The meaning of living with myotonic dystrophy for couples

Myotonic dystrophy type I is the most common type of muscular dystrophy in
adults. This hereditary progressive neuromuscular disorder has been recognized
as a multisystemic disorder with various impairments, especially in the
muscular, respiratory, cardiac, central nervous, endocrine and ocular systems.
Typical symptoms of the disease include myotonia and progressive loss of muscle
strength, usually distal to proximal, and also weakness of facial and anterior
neck muscles. Other symptoms include cataracts, frontal boldness, dysarthria,
fatigue and daytime somnolence. Also cognitive decline and specific personality
traits are related to myotonic dystrophy. Myotonic dystrophy often results in
restrictions in the performance of daily activities and participation in social
roles. By comparison with other types of muscular dystrophy, persons with
myotonic dystrophy have the greatest functional disabilities and lower social
participation. When learning about the disease, persons are told that it is
hereditary, progressive and that there is no treatment. Often, the problems in
the performance of daily occupations increase until work is not possible
anymore and the lifestyle becomes increasingly passive. Persons with the
disease often avoid talking to others about their problems and have the
impression that professionals cannot help and have little knowledge about the
disease. The worst part is lack of understanding by other people.
A chronic disease like myotonic dystrophy also has major consequences for the
life of the next of kin. Worries and sorrow, a reduced social network and
emotional loneliness with the added burden of having to do the practical tasks
are some of the experiences of the next of kin. Often there is a need to
renegotiate and reinterpret family roles and responsibilities, including those
commonly associated with gender. Husbands, more often, restrict their
involvement to a problem-solving role and do not seem to take on the same
emotional burden compared to wives. Clearly the illness becomes, to a great
extent, integrated into the life of the next of kin. When one lives close to
someone with a slowly progressive disease like myotonic dystrophy, adjustments
are made constantly over time, often without reflection. Both the person with
the disease and their next of kin go through a process of learning to live
within new limits and adapting to the changes. More attention for partner
relationship was mentioned as an important topic for research among persons
with neuromuscular diseases and their partners. An example included problems
associated with daytime sleepiness and impaired initiative, which has major
impact on the person having the disease, their next of kin and for both as a
couple.
Despite the impact of myotonic dystrophy on both the person having the disease
and the partner, these couples hardly complain and do not easily make use of
rehabilitation services. One of the reasons may be that they have been told
that the disease cannot be cured and that they have the impression that nothing
can be done. Also the feeling that doctors know awfully little and generally
people do not understand may contribute to the fact that they don*t ask for
help or support. Professionals and others often focus primarily on the problems
and needs of the person with myotonic dystrophy and overlook the support needs
of next of kin.
In order to effectively target the interventions to promote participation and
enhance quality of life, it is necessary to understand the unique meaning of
participation in everyday occupations for a person and the impact of the
disease on the participation. Because a chronic disease like myotonic dystrophy
also has impact on the life of the next of kin, and for the couple, these
perspectives also need to be understood. This understanding cannot be observed,
but can only be acquired by listening to the persons telling about it.

The purpose of this study is to increase our understanding of the meaning of
myotonic dystrophy for both the person having the disease as well as for the
next of kin as for both as a couple. An additional aim is to gain understanding
of how these persons, as a couple and individually have adapted to the changes
as a result of living with myotonic dystrophy. This understanding will
contribute to improve the rehabilitation for couples with myotonic dystrophy.

Design: a qualitative study with in-depth interviews based on grounded theory
as well as hermeneutics.
Three interviews are planned with each couple, the first interview with the
person with myotonic dystrophy and the next of kin separately and a follow up
interview with the couple together. Interviews are conducted with the aid of an
interview guide. Preceding the interviews, small talk and an explanation of the
purpose and format of the interview will enable the interviewer and interviewee
to become acquainted with each other. By way of introduction, the participants
are asked to describe an ordinary day and their satisfaction with the current
routines. Other topics include the roles and relations, context of performance,
changes in occupational performance resulting from disease progression, the
meaning of these changes and the adaptations to these changes in roles and
routines and thoughts about the future. Each interview wil lasty one to a
maximum of two hours. Following the interviews the interviewers writes memos on
the impressions and reflections of the interview.
Initially a pilot interview will be conducted with a couple. The experiences
are used to adapt the interview guide. Then two other couples are interviewed.
The interviews are audio taped with the permission of the participants and then
transcribed in full for qualitative analysis. Audiotapes and memos are used for
preliminary analysis of each interview. The results of this preliminary
analysis are used to guide interviews with three more couples. Ideally the data
collection continues until saturation. In this study five couples will be
interviewed and whether or not saturation was reached will be addressed.
Data-analysis: data from the transcribed interviews and memos are analyzed
according to the constant comparative method. Concepts will be in vivo codes as
well as sensitizing concepts. The following analytical procedure will be
carried out: multiple readings of interview transcripts to gain understanding
of each participant*s experiences, codes and meaning units are identified and
related to the research questions (open coding), grouping and categorization of
meaning units will enhance understanding of the participant*s experiences
(axial coding), and core categories will be looked for by comparing,
reorganizing and interpreting (selective coding). Finally new insights will be
uncovered.
MaxQDA computer software will be used to assist in organizing the data
(www.MAXQDA.com ).

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