Table 1 Demographic profile of patients and caregivers
Patient profile | Patients with severe cognitive impairment (n = 8) | Patients with low or no cognitive impairment (n = 17) | |||
|---|---|---|---|---|---|
MPS I (np = 3) | MPS VI (np = 5) | MPS I (np = 8) | MPS II (np = 4) | MPS VI (np = 5) | |
Gender | |||||
Men | 2 | 5 | 2 | 4 | 4 |
Women | 1 | 0 | 6 | 0 | 1 |
Age | |||||
17–20 years | 1 | 2 | 2 | 0 | 1 |
21–34 years | 1 | 2 | 2 | 3 | 2 |
29–50 years | 1 | 1 | 4 | 1 | 2 |
Education | |||||
Preschool | 2 | 3 | 0 | 0 | 0 |
Primary school | 1 | 2 | 0 | 0 | 0 |
High school | 0 | 0 | 1 | 0 | 0 |
Vocational certificate | 0 | 0 | 2 | 0 | 2 |
Higher education | 0 | 0 | 5 | 3 | 3 |
Disability-friendly class | 0 | 0 | 0 | 2 | 0 |
Access to specialised classes | 2 | 1 | 0 | 0 | 0 |
Current living location | |||||
Complete dependency on external institutes* | 3 | 4 | 0 | 0 | 0 |
Complete dependency on parenteral home | 0 | 1 | 3 | 2 | 2 |
Living alone | 0 | 0 | 4 | 1 | 3 |
Living in a shared home | 0 | 0 | 1 | 1 | 0 |
Age of patient at diagnosis (years) | |||||
< 1 year | 1 | 0 | 4 | 0 | 0 |
1–10 | 2 | 5 | 4 | 9 | 3 |
> 10 | 0 | 0 | 3 | 0 | 2 |
Other family members diagnosed with MPS | 0 | 0 | 1 | 1 | 2 |
Caregiver profile | Patients with severe cognitive impairment (nc = 8) | Patients with low or no cognitive impairment forms (nc = 17) | |||
|---|---|---|---|---|---|
Relationship with patient | |||||
Father | 1 | 1 | |||
Mother | 7 | 9 | |||
Spouse | 0 | 1 | |||
Age of the parent interviewed (years)c | |||||
< 50 | 2 | 6 | |||
50–60 | 4 | 1 | |||
61–70 | 0 | 2 | |||
≥ 71 | 0 | 2 | |||
ND | 2 | 0 | |||
Professional activity | |||||
Yes (Part time/Full time) | 7 | 5 | |||
No (Retired) | 1 | 4 | |||
ND | 0 | 2 | |||
Marital status | |||||
Married | 3 | 6 | |||
Separated/widower | 5 | 3 | |||
ND | 0 | 2 | |||
Number of children | |||||
1 | 3 | 3 | |||
2 | 4 | 6 | |||
3 | 1 | 2 | |||