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The Process of Giving Meaning: Lessons Learned from the Experience of Parents of a Child with Duchenne Muscular Dystrophy

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Title: The Process of Giving Meaning: Lessons Learned from the Experience of Parents of a Child with Duchenne Muscular Dystrophy


1
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2
The Process of Giving Meaning
  • Lessons Learned from the Experience of Parents of
    a Child with Duchenne Muscular Dystrophy

October 10, 2006
3
Objectives
  • To present the rationale for a qualitative study
    of the experience of meaning in the parents of a
    child with DMD
  • To summarize the results of the study
  • To reflect upon the lessons learned in designing
    and carrying out the study
  • To introduce the design of a followup multicentre
    study to study the experience of hope in the same
    families

4
Investigators
  • Pranesh Chakraborty
  • Mireille Choquette
  • Pierre Jacob
  • Sarah Miles
  • Andre Samson
  • Eva Tomiak

5
Background
  • DMD 1/3500 male births
  • Mutation in X linked DMD gene, leading to lack of
    the protein, dystrophin
  • 1/3 mutations are de novo
  • Progressive and incurable neuromuscular disease

6
DMD History
  • Description by Duchenne in 1861
  • -paraplégie hypertrophique de lenfance de cause
    cérébrale
  • Natural history Brooke 1981
  • DMD gene Worton 1984
  • DMD protein, dystrophin (gene product) Hoffman
    1987

7
DMD Clinical Presentation
  • Duchenne initial description
  • Progressive weakness of movements, initially
    affecting the muscles of the lower limbs and
    lower spine, gradually getting worse and
    spreading to the upper limbs.
  • Enlargement of some of the paralysed muscles and
    in some cases almost all.
  • Hyperplasia of interstitial connective tissue in
    the paralysed muscle with production of abundant
    fibrous and adipose tissue in the final stages

8
DMD Evaluation
  • Clinical
  • Biochemical
  • Neurophysiological
  • Radiological
  • Genetic
  • Pathology

9
Clinical
  • Motor delay (proximal)
  • Delay walking
  • Difficulty standing
  • Difficulty with stairs
  • Positive Gowers manoeuvre
  • Cognitive delay (80)
  • ADD/ADHD
  • Muscle hypertrophy
  • Calves, thighs, forearm, masseters, temporalis
  • Cardiomyopathy and respiratory weakness

10
Gowers manoeuvre
11
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12
Biochemical
  • Elevated CK
  • 50-100 x Normal
  • N 0-1 month 30-400 U/L
  • - 50-300 U/L
  • DMD usually well over 5000 U/L
  • Will be elevated on day 1 of life
  • Higher in the preclinical phase and deceases
    afterward as muscle mass decreases
  • Elevated
  • AST, ALT, Aldolase

13
Neurophysiology
  • Nerve conduction studies
  • Electromyographic evaluation
  • Insertion activity
  • Myopathic units
  • Increase recruitment

14
Radiological
  • Ultrasound
  • Muscle MRI/CT

15
Pathology
  • Muscle biopsy
  • Regular stains
  • Necrosis
  • Regeneration
  • Fiber variation
  • Increase fibrous tissue
  • Increase adipose tissue
  • Myopathic changes
  • Specific stains (immuno chemistry)
  • Dystrophyn
  • Sarcoglycan, merosin

16
Immunocytochemical N
17
Immunocytochemical DMD
18
Genetic
  • Deletions Xp21
  • Intragenic deletions of exons 3, 8, 13, 43, 44,
    47, 50, 51 and 52 responsible for 90 of all
    cases.

19
Natural History
20
DMD Phases(NMC functional classification)
  • Early ambulation ( 2-5 years)
  • Late ambulation (6-9 years)
  • Early wheelchair (10-13 years)
  • Late wheelchair (14 years and older)

21
2-5 years (Early Ambulation)
  • Diagnosis
  • Family education
  • Social/psychological support
  • Genetic counseling
  • Therapeutic interventions
  • Orthopedic
  • Respiratory
  • Rehabilitation/physiotherapy/OT
  • Medical

22
6-9 years (Late Ambulation)
  • A Social/psychological support
  • Family
  • School
  • Transportation/teacher aid rephysical/learning

23
6-9 years (Late Ambulation)
  • B. Therapeutic interventions
  • Orthopedic
  • Respiratory-
  • Flu vaccine
  • Pulmonary function
  • Rehabilitation
  • Physiotherapy-
  • Stretching
  • Chest physio
  • Occupational therapy-
  • Orthotics AFO- Night/Day
  • House accessibility
  • Medical-
  • Deflazacort

24
10-13 years (Early Wheelchair)
  • Social/psychological support
  • Therapeutic interventions
  • Orthopedic-
  • Contractures
  • Scoliosis
  • Respiratory-
  • Pulmonary function
  • Aids to ventilation
  • Rehabilitation-
  • Physiotherapy Chest physio, joint mobilization
  • Occupational therapy orthoses, wheelchair
  • Medical-
  • Deflazacort
  • Cardiac

25
14-18 years (Late Wheelchair)
  • Social/psychological support-
  • Therapeutic interventions
  • Orthopedic-
  • Contractures
  • Scoliosis surgery
  • Respiratory-
  • Assisted ventilation
  • Rehabilitation-
  • Physiotherapy- Chest physio/mobilization
  • Occupationnal therapy-
  • equipment adjustment
  • Swallowing
  • Medical-
  • Cardiac

26
Medical therapy
  • Prednisone
  • Definite improvement over placebo
  • 0.3mg/kg/day
  • 0.75/kg/day
  • Improvement as early as 10 days which continued
    to improve over 1 month
  • Plateau that persisted for 6 months
  • Group at 0.75mg/kg/day better than 0.3mg/kg3day
    but more side effects (wt gain, cushingoid, hair
    growth)

27
Medical Therapy
  • Side effects
  • Weight gain- (up to 20 of initial weight)
  • Behavior
  • Appetite
  • Sleep
  • Psychiatric
  • Ophthalmologic-cataracts
  • Endocrine
  • Pituitary
  • Bone density

28
Other Therapies
  • Medical
  • Immunosuppressant
  • Azathioprine
  • Cyclophosphamide
  • Myoblast transfer

29
DMD What should we remember?
  • Medical Rx limited results
  • Better energy
  • Little change in overall disease progress

30
A Clinicians Perspective (1)
  • Since we do not yet have a satisfactory
    treatment for this disease, we are confined to
    trying to provide as much support for the
    patients and families as possible There is a
    wise French aphorism that sums up what we, as
    physicians, must do for our patients Guerir
    quelquefois, soulager souvent, consoler toujours.
    Since, in this disease, we are not able to cure,
    we must be able to assuage symptoms as far as
    possible and help the family deal with both the
    prolonged agony of watching their child
    deteriorate in front of them as well as the
    inevitable loss of that child.

31
A Clinicians Perspective (2)
  • While no family can emerge from the process of
    caring for a child with a fatal disease
    unscathed, some families manage better than
    others. If we can find a way to measure how the
    more successful families cope with the process,
    these approaches and emotional attitudes can
    hopefully be taught to families that are coping
    less well. Trying to identify how parents find
    hope to keep going and, as far as possible,
    accurately describe this process, would be an
    important step.

32
Study Design
  • Qualitative study of couples having a child
    living with DMD
  • Description of the lived experience
  • Semi-structured interviews with 11 parents
  • Mothers and fathers interviewed separately
  • Interviews recorded and transcribed
  • Average length of interview 90 minutes

33
Methods Choice of Methodology(1)
  • Methodology research models Deductive,
    quantitative, inductive, or qualitative
  • The hypothetical-deductive paradigm is based on
    the premise that an independent, objective
    reality exists outside the individual
  • This reality can be observed, measured and
    explained
  • Research aims to identify cause and effect, from
    which universal laws can be deduced

34
Methods Choice of Methodology (2)
  • The holistic-inductive paradigm is characterized
    by the recognition of the subjective nature of
    reality
  • Reality is viewed as a construct of the
    individual based on emotions, values and culture
  • The objective of research is to understand the
    significance to the individual of a particular
    lived experience

35
Methods Data Analysis
  • Empirical Phenomenological Psychological (EPP)
    data analysis method
  • Five stages of analysis

36
Questionnaire Sample Questions
  • When you were first given the diagnosis of DMD
    for your child, what was your first reaction?
  • How did you make sense of what was happening at
    the time of diagnosis? In other words, did you
    see a purpose in that experience?
  • As a couple, how did it affect you? Did you
    discuss your childs illness openly? How did you
    support each other?
  • On reflecting back on your entire experience, do
    you see a purpose in your childs illness? In
    other words, how did it impact you as a human
    being The way you see others? The way you see
    your family? Your own spiritual growth? The way
    you see life in general?

37
Results
  • Gender specific differences in the psychosocial
    adjustment of parents of a child with DMD Two
    points of view for a shared experience

38
Demographic Variables
39
Themes Identified
40
I. Care of the Child
41
HER
  • A lot revolves around K. I have to be there in
    the morning. I have to help her get dressed. I
    have to feed her breakfast. I have to help her
    after the showerOur whole daily life, our whole
    schedule revolves around K
  • We were so immersed and we gave up so much over
    the years
  • Im more gentle with him, pay more attention to
    his needs
  • J. prefers that Mama takes care of him at
    certain times, because Papa is less patient

42
HIM
  • I had the chance to spend time with him, to
    tease him, to put him to bed. Then he began to
    laugh at me, he found me funny It was after that
    that I realized that something had changed. I
    dont know exactly what My attitude, yes. My
    attitude changed, but how did it happen, I cant
    explain.
  • We understood that we are important in the life
    of J. It is important to be with him.

43
II. Career
44
HER
  • I am a mother on a 24 hour basis.. thats my
    work.
  • I think that once the diagnosis came, then you
    kind of put everything on hold for a long period
    of time.
  • There still seems to be a lack of respect for
    the title as a mother.

45
HIM
  • Only time I can switch off now is when I drive
    six hundred miles away.
  • She, she has to stay at home... She is
    full-time here whereas, I get to escape. 
  •  The care of J. is difficult. One doesnt take
    it into consideration, but his care puts an extra
    weight on my shoulders. 
  • The career is not a priority for me. I have to
    think about many other things apart from career.

46
III. Social Support
47
HER
  • When I think about the people who impact my life
    and give me the most pleasure and enjoyment, and
    are the most helpful to me, they are the
    physiotherapist, who became a close friend, the
    massage therapist and I who are like best
    buddiesthe hair dresser who comes to my
    housethe baby sitters that weve had who come by
    in the summer and take her out for no reason.. my
    girlfriends who will come by just drop in, and
    say Hi..
  • I think its good for families to be in touch
    with one another. I know some are open to that
    and some arent, but I really do think its a
    great support group because youre going through
    the same thing and nobody else understands unless
    youre having to do it, right?

48
HIM
  • Theres got to be parents going through the
    same, but I see them with their kids and I cant
    really relate to them because they seem to be all
    handling it in different ways.
  • Ive always been nervous that how could anybody
    possibly understand?....
  • No, it was a bunch of women, it was all women. I
    was the only man.

49
IV. Coping Styles
50
HER
  • Husband and wife deal very differently. I find
    that I had a lot of support from friends and
    family and wanted to talk about it. My husband
    was more withdrawn and kind of internalized
    things and didnt want to talk about it.
  • He had his own way of dealing with everything
    that was going onand I think weve developed a
    lot helping each other, talking and I think
    were at a comfortable level as well where were
    happy.
  • For me, its day by day. I take things as they
    come I try not to think too far ahead, because
    then I get emotional.
  • What happens is that you look too far down the
    road initially because youre just learning and
    you want to be preparedBut then what I found out
    was I didnt need to be ten years down the road
    So, I tried to find a place in the middle where I
    wouldnt be ten years down the road worrying
    about what was going to happen.

51
HIM
  • Me, I dont talk a lot. I keep it inside,
    usually Her, she needs to talk to me It helps
    her to understand.
  • Theres a spiritual search that has to be
    doneMe, I was working more on that side. Her,
    she looked more on the social side.

52
V. Partner Relationship
53
HER
  • I had the impression that he didnt understand
    me, because he wasnt expressing it the same way.
    He seemed to be much stronger than me, when it
    comes to emotion.
  • We werent good supporters for one another
    because it was hurtful for us to talk together
    about it so we kind of separated on it for a bit
    and were still there to look after the needs of
    everybody else and ourselves, but it wasnt
    something we talked about together.
  • It wasnt something that we openly talked about.
    It was put in the box on a shelf and we dealt
    with it when we had to come to CHEO.
  • I think that J. feels OK because he sees we are
    doing OK

54
HIM
  • Just too busy with family life to be a couple.
  • We talk about what needs to be done, we dont
    talk about what each other needs.
  • Its more guesswork now, trying to guess what
    the other one.
  • We always had problems but we learned with time
    how to communicate it took time.. Today it is
    going well. I would say that now, the illness
    brings us together
  • It has enriched our relationship. I see my
    spouse through others, through the children. I
    see her interacting with the children. I see that
    she has changed over the years. She has adapted.
    I too have adapted. So weve reached an
    equilibrium..

55
Discussion
56
Results (1)
  • The unique contribution of our study is its
    attention to the internal dynamic of the
    relationship between mothers and fathers
  • The dynamic is highly dependent on the respective
    roles of primary and secondary caregiver
  • Caregiving tasks are not equally shared, but are
    divided, primarily on the base of role
  • Traditional sterotypes appear to be regenerated

57
Results (2)
  • There are important differences in coping styles
    between mothers and fathers
  • These differences may be perceived as a threat,
    and may serve as a source of isolation between
    partners
  • Dialogue appears to be the key necessary to find
    the desired equilibrium where gender differences
    exist

58
Results (3)
  • Despite our current multitidisciplinary team
    management approach, the current medical model
    does not optimally address the support required
    by parents
  • Couple base counselling
  • Discussion of adaptive tasks faced by individuals
    coping with chronic illness
  • Communication skills
  • Practical help for caring for the child

59
Limits of the Study
  • Majority of mothers held traditional role (one
    stay at home Dad)
  • Two families with child with DMD adopted into
    family
  • Parents had on average 8 years of experience in
    caring for the child with DMD

60
Lessons Learned
  • 1) Difficulty in recruiting fathers
  • 2) Reflection on the experience Possible impact
    of the burden of the day to day care of the child
  • 3) Communication and isolation between members of
    the couple
  • Dont communicate effectively regarding the
    illness
  • Dont communicate effectively about the
    existential issues (death, suffering, moral pain)

61
Future Study
  • The experience of hope in the psychosocial
    adaptation of parents of a child with Duchenne
    Muscular Dystrophy
  • Collaboration with Bloorview Kids Rehab
  • 20 parents at each centre
  • 4 phases
  • CHEO Research Institute funding application
    October 1, 2006
  • CHEO REB October 17, 2006

62
Objectives of Study
  • Primary Objective To describe what hope brings
    to the experience of living with a child with
    Duchenne muscular dystrophy, and to describe the
    main sources of hope in the day to day experience
    of the illness.
  • Secondary Objectives
  • To describe the nature of hope as the disease
    progresses
  • To determine if the experience of hope is
    different depending on the medical context in
    which the family finds itself. That is, is the
    experience of hope different between families
    where the philosophy of treatment is more
    aggressive (and may include experimental
    treatment) and those families treated in a
    medical context where treatment is seen as
    palliative in intent?

63
Questionnaire Sample Questions
  • At the time of the diagnosis, could you
    describe, what hope meant for you? What were you
    hoping for yourself? For your child? For your
    family?
  • Could you describe for me one or two events, or
    a moment, or an experience, or receiving news,
    that was for you, a source of hope?
  • Today, what is your main source of hope?

64
Acknowledgements
  • Department of Genetics
  • Co-investigators
  • CHEO Research Institute and Faculty of Education,
    U of Ottawa
  • Dr. Mario Cappelli and Linda Corsini
  • Parents of children with DMD
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