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Living with deteriorating and hereditary disease: experiences over ten years of persons with muscular dystrophy and their next of kin
Örebro University, Department of Nursing and Caring Sciences.
2005 (English)Doctoral thesis, comprehensive summary (Other academic)Alternative title
Att leva med en progressiv och ärftlig sjukdom : erfarenheter utifrån ett tioårsperspektiv från personer med muskeldystrofi och deras närmaste anhörig (Swedish)
Abstract [en]

The overall aim of this thesis was to elucidate haw persona with muscular dystrophy (MD) and their next of kin experience and describe their daily lives over the last ten years. MD is a group of inherited disorders characterised by muscular weakness caused by muscle wasting. Both qualitative and quantitative methods were used. Forty-isx individuals with MD and 36 next of kin were interviewed (Studies I, III, IV). Both groups completed the questionnaire Subjective Quality of Life (SQoL), (Study V). Persons with MD also completed the questionnaires Sickness Impact Profile and Self-report ADL (Study II). The interviews were subjected to qualitative content analysis. The persons with MD described the change from being independent to being an individual in need of assistive devices and/or personal support to manage activities of every day life (StudyI). Besides muscular weakness persons with MD have hte strain it means to have a hereditary disease, which also affects the family (Study III). The deterioration during a decennium was primarily with regard to ambulation. The number of persons walking without assistive devices has decreased from 40 to 23 and that of those working has almost been halved, from 21 to 11 (Study II). Next of kin have a special vulnerability in form of an emotional and practical lonliness in addition to actual and anticipatory losses. The relationship was described as related to obligation and/or love. To have an orientation towards tasks (obligation) was linked to a feeling of being weighed down by demands, while having an orientation towards relationship (love) was linked to a feeling of quality in life (Study IV). The persons with MD scored lower on most items in the SQoL than the next of kin (Study V). Patients without a partner scored lower on QoL as a whole than those with a partner. Those with an early onset of disease scored lower on personal economy. Next of kin who gave daily help assessed their relationships to friends lower than those who gave weekly help (Study V). This thesis provides knowledge of the trajectory with MD over time on the basis of a ten-year follow up. It also broadens the knowledge of how the hereditary aspect is experienced both by those with MD and their next of kin. How the next of kin of adult persons with MD experience their situation is an issue that has been overlooked in previous research.

Place, publisher, year, edition, pages
Örebro: Örebro universitetsbibliotek , 2005. , p. 88
Series
Studies from The Swedish Institute for Disability Research, ISSN 1650-1128 ; 15
Keywords [en]
Social sciences, muscular dystrophy, chronic disease, ten year follow up, next of kin, ICF, activity, sickness impact, hereditary aspects, illness related problems, quality of life, multimethod, content analysis
National Category
Social Sciences Social Work
Research subject
Disability Research
Identifiers
URN: urn:nbn:se:oru:diva-112ISBN: 91-7668-427-X (print)OAI: oai:DiVA.org:oru-112DiVA, id: diva2:134670
Public defence
2005-06-03, Wilandersalen, Universitetssjukhuset, Örebro, 13:00
Opponent
Supervisors
Available from: 2005-05-04 Created: 2005-05-04 Last updated: 2017-10-18Bibliographically approved
List of papers
1. Living with a chronic deteriorating disease: the trajectory with muscular dystrophy over ten years
Open this publication in new window or tab >>Living with a chronic deteriorating disease: the trajectory with muscular dystrophy over ten years
2004 (English)In: Disability and Rehabilitation, ISSN 0963-8288, E-ISSN 1464-5165, Vol. 26, no 23, p. 1388-1398Article in journal (Refereed) Published
Abstract [en]

Purpose: The aim of the study was to elucidate experiences of living with muscular dystrophy in terms of consequences for activity over 10 years. Methods: The study population was identified in a prevalence study in a county of Sweden. Forty-six persons of this cohort with MD were interviewed. A qualitative research approach was chosen. The World Health Organization's International Classification of Functioning, Disability and Health (ICF) was used for categorization. Results: Nearly all the subjects experienced a deterioration of physical capacity. Most obvious were the restrictions on mobility and increased fatigue and feebleness. The persons described psychosocial consequences of the muscular dystrophy as well as stigma when the disability had become more obvious. In spite of reported distress several persons experienced better psychological adaptation over time. The image of the future was often dark but several focus on today and avoid thoughts about the future. ICF showed some limitation with regard to temporal aspects, emotions and the subjective perspective. Conclusions: The knowledge of the trajectory with MD is important in order to offer the best possible treatment and support. Repeated assessment by ICF can serve as a valuable source of such knowledge, and a development of the classification would increase its usefulness in future analysis of functioning and disability.

National Category
Nursing Social Sciences Interdisciplinary
Research subject
Nursing Science
Identifiers
urn:nbn:se:oru:diva-2818 (URN)10.1080/0963-8280400000898 (DOI)
Available from: 2005-05-04 Created: 2005-05-04 Last updated: 2018-01-13Bibliographically approved
2. Sickness impact in people with muscular dystrophy: a longitudinal study over 10 years
Open this publication in new window or tab >>Sickness impact in people with muscular dystrophy: a longitudinal study over 10 years
2005 (English)In: Clinical Rehabilitation, ISSN 0269-2155, E-ISSN 1477-0873, Vol. 19, no 6, p. 686-694Article in journal (Refereed) Published
Abstract [en]

OBJECTIVE: To describe changes of function in terms of sickness impact over 10 years in adult patients with different types of muscular dystrophy. DESIGN: Patients with muscular dystrophy answered the Sickness Impact Profile and Self-report ADL questionnaires in 1991 and 2001. SETTING: The study population was identified in a comprehensive prevalence study in the county of Orebro, Sweden. SUBJECTS: The study group comprised 44 people grouped according to whether they had myotonic dystrophy or muscular dystrophy with proximal or distal muscles affected. MAIN MEASURES: Comparison was made between assessments of sickness impact in terms of function at the two time points. RESULTS: Most obvious deterioration over time was in activities of daily living that require finger and arm strength. Ambulation was significantly decreased in myotonic dystrophy and proximal muscular dystrophy. Those walking without assistive devices decreased from 91% to 52%, and the number with a disability pension increased from 36 to 55%. There was a relatively small influence with regard to psychosocial dysfunction assessed by the Sickness Impact Profile. CONCLUSIONS: This longitudinal study shows the deteriorating functions reported by patients with muscular dystrophy. This knowledge could be used to formulate new interventions in order to offer appropriate support and treatment to this patient group.

Place, publisher, year, edition, pages
London: Edward Arnold, 2005
National Category
Social Sciences Interdisciplinary Nursing
Research subject
Nursing Science
Identifiers
urn:nbn:se:oru:diva-2819 (URN)10.1191/0269215505cr866oa (DOI)16180606 (PubMedID)
Note
Artikelns titel i avhandlingen: Sickness impact among persons with muscular dystrophy: a longitudinal study over ten yearsAvailable from: 2005-05-04 Created: 2005-05-04 Last updated: 2018-01-13Bibliographically approved
3. Living with a hereditary disease: persons with muscular dystrophy and their next of kin
Open this publication in new window or tab >>Living with a hereditary disease: persons with muscular dystrophy and their next of kin
2005 (English)In: American Journal of Medical Genetics, ISSN 0148-7299, E-ISSN 1096-8628, Vol. 136A, no 1, p. 17-24Article in journal (Refereed) Published
Abstract [en]

This qualitative study describes conceptions and experiences of the hereditary aspect of muscular dystrophy (MD) from both the patients' and the next of kin's perspective. Different diagnoses of MD are included: dystrophia myotonica, myopathia distalis tarda hereditaria, Becker MD, facioscapulohumeral MD, limb-girdle MD, Emery-Dreifuss and undetermined proximal MD (Duchenne MD is not included). Interviews were conducted with 46 persons with MD and 36 next of kin. The interviews were subjected to inductive content analysis. Only two in each group did not spontaneously mention anything related to the fact that MD is disease with dominant or recessive inheritance. It was found that heredity has a prominent place in the thoughts and feelings of the family. These thoughts were classified as Becoming aware of MD and its hereditary nature, looking into the pedigree, acquiring an understanding of MD, thoughts about genetic testing, interpreting the risk, whether to have children or not, feelings related to the future, and feelings of responsibility and guilt. Families with MD need medical information and the opportunity for genetic testing as well as support and counseling in coming to terms with living with a hereditary disease, whether or not that includes a decision to take a test.

Place, publisher, year, edition, pages
Hoboken, N.J.: Wiley-Liss, 2005
National Category
Social Sciences Interdisciplinary Nursing
Research subject
Nursing Science
Identifiers
urn:nbn:se:oru:diva-2820 (URN)10.1002/ajmg.a.30762 (DOI)15889411 (PubMedID)
Available from: 2005-05-04 Created: 2005-05-04 Last updated: 2018-01-13Bibliographically approved
4. Being kin of an adult person with muscular dystrophy
Open this publication in new window or tab >>Being kin of an adult person with muscular dystrophy
(English)Manuscript (preprint) (Other academic)
National Category
Social Sciences Interdisciplinary Nursing
Research subject
Nursing Science
Identifiers
urn:nbn:se:oru:diva-2821 (URN)
Available from: 2005-05-04 Created: 2005-05-04 Last updated: 2018-01-13Bibliographically approved
5. Quality of life in patients with muscular dystrophy and their next of kin
Open this publication in new window or tab >>Quality of life in patients with muscular dystrophy and their next of kin
2005 (English)In: International Journal of Rehabilitation Research, ISSN 0342-5282, E-ISSN 1473-5660, Vol. 28, no 2, p. 103-109Article in journal (Refereed) Published
Abstract [en]

The aims of this study were to investigate quality of life (QoL) among adult patients with muscular dystrophy (n=46) and their next of kin (n=36) and to investigate the influence of disease-related and demographic factors on QoL. The questionnaire 'Subjective estimation of quality of life' was used. The results show that patients had lower QoL than their next of kin regarding having no work or meaningful occupation, energy, self-assuredness, self-acceptance and emotional experiences. Age of onset of disease had an impact on QoL. The need for a ventilator had an influence only on assessment of energy. Patients without a partner assessed lower than those who had a partner. In the case of a person who is young and single the onset of muscular dystrophy reduces the likelihood of having a partner or children and affects personal economy negatively. Assessment of relationship to friends was lower among next of kin who provided daily help than among those who provided help once a week. There is a need for recurrent rehabilitation during life-long disabilities and a need to give particular support to those with early onset of disease, those who are single and those who are childless. It is also important to include the patient's close relations when giving rehabilitation.

National Category
Social Sciences Interdisciplinary Nursing
Research subject
Nursing Science
Identifiers
urn:nbn:se:oru:diva-2822 (URN)15900179 (PubMedID)
Available from: 2005-05-04 Created: 2005-05-04 Last updated: 2018-01-13Bibliographically approved

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