Is functional dependence of Duchenne muscular dystrophy patients determinant of the quality of life and burden of their caregivers?

Moura, Maria Clara Drummond Soares de; Wutzki, Hanna C.; Voos, Mariana Callil; Resende, Maria Bernadete Dutra de; Reed, Umbertina Conti; Hasue, Renata Hydeé

doi:10.1590/0004-282x20140194

Cited by 34 publications

(40 citation statements)

References 29 publications

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“…One French study (Martigne [28]) reported a mean age of ambulation loss of 10 years. Unsurprisingly similar results were found in six studies reporting wheelchair use, four as percentage [29–32] and two as time to first use [19, 33]. Percentage use and time to use were unsurprisingly similar to those for loss of ambulation.…”

Section: Resultssupporting

confidence: 74%

“…The percentage of all DMD patients on assisted ventilation varied very widely from 0% in a Brazilian study of boys (mean age 11 years) by de Moura [29],0.7% in a multinational European study (mean age 13 years) by Vry [40] and 22% in a Japanese study (mean age not reported but most individuals described as less than 20 years old) (Nakamura [26]). Variation by disease progression was shown in the US study by Mayer [27] with a gradual decline from 126.6% predicted forced vital capacity (FVC) in the under 6 years age group to 7.3% predicted FVC in those aged 20 to 22 years.…”

Section: Resultsmentioning

confidence: 99%

“…Simon [55] reported the Life Satisfaction Index for Adolescents (LSI-A) in four age groups of boys in Brazil, Baiardini [56] reported the Children Health Questionnaire - Parent Form 50 in Italian boys, Bendixen [51] reported the CAPE (0 to 5 scale) in the US and Canada, de Moura [29] reported the Autoquestionnaire Qualité de vie Enfant Imagé (AUQEI) in Brazil and Houwen-van Opstal [57] reported the KIDSCREEN-52 physical domain in the Netherlands. Interestingly, Pangalila [54] concluded that adults with DMD are ‘generally satisfied with their overall quality of life.’ Also, there was little variation according to stage as shown in both Houwen-van Opstal [57] on the KIDSCREEN instrument and Simon [55] on the LSI-A instrument with no clear trend.…”

Section: Resultsmentioning

confidence: 99%

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The burden, epidemiology, costs and treatment for Duchenne muscular dystrophy: an evidence review

Ryder

Leadley

Armstrong

et al. 2017

Orphanet J Rare Dis

348

323

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BackgroundDuchenne Muscular Dystrophy (DMD) is a rapidly progressive, lethal neuromuscular disorder, present from birth, which occurs almost exclusively in males. We have reviewed contemporary evidence of burden, epidemiology, illness costs and treatment patterns of DMD.This systematic review adhered to published methods with information also sought from the web and contacting registries. Searches were carried out from 2005 to June 2015. The population of interest was individuals with clearly defined DMD or their carers.ResultsNine thousand eight hundred fifty titles were retrieved from searches. Fifty-eight studies were reviewed with three assessed as high, 33 as medium and 22 as low quality. We found two studies reporting birth and four reporting point prevalence, three reporting mortality, 41 reporting severity and/or progression, 18 reporting treatment patterns, 12 reporting quality of life, two reporting utility measures, three reporting costs of illness and three treatment guidelines.Birth prevalence ranged from 15.9 to 19.5 per 100,000 live births. Point prevalence per 100,000 males was for France, USA, UK and Canada, 10.9, 1.9, 2.2 and 6.1 respectively. A study of adult DMD patients at a centre in France found median survival for those born between 1970 and 1994 was 40.95 years compared to 25.77 years for those born between 1955 and 1969. Loss of ambulation occurred at a median age of 12 and ventilation starts at about 20 years. There was international variation in use of corticosteroids, scoliosis surgery, ventilation and physiotherapy. The economic cost of DMD climbs dramatically with disease progression – rising as much as 5.7 fold from the early ambulatory phase to the non-ambulatory phase in Germany.ConclusionsThis is the first systematic review of treatment, progression, severity and quality of life in DMD. It also provides the most recent description of the burden, epidemiology, illness costs and treatment patterns in DMD. There are evidence gaps, particularly in prevalence and mortality. People with DMD seem to be living longer, possibly due to corticosteroid use, cardiac medical management and ventilation. Future research should incorporate registry data to improve comparability across time and between countries and to investigate the quality of life impact as the condition progresses.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-017-0631-3) contains supplementary material, which is available to authorized users.

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Section: Resultssupporting

confidence: 74%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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The burden, epidemiology, costs and treatment for Duchenne muscular dystrophy: an evidence review

Ryder

Leadley

Armstrong

et al. 2017

Orphanet J Rare Dis

348

323

View full text Add to dashboard Cite

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“…It is usual that the mother takes on the job of the main caregiver of patients with DMD. Studies conducted in Brazil demonstrated that the mother is the main caregiver of children with DMD [18,19]. Data from our group confirm these findings.…”

Section: The Caregiver In Duchenne Muscular Dystrophysupporting

confidence: 88%

Caregivers’ sleep of Duchenne Muscular Dystrophy’s patients: a short review

Kt¹,

Editorial²,

Tufik³

et al. 2017

Transl Brain Rhythmicity

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The task of a caregiver might exert several effects upon his/her physical and psychological health. It should be highlight the role of the caregiving mother (CM) of patients with Duchenne Muscular Dystrophy (DMD). DMD is a genetic-character disease with a prevalence of 1 to every 3,500 boys live births and with high mortality rate. The disease´s progression significantly compromises the quality of life and mainly the patient´s independence, making it indispensible the presence of a caregiver. The overload of physical and emotionoal work, including nocturnal periods, may interfere with the CM´s sleep. CM with a son with DMD demonstrated lower sleep efficiency, higher sleep latency, and reported poor sleep quality. These modifications might negatively interfere with health and quality of life of these CMs. Therefore, the evaluation of sleep pattern and quality in this subjects is most important and essential, being for their own global health, as well as for the patient´s well-being. We point out the need for a major interest from the scientific community and health professionals in better understand the impact of chronic diseases on health and quality of life of caregivers and patients..

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“…In total, seven articles measured overall caregiver burden, [9][10][11][12][13][14]22 five HRQoL, [10][11][12][13][14] four stress, 14,[16][17][18] six work life, informal care, and/or associated cost burden, 11,[19][20][21][22]28 five family problems and functioning, 9,14,15,23,24 six sleep, 13,15,21,[25][26][27] five pain and/or discomfort, 12,13,21,23,24 two strain, 13,28 nine anxiety and/or depression, [12][13][14][15]23,24,[26][27][28] two overall caregiver health, 23,24 one self-esteem and mastery (i.e. that life chances are under one's own control), <...>…”

Section: Resultsmentioning

confidence: 99%