The Process of The Process of Giving MeaningGiving Meaning

Lessons Learned from the Lessons Learned from the Experience of Parents of a Experience of Parents of a

Child with Duchenne Child with Duchenne Muscular DystrophyMuscular Dystrophy

October 10, 2006

ObjectivesObjectives

• To present the rationale for a To present the rationale for a qualitative study of the experience of qualitative study of the experience of meaning in the parents of a child with meaning in the parents of a child with DMDDMD

• To summarize the results of the studyTo summarize the results of the study• To reflect upon the lessons learned in To reflect upon the lessons learned in

designing and carrying out the studydesigning and carrying out the study• To introduce the design of a followup To introduce the design of a followup

multicentre study to study the multicentre study to study the experience of hope in the same familiesexperience of hope in the same families

InvestigatorsInvestigators

• Pranesh ChakrabortyPranesh Chakraborty• Mireille ChoquetteMireille Choquette• Pierre JacobPierre Jacob• Sarah MilesSarah Miles• Andre SamsonAndre Samson• Eva TomiakEva Tomiak

BackgroundBackground

• DMD 1/3500 male birthsDMD 1/3500 male births• Mutation in X linked DMD gene, Mutation in X linked DMD gene,

leading to lack of the protein, leading to lack of the protein, dystrophindystrophin

• 1/3 mutations are de novo1/3 mutations are de novo• Progressive and incurable Progressive and incurable

neuromuscular diseaseneuromuscular disease

DMDDMD HistoryHistory

• Description by Duchenne in 1861Description by Duchenne in 1861-paraplégie hypertrophique de l’enfance -paraplégie hypertrophique de l’enfance

de cause cérébralede cause cérébrale

• Natural history: Brooke 1981Natural history: Brooke 1981• DMD gene: Worton 1984DMD gene: Worton 1984• DMD protein, dystrophin (gene DMD protein, dystrophin (gene

product) : Hoffman 1987product) : Hoffman 1987

DMD: Clinical PresentationDMD: Clinical Presentation

• Duchenne initial description:Duchenne initial description:– Progressive weakness of movements, Progressive weakness of movements,

initially affecting the muscles of the lower initially affecting the muscles of the lower limbs and lower spine, gradually getting limbs and lower spine, gradually getting worse and spreading to the upper limbs.worse and spreading to the upper limbs.

– Enlargement of some of the paralysed Enlargement of some of the paralysed muscles and in some cases almost all.muscles and in some cases almost all.

– Hyperplasia of interstitial connective Hyperplasia of interstitial connective tissue in the paralysed muscle with tissue in the paralysed muscle with production of abundant fibrous and production of abundant fibrous and adipose tissue in the final stagesadipose tissue in the final stages

DMD EvaluationDMD Evaluation

• ClinicalClinical• BiochemicalBiochemical• NeurophysiologicalNeurophysiological• RadiologicalRadiological• GeneticGenetic• PathologyPathology

ClinicalClinical

• Motor delay (proximal)Motor delay (proximal)– Delay walkingDelay walking– Difficulty standingDifficulty standing– Difficulty with stairs*Difficulty with stairs*– Positive Gowers’ manoeuvrePositive Gowers’ manoeuvre

• Cognitive delay (80%)Cognitive delay (80%)– ADD/ADHDADD/ADHD

• Muscle hypertrophy Muscle hypertrophy – Calves, thighs, forearm, masseters, temporalisCalves, thighs, forearm, masseters, temporalis

• Cardiomyopathy and respiratory weaknessCardiomyopathy and respiratory weakness

Gowers’ manoeuvreGowers’ manoeuvre

BiochemicalBiochemical

• Elevated CK Elevated CK – 50-100 x Normal50-100 x Normal

• N:N: 0-1 month 30-400 U/L 0-1 month 30-400 U/L- 50-300 U/L- 50-300 U/L

• DMD: usually well over 5000 U/LDMD: usually well over 5000 U/L• Will be elevated on day 1 of lifeWill be elevated on day 1 of life• Higher in the preclinical phase and Higher in the preclinical phase and

deceases afterward as muscle mass deceases afterward as muscle mass decreasesdecreases

• Elevated Elevated – AST, ALT, AldolaseAST, ALT, Aldolase

NeurophysiologyNeurophysiology

• Nerve conduction studiesNerve conduction studies• Electromyographic evaluationElectromyographic evaluation

– Insertion activityInsertion activity– Myopathic unitsMyopathic units– Increase recruitmentIncrease recruitment

RadiologicalRadiological

• UltrasoundUltrasound• Muscle MRI/CTMuscle MRI/CT

PathologyPathology

• Muscle biopsyMuscle biopsy– Regular stainsRegular stains

• NecrosisNecrosis• RegenerationRegeneration• Fiber variationFiber variation• Increase fibrous tissueIncrease fibrous tissue• Increase adipose tissue Increase adipose tissue • Myopathic changesMyopathic changes

– Specific stains (immuno chemistry)Specific stains (immuno chemistry)• DystrophynDystrophyn• Sarcoglycan, merosinSarcoglycan, merosin

Immunocytochemical: Immunocytochemical: NN

Immunocytochemical: Immunocytochemical: DMDDMD

GeneticGenetic

• Deletions Xp21Deletions Xp21• Intragenic deletions of exons 3, 8, Intragenic deletions of exons 3, 8,

13, 43, 44, 47, 50, 51 and 52 13, 43, 44, 47, 50, 51 and 52 responsible for 90% of all cases.responsible for 90% of all cases.

Natural HistoryNatural History

DMD PhasesDMD Phases((NMC functional classification)NMC functional classification)

• Early ambulationEarly ambulation ( 2-5 years) ( 2-5 years)• Late ambulationLate ambulation (6-9 years) (6-9 years)• Early wheelchairEarly wheelchair (10-13 years) (10-13 years) • Late wheelchairLate wheelchair (14 years and (14 years and

older)older)

2-5 years (2-5 years (Early AmbulationEarly Ambulation))

• DiagnosisDiagnosis– Family education*Family education*– Social/psychological support*Social/psychological support*– Genetic counseling*Genetic counseling*

• Therapeutic interventionsTherapeutic interventions– OrthopedicOrthopedic– RespiratoryRespiratory– Rehabilitation/physiotherapy*/OTRehabilitation/physiotherapy*/OT– MedicalMedical

6-9 years6-9 years ( (Late AmbulationLate Ambulation))

A: Social/psychological support*A: Social/psychological support*• FamilyFamily• SchoolSchool• Transportation/teacher aid Transportation/teacher aid

re:physical/learningre:physical/learning

6-9 years6-9 years ( (Late AmbulationLate Ambulation))

• B. Therapeutic interventionsB. Therapeutic interventions– OrthopedicOrthopedic– Respiratory- Respiratory-

• Flu vaccineFlu vaccine• Pulmonary functionPulmonary function

– RehabilitationRehabilitation• Physiotherapy-*Physiotherapy-*

– StretchingStretching– Chest physioChest physio

• Occupational therapy-*Occupational therapy-*– Orthotics AFO-Orthotics AFO- Night/DayNight/Day– House accessibilityHouse accessibility

– Medical-*Medical-*• DeflazacortDeflazacort

10-13 years10-13 years ( (Early WheelchairEarly Wheelchair))

A.A. Social/psychological supportSocial/psychological supportB.B. Therapeutic interventionsTherapeutic interventions– Orthopedic-*Orthopedic-*

• ContracturesContractures• Scoliosis**Scoliosis**

– Respiratory-*Respiratory-*• Pulmonary functionPulmonary function• Aids to ventilationAids to ventilation

– Rehabilitation-*Rehabilitation-*• Physiotherapy*: Chest physio, joint Physiotherapy*: Chest physio, joint

mobilizationmobilization• Occupational therapy*: orthoses, wheelchair Occupational therapy*: orthoses, wheelchair

– Medical-Medical-• DeflazacortDeflazacort• CardiacCardiac

14-18 years14-18 years ( (Late Wheelchair)Late Wheelchair)

A.A. Social/psychological support-*Social/psychological support-*

B.B. Therapeutic interventions:Therapeutic interventions:– Orthopedic-*Orthopedic-*

• Contractures*Contractures*• Scoliosis surgery**Scoliosis surgery**

– Respiratory-*Respiratory-*• Assisted ventilationAssisted ventilation

– Rehabilitation-*Rehabilitation-*• Physiotherapy- Chest physio/mobilizationPhysiotherapy- Chest physio/mobilization• Occupationnal therapy-Occupationnal therapy-

– equipment adjustmentequipment adjustment– SwallowingSwallowing

– Medical-Medical-• Cardiac Cardiac

Medical therapyMedical therapy

• PrednisonePrednisone– Definite improvement over placeboDefinite improvement over placebo

• 0.3mg/kg/day0.3mg/kg/day• 0.75/kg/day0.75/kg/day

– Improvement as early as 10 days Improvement as early as 10 days which continued to improve over 1 which continued to improve over 1 monthmonth

– Plateau that persisted for 6 monthsPlateau that persisted for 6 months– Group at 0.75mg/kg/day better than Group at 0.75mg/kg/day better than

0.3mg/kg3day but more side effects 0.3mg/kg3day but more side effects (wt gain, cushingoid, hair growth)(wt gain, cushingoid, hair growth)

Medical TherapyMedical Therapy

• Side effects:Side effects:– Weight gain- (up to 20% of initial weight)Weight gain- (up to 20% of initial weight)– BehaviorBehavior– AppetiteAppetite– SleepSleep– PsychiatricPsychiatric– Ophthalmologic-cataractsOphthalmologic-cataracts– EndocrineEndocrine

• PituitaryPituitary• Bone densityBone density

Other TherapiesOther Therapies

• Medical:Medical:– ImmunosuppressantImmunosuppressant

• AzathioprineAzathioprine• CyclophosphamideCyclophosphamide

– Myoblast transferMyoblast transfer

DMD: What should we remember?DMD: What should we remember?

• Medical Rx: limited resultsMedical Rx: limited results– Better energyBetter energy– Little change in overall disease progressLittle change in overall disease progress

A Clinician’s A Clinician’s Perspective (1)Perspective (1)

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

A Clinician’s Perspective A Clinician’s Perspective (2)(2)

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

Study DesignStudy Design

• Qualitative study of couples having a Qualitative study of couples having a child living with DMDchild living with DMD

• Description of the lived experience Description of the lived experience • Semi-structured interviews with 11 Semi-structured interviews with 11

parentsparents• Mothers and fathers interviewed Mothers and fathers interviewed

separatelyseparately• Interviews recorded and transcribedInterviews recorded and transcribed• Average length of interview 90 minutesAverage length of interview 90 minutes

Methods: Choice of Methods: Choice of Methodology(1)Methodology(1)

• Methodology research models: Deductive, Methodology research models: Deductive, quantitative, inductive, or qualitativequantitative, inductive, or qualitative

• The The hypothetical-deductive paradigmhypothetical-deductive paradigm is is based on the premise that an based on the premise that an independent, objective reality exists independent, objective reality exists outside the individualoutside the individual

• This reality can be observed, measured This reality can be observed, measured and explainedand explained

• Research aims to identify cause and Research aims to identify cause and effect, from which universal laws can be effect, from which universal laws can be deduceddeduced

Methods: Choice of Methods: Choice of Methodology (2)Methodology (2)

• The The holistic-inductive paradigmholistic-inductive paradigm is is characterized by the recognition of the characterized by the recognition of the subjective nature of realitysubjective nature of reality

• Reality is viewed as a construct of the Reality is viewed as a construct of the individual based on emotions, values and individual based on emotions, values and cultureculture

• The objective of research is to The objective of research is to understand the significance to the understand the significance to the individual of a particular lived individual of a particular lived experienceexperience

Methods: Data AnalysisMethods: Data Analysis

• Empirical Phenomenological Empirical Phenomenological Psychological (EPP) data analysis Psychological (EPP) data analysis methodmethod

• Five stages of analysisFive stages of analysis

Questionnaire: Sample Questionnaire: Sample QuestionsQuestions

• ““When you were first given the diagnosis of DMD When you were first given the diagnosis of DMD for your child, what was your first reaction?”for your child, what was your first reaction?”

• ““How did you make sense of what was happening at How did you make sense of what was happening at the time of diagnosis? In other words, did you see a the time of diagnosis? In other words, did you see a purpose in that experience?”purpose in that experience?”

• ““As a couple, how did it affect you? Did you discuss As a couple, how did it affect you? Did you discuss your child’s illness openly? How did you support your child’s illness openly? How did you support each other?”each other?”

• ““On reflecting back on your entire experience, do On reflecting back on your entire experience, do you see a purpose in your child’s illness? In other you see a purpose in your child’s illness? In other words, how did it impact you as a human being: The words, how did it impact you as a human being: The way you see others? The way you see your family? way you see others? The way you see your family? Your own spiritual growth? The way you see life in Your own spiritual growth? The way you see life in general?”general?”

ResultsResults

““Gender specific differences in the Gender specific differences in the psychosocial adjustment of parents psychosocial adjustment of parents of a child with DMD: Two points of of a child with DMD: Two points of

view for a shared experience”view for a shared experience”

Demographic VariablesDemographic Variables

MothersMothers FathersFathers

Current ageCurrent age 40-49 40-49 (40.5)(40.5)

44-58(47)44-58(47)

Age at child’s diagnosisAge at child’s diagnosis 31-31-41(32.5)41(32.5)

34-52(40)34-52(40)

Identified as primary Identified as primary caregivercaregiver

2/62/6 0/60/6

Viewed caregiving as Viewed caregiving as equally sharedequally shared

3/63/6 4/54/5

College or universityCollege or university 4/64/6 5/55/5

Working outside homeWorking outside home 3/63/6 4/54/5

Carrier of genetic Carrier of genetic changechange

2/62/6 N/AN/A

Themes IdentifiedThemes Identified

I. Care of the ChildI. Care of the Child

II. CareerII. Career

III. Social Support III. Social Support

IV. Coping StylesIV. Coping Styles

V. Partner RelationshipV. Partner Relationship

I. Care of the ChildI. Care of the Child

HERHER• ““A lot revolves around K. I have to be there in A lot revolves around K. I have to be there in

the morning. I have to help her get dressed. I the morning. I have to help her get dressed. I have to feed her breakfast. I have to help her have to feed her breakfast. I have to help her after the shower…Our whole daily life, our after the shower…Our whole daily life, our whole schedule revolves around K…”whole schedule revolves around K…”

• ““We were so immersed and we gave up so We were so immersed and we gave up so much over the years…”much over the years…”

• ““I’m more gentle with him, pay more attention I’m more gentle with him, pay more attention to his needs”to his needs”

• ““J. prefers that Mama takes care of him at J. prefers that Mama takes care of him at certain times, because Papa is less patient”certain times, because Papa is less patient”

HIMHIM

• ““I had the chance to spend time with him, I had the chance to spend time with him, to tease him, to put him to bed. Then he to tease him, to put him to bed. Then he began to laugh at me, he found me funny… began to laugh at me, he found me funny… It was after that that I realized that It was after that that I realized that something had changed. I don’t know something had changed. I don’t know exactly what… My attitude, yes. My attitude exactly what… My attitude, yes. My attitude changed, but how did it happen, I can’t changed, but how did it happen, I can’t explain.”explain.”

• ““We understood that we are important in We understood that we are important in the life of J. It is important to be with him.”the life of J. It is important to be with him.”

II. CareerII. Career

HERHER

• ““I am a mother on a 24 hour basis.. I am a mother on a 24 hour basis.. that’s my work.” that’s my work.”

• “ “I think that once the diagnosis I think that once the diagnosis came, then you kind of put came, then you kind of put everything on hold for a long period everything on hold for a long period of time.”of time.”

• ““There still seems to be a lack of There still seems to be a lack of respect for the title as a mother.”respect for the title as a mother.”

HIMHIM• ““Only time I can switch off now is when I Only time I can switch off now is when I

drive six hundred miles away.”drive six hundred miles away.”

• “ “She, she has to stay at home... She is full-She, she has to stay at home... She is full-time here… whereas, I get to escape. ”time here… whereas, I get to escape. ”

• “ “ The care of J. is difficult. One doesn’t take it The care of J. is difficult. One doesn’t take it into consideration, but his care puts an extra into consideration, but his care puts an extra weight on my shoulders. ”weight on my shoulders. ”

• ““The career is not a priority for me. I have to The career is not a priority for me. I have to think about many other things apart from think about many other things apart from career.”career.”

III. Social SupportIII. Social Support

HERHER• ““When I think about the people who impact my When I think about the people who impact my

life and give me the most pleasure and enjoyment, life and give me the most pleasure and enjoyment, and are the most helpful to me, they are the and are the most helpful to me, they are the physiotherapist, who became a close friend, the physiotherapist, who became a close friend, the massage therapist and I who are like best massage therapist and I who are like best buddies…the hair dresser who comes to my buddies…the hair dresser who comes to my house…the baby sitters that we’ve had who come house…the baby sitters that we’ve had who come by in the summer and take her out for no reason.. by in the summer and take her out for no reason.. my girlfriends who will come by just drop in, and my girlfriends who will come by just drop in, and say Hi..”say Hi..”

• ““I think it’s good for families to be in touch with I think it’s good for families to be in touch with one another. I know some are open to that and one another. I know some are open to that and some aren’t, but I really do think it’s a great some aren’t, but I really do think it’s a great support group because you’re going through the support group because you’re going through the same thing and nobody else understands unless same thing and nobody else understands unless you’re having to do it, right?you’re having to do it, right? “ “

HIMHIM

• ““There’s got to be parents going There’s got to be parents going through the same, but I see them with through the same, but I see them with their kids and I can’t really relate to their kids and I can’t really relate to them because they seem to be all them because they seem to be all handling it in different ways.”handling it in different ways.”

• ““I’ve always been nervous that how I’ve always been nervous that how could anybody possibly understand?....could anybody possibly understand?....””

• ““No, it was a bunch of women, it was all No, it was a bunch of women, it was all women. I was the only man.”women. I was the only man.”

IV. Coping StylesIV. Coping Styles

HERHER• ““Husband and wife deal very differently. I find that I had a Husband and wife deal very differently. I find that I had a

lot of support from friends and family and wanted to talk lot of support from friends and family and wanted to talk about it. My husband was more withdrawn and kind of about it. My husband was more withdrawn and kind of internalized things and didn’t want to talk about it.” internalized things and didn’t want to talk about it.”

• ““He had his own way of dealing with everything that was He had his own way of dealing with everything that was going on…and I think we’ve developed a lot helping each going on…and I think we’ve developed a lot helping each other, talking… and I think we’re at a comfortable level as other, talking… and I think we’re at a comfortable level as well where we’re happy.”well where we’re happy.”

• ““For me, it’s day by day. I take things as they come… I try For me, it’s day by day. I take things as they come… I try not to think too far ahead, because then I get emotional.”not to think too far ahead, because then I get emotional.”

• ““What happens is that you look too far down the road What happens is that you look too far down the road initially because you’re just learning and you want to be initially because you’re just learning and you want to be prepared…But then what I found out was I didn’t need to prepared…But then what I found out was I didn’t need to be ten years down the road… So, I tried to find a place in be ten years down the road… So, I tried to find a place in the middle where I wouldn’t be ten years down the road the middle where I wouldn’t be ten years down the road worrying about what was going to happen.”worrying about what was going to happen.”

HIMHIM

• ““Me, I don’t talk a lot. I keep it Me, I don’t talk a lot. I keep it inside, usually… Her, she needs to inside, usually… Her, she needs to talk to me… It helps her to talk to me… It helps her to understand.”understand.”

• ““There’s a spiritual search that There’s a spiritual search that has to be done…Me, I was working has to be done…Me, I was working more on that side. Her, she looked more on that side. Her, she looked more on the social side.”more on the social side.”

V. Partner V. Partner RelationshipRelationship

HERHER• ““I had the impression that he didn’t understand me, I had the impression that he didn’t understand me,

because he wasn’t expressing it the same way. He because he wasn’t expressing it the same way. He seemed to be much stronger than me, when it comes to seemed to be much stronger than me, when it comes to emotion.”emotion.”

• ““We weren’t good supporters for one another because We weren’t good supporters for one another because it was hurtful for us to talk together about it so we 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 kind of separated on it for a bit and were still there to look after the needs of everybody else and ourselves, look after the needs of everybody else and ourselves, but it wasn’t something we talked about together.”but it wasn’t something we talked about together.”

• ““It wasn’t something that we openly talked about. It It wasn’t something that we openly talked about. It was put in the box on a shelf and we dealt with it when was put in the box on a shelf and we dealt with it when we had to come to CHEO.”we had to come to CHEO.”

• “ “I think that J. feels OK because he sees we are doing I think that J. feels OK because he sees we are doing OK”OK”

HIMHIM• ““Just too busy with family life to be a couple.”Just too busy with family life to be a couple.”

• ““We talk about what needs to be done, we don’t talk We talk about what needs to be done, we don’t talk about what each other needs.”about what each other needs.”

• ““It’s more guesswork now, trying to guess what the other It’s more guesswork now, trying to guess what the other one….”one….”

• ““We always had problems… but we learned with time how We always had problems… but we learned with time how to communicate… it took time.. Today it is going well. I to communicate… it took time.. Today it is going well. I would say that now, the illness brings us together”would say that now, the illness brings us together”

• ““It has enriched our relationship. I see my spouse It has enriched our relationship. I see my spouse through others, through the children. I see her through others, through the children. I see her interacting with the children. I see that she has changed interacting with the children. I see that she has changed over the years. She has adapted. I too have adapted. So over the years. She has adapted. I too have adapted. So we’ve reached an equilibrium..”we’ve reached an equilibrium..”

DiscussionDiscussion

Results (1)Results (1)

• The unique contribution of our study is its The unique contribution of our study is its attention to the internal dynamic of the attention to the internal dynamic of the relationship between mothers and fathersrelationship between mothers and fathers

• The dynamic is highly dependent on the The dynamic is highly dependent on the respective roles of primary and secondary respective roles of primary and secondary caregivercaregiver

• Caregiving tasks are not equally shared, Caregiving tasks are not equally shared, but are divided, primarily on the base of but are divided, primarily on the base of rolerole

• Traditional sterotypes appear to be Traditional sterotypes appear to be regeneratedregenerated

Results (2)Results (2)

• There are important differences in There are important differences in coping styles between mothers and coping styles between mothers and fathersfathers

• These differences may be perceived as a These differences may be perceived as a threat, and may serve as a source of threat, and may serve as a source of isolation between partnersisolation between partners

• Dialogue appears to be the key necessary Dialogue appears to be the key necessary to find the desired equilibrium where to find the desired equilibrium where gender differences existgender differences exist

Results (3)Results (3)

• Despite our current multitidisciplinary Despite our current multitidisciplinary team management approach, the team management approach, the current medical model does not current medical model does not optimally address the support required optimally address the support required by parentsby parents

• Couple base counsellingCouple base counselling• Discussion of adaptive tasks faced by Discussion of adaptive tasks faced by

individuals coping with chronic illnessindividuals coping with chronic illness• Communication skillsCommunication skills• Practical help for caring for the childPractical help for caring for the child

Limits of the StudyLimits of the Study

• Majority of mothers held traditional Majority of mothers held traditional role (one stay at home Dad)role (one stay at home Dad)

• Two families with child with DMD Two families with child with DMD adopted into familyadopted into family

• Parents had on average 8 years of Parents had on average 8 years of experience in caring for the child experience in caring for the child with DMDwith DMD

Lessons LearnedLessons Learned

• 1) Difficulty in recruiting fathers1) Difficulty in recruiting fathers• 2) Reflection on the experience: Possible 2) Reflection on the experience: Possible

impact of the burden of the day to day impact of the burden of the day to day care of the childcare of the child

• 3) Communication and isolation between 3) Communication and isolation between members of the couple:members of the couple:– Don’t communicate effectively regarding the Don’t communicate effectively regarding the

illnessillness– Don’t communicate effectively about the Don’t communicate effectively about the

existential issues (death, suffering, moral pain)existential issues (death, suffering, moral pain)

Future StudyFuture Study

• ““The experience of hope in the The experience of hope in the psychosocial adaptation of parents of a psychosocial adaptation of parents of a child with Duchenne Muscular child with Duchenne Muscular Dystrophy”Dystrophy”

• Collaboration with Bloorview Kids RehabCollaboration with Bloorview Kids Rehab• 20 parents at each centre20 parents at each centre• 4 phases4 phases• CHEO Research Institute funding CHEO Research Institute funding

application October 1, 2006application October 1, 2006• CHEO REB October 17, 2006CHEO REB October 17, 2006

Objectives of StudyObjectives of Study• Primary Objective: Primary Objective: To describe what hope To describe what hope

brings to the experience of living with a child brings to the experience of living with a child with Duchenne muscular dystrophy, and to with Duchenne muscular dystrophy, and to describe the main sources of hope in the day to describe the main sources of hope in the day to day experience of the illness.day experience of the illness.

• Secondary Objectives: Secondary Objectives: – To describe the nature of hope as the disease To describe the nature of hope as the disease

progressesprogresses – To determine if the experience of hope is different To determine if the experience of hope is different

depending on the medical context in which the family depending on the medical context in which the family finds itself. That is, is the experience of hope different finds itself. That is, is the experience of hope different between families where the philosophy of treatment between families where the philosophy of treatment is more aggressive (and may include experimental is more aggressive (and may include experimental treatment) and those families treated in a medical treatment) and those families treated in a medical context where treatment is seen as palliative in context where treatment is seen as palliative in intent? intent?

Questionnaire: Sample Questionnaire: Sample QuestionsQuestions

• ““At the time of the diagnosis, could you At the time of the diagnosis, could you describe, what hope meant for you? describe, what hope meant for you? What were you hoping for yourself? For What were you hoping for yourself? For your child? For your family?”your child? For your family?”

• ““Could you describe for me one or two Could you describe for me one or two events, or a moment, or an experience, events, or a moment, or an experience, or receiving news, that was for you, a or receiving news, that was for you, a source of hope?”source of hope?”

• ““Today, what is your main source of Today, what is your main source of hope?”hope?”

AcknowledgementsAcknowledgements

• Department of GeneticsDepartment of Genetics• Co-investigatorsCo-investigators• CHEO Research Institute and CHEO Research Institute and

Faculty of Education, U of OttawaFaculty of Education, U of Ottawa• Dr. Mario Cappelli and Linda CorsiniDr. Mario Cappelli and Linda Corsini• Parents of children with DMDParents of children with DMD