Alison Metcalfe presented her research on talking to children and young people about Familial Alzheimer's Disease and Familial Frontotemporal Dementia.

1. Talking to your children
and young people about
Familial Alzheimer’s
Disease / Familial
Frontotemporal Dementia
Alison Metcalfe
Professor of Health Care Research

2. Overview
• Focusing on parents, children and young
people.
• Briefly cover the issues for parents,
children and young people.
• Practical pointers for families.

3. Background
Briefly outline the research activities
• Research work with families affected by
range of genetic / familial conditions.
• Working to co-design interventions to
facilitate family coping and adaptation to
living with the condition.

4. Family
structure
Family
interactions
Family
functions
Family life cycle

5. Family Systems Theory
• What happens to one family member will
have repercussions for all other family
members (often v different repercussions).
• Transgenerational stressors.
• Facilitation of families coping and adapting
to living with the genetic condition and / or
its risks – improved communication is
essential.

6. Factors affecting family communication
• Emotional impact of diagnosis.
• Guilt and blame.
• Parents knowledge and understanding.
• Care giver burden - care fatigue.
• Grief – complex grief reactions.
• Key transition points for children and
genetic testing

7. Family roles
in the
communication
of genetic risk
information

8. Children's developing understanding and response to genetic risk
Children begin to notice
and question visible
difference and some
asked if they were likely
to be affected in the
same way to what they
observed. Some
understood that the
condition was a result of
the biological relationship
between parents and
child in the same way as
eye colour.
Children understood the
condition in terms of
what they could see and
how it currently impacted
on their daily lives. Most
understood the notion of
hereditary in terms of the
condition being passed
down through the family
but had no idea of
hereditary patterns.
Children began to
understand more about the
condition in terms of
expected future symptoms
and about mortality. Most
understood hereditary in
terms of their relationship to
their parent(s) but not to
their future children. Most
used genetic language but
without an underlying
understanding and could
not describe hereditary
patterns accurately or
quantify risk
Young people understand
that they may carry a gene
that could or would affect
their health. They had a
clearer understanding of
mortality although still not
always the stages of
progression. They were
more able to described
hereditary patterns and
begin to realise the
implications for their own
future children.
Young adults began to
understand the wider
implications of the
condition and relate it
more to themselves. They
realised how it might
impact on their career
choices and on their
personal relationships and
how their decisions
relating to genetic testing
impacted on other family
members.
Up to 7 years 8 - 11 years 12 - 14 years 15 - 17 years 18 years plus
Children seemed to
accept information at a
relatively superficial level.
There were some
behavioural problems or
signs of stress when they
were not given
information in difficult
family situations.
Children continued to
accept information at a
relatively superficial level
and looked for positives
and did not let the
condition become the
central focus of their
lives
A period of rebellion for
some children who became
angry and questioned ‘why
me?’ Some children
challenged treatments and
routines.
Young people reported
upset and shock as they
better understood the
mortality or responded to
test results or faced surgery
but they continued to look
for positives and not let the
condition monopolise their
life.
The most difficult time
emotionally. Young adults
were coming to terms with
the implications of the
condition at a time when
so many other important
life decisions and choices
are being made.
Understanding of genetic condition and risks
Emotional reaction

9. Observations
• Children will need different information at different times,
whilst discussing the IGC as a family its important to
check individual child’s understanding.
• Talking about the genetic condition is a process – reveal
information in response to children’s needs and
questions.
• Encourage children to ask questions (at appropriate
times).
• But check what children REALLY want to know
• Honesty and trust.
• Reassurance and reminders.
• Name the condition then at least children can discuss it

10. … I think the parents have to really
really understand a lot of psychology.
Its not just the physical aspects of
feeding and going through a major
operation…. All that is secondary I
feel to how a mother has to
understand and its a lot of
psychological understanding cos if its
not there, the the children are
damaged…

11. Communication tips children, young people
and parents have found useful.
• Children and young people prefer informal discussion often whilst doing other things
together eg: driving, cooking or gardening.
• Check their understanding because children worry about upsetting their parents and
so may not always ask.
• Talking about the genetic condition is an ongoing discussion rather than a one off
conversation. Like adults, children probably need information given to them more
than once. They may need time to digest information and then want to come back
and discuss it.
• Discuss information young people find on the Internet or in newspapers
• Discuss emotions – provide reassurance they are not alone.
• Explain parents behaviour if they are anxious or upset.
• Being with peers eg cousins in similar circumstances might be helpful
• Support and guide decision-making, especially with young people, who usually like to
make their own decisions but with advice from parents.
• If you do not know the answer, explain some questions do not have answers or that
you will try to find out for your child.
• Agree appropriate times to discuss the genetic condition if your child asks questions
at inopportune moments.

12. New interventions
• Co-design and development of Multi-
Family Discussion Groups.
• Implications for genetic nurses and
counsellors.

13. It has been observed that the following points helped parents talk to their
children:
• Younger children do not have experience to recognise and anticipate the
fuller implications therefore there is a gradual realisation.
• Not feeling pressurised to talk by an impending event eg a school science
lesson.
• Talking was a relief for parents and ultimately easier than secrets.
• Parents can be the role model for young people – giving them insight into
how to cope with the risk.
• Recognising siblings may all have different needs, try to find out what each
understands at different times in their development
• Ensure children and young people understand a positive genetic test is not
necessarily a diagnosis (unless genetic testing is specifically used to assist
with diagnosing an illness) – some get quite confused about this.
• Belief in a child’s right to know.
• Support of other family members, friends and health professionals.
• Attendance at support groups gave focus to regular discussion with children
and young people with parents discussing where they were going and what
had been discussed when they returned.
What helps parents talk to their children?

14. Preparing parents to talk to children
• Makes family closer
• Support for children
• Gives insight and helps them
realise that parent’s being upset
about the genetic condition is not
down to them or their behaviour
i.e.‘no fault’ of theirs
• Confidence to talk to close friends
• Children and young people feel
valued by parent(s)
• Allows discussion of the genetic
condition and its risk without
centralising it to life
• A shared reality and
understanding helps children and
young people cope
• Reduces risk of children getting
inaccurate information from
elsewhere.
• It can be emotionally taxing
dealing with questions
• Children and young people can
remind you about the genetic
condition, when you do not want
to be reminded
• Questions can arise at
inopportune moments – explain
when it is appropriate to discuss it
• Wanting to talk to peers but
networks can be limited
• Can affect school work for a short
time (but so can worrying about
what’s happening in their family if
there is secrecy).
Benefits Drawbacks

15. Conclusions
• If parents are able it is better to talk to their
children and young people.
• Good communication assists the family in
coping and adapting to the genetic condition –
resilience.
• Getting communication better between
parents and children will lead to stronger
family relationships in the longer-term.

16. Questions?
alison.metcalfe@kcl.ac.uk

17. References - selection
Biesecker B. Erby L. (2008) Adaptation to living with a genetic condition or risk: a mini-review. Clinical Genetics. 74(5):401-7.
Brouer-DudokdeWit A, Savenije A, Zoeteweij M, Maat-Kievit A. and Tibben A. (2002). A Hereditary Disorder In the Family and the
Family Life Cycle: Huntington Disease as a Paradigm. Family Process 41(4): 677-692.
Conkie-Rosell, A., E. M. Heise, et al. (2009). "Genetic Risk Communication: Experiences of Adolescent Girls and Young Women
from Families with Fragile X Syndrome." J Genet Couns.
Conkie-Rosell, A., G. A. Spiridigliozzi, et al. (2008). "Living with genetic risk: effect on adolescent self-concept." American Journal
of Medical Genetics Part(1): 56-69.
Fanos JH, Davis J, Puck JM. Sib understanding of genetics and attitudes toward carrier testing for X-linked severe combined
immunodeficiency. American Journal of Medical Genetics Part A 2001(98): 46-56.patterns in families with a history of
breast/ovarian cancer (HBOC). Psychooncology; 13: 335–345
Forrest, K. K., T. E. van, et al. (2009). "How young people find out about their family history of Huntington's disease." Social
Science & Medicine 68(10): 1892-1900. Kenen R, Arden-Jones A, Eeles R (2004) We are talking, but are they listening?
Communication
Mathers J, Greenhough S, Metcalfe A, Cole T, Flanagan S, Wilson S (2010) Family history in primary care: Understanding
General Practitioners resistance to clinical genetics: Qualitative study. British Journal of General Practice, 60(574), 221-230
McAllister M, Davies L, Payne K, Nicholls S, Donnai D, MacLeod R. (2007). The emotional effects of genetic diseases:
implications for clinical genetics. American Journal of Medical Genetics Part A;143A(22):2651-61.
Metcalfe A, Plumridge G, Coad J, Shanks A, Gill P (2011) Parents’ and children’s communication about genetic risk: qualitative
study learning from families’ experiences. European Journal Human Genetics 19; 640-646 (16 February 2011)
doi:10.1038/ejhg.2010.258
Pickett T Jnr, Altmaier E, Paulsen JS (2007) Caregiver Burden in Huntington’s Disease. Rehabilitation Psychology 53(3): 311-318
Plumridge G, Metcalfe A, Coad J, Gill P.(2010). Family Communication about Genetic Risk Information: Particular Issues for
Duchenne Muscular Dystrophy. American Journal of Medical Genetics. Part A 152A: 404–408.
Plumridge G, Metcalfe A (Corresponding author), Coad J, Gill P (2011) Siblings’ experiences in families affected by genetic
conditions. Journal of Genetic Counselling 20; 374-383 DOI: 10.1007/s10897-011-9361-13
Plumridge G, Metcalfe A (Corresponding author), Coad J, Gill P (2011) The role of support groups in facilitating family discussion
of genetic risk information. Health Expectations, 17 FEB 2011 DOI: 10.1111/j.1369-7625.2011.00663.x
Sobel S and Cowan C (2000).Impact of genetic testing for Huntingtons disease on the family system. American Jn of Medical
Genetics 90: 49–59.
Sobel S and Cowan (2003) Ambiguous Loss and Disenfranchised Grief: The Impact of DNA Predictive Testing on the Family as a
System. Family Process 42 1;47-57
Travers E, Jones K, Nichol J. (2007) Palliative care provision in Huntington's disease. International Journal of Palliative Nursing
13(3):125-30.