Background
Methods
Quality of Life (QoL) Survey
SMA awareness video contest
Results
Quality of life survey
Participation rates and sample characteristics
Total age cohort | Adolescents (12–18) | Young adults (19–24) | |
---|---|---|---|
Total | 85 | 44 (52%) | 41 (48%) |
SMA type | |||
Type I | 6 (7%) | 4 (5%) | 2 (2%) |
Type II | 56 (66%) | 28 (33%) | 28 (33%) |
Type III | 23 (27%) | 12 (14%) | 11 (13%) |
Gender | |||
Female | 53 (62%) | 28 (33%) | 25 (29%) |
Male | 31 (37%) | 15 (18%) | 16 (19%) |
Non-binary | 1 (1%) | 1 (1%) | 0 |
Motor function at time of diagnosisa | |||
Non-independent sitter | 25 (29%) | 13 (15%) | 12 (14%) |
Sitter/non-independent walker | 37 (44%) | 20 (24%) | 17 (20%) |
Walker | 23 (27%) | 11 (13%) | 12 (14%) |
Motor function at time of survey completion* | |||
Non-independent sitter | 31 (37%) | 17 (20%) | 14 (17%) |
Sitter/non-independent walker | 40 (47%) | 19 (22%) | 21 (25%) |
Walker | 14 (16%) | 8 (9%) | 6 (7%) |
What is the most difficult aspect of balancing SMA symptoms with everyday life?
What is the most difficult aspect of balancing your SMA/SMA symptoms with everyday life? | |||||||
---|---|---|---|---|---|---|---|
Age | Motor function | ||||||
Adolescents n = 44% (95% CI) | Young Adults n = 41% (95% CI) | p value | Non independent sitter n = 31% (95% CI) | Sitter/non-independent walker n = 40% (95% CI) | Independent walker n = 14% (95% CI) | p value | |
Accessibility | 15.91 (6.64–30.07) | 14.63 (5.57–29.17) | 0.87 | 0 | 20 (9.05–35.65) | 35.71 (12.76–64.86) | 0.002* |
Advocating | 2.27 (0.06–12.02) | 2.44 (0.06–12.9) | 1 | 3.23 (0.08–16.70) | 0 | 7.14 (0.18–33.9) | 0.147 |
Balance | 6.82 (1.43–18.66) | 7.32 (1.54–19.92) | 1 | 3.23 (0.08–16.70) | 12.5 (4.2–26.8) | 0 | 0.256 |
Dependence | 22.73 (11.47–37.84) | 24.39 (12.36–40.30) | 0.857 | 16.13 (5.45–33.73) | 37.5 (22.73–54.20) | 0 | 0.008^ |
Everyday | 9.09 (2.53–21.67) | 9.76 (2.72–23.13) | 1 | 9.68 (2.04–25.75) | 5 (0.61–16.92) | 21.43 (4.66–50.80) | 0.151 |
Fatigue | 6.82 (1.43–18.66) | 17.07 (7.15–32.06) | 0.186 | 19.35 (7.45–37.47) | 7.5 (1.57–20.39) | 7.14 (0.18–33.9) | 0.258 |
Finding care | 0 | 9.76 (2.72–23.13) | 0.05* | 9.68 (2.04–25.75) | 2.5 (0.06–13.16) | 0 | 0.374 |
Mental health | 6.82 (1.43–18.66) | 0 | 0.242 | 6.45 (0.79–21.42) | 0 | 7.14 (0.18–33.9) | 0.181 |
other | 6.82 (1.43–18.66) | 0 | 0.242 | 3.23 (0.08–16.7) | 2.5 (0.06–13.16) | 7.14 (0.18–33.9) | 0.567 |
Pain | 9.09 (2.53–21.67) | 7.32 (1.54–19.92) | 1 | 12.9 (3.63–29.83) | 7.5 (1.57–20.39) | 0 | 0.451 |
Social | 13.64 (5.17–27.35) | 7.32 (1.54–19.92) | 0.486 | 16.13 (5.45–33.73) | 5 (0.61–16.92) | 14.29 (1.78–42.81) | 0.25 |
How does SMA affect schooling?
How does SMA affect your schooling? | |||||||
---|---|---|---|---|---|---|---|
Age | Motor function | ||||||
Adolescents n = 44% (95% CI) | Young Adults n = 41% (95% CI) | p value | Non independent sitter n = 31% (95% CI) | Sitter/non-independent walker n = 40% (95% CI) | Independent walker n = 14% (95% CI) | p value | |
Accessibility | 11.36 (3.79–24.56) | 9.76 (2.72–23.13) | 0.55 | 6.45 (0.79–21.42) | 7.5 (1.57–20.39) | 28.57 (8.39–58.10) | 0.077 |
Activity limitation | 13.64 (5.17–27.35) | 2.44 (0.06–12.9) | 0.11 | 3.23 (0.08–16.70) | 15 (5.71–29.84) | 0 | 0.163 |
Aid | 9.09 (2.53–21.67) | 12.2 (4.08–26.20) | 0.733 | 12.9 (3.63–29.83) | 12.5 (4.2–26.8) | 0 | 0.457 |
Everyday | 11.36 (3.79–24.56) | 4.88 (0.60–16.53) | 0.435 | 9.68 (2.04–25.75) | 2.5 (0.06–13.16) | 21.43 (4.66–50.80) | 0.073 |
Fatigue | 2.27 (0.06–12.02) | 17.07 (7.15–32.06) | 0.026* | 6.45 (0.79–21.42) | 12.5 (4.2–26.8) | 7.14 (0.18–33.9) | 0.79 |
Homeschool | 18.18 (8.19–32.71) | 0 | 0.006* | 6.45 (0.79–21.42) | 12.5 (4.2–26.8) | 7.14 (0.18–33.9) | 0.79 |
Keep up | 6.82 (1.43–18.66) | 9.76 (2.72–23.13) | 0.707 | 9.68 (2.04–25.75) | 7.5 (1.57–20.39) | 7.14 (0.18–33.9) | 1 |
More effort | 4.55 (0.56–15.47) | 14.63 (5.57–29.17) | 0.147 | 16.13 (5.45–33.73) | 7.5 (1.57–20.39) | 0 | 0.255 |
No effect | 15.91 (6.64–30.07) | 14.63 (5.57–29.17) | 0.87 | 16.13 (5.45–33.73) | 17.5 (7.34–32.78) | 7.14 (0.18–33.9) | 0.642 |
Other | 4.55 (0.56–15.47) | 7.32 (1.54–19.9) | 0.669 | 9.68 (2.04–25.75) | 2.5 (0.06–13.16) | 7.14 (0.18–33.9) | 0.381 |
Writing | 2.27 (0.06–12.02) | 7.32 (1.54–19.9) | 0.349 | 3.23 (0.08–16.70) | 2.5 (0.06–13.16) | 14.29 (1.78–42.81) | 0.217 |
How does SMA affect socialization?
How does SMA affect your social life? | |||||||
---|---|---|---|---|---|---|---|
Age | Motor function | ||||||
Adolescents n = 44% (95% CI) | Young Adults n = 41% (95% CI) | p value | Non independent sitter n = 31% (95% CI) | Sitter/non-independent walker n = 40% (95% CI) | Independent walker n = 14% (95% CI) | p value | |
Accessibility | 11.36 (3.79–24.56) | 26.83 (14.22–42.94) | 0.068 | 16.13 (5.45–33.73) | 22.5 (10.84–38.45) | 14.29 (1.78–42.81) | 0.708 |
Activity limitation | 18.18 (8.19–32.71) | 14.63 (5.57–29.17) | 0.659 | 12.9 (3.63–29.83) | 15 (5.71–29.84) | 28.57 (8.39–58.10) | 0.423 |
Communication difficulties | 4.55 (0.56–15.47) | 9.76 (2.72–23.13) | 0.349 | 12.9 (3.63–29.83) | 5 (0.61–16.92) | 0 | 0.312 |
Dating | 2.27 (0.06–12.02) | 2.44 (0.06–12.9) | 1 | 0 | 5 (0.61–16.92) | 0 | 0.653 |
Dependence | 6.82 (1.43–18.66) | 7.32 (1.54–19.9) | 1 | 0 | 15 (5.71–29.84) | 0 | 0.04* |
Feeling judged | 11.36 (3.79–24.56) | 0 | 0.056 | 3.23 (0.08–16.70) | 7.5 (1.57–20.39) | 7.14 (0.18–33.9) | 0.705 |
Misunderstood | 6.82 (1.43–18.66) | 12.2 (4.08–26.20) | 0.474 | 19.35 (7.45–37.47) | 2.5 (0.06–13.16) | 7.14 (0.18–33.9) | 0.056 |
No effect | 27.27 (14.96–42.79) | 9.76 (2.72–23.13) | 0.053 | 19.35 (7.45–37.47) | 15 (5.71–29.84) | 28.57 (8.39–58.10) | 0.533 |
No social life | 9.09 (2.53–21.67) | 4.88 (0.60–16.53) | 0.677 | 12.9 (3.63–29.83) | 2.5 (0.06–13.16) | 7.14 (0.18–33.9) | 0.209 |
Other | 2.27 (0.06–12.02) | 12.2 (4.08–26.20) | 0.102 | 3.23 (0.08–16.70) | 10 (2.79–23.66) | 7.14 (0.18–33.9) | 0.542 |
What resources should exist for teens and young adults with SMA?
What resources do you think should exist for teens and young adults with SMA? | |||||||
---|---|---|---|---|---|---|---|
Age | Motor function | ||||||
Adolescents n = 44% (95% CI) | Young Adults n = 41% (95% CI) | p value | Non independent sitter n = 31% (95% CI) | Sitter/non-independent walker n = 40% (95% CI) | Independent walker n = 14% (95% CI) | p value | |
A Cure | 9.09 (2.53–21.67) | 0 | 0.117 | 0 | 5 (0.61–16.92) | 14.29 (1.78–42.81) | 0.116 |
Accessible activities | 6.82 (1.43–18.66) | 0 | 0.242 | 6.45 (0.79–21.42) | 0 | 7.14 (0.18–33.9) | 0.181 |
Accessible transit | 0 | 7.32 (1.54–19.92) | 0.108 | 6.45 (0.79–21.42) | 2.5 (0.06–13.16) | 0 | 0.755 |
Big/little support | 6.82 (1.43–18.66) | 2.44 (0.06–12.9) | 0.617 | 3.23 (0.08–16.70) | 7.5 (1.57–20.39) | 0 | 0.654 |
Caregivers | 0 | 7.32 (1.54–19.92) | 0.108 | 6.45 (0.79–21.42) | 2.5 (0.06–13.16) | 0 | 0.755 |
College/independence materials | 0 | 100.00% | 0* | 12.9 (3.63–29.83) | 12.5 (4.2–26.8) | 14.29 (1.78–42.81) | 1 |
Equipment/technology | 18.18 (8.19–32.71) | 4.88 (0.60–16.53) | 0.057 | 6.45 (0.79–21.42) | 15 (5.71–29.84) | 14.29 (1.78–42.81) | 0.591 |
Other | 2.27 (0.06–12.02) | 4.88 (0.60–16.53) | 0.607 | 3.23 (0.08–16.70) | 5 (0.61–16.92) | 0 | 1 |
Support groups | 40.91 (26.34–56.75) | 36.59 (22.12–53.06) | 0.683 | 38.71 (21.85–57.81) | 42.5 (27.04–59.11) | 28.57 (8.39–58.10) | 0.655 |
Unknown | 6.82 (1.43–18.66) | 9.76 (2.72–23.13) | 0.707 | 9.68 (2.04–25.75) | 5 (0.61–16.92) | 14.29 (1.78–42.81) | 0.516 |
Video games | 9.09 (2.53–21.67) | 0 | 0.117 | 6.45 (0.79–21.42) | 2.5 (0.06–13.16) | 7.14 (0.18–33.9) | 0.502 |
Awareness video contest
Awareness video contest demographics | ||||
---|---|---|---|---|
Age | Gender | Type | Ambulatory status | |
Respondent 1 | 15 | Female | II | Non-ambulant |
Respondent 2 | 15 | Female | II | Non-ambulant |
Respondent 3 | 17 | Female | II | Non-ambulant |
Respondent 4 | 22 | Female | III | Ambulant |
Respondent 5 | 20 | Female | II | Non-ambulant |
Respondent 6 | 17 | Male | II | Non-ambulant |
Awareness video contest topic breakdown (n = 6) | |||
---|---|---|---|
Theme | % of participants addressing theme | Subtheme | Sample quote |
Social engagement | |||
100 | Friendship | “My friends treat me the same way they treat anyone else, that’s why we’re best friends” | |
100 | Key relationship (family, pet, helper) | “My brother is a runner and he runs for me” | |
50 | Lack of social engagement | “No one invited me to be their friend” | |
Everyday life | |||
100 | Schooling | “With school, I have to have a full-time para” | |
100 | Activities | “One of my passions is the arts, I like to paint” | |
83.3 | Everyday routine | “I still do chores, I still help out around the house” | |
Physical health | |||
100% | Mobility | “A wheelchair can’t go that way” | |
100% | Overall disease burden | “I have all the physical demands of being disabled” | |
Emotional health | |||
50 | Frustration | “It’s hard to go upstairs, and I get mad at myself sometimes when I can’t do it” | |
33.3 | Depression | “Realizing how my peers treated me, depression overcame me” | |
Other obstacles | |||
100 | Dependence | “I have to have someone do everything for me” | |
83.3 | Being labeled/treated “disabled” | “Stop with these misconceptions that everyone who is disabled is mentally slow or just sitting around” | |
Overcoming obstacles | |||
100 | Defying other’s expectations | “Just because you can’t physically do something doesn’t mean you can’t follow your dreams” | |
100 | Accepting + adapting | “I’ve learned to adapt and teach myself to write with both hands” |