuals with
DS that families have encountered, often stemming from a lack of understanding
or education.
“Most doctors we saw said ‘put him away.’”
"When she was born it was kind of tough on both of us…the name that they
used at the time…it was mongoloid idiot."
82.9%
63.2%
Common Themes Across Groups
Aims
Mothers
Siblings
Dementia
Mother
(n=36)
Social Personality Characteristics: The qualities of an individual’s
personality that are evident in their social interactions (ie. easy-going,
caring).
“He has a wonderful personality….a good sense of humor.”
“She is a very caring, loving young lady. She is always concerned
about everyone else’s feelings.”
Functional/Adaptive Personality Characteristics: The qualities of an
individual’s personality that help them with daily living and allow them to
be productive members of society (ie. hard-working, organized).
“He’s a hard worker and they can put him at any job they’ve got. And
he will do it, and do it well.”
“He’s very helpful around the house; if I ask him to do something he’s
always ready to pitch in and do whatever has to be done.”
Maladaptive Personality Characteristics: The qualities of an individual’s
personality that hinder them in daily living and interfere with their
integration into society (ie. stubborn, rigid).
“He does things a certain way, and sometimes we have to adjust
rather than he adjust”
“She gets a little stubborn at times. When she wants something, she
wants it, whether it’s the proper time or not.”
Hobbies and Interests: The activities and interests that an individual
enjoys for recreation and/or socialization.
“Couple times a week he goes to the art center, that's his big love in
his life.”
“And he’s really gung-ho on sports like any other guy would be.”
Good self-image: An individual’s pride in their successes and sense of self-worth.
“He has a remarkable self-image. I mean, he just thinks he’s quite alright, and
quite frankly, he is.”
Communication: The impact of communication challenges on interpersonal
interactions.
“His handicap more than anything is his speech, which makes it hard for him to
communicate to people who don’t know him very well.”
Health issues: Problems with medical, physical, or mental well-being.
“He’s overweight and we’re trying to bring it down.”
Themes Specific to Siblings
Impact of caregiving on caregiver: Definition same as above
“And it’s just amazing living with him. When he’s gone for a few days, it’s like
there’s a light out in my life.”
Nostalgia of childhood: Recollections of the past and of growing up with a sibling
with DS.
“And then when he finally did learn to walk I remember that was just such a thrill
for all of us.”
“And we just loved him to pieces right from the start.”
Decline in health: Life changes associated with medical, physical, or mental
decline.
“I don’t know if she necessarily recognizes people as much now.”
Themes Specific to Dementia
Impact of dementia on family: The challenges and emotions experienced by
families caring for an individual with dementia.
“It's as always hard to understand what's going on and to accept things that he
used to be able to do and he just does not understand anymore."
Decline in health: Life changes associated with medical, physical, or mental
decline, including those related to dementia.
“Some days he's very good, so very much like he used to be, and other days
he's distant and you know, a lot of confusion going on there."
Acknowledgements
Research was supported by NICHD (R03 HD59848, Esbensen; P30 HD03352, Seltzer) and NIA (R01 AG08768, Seltzer).
Families who participated in this research.
The DS behavioral phenotype has been characterized in previous literature. Visual
processing, social functioning, self help, and daily living skills are relative strengths in
comparison to weaknesses in speech and language, verbal processing and motor
functioning. Personality characteristics extracted as themes from the FMSS in this
study were in-line with this established behavioral phenotype.
Mothers
The familial environment of individuals with DS is different from that of individuals with
intellectual disability (ID) due to other causes. Such families are said to experience the
“DS advantage,” which refers to the more favorable pattern of adaptations in families of
individuals with DS as compared to families of individuals with ID due to other causes
(Hodapp et al., 2001). Maternal well-being is a large component of the family
environment and creates a positive environmental force in the lives of individuals with
DS (Hauser-Cram et al., 1999). In this way, positive comments seen in themes from
the mothers FMSS group suggest a positive related home environment. Presumably,
this “advantage” has had a long-term positive impact for the individual with DS.
Siblings
As adults with DS live increasingly longer lives, their adult siblings often assume
caregiving responsibilities. Literature has demonstrated a similar “advantage” for these
sibling caregivers of individuals with DS as seen with mothers (Hodapp et al., 2007).
Themes from the sibling group in this study are consistent with these findings and
contribute to the dearth of information about sibling caregivers of adults with DS.
Dementia
Research looking at the effect of life-long caregiving on the caregiver has shown that
most families adapt well to having a family member with disabilities (Seltzer et al.,
2001). Themes from this sample depict the positive emotions as well as the
apprehension and worry associated with caring for an aging family member with DS.
This is also an essential area for future research.
Themes extracted from all three groups bear witness to the unique characteristics of
caring for an aging individual with DS. As mentioned previously, this is an area where
there is a large need for further investigation. Parents and siblings in this study
described characteristics and experiences related to the DS phenotype and
“advantage,” and further research will potentially discover that individuals with DS have
benefitted from this enriching environment throughout their lives.
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