The quality of life of parents caring for a child with a Lysosomal Storage Disorder LSD Research Ass

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The quality of life of parents caring for a child
with a Lysosomal Storage Disorder (LSD) Research
Assistant Leanne Whaites Prof Michael
SawyerProf John HopwoodDr Janice Fletcher
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Aim of the current study

• To develop a questionnaire to measure the quality
  of life (QOL) of parents caring for a child with
  a LSD

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Quality of Life

• A multi-factorial construct that focuses on an
  individuals perception of their own physical,
  psychological and social functioning (Schipper et
  al, 1996)

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Subtypes

• Currently, 46 different LSDs have been
  identified, each has a different enzyme
  deficiency
• Low prevalence disorders approx 1 in 5,000
  birthsgt50 new cases each year in Australia (most
  prevalent subtype is Gaucher Disease 1 in 57,000
  in Australia)

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Subtypes (cont.)

• In the present study we have spoken to parents of
  children who suffer from
• Hunter Syndrome
• Hurler Syndrome
• Sanfilipo Syndrome
• Morquio Syndrome
• Batten Disease
• Fabry Disease
• Gaucher Disease
• Krabbe Disease

Mucopolysaccharidoses (MPS)
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Impact of LSDs

• Large impact on the well-being and quality of
  life of children suffering from LSDs
• Family is also affected to a large extent
  especially parents who must change their lives to
  care for the child
• Currently there are NO instruments available to
  assess the quality of life of these very special
  dedicated parents

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Why measure QOL of parents?

• An important current issue because
• improved medical care is now prolonging the lives
  of children with chronic illness
• treatment intensity for children with chronic
  illness has increased
• this has led to an increased care-giver burden
  for parents

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Why measure QOL of parents? (cont.)

• Increased care-giver burden has many impacts on
  parents
• Possible negative impact may be minimised by
  identification of areas of concern and provision
  of help, e.g.
• Financial assistance
• Psychological counselling (grief, relationships)
• Greater availability of services
• Communication of results of QOL measures to
  policy makers

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Why measure QOL of parents? (cont.)

• It is also important that we are able to evaluate
  the effects of new treatments for children on the
  quality of life of parents

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Methodology

• Literature review to identify possible
  questionnaire domains (Fiona Arney)
• Focus groups with parents, Interviews with
  professionals to confirm domains identified in
  literature review and generate new domains

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Focus Groups

• 4 focus groups conducted 2 in Adelaide, 2 in
  Sydney (total N20)
• Questions asked generated from literature review
• Each focus group was transcribed and a content
  analysis was conducted
• Each separate issue was converted into a
  preliminary pilot questionnaire item

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Domains Identified for Questionnaire
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Time Available

• a. Recreation/Social
• b. Family Activities
• c. Marriage
• d. Other Children
• e. Employment
• f. Causes of Lack of Time
• (i) child care
• (ii) services
• (iii) medical care
• g. Time for Yourself

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Time Available eg.

• you cant do anything spontaneous, you cant
  just say lets go to the pictures cause its just
  not as easy as that

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Role Satisfaction

• a. Marital/Partner
• b. Parental
• (i) Affected Child
• (ii) Other Children
• c. Social
• d. Work
• e. Individual/Personal

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Role Satisfaction eg.

• my relationship with my husband is probably
  closer now because of what weve been through
• You need an outlet, otherwise you feel like you
  could scream, youll go mad cause it is 24h a
  day, 7 days a week

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Financial Health

• a. Extra Expenditure
• (i) Child Care
• (ii) Medical Care
• (iii)Transport
• b. Loss of Income
• c. Financial Assistance
• d. Financial Sacrifices

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Financial Health eg.

• you have added expenses - petrol, running kids
  to the doctors and specialists
• the fact that I can only work sporadically has
  the most impact on our finances

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Experience of the System and Public Policy

• a. Medical Staff
• (i) information given to parents
• (ii) feedback given to parents
• (iii) problems with medical staff/hospitals
• b. Services
• (i) access to services
• (ii) problems with services
• c. Government Agencies
• (i) worries about government benefits
• (ii) problems with government agencies
• (iii) problems with public infrastructure

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Experience of the System and Public Policy eg.

• I feel that you get lost in the system a bit -
  because not enough is known about it and the
  money cant go into it because theres not enough
  people
• Ive got nothing but praise for the public
  hospital system
• You dont get support. If they cant see it or
  it hasnt got a name, they dont know about you

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Family Functioning

• a. Family Activities
• b. Family Relationships
• c. Family Structure

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Family Functioning eg.

• we dont do anything together someone is always
  too tired
• I guess the wheelchair is the biggest problem,
  we cant go into town
• you can either let it beat you or let it bring
  you closer together as a family unit

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Emotional Health

• a. General Emotions
• (i) Feelings about self
• (ii) Feelings about child
• b. Pregnancy
• c. Diagnosis
• d. Treatment
• e. Family Issues
• f. Child-care
• g. Future

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Emotional Health eg.

• the first 12 months of her life, wondering
  whether wed done the right thing by having her
  was the most difficult for me - emotionally and
  the guilt
• in the beginning it was shocking and I cried for
  at least 12 months, I was a mess

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Physical Health eg.

• Ive got a prolapsed disc in my back
• We just dont sleep any more, it doesnt worry
  me as much but it worries my wife
• Ive got a hernia cause I dont use a hoist

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Structure of Pilot Questionnaire

• Currently contains 212 questions (including
  demographic information, financial)
• Frequency x bother/importance/emotional intensity
• Response format eg.

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Future Methodology

• 1. Pilot of questionnaire 1 - SA (N13), NSW
  (N19), VIC (approx N35) - both parents (max
  N67)
• 2. Perform statistical analyses on items to
  determine which items will remain in final
  questionnaire (impact rating, item construct
  correlation)
• 3. Change format of remaining items to allow for
  a greater range of responses in face-to-face
  final questionnaire format.
• 4. Administer final questionnaire with another
  established measure to evaluate psychometric
  properties of final questionnaire (same
  participants as for questionnaire 1, also
  participants in the UK)

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Preliminary Results
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Impact scores for main caregiver (N26) vs other
parent (N14)
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Impact scores for MPS (N19) vs other LSDs (N7)
(3 Fabry, 1 Batten, 3 Gaucher)
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THANKYOU TO ALL PARENTS WHO HAVE HELPED WITH THIS
STUDY!
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Acknowledgements

• Fiona Arney
• Ros Smith
• Sophie Lazenkas
• Margaret Sahhar
• Bronwynne Gitsham
• Justine Whitham