Few studies have reported on the effect of @entity1120 on @entity1 -reported, health-related quality of life (HRQoL) and we do not know how the HRQoL burden of @entity1120 compares to that of other chronic conditions, what areas of HRQoL are most affected or how the course of change in HRQoL compares with that observed for other conditions or for the general adult population. The purpose of this study was to estimate (1) the HRQoL burden associated with @entity1120 managed by enzyme replacement therapy (ERT), (2) recalled changes in HRQoL since ERT initiation and (3) risk factors predictive of HRQoL outcomes. We sampled 212 @entity1 with @entity1120 recruited from 146 physicians prescribing ERT in the US. The @entity1 were at least 14 years of age and had been on ERT from 1 to 51 months. The mean (SD) age of the @entity1 was 45 (17) years. Forty-nine percent had had a prior splenectomy and 26% had had a joint replacement. We administered the SF-36 Health Survey (SF-36) and three questions about changes in physical, mental and general HRQoL since starting ERT. The @entity1 with @entity1120 scored significantly worse than the age- and gender-adjusted US norms on five of the eight SF-36 subscales (p < 0.05). Age (p < 0.0001) and joint replacement (p < 0.001) were negatively associated with physical health. The presence of an intact spleen (p < 0.01) and a longer duration of ERT (p < 0.01) were associated with better mental health. When asked about changes in HRQoL since starting ERT, at least half of the @entity1 reported fewer limitations in physical activities (53%), better general health perceptions (77%) and @entity549 (49%) at the time of the interview. @entity1 who had been receiving ERT for approximately 4 years recalled four and five times more @entity283 in comparison with recalled changes over a 4 year period among adults in the general population (p < 0.001) and a @entity712 population (p < 0.01), respectively. Odds ratios (ORs) revealed that female @entity1 were more likely to report improvements in general HRQoL than males (OR = 4.50 and 95% CI = 2.19-9.25) and 45 year old @entity1 were less likely to report improvements than 35 year olds (OR = 0.76 and 95% CI = 0.62-0.94). Relative to @entity1 who had been receiving ERT for 1 year, those who had been receiving ERT for 2 and 4 years were 1.40 (95% CI = 1.06-1.84) and 2.75 (95% CI = 1.20-6.27) times more likely to report improvements in general HRQoL, respectively. In summary, @entity1 with @entity1120 on ERT reported an improvement in HRQoL that was greater than that reported by @entity1 with other @entity102 . However, @entity1120 @entity1 treated for up to 51 months scored below equivalent adults in the general population. The risk factors, including age and history of splenectomy and joint replacement, warrant further study. Standardized HRQoL measures are likely to prove useful in understanding better the outcomes from the @entity1120 @entity1 's perspective.

The health-related quality of life of adults with XXXX receiving enzyme replacement therapy: results from a retrospective study.

@entity1 :: ('9606', 'Species') :: ['participants', 'Patients', 'patients', 'patient'] @entity712 :: ('MESH:D006333', 'Disease') :: ['congestive heart failure'] @entity283 :: ('OMIM:603663', 'Disease') :: ['improvement in general HRQoL'] @entity102 :: ('MESH:D002908', 'Disease') :: ['chronic diseases'] @entity549 :: ('MESH:D064726', 'Disease') :: ['less negative emotions'] @entity1120 :: ('MESH:D005776', 'Disease') :: ["Gaucher's", "Gaucher's disease"]
@entity1120 :: (MESH:D005776,Disease) :: ['Gaucher's disease']
