S. Griffiths et al.

/ APCP Journal Volume 4 Number 1 (2013) 7-18 APCP Journal Volume 4 Number 1 (2013) 19-26

Living with Arthrogryposis Multiplex Congenita: A Survey

Histogram with bell shaped curve, demonstrating A histogram and bell shaped curve, to demonstrate the
distribution of data for right multifidus muscle during distribution of data for the left multifidus muscle during Jeanne Hartley MSc MCSP*, Sharon Baker, Kevin Whittaker
the 15% BW loaded backpack condition. the unloaded backpack condition.
The Arthrogryposis Group

*Corresponding author: email: jeannehartley@hotmail.co.uk

________________________________________________________________________________

ABSTRACT

Aims
To explore the views and opinions of adults with Arthrogryposis Multiplex Congenita (AMC) about their
lives.

Background
There is sparse literature about life as an adult with a congenital condition. Adults with AMC were keen to
discover if problems and experiences were common amongst others with AMC. Parents of children with
AMC were also interested to know what the future may hold for their child as an adult.

Design
A postal questionnaire, including open and closed questions, was sent to 295 adults with AMC who were
members of The Arthrogryposis Group (TAG). The questionnaire included domains on demographics,
education, family life, employment, physical abilities, general health, surgery, therapies, pain, disability and
life.

Results
A wide variation of opinions and experiences were recorded amongst the group of 96 adults with AMC who
responded, both from their childhood years and an overview of their achievements and challenges as adults.

A histogram and bell shaped curve, to demonstrate the
distribution of data for the left multifidus muscle during
the 10% BW loaded backpack condition.
A histogram and bell shaped curve, to demonstrate the
distribution of data for the left multifidus muscle during
the 15% BW loaded backpack condition.
Conclusions
There was a wide range of experiences amongst this group of adults with AMC whose ages ranged from 18
to 83 years. Attitudes towards life with AMC were broadly positive with many reporting acceptance and
resilience to cope with personal, social and environmental challenges. Lack of knowledge and expertise about
AMC amongst health care professionals and others in adult services was a common observation, as were
difficulties securing funding to support living with a disability, access, and opportunities.
________________________________________________________________________________

Introduction infection, in animal studies, produces a similar

Arthrogryposis Multiplex Congenita (AMC) is a
descriptive term, which literally means multiple
curved joints. The reported incidence varies widely.
This is possibly because the term could be applied to
all patients with multiple curved joints (1:3,000) or
only to those with no other possible diagnosis after
extensive investigation (Fixsen, 2010). Hall (1998), a
geneticist, recorded at least 150 diagnoses in which a
baby is born with multiple curved joint deformities
but quoted an incidence of classical AMC
(amyoplasia congenita) of 1 in 10,000 live births.
Wynne Davies et al (1981), when looking at the
incidence of AMC in the UK, Australia and the USA,
found an apparent increase in AMC in the 1960s,
suggesting an infective cause. Intrauterine viral
condition to classical arthrogryposis (Fixsen, 2010).
Limitation of movement in utero has been shown,
again in animal studies, to result in contractures at
birth. Decreased foetal movement during pregnancy
is often reported by mothers of babies born with
AMC. Predisposing factors that reduce intrauterine
mobility include neuromuscular and connective
tissue disorders, foetal crowding, oligohydraminos,
etc. Wynne-Davies et al (1981) aptly described the
aetiology as an environmental disease of early
pregnancy associated with one or more
unfavourable intrauterine factors.
The features of classical AMC include not only
multiple curved stiff joints but also defective
muscles, usually affecting all 4 limbs, but may also

18 19
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
involve only joints in the upper or the lower limbs.
There may also be other congenital abnormalities
such as bowel atresia, hernias, etc. Sensation is
normal. It is usually a sporadic occurrence and is not
thought to be inherited. Distal arthrogryposis,
however, does have an autosomal dominant
background (Hall et al, 1982). In these cases there are
severe deformities in the hands and feet, with
perhaps minor contractures proximally. These
children may develop scoliosis later.
Enabling children born with AMC to achieve
optimal participation levels in their daily life
requires a large multi-disciplinary team physicians,
surgeons, therapists, orthotists, dieticians, etc. with
specialised input throughout their childhood years.
For most young people with disabilities, and their
families, transition into adult services is fraught,
with reports of lack of therapy input and lack of
knowledge concerning congenital conditions from
those working in adult services. For young adults
with AMC this change can be particularly difficult,
as many have transferred from dedicated AMC
clinics held at some of the major childrens hospitals
in England and Scotland.
Fassier et al (2009) published a long term AMC
study but sadly only covered the years from birth
until skeletal maturity. In fact most literature seems
to be paediatric and medically-centred and ignores
the impact of every day living with AMC. Staheli et
als useful book on AMC (1998) covers all paediatric
aspects of the condition and is a valuable source of
current knowledge but has no information or
discussion of AMC in adulthood. A final chapter
dealing with the long-term effects of AMC and adult
issues, such as employment and other resources
available, would have completed this book.
Information and literature about AMC in adult life is
sadly lacking and it is therefore understandable that
those providing services to adults with AMC appear
to have a poor knowledge or understanding of the
condition.
The Arthrogryposis Group (TAG) is a UK charity
providing valuable support and advice for families
who have a child with AMC and also for adults with
the condition. An annual weekend conference
allows those with AMC and their families to meet
and attend lectures on various aspects of the
medical, surgical and therapeutic management of
this condition, as well as covering other aspects such
as psychological, educational and social issues. This
conference is also open to interested healthcare
professionals and can give useful insight into the
long-term ramifications of living with the condition,
as well as a chance to meet adults with AMC.
20
Adults with AMC are often isolated and value the
annual conference as a chance to meet others with
the condition. Through discussions many of them
wondered whether many of the problems they were
experiencing, particularly physical symptoms, were
common to others with AMC or just related to
natural aging. Parents of children with AMC were
also keen to know what the challenges may be once
their child becomes an adult. From these
conversations it was decided that a survey of adults
with AMC would be useful to address some of the
unanswered questions. A similar survey has been
carried out by Avenues, the AMC support group in
the USA and also addressed similar concerns as
those included in this study (Sneddon, 1999).
Method
Adult members of TAG with AMC were invited to
attend a workshop held during the TAG annual
conference. As the result of discussion they
identified issues that they wanted to explore about
AMC. A questionnaire was developed to capture
information about the following domains:
demographic information - age, sex, ethnicity, type
of AMC, body parts affected;
education - type of school attended, leaving age,
academic qualifications, special educational needs,
other support, further education;
family life - family history of AMC, marital status,
children, issues around independent living;
employment - occupation, discrimination, support,
income, benefits, impact of AMC on employment
opportunities;
physical abilities - abilities, mobility aids, prob-
lems;
general health - general health, impact of AMC,
childhood problems, problems as an adult;
surgery - in childhood and as an adult;
therapies - physiotherapy input, other therapies,
orthotics, perceived therapy needs as an adult;
pain - frequency, intensity and location of pain,
medication, strategies;
disability and life - impact of AMC on social life,
hobbies, sports, self-esteem, personal relationships,
thoughts about the future and disability, achieve-
ments.
The questionnaire consisted of closed and open
questions and was piloted by 6 adults with AMC, all
members of TAG. Changes were made in response
to their feedback. A final page was left blank so that
thoughts and comments could be added should the
respondents wish to provide further information.
The finalised questionnaire was posted to 295 adult
members of TAG with AMC, who were 18 years and
over. An accompanying letter of invitation to take
J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
part in the survey was included. This emphasised
that participation was voluntary and reassurance
was given that information provided would be
anonymous. A stamped addressed envelope was
included to facilitate return of the questionnaire.
Results
Ninety-six questionnaires (34%) were returned and
analysed, using simple descriptive statistics due to
the heterogeneity of the study group.
Demographics: n=96
Sixty-two females and 34 males responded with ages
ranging from 18-83 years, with a mean age of 39
years 6 months. Of these the majority were born in
the period 1960-1988 (70%).
All identified themselves as white British apart from
2 people from Eire.
Twenty-seven people had been diagnosed with
classical AMC (amyoplasia) but 53 others did not
know what type of AMC they had. From their
description of the joints involved they are included
in the classical group. Thirteen people had a
diagnosis of distal AMC and 3 had syndromes
associated with AMC.
Table 1: Age distribution n=96
Decade born No. of respondents
1930s 4 Fourteen thought it had made securing a job more
1940s 12
1950s 14
1950s 20
1960s 18
1980s 28
Education : n=96
From 1950 to 1970 the majority attended schools for
physically handicapped children. Before 1950, and
after 1970, most children were educated in
mainstream establishments with increasing
additional support such as the provision of suitable
access, adjustments to timetables and examinations,
and learning support - 1970s (8/18), 1980s (19/26).
Sixty-four (66%) people had gone on to further
education.
Family Life: n=96
For 8 of the respondents with AMC there was a
family history of the condition. Thirty-two people
were married 11 of these had a partner with a
disability. Eleven lived with a partner whilst 48
people were single. Five people had separated or
were divorced. There were 27 families with a total of
57 children 33 girls and 24 boys. Six children also
had AMC.
Fifty-one people lived independently whilst 40
others managed with family support. Five people
were accommodated in sheltered housing.
Additional support was provided, as needed, by
social services (care and nursing agencies) or paid
private care. Twenty people required additional
night-time support.
Work: n=91
Of those that completed this section, 35 people were
employed and 3 self-employed. Ten people carried
out voluntary work. Fourteen of the respondents
were students. Six people said they were retired
with a further 6 stating they were homemakers.
Seventeen people were unable to work because of
their condition.
Gaps in employment because of AMC were cited by
16 people with health, limited opportunities, and
problems with access identified as challenges. Low
expectations by employers or work colleagues and
work place bullying were also identified as major
problems. Personal difficulties were acknowledged
with body image, pain and tiredness impacting on
daily work experiences.
Thirty people stated that they had experienced
discrimination, which they put down to their AMC.
difficult. In the work place, 4 had experienced
discrimination from staff and another 4 thought that
keeping a job once appointed had been challenging.
Seven of the respondents also stated that seeking or
achieving promotion was possibly compromised by
them having AMC.
Sixty-one people volunteered information about
their income. Of these the majority earned less than
30,000 per annum, with only 3 people with earnings
up to 40,000 and one with earnings over 50,000.
When asked if they viewed that their earning
potential had been affected by their AMC, 40 people
who answered the question thought it had and 42
thought that it had not. Two people were uncertain.
Seventy-four people claimed disability benefits.
Time off from work due to problems of pain or
tiredness caused by their AMC was identified by 15
people. Seven people stated that they used holiday
as rest days to charge batteries, because of pain, or to
attend hospital appointments.
Physical abilities: n=96
Sixty of the respondents reported that they had
21
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
involve only joints in the upper or the lower limbs.
There may also be other congenital abnormalities
such as bowel atresia, hernias, etc. Sensation is
normal. It is usually a sporadic occurrence and is not
thought to be inherited. Distal arthrogryposis,
however, does have an autosomal dominant
background (Hall et al, 1982). In these cases there are
severe deformities in the hands and feet, with
perhaps minor contractures proximally. These
children may develop scoliosis later.
Enabling children born with AMC to achieve
optimal participation levels in their daily life
requires a large multi-disciplinary team physicians,
surgeons, therapists, orthotists, dieticians, etc. with
specialised input throughout their childhood years.
For most young people with disabilities, and their
families, transition into adult services is fraught,
with reports of lack of therapy input and lack of
knowledge concerning congenital conditions from
those working in adult services. For young adults
with AMC this change can be particularly difficult,
as many have transferred from dedicated AMC
clinics held at some of the major childrens hospitals
in England and Scotland.
Fassier et al (2009) published a long term AMC
study but sadly only covered the years from birth
until skeletal maturity. In fact most literature seems
to be paediatric and medically-centred and ignores
the impact of every day living with AMC. Staheli et
als useful book on AMC (1998) covers all paediatric
aspects of the condition and is a valuable source of
current knowledge but has no information or
discussion of AMC in adulthood. A final chapter
dealing with the long-term effects of AMC and adult
issues, such as employment and other resources
available, would have completed this book.
Information and literature about AMC in adult life is
sadly lacking and it is therefore understandable that
those providing services to adults with AMC appear
to have a poor knowledge or understanding of the
condition.
The Arthrogryposis Group (TAG) is a UK charity
providing valuable support and advice for families
who have a child with AMC and also for adults with
the condition. An annual weekend conference
allows those with AMC and their families to meet
and attend lectures on various aspects of the
medical, surgical and therapeutic management of
this condition, as well as covering other aspects such
as psychological, educational and social issues. This
conference is also open to interested healthcare
professionals and can give useful insight into the
long-term ramifications of living with the condition,
as well as a chance to meet adults with AMC.
20
Adults with AMC are often isolated and value the
annual conference as a chance to meet others with
the condition. Through discussions many of them
wondered whether many of the problems they were
experiencing, particularly physical symptoms, were
common to others with AMC or just related to
natural aging. Parents of children with AMC were
also keen to know what the challenges may be once
their child becomes an adult. From these
conversations it was decided that a survey of adults
with AMC would be useful to address some of the
unanswered questions. A similar survey has been
carried out by Avenues, the AMC support group in
the USA and also addressed similar concerns as
those included in this study (Sneddon, 1999).
Method
Adult members of TAG with AMC were invited to
attend a workshop held during the TAG annual
conference. As the result of discussion they
identified issues that they wanted to explore about
AMC. A questionnaire was developed to capture
information about the following domains:
demographic information - age, sex, ethnicity, type
of AMC, body parts affected;
education - type of school attended, leaving age,
academic qualifications, special educational needs,
other support, further education;
family life - family history of AMC, marital status,
children, issues around independent living;
employment - occupation, discrimination, support,
income, benefits, impact of AMC on employment
opportunities;
physical abilities - abilities, mobility aids, prob-
lems;
general health - general health, impact of AMC,
childhood problems, problems as an adult;
surgery - in childhood and as an adult;
therapies - physiotherapy input, other therapies,
orthotics, perceived therapy needs as an adult;
pain - frequency, intensity and location of pain,
medication, strategies;
disability and life - impact of AMC on social life,
hobbies, sports, self-esteem, personal relationships,
thoughts about the future and disability, achieve-
ments.
The questionnaire consisted of closed and open
questions and was piloted by 6 adults with AMC, all
members of TAG. Changes were made in response
to their feedback. A final page was left blank so that
thoughts and comments could be added should the
respondents wish to provide further information.
The finalised questionnaire was posted to 295 adult
members of TAG with AMC, who were 18 years and
over. An accompanying letter of invitation to take
J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
part in the survey was included. This emphasised
that participation was voluntary and reassurance
was given that information provided would be
anonymous. A stamped addressed envelope was
included to facilitate return of the questionnaire.
Results
Ninety-six questionnaires (34%) were returned and
analysed, using simple descriptive statistics due to
the heterogeneity of the study group.
Demographics: n=96
Sixty-two females and 34 males responded with ages
ranging from 18-83 years, with a mean age of 39
years 6 months. Of these the majority were born in
the period 1960-1988 (70%).
All identified themselves as white British apart from
2 people from Eire.
Twenty-seven people had been diagnosed with
classical AMC (amyoplasia) but 53 others did not
know what type of AMC they had. From their
description of the joints involved they are included
in the classical group. Thirteen people had a
diagnosis of distal AMC and 3 had syndromes
associated with AMC.
Table 1: Age distribution n=96
Decade born No. of respondents
1930s 4 Fourteen thought it had made securing a job more
1940s 12
1950s 14
1950s 20
1960s 18
1980s 28
Education : n=96
From 1950 to 1970 the majority attended schools for
physically handicapped children. Before 1950, and
after 1970, most children were educated in
mainstream establishments with increasing
additional support such as the provision of suitable
access, adjustments to timetables and examinations,
and learning support - 1970s (8/18), 1980s (19/26).
Sixty-four (66%) people had gone on to further
education.
Family Life: n=96
For 8 of the respondents with AMC there was a
family history of the condition. Thirty-two people
were married 11 of these had a partner with a
disability. Eleven lived with a partner whilst 48
people were single. Five people had separated or
were divorced. There were 27 families with a total of
57 children 33 girls and 24 boys. Six children also
had AMC.
Fifty-one people lived independently whilst 40
others managed with family support. Five people
were accommodated in sheltered housing.
Additional support was provided, as needed, by
social services (care and nursing agencies) or paid
private care. Twenty people required additional
night-time support.
Work: n=91
Of those that completed this section, 35 people were
employed and 3 self-employed. Ten people carried
out voluntary work. Fourteen of the respondents
were students. Six people said they were retired
with a further 6 stating they were homemakers.
Seventeen people were unable to work because of
their condition.
Gaps in employment because of AMC were cited by
16 people with health, limited opportunities, and
problems with access identified as challenges. Low
expectations by employers or work colleagues and
work place bullying were also identified as major
problems. Personal difficulties were acknowledged
with body image, pain and tiredness impacting on
daily work experiences.
Thirty people stated that they had experienced
discrimination, which they put down to their AMC.
difficult. In the work place, 4 had experienced
discrimination from staff and another 4 thought that
keeping a job once appointed had been challenging.
Seven of the respondents also stated that seeking or
achieving promotion was possibly compromised by
them having AMC.
Sixty-one people volunteered information about
their income. Of these the majority earned less than
30,000 per annum, with only 3 people with earnings
up to 40,000 and one with earnings over 50,000.
When asked if they viewed that their earning
potential had been affected by their AMC, 40 people
who answered the question thought it had and 42
thought that it had not. Two people were uncertain.
Seventy-four people claimed disability benefits.
Time off from work due to problems of pain or
tiredness caused by their AMC was identified by 15
people. Seven people stated that they used holiday
as rest days to charge batteries, because of pain, or to
attend hospital appointments.
Physical abilities: n=96
Sixty of the respondents reported that they had
21
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26

noticed that their mobility was decreasing as they Table 5: Assessment of present physical problems n=64 Sixty people thought that the physiotherapy input as were worried about having children with concerns
aged, including 16 out of 20 born in the 1960s, 14 out a child was helpful, but 24 did not agree. Thirty-four about coping 3 of these worried about having
of 18 born in the 1970s, and 6 out of 28 of those born Problem 1930s 1940s 1950s 1960s 1970s 1980s children had occupational therapy and 22 had children with AMC and 1 stated that they may prefer
in the 1980s. Some reported increasing reliance on ing muscle weakness 1 7 4 7 8 5 speech and language therapy. Three were seen by to adopt or foster a child.
mobility aids, particularly after the age of 40 years. ing joint stiffness 8 11 13 12 7 dieticians.
Assessment of mobility levels showed that 22 Thoughts about the future were of no concern for 21
Joint wear and tear 1 8 12 15 9 4
viewed that they were community walkers, whilst 18 The majority of the respondents used some form of people live one day at a time, keep going, the
were unable to walk at all. The remaining ing tiring 2 7 9 10 9 4 splintage as a child, more commonly in those born priority is to enjoy life. Leaving home, finding work
respondents were limited walkers, some able to walk ing problems breathing 3 3 3 3 2 from the 1950s onwards. 29 people used Ankle Foot or changing jobs to match physical needs challenged
short distances outside and some household walkers Back/ neck problems 6 8 11 12 7 Orthoses and 20 people used Knee Ankle Foot 12 people.
only, as illustrated in Table 2. Orthoses. Twenty-two used upper limb splints and
ing weight 1 7 5 14 7 8
1 used a spinal brace. Eighteen used splints only Mobility concerns were expressed by 48 people
Of those that owned cars, 34 out of 49 drivers had Other 6 12 8 10 10 during the day and 12 used them only during the (50%) with issues around decreasing independence
cars with automatic transmission, 13 used cars with night. Both day and night splintage was used by 15. alongside increasing dependence (more reliance on
adaptations, and a further 13 were driven by carers. Under the heading of Other, pain that impacted on Seventeen adults still used splints. others, wheelchairs, etc.) expressed by 25. Mobility
their daily life was cited as a problem for 21 people. problems at a relatively young age were identified
Sixteen people complained of general slowing As adults, 13 people used a physiotherapy service; 9 by 15 people. Twenty individuals were concerned
Table 2: Distribution of reported mobility levels for each decade down, including 9 people born in the 1970s and used occupational therapy; and 4 used chiropody. about the effects of increasing weight gain on their
of birth. n=60 1980s. Three people had noticed an increasing Ten people used complementary therapies such as joints and affecting their mobility levels.
dependence on others whilst 2 complained of mental acupuncture, Chinese medicine, massage,
Mobility level 1930s 1940s 1950s 1960s 1970s 1980s fatigue, and 2 of stress. meditation and counselling. In reply to the question 72 people expressed positive comments about their
Walk anywhere 1 1 4 6 10 Do you think you should have some therapy input? disability deal with it / live with it (12), part of who
Short distance outside 2 5 7 8 6 10 Questions regarding health as a child showed that 10 24 people agreed, with 4 suggesting that they would I am (27), it does not stop me (22), makes me more
In house mainly 1 5 7 8 3 had experienced floppy or blank episodes, and 5 had particularly like hydrotherapy. Reasons for wanting determined (11). Negative comments from the
Unable to walk 4 2 6 2 4 had fits. Thirty, as children, had problems with body physiotherapy were to help maintain mobility, and remainder included frustrations regarding physical
temperature regulation, and 20 had problems with to help with pain and muscle weakness. Difficulties limitations (11), body image, limited choices,
Questions focusing on day-to-day function showed a general anaesthesia. Fifteen people had eye with obtaining a referral, and lack of knowledge and emotional problems (6), sadness, anger and
wide variety of ability and disability at various tasks problems related to AMC. Of the 33 people who had experience of the condition amongst health resignation by others I hate it, what would life be
amongst those that responded and are illustrated by digestive system problems, 13 stated that they were professionals were seen as major obstacles to be like without AMC, ruined part of my life.
Tables 3 and 4. linked to AMC, with a further 16 unsure if there was overcome.
a link. The final question in this section asked the
Pain n=96 respondents to list achievements that they felt were
Table 3: Reported day to day abilities within the home n=94 General health as an adult was assessed as poor by Eighty-two people stated that they had experienced worthy of mention. Themes identified included:
23 of the respondents - constipation affected 17. pain over the past month and 68 of these (83%) determination and courage to overcome limitations
During the past week how easy Easy A little hard Very hard Cant do at all Diabetes, high blood pressure, osteoporosis, high stated that the pain was related to their AMC. (14);
was it too:
cholesterol, sleep apnoeas and mental health Fifteen of these had pain all the time and 29 on most finding a partner (6);
Lift a kettle of water 45 30 6 13 problems were also cited as general health problems. days. Prescription analgesia was used by 23 people having children (12);
Pour from a pint of milk 62 21 3 8
with a further 18 relying on over-the-counter living independently (15);
Open a jar that has been opened before 50 22 15 10 Surgery n=96: products. For 48 people, pain stopped their activity. educational achievements (18);
Use a fork and spoon 70 16 3 5 Those requiring surgery in childhood had work - record (20), running a business (6), volun-
Comb your hair 56 18 5 15 undergone corrective procedures to their knees and Disability and Life n=96 tary work (10);
Manage buttons 49 13 15 17 feet. Two had had below knee amputations and 4 Fifty-nine people thought that having AMC did not driving (11).
spinal fusions had been carried out. affect their social lives as long as forward planning
and access had been considered. The remaining 39 General achievements included a wide variety of
Table 4: Respondents assessment of their mobility levels n=94 In adulthood further procedures had been needed to identified issues such as low self-esteem, shyness, challenging activities such as abseiling from the
the foot and ankle for 5 people, and 5 had needed body image, pain and tiredness as major problems in Humber Bridge, sky diving, mountain climbing,
During the past week how easy Easy A little hard Very hard Cant do at all surgery to improve function at the knees. Two socialising, as well as needing to ask for help and water skiing, 48-hour sponsored wheelie, and
was it too:
people had spinal surgery and 2 others, surgery for difficulties with access. obtaining several Olympic Paralympic Dressage
Walk 100 m. 29 20 14 31 their upper limbs. Four people had hip joint gold medals; as well as getting published, being a
Go up and down stairs 23 20 18 33 replacement surgery, with 2 requiring repeat Personal relationships were not an issue for 30 foot and mouth artist, and winning a Blue Peter
Get in and out of bed 45 31 7 11 replacements. people I am who I am and 3 stated that their Badge.
Move between rooms 60 23 4 7 disability had helped with meeting people. Seven
Run for a bus 13 9 6 66 Therapies n=96: respondents stated that they had found their social The final blank page was used by 43 people to
Walk a mile 18 8 7 61 As children, 88 had physiotherapy input, but of these life difficult as a teenager, but as adults they found express issues and opinions not covered in the
3 had input only after surgery. In secondary school people less judgemental. Two people found it easier questionnaire. Obtaining funding for equipment,
Opinions regarding problems being experienced at 45/96 were seen by a physiotherapist, but again 3 to have close relationships with other disabled adaptations to housing, personal care, etc. was seen
the time of the study are illustrated in Table 5 below. only received input after surgery. people, but 4 worried about intimacy and felt they as too complex, and means testing, as unfair. Several
had missed part of life by not marrying. Eight people complained about the provision of shoes and

22 23
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26

noticed that their mobility was decreasing as they Table 5: Assessment of present physical problems n=64 Sixty people thought that the physiotherapy input as were worried about having children with concerns
aged, including 16 out of 20 born in the 1960s, 14 out a child was helpful, but 24 did not agree. Thirty-four about coping 3 of these worried about having
of 18 born in the 1970s, and 6 out of 28 of those born Problem 1930s 1940s 1950s 1960s 1970s 1980s children had occupational therapy and 22 had children with AMC and 1 stated that they may prefer
in the 1980s. Some reported increasing reliance on ing muscle weakness 1 7 4 7 8 5 speech and language therapy. Three were seen by to adopt or foster a child.
mobility aids, particularly after the age of 40 years. ing joint stiffness 8 11 13 12 7 dieticians.
Assessment of mobility levels showed that 22 Thoughts about the future were of no concern for 21
Joint wear and tear 1 8 12 15 9 4
viewed that they were community walkers, whilst 18 The majority of the respondents used some form of people live one day at a time, keep going, the
were unable to walk at all. The remaining ing tiring 2 7 9 10 9 4 splintage as a child, more commonly in those born priority is to enjoy life. Leaving home, finding work
respondents were limited walkers, some able to walk ing problems breathing 3 3 3 3 2 from the 1950s onwards. 29 people used Ankle Foot or changing jobs to match physical needs challenged
short distances outside and some household walkers Back/ neck problems 6 8 11 12 7 Orthoses and 20 people used Knee Ankle Foot 12 people.
only, as illustrated in Table 2. Orthoses. Twenty-two used upper limb splints and
ing weight 1 7 5 14 7 8
1 used a spinal brace. Eighteen used splints only Mobility concerns were expressed by 48 people
Of those that owned cars, 34 out of 49 drivers had Other 6 12 8 10 10 during the day and 12 used them only during the (50%) with issues around decreasing independence
cars with automatic transmission, 13 used cars with night. Both day and night splintage was used by 15. alongside increasing dependence (more reliance on
adaptations, and a further 13 were driven by carers. Under the heading of Other, pain that impacted on Seventeen adults still used splints. others, wheelchairs, etc.) expressed by 25. Mobility
their daily life was cited as a problem for 21 people. problems at a relatively young age were identified
Sixteen people complained of general slowing As adults, 13 people used a physiotherapy service; 9 by 15 people. Twenty individuals were concerned
Table 2: Distribution of reported mobility levels for each decade down, including 9 people born in the 1970s and used occupational therapy; and 4 used chiropody. about the effects of increasing weight gain on their
of birth. n=60 1980s. Three people had noticed an increasing Ten people used complementary therapies such as joints and affecting their mobility levels.
dependence on others whilst 2 complained of mental acupuncture, Chinese medicine, massage,
Mobility level 1930s 1940s 1950s 1960s 1970s 1980s fatigue, and 2 of stress. meditation and counselling. In reply to the question 72 people expressed positive comments about their
Walk anywhere 1 1 4 6 10 Do you think you should have some therapy input? disability deal with it / live with it (12), part of who
Short distance outside 2 5 7 8 6 10 Questions regarding health as a child showed that 10 24 people agreed, with 4 suggesting that they would I am (27), it does not stop me (22), makes me more
In house mainly 1 5 7 8 3 had experienced floppy or blank episodes, and 5 had particularly like hydrotherapy. Reasons for wanting determined (11). Negative comments from the
Unable to walk 4 2 6 2 4 had fits. Thirty, as children, had problems with body physiotherapy were to help maintain mobility, and remainder included frustrations regarding physical
temperature regulation, and 20 had problems with to help with pain and muscle weakness. Difficulties limitations (11), body image, limited choices,
Questions focusing on day-to-day function showed a general anaesthesia. Fifteen people had eye with obtaining a referral, and lack of knowledge and emotional problems (6), sadness, anger and
wide variety of ability and disability at various tasks problems related to AMC. Of the 33 people who had experience of the condition amongst health resignation by others I hate it, what would life be
amongst those that responded and are illustrated by digestive system problems, 13 stated that they were professionals were seen as major obstacles to be like without AMC, ruined part of my life.
Tables 3 and 4. linked to AMC, with a further 16 unsure if there was overcome.
a link. The final question in this section asked the
Pain n=96 respondents to list achievements that they felt were
Table 3: Reported day to day abilities within the home n=94 General health as an adult was assessed as poor by Eighty-two people stated that they had experienced worthy of mention. Themes identified included:
23 of the respondents - constipation affected 17. pain over the past month and 68 of these (83%) determination and courage to overcome limitations
During the past week how easy Easy A little hard Very hard Cant do at all Diabetes, high blood pressure, osteoporosis, high stated that the pain was related to their AMC. (14);
was it too:
cholesterol, sleep apnoeas and mental health Fifteen of these had pain all the time and 29 on most finding a partner (6);
Lift a kettle of water 45 30 6 13 problems were also cited as general health problems. days. Prescription analgesia was used by 23 people having children (12);
Pour from a pint of milk 62 21 3 8
with a further 18 relying on over-the-counter living independently (15);
Open a jar that has been opened before 50 22 15 10 Surgery n=96: products. For 48 people, pain stopped their activity. educational achievements (18);
Use a fork and spoon 70 16 3 5 Those requiring surgery in childhood had work - record (20), running a business (6), volun-
Comb your hair 56 18 5 15 undergone corrective procedures to their knees and Disability and Life n=96 tary work (10);
Manage buttons 49 13 15 17 feet. Two had had below knee amputations and 4 Fifty-nine people thought that having AMC did not driving (11).
spinal fusions had been carried out. affect their social lives as long as forward planning
and access had been considered. The remaining 39 General achievements included a wide variety of
Table 4: Respondents assessment of their mobility levels n=94 In adulthood further procedures had been needed to identified issues such as low self-esteem, shyness, challenging activities such as abseiling from the
the foot and ankle for 5 people, and 5 had needed body image, pain and tiredness as major problems in Humber Bridge, sky diving, mountain climbing,
During the past week how easy Easy A little hard Very hard Cant do at all surgery to improve function at the knees. Two socialising, as well as needing to ask for help and water skiing, 48-hour sponsored wheelie, and
was it too:
people had spinal surgery and 2 others, surgery for difficulties with access. obtaining several Olympic Paralympic Dressage
Walk 100 m. 29 20 14 31 their upper limbs. Four people had hip joint gold medals; as well as getting published, being a
Go up and down stairs 23 20 18 33 replacement surgery, with 2 requiring repeat Personal relationships were not an issue for 30 foot and mouth artist, and winning a Blue Peter
Get in and out of bed 45 31 7 11 replacements. people I am who I am and 3 stated that their Badge.
Move between rooms 60 23 4 7 disability had helped with meeting people. Seven
Run for a bus 13 9 6 66 Therapies n=96: respondents stated that they had found their social The final blank page was used by 43 people to
Walk a mile 18 8 7 61 As children, 88 had physiotherapy input, but of these life difficult as a teenager, but as adults they found express issues and opinions not covered in the
3 had input only after surgery. In secondary school people less judgemental. Two people found it easier questionnaire. Obtaining funding for equipment,
Opinions regarding problems being experienced at 45/96 were seen by a physiotherapist, but again 3 to have close relationships with other disabled adaptations to housing, personal care, etc. was seen
the time of the study are illustrated in Table 5 below. only received input after surgery. people, but 4 worried about intimacy and felt they as too complex, and means testing, as unfair. Several
had missed part of life by not marrying. Eight people complained about the provision of shoes and

22 23
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
orthoses as they felt there was no discussion and no
choice: Professionals have the technical expertise but
often we know what is best for us. Listen to us.
Four people wrote about their late diagnosis of
AMC. Of these two had been given the diagnosis in
their 50s, and 1 after giving birth to a child with
AMC with similar joint problems.
Two people, born in the 1980s, had experienced
bullying in mainstream school and thought that their
academic progress had been affected. Both said that
they would have been better off in a PH school.
Seven people expressed gratitude for the support
provided by TAG and some suggested that local
groups could be set up for socialising. Four people
had felt isolated and had never seen anyone else
with AMC until attending a TAG annual conference.
Five people commented on this survey as a chance to
put thoughts on paper after a life with AMC, and 3 said
that they were glad there is a survey of adult life as there
is only information about children.
Discussion
Hall (1988) stated that the study of natural history
from individual parents and their records is
complementary to population or registry-based
studies because it identifies individual variations
and heterogeneity. The understanding of the natural
history of a particular disorder is of importance both
to the affected person and their family, and to the
professionals caring for them. Older affected
subjects and unique cases with unusual features are
often most important in unravelling the normal
course of a disease or recognising the basic defect.
The ultimate goal of treatment for children with
AMC is for them to develop into self-confident
adults who can cope with life despite their
handicaps. This is reflected in the literature, which
concentrates on AMC during childhood and
adolescence, but there is a dearth of information
about living with AMC in adult life. These opinions
are mirrored by Sneddons survey in 1999.
This study arose out of conversations with, and
amongst, adults with AMC attending TAGs annual
weekend conference. Through these conversations
and discussions it was apparent that many were
concerned to discover if symptoms they were
experiencing in adulthood were common amongst
those affected by AMC or were they not even related
to their condition.
This survey gathered information about childhood
24
and adult life experiences and provided a historical
view; for example about educational provision
changing to attendance at schools for children with
physical handicap in the 1960s and then reflecting
the move to inclusive education during the 1980s in
mainstream school with learning and therapy
support.
Most had physiotherapy as a child but input
dropped to less than half in adolescence. This
reflects, in the main, the attention and focus in the
early years on function and mobility against the
demand of the school day structure and academic
workload in adolescence. Jaffe (1998) cautioned on
attention being focused only on childhood and
adolescent years, as it was easy to lose sight of the
fact that adulthood approaches rapidly.
There is now an increasing interest in transition
services to provide a co-ordinated set of activities
that promote the move from school to adult services
and settings. For some adolescents with congenital
conditions, the move from paediatric to adult care is
seen as a natural progression to a service with
knowledge and experience (Moons et al, 2009). The
experience of adults with intellectual disabilities
showed insufficient knowledge and lack of expertise
amongst health professionals (van Schrojenstein
Lan-de Valk, 2005) and is more in line with the
opinions of adults with AMC. Difficulties in
accessing adult healthcare provision were
highlighted in this study, especially a general lack of
knowledge of AMC amongst therapists and doctors
working in adult services. This is not surprising
since the reported incidence of classical AMC is
1:10,000 live births. Children with AMC now tend to
be managed in dedicated clinics within specialist
childrens services, with experienced multi-
disciplinary teams to support them and the family;
but this has not been replicated in adult services.
Some adults with AMC thought that their diagnosis
was often a barrier to help with problems they were
experiencing - such as joint and generalised pain,
and muscle weakness even though these issues
were similar to those experienced by other adults as
part of the aging process.
From opinions garnered from this survey, as well as
discussion and lobbying amongst TAG members and
health professionals, the first dedicated clinic for
adults with AMC has been set up at the Robert Jones
and Agnes Hunt Hospital in Oswestry.
Attitudes towards life with AMC were broadly
positive with many reporting acceptance and the
view that there is always someone worse off and that
their disability made them more determined and
resilient to cope with personal, social and
J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
environmental challenges. This is a similar finding
to a study by Resende and Neri (2009) who found
that people with a congenital disability reported
higher psychological adjustment and showed more
positive perspectives of personal aging. There were
many concerns around difficulties in securing
funding to support living with a disability, access
and opportunities, plus low expectations and the
need to modify lifestyle to cope with work demands.
Limitations to the study
The desire to capture a snapshot of the life
experiences of adults with AMC has shown several
limitations. The age group (18-83) and its
heterogeneity have made it difficult to analyse in any
statistical form apart from using the narratives
provided. Narratives, however, can be powerful
tools in conveying experiences and opinions.
Lack of a control group, perhaps with age matching,
could have provided deeper analysis but this survey
was devised to answer questions that TAG members
viewed as important at that time and perhaps,
therefore, specific to their condition.
Uncertainty regarding specific diagnoses of the type
of AMC for some of the participants did not allow
true comparison between, for example, those with
distal AMC and those with classical AMC.
There was a disappointing low response rate of 34%,
despite the survey being carried out 6 months after
the questionnaire workshop and publicity in the
TAG magazine. As this was a self-selected group of
people with AMC (i.e. all were members of TAG and
had AMC), it may be due to the length of the survey
itself as well as individuals perceptions that their
situation was exceptional and would not be relevant.
It has been found that response rate increases if
questionnaires are short and participants are
contacted beforehand (Edwards et al, 2002). It is
possible that this study tried to cover too wide a
remit and this may have discouraged some to
respond.
The age span from 18-83 years has allowed a long-
term view of living with AMC although the
manifestations of the condition (physically,
economically and psycho-socially) obviously varied
widely amongst the study group. Any future study
into adult issues around AMC would benefit from
being more focused so that, for example, physical
function and limitation could be investigated in
greater depth.
Conclusion
This survey was an attempt to find out about the
lives of adults with AMC and highlights many issues
that are common amongst all with disability
(funding, access, limited opportunities, etc.). The
literature about living with congenital disabilities as
an adult is sparse and there is a great desire amongst
TAG members to discover whether their life
experiences are common to others with the
condition. It was not an attempt at scientific
analysis, or to look at outcomes from interventions
or to make comparisons, but to provide a picture of
the status quo at the time of the survey. It is hoped
that the information provided about the common
problems and symptoms experienced in adulthood
will be useful to others with AMC, the parents of
children with AMC, and healthcare professionals.
This information may also provide an insight into
the longer-term view of a challenging congenital
condition seen by paediatric physiotherapists.
Acknowledgements
This study was funded by The Arthrogryposis
Group. The authors would like to thank TAG
members who helped devise the survey and also to
those who responded.
For further information on The Arthrogryposis
Group please visit www.tagonline.org.uk
References
Edwards. Roberts I, Clarke M, Di Giuseppi C, Pratap S,
Wentz M, Kwan I (2002). Increasing response rates to
postal questionnaires: systematic review. BMJ 2002: 324:
1183.
Fassier A, Wicart P, Dubbousset J, Seringe R (2008).
Arthrogryposis multiplex congenital. Long term follow-up
from birth until skeletal majority. J Child Orthop, 3(5): 383-
390.
Fixsen JA (2010). Athrogryposis multiplex congenital. In:
Benson M, Fixsen J, Macnicol M, Parsch K, eds. Childrens
Orthopaedics and Fractures 3rd Edition. Springer. Chapter 20
327-334.
Hall JG, Reed SG, Green G. (1982). The distal
arthrogryposis: delineation of new entities: review and
nosologic discussion. Am J Med Gen, 11: 185-239.
Hall JG (1988). The value of the study of natural history in
genetic disorders and congential anomaly syndromes. J.
Med. Genet. 1988;25:434-444.
Hall JG (1998). Overview of arthrogryposis. In: Staheli LT,
Hall JG, Jaffe KM, Pahoike DE, eds. Arthrogryposis: A Text
Atlas. Cambridge: Cambridge University Press: 1-25.
25
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
orthoses as they felt there was no discussion and no
choice: Professionals have the technical expertise but
often we know what is best for us. Listen to us.
Four people wrote about their late diagnosis of
AMC. Of these two had been given the diagnosis in
their 50s, and 1 after giving birth to a child with
AMC with similar joint problems.
Two people, born in the 1980s, had experienced
bullying in mainstream school and thought that their
academic progress had been affected. Both said that
they would have been better off in a PH school.
Seven people expressed gratitude for the support
provided by TAG and some suggested that local
groups could be set up for socialising. Four people
had felt isolated and had never seen anyone else
with AMC until attending a TAG annual conference.
Five people commented on this survey as a chance to
put thoughts on paper after a life with AMC, and 3 said
that they were glad there is a survey of adult life as there
is only information about children.
Discussion
Hall (1988) stated that the study of natural history
from individual parents and their records is
complementary to population or registry-based
studies because it identifies individual variations
and heterogeneity. The understanding of the natural
history of a particular disorder is of importance both
to the affected person and their family, and to the
professionals caring for them. Older affected
subjects and unique cases with unusual features are
often most important in unravelling the normal
course of a disease or recognising the basic defect.
The ultimate goal of treatment for children with
AMC is for them to develop into self-confident
adults who can cope with life despite their
handicaps. This is reflected in the literature, which
concentrates on AMC during childhood and
adolescence, but there is a dearth of information
about living with AMC in adult life. These opinions
are mirrored by Sneddons survey in 1999.
This study arose out of conversations with, and
amongst, adults with AMC attending TAGs annual
weekend conference. Through these conversations
and discussions it was apparent that many were
concerned to discover if symptoms they were
experiencing in adulthood were common amongst
those affected by AMC or were they not even related
to their condition.
This survey gathered information about childhood
24
and adult life experiences and provided a historical
view; for example about educational provision
changing to attendance at schools for children with
physical handicap in the 1960s and then reflecting
the move to inclusive education during the 1980s in
mainstream school with learning and therapy
support.
Most had physiotherapy as a child but input
dropped to less than half in adolescence. This
reflects, in the main, the attention and focus in the
early years on function and mobility against the
demand of the school day structure and academic
workload in adolescence. Jaffe (1998) cautioned on
attention being focused only on childhood and
adolescent years, as it was easy to lose sight of the
fact that adulthood approaches rapidly.
There is now an increasing interest in transition
services to provide a co-ordinated set of activities
that promote the move from school to adult services
and settings. For some adolescents with congenital
conditions, the move from paediatric to adult care is
seen as a natural progression to a service with
knowledge and experience (Moons et al, 2009). The
experience of adults with intellectual disabilities
showed insufficient knowledge and lack of expertise
amongst health professionals (van Schrojenstein
Lan-de Valk, 2005) and is more in line with the
opinions of adults with AMC. Difficulties in
accessing adult healthcare provision were
highlighted in this study, especially a general lack of
knowledge of AMC amongst therapists and doctors
working in adult services. This is not surprising
since the reported incidence of classical AMC is
1:10,000 live births. Children with AMC now tend to
be managed in dedicated clinics within specialist
childrens services, with experienced multi-
disciplinary teams to support them and the family;
but this has not been replicated in adult services.
Some adults with AMC thought that their diagnosis
was often a barrier to help with problems they were
experiencing - such as joint and generalised pain,
and muscle weakness even though these issues
were similar to those experienced by other adults as
part of the aging process.
From opinions garnered from this survey, as well as
discussion and lobbying amongst TAG members and
health professionals, the first dedicated clinic for
adults with AMC has been set up at the Robert Jones
and Agnes Hunt Hospital in Oswestry.
Attitudes towards life with AMC were broadly
positive with many reporting acceptance and the
view that there is always someone worse off and that
their disability made them more determined and
resilient to cope with personal, social and
J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26
environmental challenges. This is a similar finding
to a study by Resende and Neri (2009) who found
that people with a congenital disability reported
higher psychological adjustment and showed more
positive perspectives of personal aging. There were
many concerns around difficulties in securing
funding to support living with a disability, access
and opportunities, plus low expectations and the
need to modify lifestyle to cope with work demands.
Limitations to the study
The desire to capture a snapshot of the life
experiences of adults with AMC has shown several
limitations. The age group (18-83) and its
heterogeneity have made it difficult to analyse in any
statistical form apart from using the narratives
provided. Narratives, however, can be powerful
tools in conveying experiences and opinions.
Lack of a control group, perhaps with age matching,
could have provided deeper analysis but this survey
was devised to answer questions that TAG members
viewed as important at that time and perhaps,
therefore, specific to their condition.
Uncertainty regarding specific diagnoses of the type
of AMC for some of the participants did not allow
true comparison between, for example, those with
distal AMC and those with classical AMC.
There was a disappointing low response rate of 34%,
despite the survey being carried out 6 months after
the questionnaire workshop and publicity in the
TAG magazine. As this was a self-selected group of
people with AMC (i.e. all were members of TAG and
had AMC), it may be due to the length of the survey
itself as well as individuals perceptions that their
situation was exceptional and would not be relevant.
It has been found that response rate increases if
questionnaires are short and participants are
contacted beforehand (Edwards et al, 2002). It is
possible that this study tried to cover too wide a
remit and this may have discouraged some to
respond.
The age span from 18-83 years has allowed a long-
term view of living with AMC although the
manifestations of the condition (physically,
economically and psycho-socially) obviously varied
widely amongst the study group. Any future study
into adult issues around AMC would benefit from
being more focused so that, for example, physical
function and limitation could be investigated in
greater depth.
Conclusion
This survey was an attempt to find out about the
lives of adults with AMC and highlights many issues
that are common amongst all with disability
(funding, access, limited opportunities, etc.). The
literature about living with congenital disabilities as
an adult is sparse and there is a great desire amongst
TAG members to discover whether their life
experiences are common to others with the
condition. It was not an attempt at scientific
analysis, or to look at outcomes from interventions
or to make comparisons, but to provide a picture of
the status quo at the time of the survey. It is hoped
that the information provided about the common
problems and symptoms experienced in adulthood
will be useful to others with AMC, the parents of
children with AMC, and healthcare professionals.
This information may also provide an insight into
the longer-term view of a challenging congenital
condition seen by paediatric physiotherapists.
Acknowledgements
This study was funded by The Arthrogryposis
Group. The authors would like to thank TAG
members who helped devise the survey and also to
those who responded.
For further information on The Arthrogryposis
Group please visit www.tagonline.org.uk
References
Edwards. Roberts I, Clarke M, Di Giuseppi C, Pratap S,
Wentz M, Kwan I (2002). Increasing response rates to
postal questionnaires: systematic review. BMJ 2002: 324:
1183.
Fassier A, Wicart P, Dubbousset J, Seringe R (2008).
Arthrogryposis multiplex congenital. Long term follow-up
from birth until skeletal majority. J Child Orthop, 3(5): 383-
390.
Fixsen JA (2010). Athrogryposis multiplex congenital. In:
Benson M, Fixsen J, Macnicol M, Parsch K, eds. Childrens
Orthopaedics and Fractures 3rd Edition. Springer. Chapter 20
327-334.
Hall JG, Reed SG, Green G. (1982). The distal
arthrogryposis: delineation of new entities: review and
nosologic discussion. Am J Med Gen, 11: 185-239.
Hall JG (1988). The value of the study of natural history in
genetic disorders and congential anomaly syndromes. J.
Med. Genet. 1988;25:434-444.
Hall JG (1998). Overview of arthrogryposis. In: Staheli LT,
Hall JG, Jaffe KM, Pahoike DE, eds. Arthrogryposis: A Text
Atlas. Cambridge: Cambridge University Press: 1-25.
25
 J. Hartley et al. / APCP Journal Volume 4 Number 1 (2013) 19-26 APCP Journal Volume 4 Number 1 (2013) 27-36

Jaffe KM (1998). Rehabilitation: Scope and Principles. In: Does a Postural Management Training Programme Improve Understanding of the
Staheli L, Hall, JG, Jaffe KM, Pahoike DE, eds.
Arthrogryposis: A Text Atlas. Cambridge: Cambridge
Importance of Postural Management for Children with Complex Movement Disorders?
University Press. p82
Holly L Bacon BSc (Hons) MCSP *
Moons P, Pinxton S, Dedroog D, Van Deyk K, Gewillig M, Jenny Lind Childrens Hospital, Norfolk and Norwich University Hospital, UK
Hilderson D, Budts W (2009). Journal of Adolescent Health 1-
7 *Corresponding author: email: holly.bacon@nnuh.nhs.uk

Resende MC and Neri,A L (2009). Psychological

adjustment and personal aging perspective in adults and
older adults with physical disability. Psicol. estud. [online].
2009, vol.14, n.4, pp. 767-776. ISSN 1413-7372
http://dx.doi.org/10.1590/S1413-73722009000400017.
Van Schrojenstein Lantman-de-Valk (2005). Journal of
research into Intellectual Disabilities. Vol 8: issue 4. 325-
333.
Sneddon J (1999). AMC and aging survey.
Avenues Newsletter: Volume xx: No 1, May.
Staheli L, Hall, JG, Jaffe KM, Pahoike DE, eds. (1998).
Arthrogryposis: A Text Atlas. Cambridge: Cambridge
University Press.
Wynne-Davies R, Williams PF, OConnor JCB (1981). The
1960s epidemic of arthrogryposis multiplex congenita. A
survey from the United Kingdom, Australia and the
United States of America. J. Bone Joint Surg 63B: 76-82.
________________________________________________________________________________
ABSTRACT
Aims
To evaluate a locally developed and tailored postural management training programme.
Method
A service evaluation questionnaire.
Results
77 of the 101 participants completed the questionnaire. Participants were predominantly parents (31%),
nursing staff (23%), and allied health professionals (22%). All respondents indicated that the programme was
useful, 97% found it easy to understand, and 91% indicated that they would like updates. 84% reported that
following training they had changed how they looked after the children in their care.
Conclusion
A well-designed tailored postural management training programme can be an effective method of improving
awareness and understanding of postural management. Further research is needed to establish whether this
type of training improves outcomes for children with complex movement disorders.
________________________________________________________________________________

Introduction approach. Therapy support and education may also

Over the last 10 years, postural management
programmes have been recommended for children
with complex movement disorders. A number of
studies have assessed conservative approaches to
managing postural deformity in children with
neurological conditions (Pountney, 2009). Many of
these studies discuss postural management and
what, if any, impact it has on the development of
structural deformity when used for individuals with
movement disorders (Gough, 2009; Lloyd et al, 2011;
Hutton and Coxen, 2011; Maher et al, 2011).
Gough (2009) suggests that there may be a very
specific group of children with a movement disorder
who will benefit from adopting a postural
management programme. He noted that the
increase in demands on caregivers and problems
associated with using postural management
equipment suggest that a full programme may not
be beneficial to all, and might help to explain
difficulties with compliance. He concludes that
abandoning the use of postural management
completely may be as detrimental as recommending
its use for all. This suggests that flexibility is
required and that taking more account of the needs
of the individual family and child could be the best
help to improve understanding and compliance with
postural management programmes.
Postural management is defined by Farley et al
(2003) as, the use of any technique to minimise
postural abnormality and enhance function. It has
been suggested that for children with neurological
dysfunction positioning techniques can be used to
enhance functional ability and should therefore be
implemented as early as possible in order to promote
development (Wandel, 2000). There remains a
limited awareness amongst health professionals
concerning postural management and this could
lead to some childrens health and life expectancy
being compromised (Waugh and Hill, 2009).
Goldsmith (2000) suggests that postural care relies
on the availability of appropriate equipment and
training to ensure that all carers are skilled in its use.
She argues that parents/guardians should be
acknowledged as experts on their own child and
need to be empowered by training sessions to have a
greater understanding of what postural
management is, and their role in its provision.
Humphreys and Pountney (2006) state that service
provision for postural management equipment has

26 27