Table 3 Studies describing the lived experiences
From: Health, wellbeing and lived experiences of adults with SMA: a scoping systematic review
Reference/ country | Study design | Sample | Key findings | QUALSYST scorea |
|---|---|---|---|---|
Ho et al, 2016 [69] Taiwan | Qualitative study Purposive sampling of cross-sectional cohort | Adults (Age = 25–54 years, mean 34.4) N = 9, (2 SMA II; 7 SMA III) Mandarin/Taiwanese speaking | Experienced a loss of control from declining muscular strength and independence. Utilised assistive devices and environmental manipulation to maximise function. Transcended limitations through striving to maximise independence and continue achieving key financial, educational and relational goals. | 0.9 |
Lamb & Peden, 2008 [70] USA | Qualitative study Recruitment through patient support group | Adults (Age = 26+ years) N = 11 (4 SMA II; 7 SMA III) English speaking | People with SMA utilised creative and innovative methods for overcoming physical challenges. Maintaining strong relationships with family, friends and community and an optimistic life view was important. | 0.8 |
Jeppesen et al., 2010 [68] Denmark | Cross-sectional mixed methods design, semi-structured survey with narrative inquiry | Adults ≥ 18 years N = 29 (All SMA II) | People with SMA faced multiple difficulties but managed to achieve large landmark “goals/achievements”; are in a state of striving to maintain optimism in spite of constant stressors. | 0.79/0.75 |
Hunter et al., 2016 [67] USA | Qualitative study Purposive sampling | Adults (Age = 18–69 years, mean = 34) N = 15 (5 SMA II; 10 SMA III) | Most concerning issues included personal hygiene, dressing, walking and independence. Social and emotional issues included mainstreaming into society and self-confidence. | 0.65 |
Qian et al., 2015 [116] USA | Qualitative study Purposive sampling | Children and adults with SMA (Age = 8–46 years, 33% were > 18 years). N = 21 (1 SMA I; 8 SMA II; 12 SMA III) Parents of people with SMA (N = 64) | SMA left a constant psychological threat of disease progression and premature death. High level of burden included social limitations, difficulties achieving independence, and financial pressures. | 0.82 |
Rouault et al., 2017 [60] Countries across Europe | Cross-sectional survey Purposive sampling | People with SMA (n = 436; 2–65 years; 52% ≥ 19 years); Parents of people with SMA (n = 370); Other or unknown(N = 16) | Activities with the biggest impact on QoL were: ‘using the rest-room independently’, ‘self-feeding’, ‘turning in bed’, ‘washing’ and ‘transferring independently’. | 0.87 |
Kruitwagen-van Reenen et al., 2018 [111] The Netherlands | Cross-sectional surveys Recruitment through patient support groups and clinics. | Adults with SMA (20–70 years); n = 62 (4 SMA I; 21 SMA II; 13 SMA IIIa; 20 SMA IIIb; 4 SMA IV) | People with early onset SMA experience more participation restrictions but similar levels of satisfaction compared with people with later onset SMA. Motor skills, feelings of depression and fatigue are correlates of participation in daily life. | 0.93 |
Mongiovi et al., 2018 [27] 34 countries world-wide | Cross-sectional study Recruitment through the International SMA Patient Registry | Adults with SMA (18–81 years); n = 359 (16 SMA I; 132 SMA II; 144 SMA type III; 30 SMA IV; 34 unknown) | Limitations with mobility or walking, inability to do activities, weakness, and fatigue. Limitations with mobility had the greatest impact on the lives of adults with SMA. | 0.87 |
Wan et al., 2019 [117] Australia | Qualitative study Purposive sampling | Adults and adolescents with SMA, parents and partners of people with SMA; n = 25 (19 people with SMA, 5 parents, 1 partner) | Participants report widespread unmet physical and mental healthcare needs, disengagement from adult healthcare services, as well as pride in resilience, personal accomplishments and social relationships. | 0.93 |