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It’s a busy life for an active member of the community with special needs, as John Feighery says in his first article for Frontline Magazine.

My name is John Feighery. I am 24 years old, I have special needs. I was born with Down’s Syndrome but that has not stopped me from growing into an active member of my community.

I am a volunteer in Porterstown Pastoral Centre. Every Sunday from 11:00am-12.30pm, the community meet for a cup of tea and a chat after Mass. My role is to give out the tea and biscuits, I also collect rubbish and wash the dishes. I am very friendly and I talk to everybody, especially the children.

I am also a Minister of the Eucharist for the church. I take part in the local pantomime every year. I sing and dance and have a part to play. I am the same as everyone else in the pantomime and we make the audiences very happy.

There is a lot of sadness in the world so when my friends and I in the local Special Olympics ALPs decided to have a Book and Art sale, we decided to give the money we made to a good cause. We picked the Laura Lynn Hospice for sick children. We made €1,700- The director of Laura Lynn was delighted with that donation from our group and we were happy to help sick children.

I often give presentations to Transition Year students about my life so that they may understand that just because I have special needs, it does not make me any different from them.

My Dad says that it is good to help out in the community, because it encourages other people to help out also. My mentor and I asked my parents what my good points were, and they said that I was a very good listener, that I would talk and listen to all the families who come to church for Mass, Christenings and Marriages.

When I talk to people and they have worries or sick family, I pray for them every night. People like to talk to me because I am a good listener and I make friends easily with everyone.

I work for two hours every week in a Starbucks Cafe. I love when people I know come in for a cup of coffee. I also have a work placement in the Elbow Room in North Brunswick Street. I am able to get there by bus which makes me very independent. I would enjoy having another job near home.

I think that people are comfortable talking to me. When I go to the local shop and shopping centre I make lots of friends. I love the Golden Discs shop and I know all the people who work there. When I go to the town centre people from the Cinema, Eason’s Book shop and clothes shops all know me by name and talk to me.

I wanted to write this article to let people know that I, as a special needs person, have a lot to offer in my community.

Author Bio

5 - Citizen John - John Feighery photoWhen he’s not busy working and helping in the community, John Feighery lives in Clonsilla, Dublin, 15.

Eithne Clarke (1923 – 2011)

Intellectual disability was not an easily understood, or comfortably discussed, subject in 1955, when Eithne Clarke’s third child, Mary, was born with Down Syndrome. Eithne searched for advice, even reading Angel unaware, a book published in 1950 by Dale Evans Rogers (wife of the cowboy singer-star Roy Rogers). While Mary was still a baby, Eithne began meeting with Patsy Farrell and others at the Irish Country Women’s Association Country Shop in Stephen’s Green, and at the Mansion House—where the idea for St Michael’s House was born.

Eithne maintained her work with St Michael’s House throughout her life. She and her husband Judge Gerard Clarke served on the board of the organisation over several years. She was also on the board of Cheeverstown House, in its transition from convalescent home to residential service for people with intellectual disability. Eithne had a special concern about suitable residential care for persons with intellectual disability as their parents aged (see Frontline, Issue 19, 1993). In 1990, when St Michael’s House was still primarily providing day-services, she led a small group of parents in purchasing ‘The Bungalow’ in Goatstown, Dublin (see Frontline Issue 7, 1990, and Issue 28, 1996.) Developed in cooperation with the Department of the Environment, the local health authority and St Michael’s House, the Bungalow provided a home in their community for Mary and five other service users.

In 1996, Eithne Clarke was one of eight Irish women who received the Rose Fitzgerald Kennedy Award from Jean Kennedy Smith, then US Ambassador to Ireland. Mary Clarke lived at The Bungalow until 2007, when she moved to Cara, the St Michael’s House centre for Alzheimer care. Mary died (aged 55) in April 2010—just over a year before her mother’s death. At Mary’s funeral, Eithne honoured her daughter by reading ‘Welcome to Holland’ (by Emily Perl Kingsley, see http://www.our.kids.org/archives/Holland.html), the much.quoted allegory to describe the experience of having a child with a disability.

Declan Costello (1923 – 2011)

As a young TD in 1955, Declan Costello chaired the public meeting of parents in the Mansion House in Dublin, which launched St Michael’s House. He served as the organisation’s President from its early days, until his death last June. He also helped to found Ireland’s national organisation for intellectual disability, NAMHI (now Inclusion Ireland), becoming its first president in 1961. Declan championed the rights of children with an intellectual disability to be educated as equal members of society, and he successfully lobbied then Minister for Education Dr Patrick Hillery to formally recognise and fund Ireland’s first school for children with a moderate intellectual disability (St Michael’s House school on Grosvenor Road).

Universally known across Ireland, Declan served as Attorney General and President of the High Court, and he was the author of Fine Gael’s ‘Just Society’ policy document in the 1960s. In later life, Declan said: ‘Of all the things that I in my life, being associated with St Michael’s House was the one I regard as the most important of all. The story of St Michael’s House is an example of how opinion and people working together changed public attitudes.’

by Miriam Masterson, Dublin

Let’s start with just before Julia was born—the reason being that a funny thing happened to me about 4 weeks beforehand.

Julia’s father, Timmy, and I were in town doing some last-minute Christmas shopping. We were in Arnotts and, being 8 months pregnant, I was leaning against a railing by the escalator while Timmy browsed through some clothes. I was daydreaming and two women walking by caught my eye. They were probably a mother with her daughter, in her 20s. They caught my eye because the younger woman had Down Syndrome. Nothing unusual about that. But as got the bus home—and not our usual one, but the first that came along (which would mean quite a long waddle for me) who should get on the same bus, only my two ladies from Arnotts. With hand on heart I did wonder for a few minutes about this coincidence, and I have never forgotten it!

It’s January 22nd and I am in labour. We head for Holles St and go straight to the Labour Ward. I scream for the epidural and then sit back and wait. I had an epidural with our first child Sadie who is 4 years of age and fast asleep at home with Nana and Grandad who live next door to us. All is good and we are so happy. We are giddy and having a right laugh. The midwife is a lovely woman and time ticks away. Bang, the epidural is losing its effect and unbelievable pain is kicking in. I cannot take much more and start giving out. Nurse-in.charge comes in and tells me to get my act together and start pushing. That wonderful moment arrives and Julia is born. Nurse is holding my bundle and checking her out. Now she has gone over to the far side of the room and has put her on a table and is examining her again. I look at Timmy. His head is bowed slightly and his face looks funny. He’s very overcome and there is a funny colour to his face. He is quiet and SAD looking? ‘What is wrong, I know something is wrong, tell me, you can tell me’, I say in a very calm but loud voice to get attention immediately. ‘I’m fairly sure I am right, but I think your little baby has Down Syndrome, and I am going to ask the Doctor to take a look for me’, Nurse in charge tells me with huge compassion in her voice. ‘So that’s it,’ I think to myself. God has caught up with me for all my sins. I start telling anyone in the room that it’s fine and everything is alright. Timmy is very quiet, he knew the minute she was born, he knew by her eyes. This is my fault. I am 39 and I am to blame.

I ring around to tell my gang the news, my poor mother is very upset. My sister Liz, who was out the night before, tells me she will come in asap. She arrives with Gina, still a bit hungover, and they have me in stitches as they try to battle nausea and lack of balance. We give Julia a little bath and in time head to the ward. Liz and Gina watch Julia while I have my shower and Timmy goes home to see Sadie and ring his own family. None of those phone calls were easy.

The first four months

I have to keep this part short and sweet as quite simply it was horrific. We found out that Julia had a large hole in the centre of her heart. She could not feed because of this and was admitted to Temple St Hospital for tube feeding, which they would train me to manage. This involves putting a tube up Julia’s nose and down into her stomach. You do a test to make sure the tube has gone down the right way and then connect it to a feeding pump. Everytime I had to do this I wanted to vomit. The morning we were due to go home I was told that Julia had contracted the Rota Virus. A week later she was rushed to theatre to have the infected part of her bowel removed and she spent the next five weeks in ICU fighting for her life. We had her baptised as soon as she had come out of theatre. It was that bad. ICU could do no more so we were sent back to the High Dependency Unit. A few weeks later Dr Duff decided she was ready for a banding procedure which would reduce the pressure on her heart. It would be carried out in Our Lady’s Hospital Crumlin. A team from Temple St would take her by ambulance and we would be informed when they were coming as this in itself would be very involved. All goes well in that operation, but from the previous operation, her bowel had prolapsed and because that was done in Temple St, she had to go back there. So, we were back in the High Dependency Ward again. A couple of weeks later one of the surgeons remarked, ‘Is that child still here, let’s get her back to theatre, reverse the bowel and send her home. The wonderful nurses fought over who was going to give me the good news. I had spent every day in this hospital, with Timmy coming in every evening straight after a bite to eat and a few minutes with Sadie. One more trip to the theatre and three weeks later we went home. When I say I cried during this period, it just does not do justice for the pain and grief I felt in my chest every day. I had to stop the car more times, not able to drive with tears blocking my sight! After begging for the truth, I was told that, yes, she could very well die and she was a very sick little girl. I fought with God so many times and tried to take up smoking again—that just made me feel sick.

However, in the end Julia showed us all—she did come home and, very slowly, began to thrive. Our wonderful public health nurse Susie eased our panic at home. The months ticked by and we were on the waiting list for major open heart surgery as soon as Dr Duff reckoned Julia could withstand the ‘big one’. We had glitches along the way, pneumonia and other small bouts, but they were manageable at home. The following January we got the call, but the operation was cancelled at the last minute. A week later we were back and this time we were good to go. The operation was a great success and we were home a week later. Throughout all of this, the fact that Julia had Down Syndrome became less and less important, though not irrelevant.

Now

After two years attending a mainstream playgroup, Julia has just moved from the baby room up to the Montessori room. We are very lucky with this place, and I have total trust and faith in the staff. We are working towards mainstream school, but we live in the present as much as possible. Julia was recently diagnosed with a leaking heart valve, so she is on medication, with regular check-ups. This has knocked me back, as we could be facing more surgery—you just have to put it to the back of your mind. I would be lost without Timmy—I do not know where he gets his positive outlook from—my other beautiful daughter Sadie, my deeply treasured parents, Betty and Ernie, my wonderful family popping in and out daily, the mums from Cheeverstown who I meet regularly—and all the great friends I have made through the Dublin Branch of Down Syndrome Ireland and the NIID at Trinity College..

Julia puts manners on me every day. She is full of love for everyone and has the biggest smile going. What more could I ask!

The greatest gift we can give a child is help in learning to communicate and connect with the world by L. Marsh, E. Drummond and V. Mc Manus, Catherine McAuley School of Nursing and Midwifery, University College Cork. Email correspondence to l.marsh@ucc.ie

Introduction

There can be little doubt that the use of effective communication has major implications on how individuals can exercise choice and control over their lives, gain independence and social inclusion leading to real improvements in quality of life (Thurman et al. 2003). The core meaning of the word communicate is ‘to place in common’, and language is said to communicate when the individual speaking and the individual listening come to understand and interpret correctly what has been said (Cole and Cole 1996). Kaiser et al.(2001, p.144) define communication as ‘the exchange of shared meanings using conventional, mutually recognized verbal and nonverbal forms’ and Kumin (1998) further suggests that communication involves not only speech but also facial expressions, smiles, gestures, pointing, high-fives and alternative aided or unaided communication systems.

By the age of three and a half most typically developing children have mastered the skills of communication, as they have participated in everyday family life and community activities (Cole and Cole 1996). They also assert that if children are to master language, not only do they have to experience and participate in family and community living, but they must also hear or see language as they grow (Cole and Cole 1996). Equally the development of communication in children with Down Syndrome is comparable to typically developing children with spoken language, tending to emerge between two and a half and four years of age (Buckley 1993; Watson 1996). The production of speech and language, however, presents many challenges for children with Down Syndrome and many may not experience the levels of fluency, complexity and skill in keeping with their peers (Kaiser et al. 2001). There is wide acknowledgement in the literature that children with Down Syndrome experience significant delays and difficulties with learning to talk and are at a higher risk of communication difficulties, thus leading to problems in the production of speech and language even into later life (Cockerill 2002; Buckley 1993; Lorenz 2006).

Some reasons cited as attributing to these communication difficulties are that as many as 75% of young children with Down Syndrome have mild hearing problems attributed to the higher than average incidence of ear infections (specifically otitis media) (Miller and Rosin 1998). This commonly recurring chronic health condition is identified as leading to fluctuations in auditory processes resulting in short telegraphic speech often difficult for others to understand (Buckley 1993; Bosch 2003). Additionally children with Down Syndrome have small oral cavities and large coarse protruding tongues, so much of their speech production is Manolson 1992 often found to be unintelligible and indistinct (Buckley and Bird 2001). This situation further compounded by poor listening skills (auditory memory) and visual problems (Cockerill 2002; Buckley 1993). These combined difficulties create significant barriers to effective communication and language development and leave much of the interpretative work to parents who can best interpret their children’s needs (Wishart 2001). Nonetheless, some children will require Augmentative and Alternative Communication (AAC) to be implemented to ensure they learn to use speech and language effectively for functional communication (Duker et al. 2002; Cockerill 2002).

Total or inclusive communication

The introduction of total or inclusive communication approaches are increasingly recognised as best practice (Bradshaw 2000; Jones 2000) and can be described as an ‘approach that seeks to create a supportive and effective communication environment, using every available means of communication to understand and to be understood’ (Thurman et al. 2003). It is an approach by which a variety of different methods can be used to convey meaning including speech to teach language, touch, body clues, natural gestures, real objects (objects of reference) and formal signs to support communication (Jones 2000). Linder (1993) advocates the use of play as it is the most natural activity for the child and encourages communication and language development as well as cognition and social-emotional development. When analysing the child’s communication, videotaping the play session is considered an integral aspect of the assessment as it is a useful mechanism for parents and other professionals to discuss if the recording was wholly representative of the child’s current levels of communication (Linder 1993).

This natural play environment further provides an important opportunity for the child to learn about the world in a fun and relaxed setting (Pepper and Weitzman 2004). Thus AAC is not ‘a one size fits all’, and is largely dependent on the individual child and where s/he is at in relation to their own development. This further supports a child-centred approach to communication and essentially any intervention will be determined by the child themselves. The approach shifts and adapts as the child’s communication skills improve. Other factors such as the child’s age, level of ability, current communication skills and the environment have to be considered prior to the introduction of AAC. Indeed, children with Down Syndrome who utilise AAC are more likely to interact with their environment when they can understand and be understood, and they become more motivated to learn (Wishart 2001).

In essence, by adopting an integrative approach to AAC, children learn to communicate effectively by every means possible as they are active participants in their environment. This leads to an enhanced knowledge and understanding for the child of the world around them. Such understanding supports the inclusion and integration of children with Down Syndrome in their community, enabling them to have their needs met (Kumin 1998; Buckley 1993). Indeed, Cockerill (2002) identifies signing as a positive influence on the development of children’s speech and language development. Moreover Chapman (1997, pg.311) suggests that most of these children drop the signs ‘as intelligibility, number of communicative partners, and vocabulary size increase’. Hence, the practical application of communication interventions has shown that provided the right supports are afforded to children with Down Syndrome, they can influence their immediate environment, thus leading to real improvements in life opportunities and quality of life (Foreman and Crews 1998).

Augmentative and alternative communication (aac)

The last thirty years have witnessed an increase in the use of augmentative and alternative communication techniques (AAC) which have been acknowledged as having a major role in the development of ‘effective social behaviour’. AAC includes the use of unaided systems such as gestures and signing and aided systems ranging from picture boards to computer technology (Romski and Sevcik 2005), leading to more effective speech, language and cognitive skills (Cockerill 2002; Buckley 1993; Foreman and Crews 1998). There have been many sign systems developed with particular interest in people with intellectual disabilities in the last two decades both, in the United Kingdom and in Ireland. One such system based on British Sign language is Margaret Walker’s Makaton Vocabulary developed in the early 1970s (Grove and Walker 1990).
Makaton vocabulary is one of the most extensively used systems of augmentative communication in use in the United Kingdom. It ‘provides an approach to the teaching of communication and language skills, using manual signs and/or graphic symbols, accompanied by speech’ (Grove and Walker 1990). The system ‘relies heavily on natural gestures, body language and facial expression… and appears to enable children and adults to master the skills quickly’ (Foreman and Crews 1988).

From an Irish perspective LAMH (Language Augmentation for the Mentally Handicapped) (Clarke et al. 1991) (‘lamh’ is the Irish word for hand; pronounced lawv) was adopted nationally in intellectual disability services in 1982, and further revised in 2002 (Le Prevost 1995). LAMH is a sign system of approximately 600 signs that can be combined to form sentences, with many signs looking like the objects they represent. It emphasises gestures which are incorporated in the development of a vocabulary and appropriate facial expressions with signs and speech must be adopted in order to convey meaning to what is being said. It is based on the premise of ‘sign and say’ and is not used to replace language, but to support and augment communication (Clibbins et al 2002).

In the context of children with Down Syndrome specifically, sign language has been identified as a good communication tool (Duker et al 2002; Donovan 1998) as these children demonstrate strengths in visual processing and are excellent at using facial expressions, gestures, and vocalizations (Duker et al. 2002; Foreman and Crews 1998). Even though speech and language production often develops more slowly than receptive language, improvements in communication can and do occur (O’Toole and Chiat 2006). Notably, children of all ages find it more difficult to use miniatures or substitute objects as symbols and demonstrate greater understanding of the ‘sign and say’ approach reflective of LAMH and Makaton (Grove and Walker 1990; Clarke et al. 1991. In conclusion, sign language involving natural gestures and signs are easier for children with Down Syndrome to use than communication systems based solely on objects (O’Toole and Chiat 2006). The following case study demonstrates how AAC was used to support Sean’s communication.

Profile of Seán

Seán is a three and a half year old child with Down Syndrome who presents with a moderate intellectual disability (35-49) as defined by the ICD-10 (WHO 1996). He lives at home with his parents and two older sisters and is a happy and alert young child. His communication is mostly in the form of using gestures or ‘body language’; he has no clear spontaneous speech. When prompted by his parents he will engage in some sign language such as waving bye-bye or blowing kisses. His expressive skills are less explicit than his receptive skills, in that he understands much of what is being said and has the ability to get his needs and wants met. This he achieves through pointing to objects or by pulling his parent to items/activities. He understands simple instructions said in context such as ‘Seán, go and get your coat’, but cannot follow more complex requests such as ‘Seán, go and get your coat and put it on.’ Seán manages to communicate well even though he has very little spoken language and relies heavily on signs, gestures, cues and body language—all of which is characteristic of children with Down Syndrome (Cockerill 2002; Lorenz 2006).

Prior to the introduction of AAC, it is suggested that those supporting any communication intervention should adhere to the following five key principles:

  1. everyone has the capacity to communicate and has something to say,
  2. opportunities are key to communication,
  3. you have to be a good listener,
  4. always assume that individuals can understand everything you say, and
  5. your communication styles need to be adjusted as necessary to suit the situation (Mirenda 1999).
Enhancing communication

Thus, prior to the planning and implementation of any communication intervention it is essential that a holistic assessment with the child and their family be undertaken. The assessment is the first step in planning any communication intervention, with the second step identifying what it is the child needs to acquire, and finally that the needs are acquired in the immediate and not the distant future (Cunningham and Sloper 1985). This will assist in identifying the child’s current communication skills, thereby establishing a baseline from which other skills can be taught (Frost and Bondy 2002).

Methods of assessment
Detailed information about Seán and his environment was collected using the LAMH assessment checklist (Clarke et al. 1991) and the Interactive Communication and Play Checklist (Down Syndrome Trust 2001) (Table 1.0), as well as direct observations and information gleaned from Seán, his parents and staff. Informal observations of his physical, social and emotional health and well being during his regular routine and play sessions and formal assessments of his medical history and cognitive development were also reviewed.
The LAMH assessment checklist:

  • To establish a baseline of Seán’s current communication skills.
  • To identify if Seán requires AAC.
  • To identify if Seán can benefit from AAC.
  • To identify which kind of AAC Seán can use and learn.
  • To identify which system will meet his future needs.
The Interactive Communication and Play Checklist

This tool is completed by parents and it ‘provides a guide to the range of communicative functions that children use and to their ability to join in and initiate conversation’ (Buckley and Bird 2001).

TABLE 1.0—ASSESSMENT CHECKLIST

The information gleaned from the assessments showed that Seán vocalises to gain attention. He smiles at people who talk to him, but does not initiate contact with peers. Sean displays intentional communication strategies in that he points, gestures and uses ‘body language’ as well as displaying good imitation skills. He can request items/activities through pointing and will reject items/objects by throwing them on the floor. Seán will vocalise loudly when he is interested in an activity and he shows disapproval by moving away from an activity. He demonstrates a preference for his left hand. In partnership with Seán’s parents and the multidisciplinary team, it was determined that Seán would benefit from the immediate introduction of LAMH as a watchful waiting approach is no longer acceptable for any child who is identified as being at risk of communication difficulties (Cockerill 2002).

The introduction of AAC in this case resulted in Seán’s parents being asked to consider twenty signs which they considered to be important to learn, and from this list they were asked to identify six signs that Seán would benefit from. When choosing signs it is vital that they are interesting, motivating and functional, thus giving the child control over his environment. Initially Seán’s parents had requested signs for both mother and father, but as these signs are very similar they are not taught together. Seán’s preference for animal storybooks will facilitate the use of the sign for ‘book’ and ‘dog’. Repetition and re-reading the same story will encourage the use of these signs and can be built into numerous sing-along activities in the home and school environments (Donovan 1998).

Discussion

A growing body of evidence suggests that interventions with parents and children in the early years can be very valuable in terms of stimulating children’s intellectual, physical and social development (Ghate and Hazel 2002; Johnson et al. 2000). As parents are the first teachers of speech and language they must be central to any communication interventions, as the most important intervention takes place in the home (Kumin 1998). However, parents’ fears regarding the introduction of LAMH are that language production may be hindered or discouraged or that they would have difficulty learning the signs themselves. Therefore training in the use of LAMH is essential for parents, siblings and staff alike, as all those supporting AAC are required to more fluent than the child in signing (Kaiser et al. 2001). Signs must be used consistently with natural speech and any attempt that the child makes to sign has to be rewarded immediately (Cockerill 2002; Le Prevost 1995).

It is essential that any communication intervention is evaluated and that everyone is afforded the opportunity to discuss the child’s progress and address any concerns or worries which may arise. AAC has not only been acknowledged as an effective communication intervention, it is also clear that using this approach will augment existing natural speech whilst reinforcing the concepts of basic language development (Romski and Sevcik 2005; Grove and Walker 1990). Indeed, it is essential that all modalities of communication are promoted enabling individuals to communicate in everyday situations, whilst preparing them to communicate in unfamiliar situations, thus transferring knowledge and skills across a wide variety of settings (Foreman and Crews 1998).

Conclusion

The understanding from a comprehensive assessment highlighted both Seán’s strengths and weaknesses with communication and language development. If effective communication interventions are implemented early on in a child’s life, then better speech, language and cognitive abilities are evident thus enabling individuals to make choices, gain independence, exercise more control over life, ultimately enhancing quality of life for all (Buckley 1993; Thurman et al. 2005). The practical application of AAC for children with Down Syndrome has shown that if children can influence their immediate environment, life opportunities can be improved provided that the right supports are afforded these children, thus promoting optimal nursing supports for the child with Down Syndrome (Foreman and Crews 1998). This case study demonstrates the importance for all health professionals who support children with Down Syndrome to be knowledgeable of communication interventions that are available to these children.

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by Steven Gannon (Postgraduate student) and Sinéad McGilloway (Senior Lecturer)

Introduction

The social relationships of a child with Down Syndrome or other Special Educational Needs (SEN) often constitute the single area of school life about which parents and carers are most concerned (Cuckle and Wilson 2002). Therefore, it is perhaps not surprising that exposure to peers and opportunities for friendships in the local community are frequently the main reasons why parents send their children with SEN to the local primary school (Nakken and Pijl 2002). However, these pupils belong to a vulnerable population of young people with learning disabilities who are more likely than their non-disabled peers to experience peer rejection, depression, anxiety, behavioural and conduct problems, delinquency, school dropout and poor academic adjustment (e.g. Sinson and Wetherick 1981; Weiner 1987; Werner, 1993; Guevremont and Dumas 1994; Laws, Taylor, Bennie and Buckley 1996; Wenz-Gross and Siperstein 1996). Furthermore, the extent to which these young people experience fully inclusive education has been the subject of considerable debate in recent years.

Despite a recent increase in research investigating the social relationships of pupils with Down Syndrome (eg. Murray and Greenberg 2006), studies of the attitudes of non-disabled children towards their peers with Down Syndrome are in their infancy and very little research of this kind has been conducted in Ireland (Gash et al 2000). The primary aim of the present study was to assess the attitudes of non-disabled children in several rural mainstream primary schools toward children with Down Syndrome. A secondary aim of the study was to examine the impact on attitudes (if any) of administering a video designed to promote inclusive attitudes toward children with Down Syndrome.

Research methodology

A convenience sample of 118 children (comprising 54 males and 64 females) was drawn from six rural mainstream primary schools in North Kildare. Participants were recruited from twelve Third and Fourth Classes and were aged between 8 and 11 years (mn=9.35; sd=0-72). Classes which included children with learning disabilities (LD) were excluded from the study due to ethical reasons. The research was conducted during the Social Personal Health Education (SPHE) class in each school. Specific objectives of this module for Third and Fourth Classes involve acknowledging the importance of friendship and the practice and recognition of the value of care and consideration, courtesy and good manners when interacting with others (Government of Ireland 1999). The sample size and age-range of the children were considered appropriate in view of other similar studies in this area conducted elsewhere (Laws, Taylor, Bennie and Buckley 1996; Gash et al 2000; Gash, Romeu and Pina 2004; Roberts and Smith 1999).

Prior to commencement of the study, a letter was sent to the parents/guardians of all potential participants informing them about the study and seeking their consent for their child to be involved on an ‘opt-out’ basis. Participants were first asked to complete a brief Background Questionnaire (BQ) which was devised to elicit details on age, sex, number of children in family and position of the child in family. Information regarding the participants’ experience of a friend or relative with Down Syndrome was also obtained. It was thought that some or all of these kinds of variables might be linked to overall attitudes.

Following this, participants completed an Attitude Questionnaire (AQ) at two points in time and each on a group basis. The AQ was based on a slightly modified version of a questionnaire devised by Gash (1993) to examine attitudes towards children with special needs. For purposes of the current study, the term ‘mental handicap’ was replaced by ‘Down Syndrome’. This 20-item measure comprises 10 items relating to the sociability of non-disabled children toward their counterparts with Down Syndrome (Scale One) and 10 items that assess the views of non-disabled children on the inclusion in schools of children with Down Syndrome (Scale Two). The children answered ‘yes’ or ‘no’ to each statement. A ‘yes’ response was scored as ‘1’; a ‘no’ response was scored as ‘2’, so that higher scores reflect more negative attitudes.

A brief excerpt from a video entitled Including children with Down Syndrome in your school (Down Syndrome Ireland 2004) was shown upon completion of the first AQ. This 10-minute excerpt depicted children with Down Syndrome in an inclusive classroom participating in everyday school activities such as reading, writing, spelling and singing. This video was sent to every school in the Republic of Ireland in 2004 by Down Syndrome Ireland in cooperation with the Department of Education and Science in order to promote the inclusion of children with Down Syndrome in Irish primary schools. The literature accompanying the video provides general information on inclusion strategies and benefits, the learning profile of a child with Down Syndrome (e.g. reading, writing, phonics and numbers) and on the motor skills and behaviour of these children. Once the videotape had concluded, the class were invited to complete the AQ for a second time (also on a group basis). Upon completion, the participants were fully de.briefed and thanked for their co-operation.

Key findings

The mean AQ scores obtained on each scale at both Time 1 and Time 2 are shown in Table 1, alongside the background data. As shown in the table, the pattern of scores was broadly similar across both time points in relation to all key variables, although scores on Scale Two (attitudes toward inclusion) were consistently higher than those for Scale One at both time points. It can be seen that those who were middle children had the lowest mean score on Scale One (at both time points), while those who were only children obtained the highest mean scores overall. The youngest children in the group (≤ 9 years) also attained marginally higher (i.e. more negative) scores than those aged 10 years and over- Closer inspection revealed the following key findings:

  • Sociability versus inclusion: Scores on Scale Two were consistently and statistically higher than those on Scale One, indicating more positive attitudes toward sociability than toward inclusion in general.
  • Gender differences: Males and females did not differ significantly in terms of their Scale One and Scale Two scores at baseline (Time One).
  • Most sociable group: Females in the over 10 years group were found, statistically, to be the most sociable toward their peers with Down Syndrome. No statistically significant differences were found for the effect of age on attitudes toward inclusion for either males or females.
  • Role of familiarity: Those who had a relative or friend with Down Syndrome did not differ significantly from those without such contact in sociability or in attitudes toward inclusion in this study.
  • Position in family (i.e. oldest, youngest, middle or only-child): This was not shown to play a role in children’s levels of sociability and attitudes toward inclusion as measured by the AQ.
  • Analysis at Time 2: This revealed that there was no statistically significant change in Scale One or Scale Two scores between the two time points.

Table 1: Mean AQ scores by background variables

Background Variable Time 1 Time 2
Scale 1 Scale 2 Scale 1 Scale 2
Gender
male 12.46 16.07 12.39 16.20
female 12.16 15.55 12.16 15.75
Age-group
9 years and under 13.17 16.50 14.83 16.83
10 years 12.28 16.19 12.33 16.58
Over 10 years 12.80 15.80 12.60 16.20
Position in Family
oldest 12.55 15.95 12.45 16.10
youngest 12.19 15.58 12.02 15.65
middle child 11.93 15.72 11.83 16.03
only child 13.17 16.50 14.83 16.83
Number of children in family
1 13.29 14.86 13.86 15.57
2-3 12.19 15.84 12.19 16.01
4-5 12.32 16.02 12.26 16.30
Friend/Relative with Down Syndrome?
Yes 12.24 15.76 11.95 15.47
No 12.33 15.80 12.41 16.19
Total
(group as a whole) 12.30 15.79 12.26 15.96

Commentary

The finding of greater sociability amongst the older females in the group is consistent with other research showing females to be generally more sociable than their male counterparts (Gash 1993; Gash et al 2000). It is possible that this also increases with age, although it has been suggested by Nowicki (2005) that girls and boys may differ in how they respond to attitude measures that relate to specific types of responses and that these differences may be more indicative of gender-based response biases rather than gender differences in their opinions. This may also explain, at least in part, the lack of differences between the younger males and females in the current study. However, the research findings in this respect are mixed and it is not clear at this stage if age and attitudes are related to any great extent (Gash and Coffey 1995; Diamond, Hestenes, Carpenter and Innes 1997; Tamm and Prellwitz 2001). Nowicki (2005) also asserts that within gender, age differences in attitudes may be greater than any attitudinal differences across gender.

One finding of particular interest in the current study was the absence of any differences between those children who had a friend or relative with Down Syndrome and those who did not. It was thought that those with such contact might hold more favourable attitudes, particularly in relation to sociability, but this was not the case. This finding contrasts with some other work including a study by Helmstetter et al (1994) who found that non-disabled children who had previous contact with a child with SEN had a more positive attitude towards those with SEN and viewed their inclusion as more favourable than those without such experience. By contrast, however, Scheepstra et al. (1999) reported that almost half of 23 students with Down Syndrome in an integrated mainstream school were rejected by their peers. Furthermore, contact alone between children with Down Syndrome (and other learning disabilities) and their non-disabled classmates may not automatically lead to enhanced social interaction, or to the development of positive attitudes (e.g. Lyons 1991).

A substantial body of evidence has suggested that children and early adolescents can provide reliable information regarding their personal relationships and their social and emotional functioning (Elliot, Huizinga and Ageton 1985; Jolliffe, Farrington, Hawkins, Catalano, Hill and Kosterman 2003; Lynch and Cicchetti 1997). Consequently, the above finding (and perhaps some additional findings also) may be due, in part, to the possibility that some of the items on the AQ may not accurately reflect the ways in which children relate to each other in social or school settings. Gash et al (2000) and Gash (1996) argue that the AQ reflects attitudes in observable ways and has good reliability, and validity, but further work is needed to establish the full range of psychometric properties of the AQ. Nonetheless, the AQ was chosen because it was brief and easy to complete (eg. containing ‘yes’ and ‘no’ answers only) and contained age-appropriate language. No other similar such measure could be identified from the literature.

It was also thought that the number of other children in the family or a child’s place in the family might have a bearing on overall levels of sociability, in particular, and perhaps also attitudes toward other children with Down Syndrome. For example, children in larger families may be generally more tolerant of differences. Again, however, no differences in sociability or inclusion were found with respect to this variable.

The current study was cross-sectional in nature and necessarily constrained by time and resources. The data were gathered from a convenience sample of children attending schools in rural settings. Whilst the sample was of a reasonable size, it is possible that the attitudes of children in larger schools based in cities or towns may differ from those of children in typically smaller rural settings. Again, future research should expand data collection beyond one geographical location (in this case rural areas) and across different settings, thereby enhancing the generalisability of the findings. For example, Nowicki (2005) argues that studies of attitudes similar to the present study might yield different results if conducted in other contexts, such as playgrounds or sports venues.

A larger, longitudinal study would also be more beneficial in assessing any changes in attitude over time (perhaps also using the AQ) across a number of different settings. Only these kinds of studies can add to the current uncertainties surrounding age and attitudes in the research literature. More specifically, as stated by Nowicki (2005), longitudinal designs provide an opportunity to examine the consistency of results across measures and potential interactions between factors that may not be uncovered when potential attitude-related variables are investigated in isolation.

Another limitation of the current study and one that was beyond the researcher’s control (owing to time constraints and restricted access to schools) was the short time period between the video and the second AQ. Ideally, the second AQ should have been administered at a greater length of time after presentation of the video (e.g. two weeks later), but this was not possible in the current study. This may also have led to some degree of response bias in that some children (particularly the older group) may have become aware of the purpose of the video (because it was administered so soon after the first AQ) and perhaps adjusted, or declined to adjust, their responses accordingly. However, there were a number of measures put in place to try to control for this (insofar as possible). For example, the children were instructed to provide their own responses without consulting others or viewing the questionnaires of other participants. Both the class teacher and researcher were also present at all times.

It is also noteworthy that difficulties arise when attempting to compare results across studies because of the variety of measures and designs available. For example, according to Gash (1996), there is considerable uncertainty surrounding whether an improvement in attitudes is due to a change in the feelings about a child with learning disability (LD) or if a child’s view of LD changes depending on the nature of the disability (eg. mild or profound). The image of a profound LD may evoke fear in children with little experience of others with LD. Therefore, if a child’s idea or image of LD changes from profound to mild, the attitude may be expected to improve.

Additional research is required to disentangle the potentially complex interplay of factors that impact on overall attitudes. There is considerable scope, in particular, for conducting qualitative research. For example, a study by Francis and Muthukrishna (2004) used unstructured interviews as a means of investigating and understanding the experiences of primary school children with disabilities in South Africa. This paper raised some interesting issues in relation to growing up with a disability including cultural attitudes toward disabilities and social relationships. Similar research conducted in Ireland could prove valuable.

Gilmore et al (2003) state that accurate knowledge and positive, but realistic, expectations are important for enhancing the acceptance of children with learning disabilities (including Down Syndrome) within their schools and in their communities. Reassuringly, Gash et al (2000) found that Irish children (and their Spanish counterparts) were found to be more favourably disposed toward inclusion than children from either France or Portugal. However, the findings of the current study and other work in this area illustrate, above all, that the attitudes of non-disabled children toward their peers with Down Syndrome and other learning disabilities are complex. Previous inconsistencies and methodological variations in research carried out in this area are common and a need for further research is highlighted.

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Dr Janette Tyrrell, MD, Consultant Psychiatry of Learning Disability, St Michael’s House, Goatstown, Dublin

People with Down Syndrome are now living longer and healthier lives. This in part is attributable to better health care and nutrition in infancy and childhood. The average life expectancy has increased dramatically over the last 60 years with many people living into their fifties and sixties. As in the general population, increased life expectancy brings with it, the risk of developing diseases of old age. One such disease is Alzheimer’s disease, an illness characterised by memory loss and behavioural change. Alzheimer’s disease is a complex disorder that affects the brain initially with short term memory loss but also affecting skills such as speech, walking, knowledge, judgement and ultimately leading the individual to complete dependence on their carers for all activities of daily living.

The exact cause of AD is unknown, but scientists have discovered an excess of two proteins Tau and Amyloid that accumulate within brain tissue and surrounding tissue leading to death of brain cells (neurones), particularly in areas responsible for speech and memory.

For most people in the general population Alzheimer’s disease is age-related, with the incidence increasing with advanced age. In some rare cases there are genetic abnormalities leading to family risk. In people with Down Syndrome there are three copies of the gene coding for the amyloid protein and simply more amyloid deposition at a younger age within the brain, with the result that about 15 – 40% of people with Down Syndrome develop AD at a younger age (usually mid-fifties). Research is currently investigating what factors protect people with Down Syndrome from Alzheimer’s disease.

Clinical features

Usually, as in the general population, the first clinical feature of dementia is memory loss, however, this may be hard to detect. Memory loss is due to impairment in the area of the Hippocampus, a small midline structure within the brain. The critical defect is one of loss of a neurotransmitter called acetylcholine leading to faulty connections between neurones and initially loss of newly acquired memories. Short-term memory loss may present as failure to remember names in the workshop or neighbourhood, forgetfulness of keys or personal objects, and forgetfulness about time of day. One common feature I have observed is a person forgetting that they have eaten a meal and asking for dinner at 2 am in the morning or other inappropriate times.

Other features are impairment of speech and gait, particularly difficulty with falling or high stepping when moving from one room to another. This is because difficulties with perception may make distance and height hard to judge. Some people may lose their initiative and need prompting to do a task, but once started they may complete that task successfully. Complex tasks that require a person to sequence the task in 2-3 steps (sorting and collating tasks in the workshop) may become difficult and frustrating.

As the illness progresses, people may develop incontinence, and may need assistance in walking and feeding. These features are not early features and the illness should have been recognised before then.

In a small proportion of people there may be psychiatric features, some people may appear depressed or some people may misinterpret their environment due to altered perception, or experience hallucinations. Such symptoms are due to an abnormal balance of neurochemicals such as noradrenaline, serotonin and dopamine within the brain. It is also important not to misdiagnose dementia—both depression and psychosis may mimic dementia; sometimes adequate treatment of psychiatric illness will differentiate the correct illness. A severe depressive illness will usually be accompanied by weight loss, anorexia, early morning waking and low mood that is characteristically worse in the morning. Whilst sleep disturbance occurs in dementia, it is usually characterised by difficulty falling and staying asleep.

Epilepsy, either in the form of a generalised seizure or in a localised form (myoclonus), is very common but is not a feature associated with early dementia. It responds well to treatment and is rarely a problem once stabilised.

One mystery in people with Down Syndrome is that several other conditions may mimic dementia. I have mentioned psychiatric illness, but any physical illness—untreated hypothyroidism, a deficiency of vitamin B12 or folate, high levels of calcium in the blood, sensory loss (particularly visual or hearing loss), bereavement or abuse—may lead to regression in skills as discussed above. Therefore a proper assessment for dementia requires a comprehensive evaluation to exclude untreated medical or social distress, remembering that a person can have more than one illness at a time and if memory loss and skill loss do not improve with, say, grief therapy, and antidepressants have failed to treat a post-bereavement depression, this may indicate that dementia should be considered.

A comprehensive assessment will consist of a multidisciplinary approach with an assessment of memory and thought, physical health and mental health and social wellbeing. Occasionally when a diagnosis is complicated extra tests such as brain scans (CAT scans or MRI scans) or EEG testing may be required. At the centre where I work, if we feel that the diagnosis is unclear because of other factors we review the person at 6-monthly intervals. Since dementia is a progressive illness, we repeat the assessment to see if decline (and therefore dementia) is present.

If dementia is present, then the management is multidisciplinary and aimed at providing a person-centred approach aiming to preserve independence for as long as possible. Specific management of mental health problems may involve the use of drugs that increase the amount of acetylcholine in the brain (eg donezipil, galantamine and rivastigmine) to improve memory. Memantine may be used in middle-stage dementia to stabilise memory. Where depression is a problem, selective serotonin reuptake inhibitors (SSRIs) may be helpful. Hallucinations and delusions may be helped by dopamine inhibitors, but these are used cautiously and at low dose because of possible side effects with older drugs in this class and because some of the newer drugs, whilst having fewer side effects, have been implicated in multi-infarct dementia.

Sleep disturbance is best treated environmentally but if it is still a problem it may be helped by trazadone which acts to increase serotonin. It is best to avoid sedatives in dementia as they may add to confusion and if hypnotics are used they should be used for a short period only and at the lowest dose.

To summarise, Alzheimer’s disease occurs with increased incidence in people with Down Syndrome, usually after age 50- Memory loss and impairments of thought and behaviour are key features and it is important to differentiate dementia from other illnesses. Whilst there is as yet no cure for AD, many symptoms may be helped by environmental, behavioural and medical intervention.

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by Jean Lane, M.B., FRCPI

The presence of symptoms of dementia in persons with Down Syndrome and with intellectual disabilities of other aetiologies poses challenges for physicians, nurses and other staff. The following observations reflect the growing literature on the health consequences of dementia, as well as the author’s own clinical experiences in working with people with Down Syndrome and dementia and participating in multidisciplinary efforts to address the needs of these individuals. Challenges to be addressed include assessment and early diagnosis, medical surveillance, identifying and responding to common health problems, and care planning considerations in the later stages of dementia

Assessment and early diagnosis

The association of Alzheimer dementia with Down Syndrome is now well recognised among healthcare professionals, carers and families of people with Down Syndrome. Dementia screening is being increasingly used to diagnose Alzheimer dementia in people over 35 years old with Down Syndrome. Where screening is available, not only are more cases of Alzheimer dementia diagnosed, but the diagnosis is made earlier in the course of the disease.

Early diagnosis is important as it gives time to investigate various forms of intervention. Medications are now available which may slow the progression of the disease; a trial of these may be considered appropriate. In addition, carers can share and receive information regarding the course of the illness. It is important that this knowledge is availed of when deciding on the management of medical complications that arise during the progression of the disease.

Medical surveillance

The importance of medical surveillance among people with Down Syndrome cannot be over emphasised. Many medical disorders that occur in the general population also occur in people with Down Syndrome, but with greater frequency. Most of these are treatable disorders, but if left undiagnosed they could potentially pose a further handicap to this group of people. Medical monitoring of people with Down Syndrome should be life long. When a diagnosis of Alzheimer dementia is made the necessity of medical surveillance is all the more important to ensure that any treatable medical or mental health conditions are recognised and promptly managed.

Identifying and responding to common health problems

There are a number of medical problems that may be encountered in the person with Down Syndrome and Alzheimer dementia. While it is beyond the scope of this article to discuss all of these conditions it may be useful to highlight some of the more common problems.

EPILEPSY

While only 10% of people with Alzheimer dementia in the general population develop epilepsy, the figure for people with Down Syndrome is reported to be as high as 80%. In people with Down Syndrome the seizures tend to appear after the onset of other features of cognitive deterioration. Many different types of seizures may be seen, the most common being myoclonic seizures.

A myoclonic seizure is a brief contraction of a muscle, muscle group or several muscle groups. It can be single or repetitive, varying in severity from tiny twitches to severe jerking. In people with Down Syndrome and Alzheimer dementia myoclonus can become quite troublesome, especially on awakening or as a startle response. As the dementia process progresses the myoclonic seizures may become quite resistant to treatment with medication.

When treating seizures in people with Down Syndrome and dementia an appropriate anti-epilepsy drug should be prescribed with the aim of optimum seizure control with the least side effects. A protocol for the use of emergency medication for prolonged seizures should be available to all.

PRESSURE ULCERS

Pressure ulcers or bedsores are not uncommon in people who are bed bound and particularly those with dementia. Pressure ulcers develop as a result of unrelieved pressure over certain parts of the body, usually bony prominences, such as the heels and the hip area. In people with Down Syndrome and Alzheimer dementia the combination of immobility, moist skin due to incontinence, possible nutritional deficits and the decreased perception of pain due to their cognitive deterioration can lead to the development of pressure sores.

Pressure ulcers can be difficult to prevent; however, if they occur there are a number of possible interventions that may help. These include the use of pressure relieving mattresses, specialist dressings, nutritional support and the institution of a programme for regular repositioning of the person may lessen the risk of pressure sores developing and also aid their treatment.

INFECTIONS

Infections such as pneumonia, urinary tract infections and septicaemia are not uncommon in late stages of dementia and are the most frequent causes of death in the terminal stages of the disease.

Several factors contribute to the increased incidence of infections and their severity in people with advanced dementia. For example, the immune response may diminish with age and underlying medical conditions. Also, the presence of urinary or faecal incontinence may predispose to infection. Aspirating the contents of the stomach into the lungs, which can occur when the person is weak and immobile, may cause pneumonia. Bladder catheterisation, which may be unavoidable, can be another source of infection.

The person with Down Syndrome and Alzheimer’s disease who has developed an infection may not present in the typical way. Even with pneumonia, the person may be unable to report their symptoms and may not present with a cough or temperature. The combination of all these factors emphasise the necessity for frequent medical and nursing assessment to detect signs and symptoms of an emerging infectious progress.

Many of the risk factors for the development of infection in this group of people are an inevitable consequence of the progression of the dementia. The persistence of these risk factors explains why it is not unusual for the person to relapse after the infection has been treated. The issue therefore arises whether the treatment of infections in terminal stage dementia is indicated. Although there are no agreed standard guidelines at present, research suggests that aggressive treatment of infections in people with terminal stage dementia does not influence the progression of the dementia or the mortality rate. It has been shown that palliative management of infections in people with terminal stage Alzheimer dementia is associated with a lower discomfort rate than aggressive medical treatment.

FEEDING DIFFICULITIES

People affected with Alzheimer dementia and Down Syndrome are prone to developing feeding problems. In the early stages memory impairment, poor judgment and difficulty feeding themselves may complicate meal times. In the mid stages, agitation and wandering may prevent the person from settling at a table to eat their meal. By the late stage, the progressive degeneration of the brain results in the person being unable to recognise the smell and taste of food. This problem is further complicated by the physical inability to chew and swallow effectively. Carers and families commonly report that this is the most traumatic issue in caring for people with dementia. Despite these difficulties many people, up to the terminal stage of the disease, do not develop any clinical or laboratory signs of malnutrition when specialist clinical input is available.

It is possible for a person with advanced dementia to be fed orally under the guidance of a speech and language therapist. The therapist will assess the person and then advise carers and families regarding the techniques of skilful feeding and food preparation. The importance of a medical assessment should not be forgotten to rule out any medical or dental condition that could be exacerbating the feeding difficulties. The input of a dietician will ensure that the person receives adequate nutritional input and advice on supplemental feeds if necessary.

The use of tube feeding (nasogastric or PEG/Gastrostomy tubes) had previously been thought to be the answer to feeding difficulties in people in the advanced stages of Alzheimer dementia. Tube feeding was thought to prevent aspiration pneumonia, pressure sores, dehydration and malnutrition and even prolong life expectancy. Research and published data now question whether tube feeding is appropriate7, 8. What has become apparent is that tube feeding in people with advanced dementia can adversely affect their quality of life. Restraints may be required to prevent the tube being pulled out, and infections may develop at the site of tube entry while the risk of aspiration still remains. In addition, account should be taken of the fact that the person is being deprived of the normal social interaction and pleasure that surrounds mealtime.

Care planning considerations in the later stages of dementia

Service providers for people with intellectual disability strive to achieve optimal care for people with Down Syndrome and Alzheimer dementia. Dementia care is clearly an area for services that will benefit from an active multidisciplinary and multifaceted approach. This is especially true when considerations for care for the end of life must be discussed, decided and implemented. That dementia is a progressive, degenerative disease requires that end-of-life considerations be discussed and preferably early in the disease process.

With a proactive dementia screening policy in the Daughters of Charity Residential Services we aim for an early diagnosis of Alzheimer dementia in the Down Syndrome population. Education of families and carers commences from the time of diagnosis and continues throughout the course of the disease. As the person approaches the late stages of dementia, a formal meeting is organised with the family and those involved with providing care to the person in question in order to discuss agreed care plans for the terminal stages of the disease. At this meeting a full and frank discussion with the family regarding the usual clinical course of Alzheimer dementia in Down Syndrome takes place. Therapeutic and management options of the different medical conditions that may arise are discussed. Families are fully informed regarding the options of a palliative care type approach or an aggressive medical and therapeutic management approach to the disease. Following this meeting, the agreed care plan is signed by the clinical team and family members. The care plan is kept in the person’s chart for the purpose of informing all staff involved. The families and carers may revisit and revise this plan at any time as desired. Such individualised care plans ensure that clear goals of care are not only identified but also delivered. From our experience to date it appears that families who have been fully informed regarding the progression of this relentless disease usually opt for a hospice care type approach.

Conclusion

The provision of care to a person with Down Syndrome and Alzheimer dementia involves families, carers and health professionals. The difficulties that present are emotive for all involved. The availability of a clinical and nursing team who are experienced in this area of medicine can ensure that patients with Down Syndrome and Alzheimer dementia are given appropriate care based on best clinical practice. Through sharing of information and support for decisions made, care can be optimised to ensure the best quality of life possible.

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by Anne Halpin, Outreach/Development Officer, Kay Devine, Director, Foyle Down's Syndrome Trust, Spencer Road, Waterside, Derry

Foyle Down’s Syndrome Trust (FDST) was formed in September 1995. The Trust has an innovative and groundbreaking approach to the educational and social inclusion of children and young people with Down’s Syndrome who are disadvantaged through the disabling attitudes in society.

The specific objectives of the Trust are:

  • to support children with Down’s Syndrome and their parents and families by providing home-based education to alleviate language delay and improve communication skills,
  • to support teaching and learning in schools,
  • to develop inclusive social programmes of activities for children and young people with Down Syndrome and their non-disabled peers,
  • to empower and assist parents in leading their children to independent living,
  • to develop inclusive good practice networks providing non-traditional education to children and young people, incorporating the social skill elements necessary to achieve independent living, and
  • to promote and increase representation of people with Down’s Syndrome in the wider community in decision-making and community activities.

Currently the Trust’s work is funded by the Department for Social Development, BBC Children in Need and Community Fund: Lottery Money Making a Difference.

Social programmes

The Trust works with and supports our partner providers in designing and developing specialist workshops for children and young people with Down’s Syndrome. Activities for the younger children are mainly centred around play and fun, and are aimed at building self-confidence and self-assurance in group situations.

The 11–16 age group look at more serious issues such as relationships and keeping themselves safe from harm in their community. They have been involved in two important projects in the past year. The first was ‘Self-Confidence/Self-Assurance’ programme with the Verbal Arts Centre, FDST and Patsy Devine (a community dramatist), The second project, jointly organised by FDST and the Cookie Company, via Challenge NI and the Foyle Parents and Friends Association, introduced young people to basic cookery skills and developed their practical life skills. The course can lead to NVQ accreditation.

Lisa Cregan (14) reports on the project: ‘Every Wednesday, Fidelma, Claire, Nicola, William, Michael, Rhona, Patricia, Emma, Laura and I went to the Cookie Club. Louise [Lyons] was our teacher. She taught us how to get ready to cook, lots of different recipes and all about hygiene and safety and preventing accidents. Here are some of the things we cooked: jams, pancakes, scrambled eggs on toast, spaghetti, stew, chicken curry, burgers, cookies, buns, rice crispies, Mars bar slices. Each week, when we finished our cooking, we ate it! Then we had to clean up! I always looked forward to going to the Cookie Club and I am sad that we have finished now. I would recommend the Cookie Club to everyone.’

Claire Thompson (14) says ‘I liked going to the Cookie Company because I like cooking with my friends. My favourite recipe was Spaghetti Bolognaise.’

Emma Diver (14) and Fidelma Coyle (17) provided the captions for the pictures. [Editor’s note: We apologise that there wasn’t enough space to show all seven pictures, but these will give ‘the flavour’.)

  1. Emma is going to eat vol au vents and orange juice which she made.
  2. Fidelma, Claire, Louise, Emma and Michael are writing about health and safety.
  3. Emma is flipping her pancakes, watched by Fidelma and Lisa.
  4. Louise is helping me read the questions about the recipe. My friend Claire is also in this photo.
  5. I’m cooking pancakes. They were delicious!
  6. Louise is teaching Claire and Emma about hygiene and safety when they are cooking.
  7. Michael is cooking pancakes, watched by Nicola and me.
Community participation

While taking part in any of the social programmes, the children and young people have been integrated into community venues and organisations. The 11–16 age group recently participated in the Carnivale of Colours, a community festival in Derry’s St Columb’s Park, and one of our young people has been invited to join a youth committee for next year’s Carnivale.

Nicola Harkin (16) explains: ‘We spent each Tuesday evening for a month before the Carnival, making our costumes at the Shantallow Community Centre, with Rhona and Jani helping us. To make my hat, I had to cut out the shape and paint it with lots of different colours. To make my skirt, I painted it with a mixture of colours. At the carnival, we walked around St Columb’s Park wearing our costumes. The walking was a bit too far for my legs, but we had a fun day!’

Dolores Murphy (15) and Nicola explain the photos from the day:

  1. This is my friend Laura. Doesn’t she look beautiful?
  2. Here’s a photo of Kay and William. Do you like their costumes?
  3. This is me (Dolores), looking lovely in my beautiful skirt. I painted my skirt with all different colours.

All of the young people thoroughly enjoyed their involvement in the Carnivale, just as they enthusiastically participate in all aspects of the Trust’s work available to each of them.

Professor Hilary Hoey & Joan Murphy, Department of Paediatrics, Trinity College, on the National Children's Hospital (AMNCH) Tallaght, Dublin 24

Tallaght Hospital, 15 June 2001
This Scientific Meeting presented the results of the first major Irish and international research project performed on children and adolescents with Down Syndrome (DS) by Ms Joan Murphy, Dr Magued Philip and Professor Hilary Hoey of the TCD Department of Paediatrics at The National Children’s Hospital. Following the research we produced the Guidelines on the Medical Management of Children and Adolescents with DS in Ireland. The results of this study give evidence-based data which support the need and appropriateness of the guidelines presented for children and adolescents with DS in Ireland.

Down Syndrome is the most common genetic cause of developmental disability in Ireland and has a birth prevalence of 1 in 580 live births—the highest in Europe. In Ireland the population of people with DS is in the region of 4,500 to 6,000- It is well recognised that the syndrome is accompanied by a high incidence of treatable medical disorders. All studies show that early intervention carries a better outcome for quality of life and life expectancy. With medical progress many persons with Down Syndrome now live into their 60s. Until now there were no consensus guidelines for the screening and management of children and adults with Down Syndrome in Ireland.

Irish and UK experts from many healthcare disciplines involved in the care and management of this group gave excellent presentations at the scientific meeting.

Research results and guidelines:
  • Joan Murphy made a very impressive presentation on the results of our research in Ireland and showed in this large cohort of children with DS the incidence and management of medical problems. A brief summary of her report is given below.
  • Professor Hilary Hoey presented the Guidelines for the Medical Management of Children and Adolescents with Down Syndrome in Ireland.
Other areas covered:
  • Breaking the news in the newborn, presented by Dr Margaret Sheridan, Consultant Neonatologist/Paediatrician, The Woman’s Hospital, Coombe,
  • Growth Problems in DS and the launch of the new growth charts (UK and Ireland), presented by Dr Liz Marder, Consultant Paediatrician (UK), and Vice Chairman Down Syndrome Medical Interest Group (DSMIG)
  • Development and early intervention, presented by Dr Sheila Macken, Consultant Developmental Paediatrician, The Children’s Hospital, Temple Street
  • Cardiac problems in children with DS, including investigations, treatment and an excellent video of a heart echo showing anomalies, presented by Dr Desmond Duff, Consultant Cardiologist, Our Lady’s Hospital for Sick Children, Crumlin
  • Thyroid problems in DS and investigations and treatment, presented by Dr Gerard Boran, Consultant Chemical Pathologist, AMNCH, and Dr Philip Maine, Consultant Chemical Pathologist, Head of the Irish Metabolic Screening programme, The Children’s Hospital, Temple Street
  • Visual disorders in DS including investigations, treatment and management, giving a very graphic picture of the many varied disorders in this group, presented by Mr Michael O’Keefe, Consultant Ophthalmologist, The Children’s Hospital, Temple Street
  • Hearing disorders in DS, including hearing screening, and surgical and medical management, presented by Mr Michael Harney, Senior Registrar ENT, and Mr Don McShane, Consultant ENT Surgeon, AMNCH
  • Speech and language difficulties in children and adolescents with DS, including services and management, presented by Ms Clothra Ní Cholmáin, Clinical Tutor, Clinical Speech & Language Studies, Trinity College.
  • Orthopaedic problems including cervical spine instability in children with DS, presented by Mr Esmond Fogarty, Consultant Orthopaedic Surgeon, The National Children’s Hospital AMNCH and Our Lady’s Hospital for Sick Children, Crumlin
  • Professor Ward gave a wonderful presentation on Dr Langdon Down and showed us super pictures of very elegant people with DS from the 1800s.
  • Dr Austin O’Carroll, who himself has a disability, gave an excellent overview of care of a child with disabilities in General Practice.
  • Mrs Maura Connolly, Matron/Manager, The National Children’s Hospital, gave an excellent presentation on the care of children with special needs in hospital.
  • Mrs Ann Haig, whose two-year-old Ali has DS, spoke about her experiences and indicated in a very positive way what she as a parent needed and still needs for the best medical care for her daughter. She stressed the need for medical management guidelines so that she would have the knowledge to collaborate and support the appropriate management together with the health care professionals.
  • Emily, a young adult with Down Syndrome, now in full-time open employment with an American company in Tipperary, delivered a wonderful speech on her varied and successful achievements and the exciting path she has planned for her future.
  • Dr Jennifer Dennis, Consultant Paediatrician (UK) and Chairman DSMIG, highly commended the quality and importance of the research presented by Joan Murphy. She fully endorsed the guidelines presented and discussed their implementation in Ireland.
The Irish research
Growth

This is the first growth study of children with DS in Ireland. Children with DS are shorter and have a smaller growth spurt at puberty. Their weight for height is greater, however, and increases with age. Therefore excessive weight gain needs to be controlled with good diet and exercise. We forwarded this Irish growth data to the UK to enable the development of specific growth charts for Irish and UK children with DS. (These are now available and are being used in the pilot project ‘Personal Held Child Record PHCR booklets from May 2001 in Limerick.)

Congenital Heart Disease

Of the 394 children examined during the study, 171(43.4%) had no cardiac anomalies. 64 (16.2%) had innocent heart murmurs which needed no intervention, but did require investigations to rule out cardiac anomalies and assure the parents that everything was normal. 151 (40-4% had cardiac anomalies, of whom 43 (10-9%) children had Atrial Ventricular Septal Defect (AVSD) requiring early intervention. 56 (14%) patients underwent surgical intervention, while 32 (8.1%) patients were treated medically.

Thyroid

Children and adolescents with DS have an increased incidence of thyroid dysfunction, which can be progressive in nature. The thyroid gland makes the hormone thyroxin which is essential for growth and mental development in children and thus is especially important for the child with DS. Clinical diagnosis is difficult, requiring a blood sample and laboratory tests. Giving a blood sample may be particularly traumatic for a young child who has intellectual disability. In this study we took blood by finger prick and used the Guthrie Card (blotting paper), which is used for screening in the newborn period. Test samples were sent to The National Screening Centre, The Children’s Hospital, Temple Street, where Dr Philip Mayne performed the analysis. We found 16 (4.1%) children to have an under-active thyroid (5 of whom had not been previously detected. The finger-prick blood sample can be taken with little trauma (only two of the children tested cried during the test), and in the centre the child attends daily—or it can be done at home. We hope that children will be tested yearly or two-yearly. Previous studies show a prevalence ranging from 0-7% in neonates to 16.4% in school-age children, with increases in adult life to 22%. (In the general population the incidence is 0-2% to 2% in adults.)

Hearing

Lifelong audiological surveillance is essential for all people with DS; studies have shown that 60–85% of children with DS have mild/moderate hearing loss, and there is increased incidence of conductive, mixed and sensorineural hearing loss in adolescents and adults. Hearing impairment can lead to problems with speech and language development, so that early identification and intervention are required. Assessing hearing is a complex procedure for any child—and particularly for a child with intellectual disability—but once it is identified, hearing impairment can be successfully managed.

This is the first time internationally that such a study has assessed the hearing function of children and adolescents with DS using otoscopy (clinical examination of the ear canal and tympanic membrane), tympanometry (to assess middle ear function) and Distortion Product Oto Acoustic Emissions (DPOAE) to assess inner-ear function. The last two tests are carried out using portable machines which provide quick, objective screening tests, are easy to use and require no patient feedback. The tests can be performed when the child is asleep, or awake and sitting quietly.

Clinical examination—65.6% abnormal (mild to severe wax, perforated tympanic membrane, small ear canals)

Middle ear function—48% abnormal tympanograms (conductive hearing loss or Eustachian tube dysfunction)

Inner ear function (cochlear)—10-8% normal inne- ear function at 3 frequencies, 89.2% abnormal inner-ear function, of whom 28.6 % had mild hearing loss, 49.7% had conductive hearing loss and 10-4% had possible sensory-neural hearing or mixed hearing loss.

DPOAEsan easy, objective non-invasive screening test that can be performed in infants shortly after birth. Absent DPOAEs may indicate hearing loss of 35 dB or greater.

Speech

A high proportion of children had speech impairment (371, i.e.94.5%). Six children (1.5%) had normal speech for their age. 16 (4%) were not tested as they were unable to cooperate on the day of the study. Parents reported difficulties in obtaining speech therapy throughout childhood. As speech developments starts in infancy, speech therapy would be most beneficial from the earliest stages of development. With the present shortage of speech therapists, it may be appropriate for parents to be trained to deliver speech therapy with ongoing professional supervision and evaluation. We are presently looking at international best practice.

Over 400 delegates attended the meeting, from twelve Irish counties and from the UK. Health professionals from a broad spectrum of care disciplines attended—psychiatry, psychology, paediatrics, neonatology, physiotherapy, speech and language therapy, occupational therapy, play therapy—and also area medical officers, clinical nurse managers, research nurses, medical directors of developmental centres, general practitioners, nurses in early intervention programmes and public health nurses.

Mr. Pat Clarke, President (ex officio) of Down Syndrome Ireland, who sponsored the research study, expressed his gratitude to the team for their work in bringing the research so far forward and expressed his hope, on behalf of DSI, that the implementation of the guidelines would soon be implemented nationally. The guidelines have been received with praise and gratitude by parents and health-care professionals. Since the meeting there have been over 500 enquiries, with requests for information and copies of the guidelines.

We are very grateful to the many health-care professionals with expertise in the management of children and adolescents with Down Syndrome in Ireland, who contributed to the Irish edition of the guidelines and to Down Syndrome Ireland for funding the research. We are grateful to the National Children’s Hospital Foundation for sponsoring the scientific meeting and for all those who took part on the day.

Julien Benoit lives in the south of France. His Irish-born mother, Siobhán Benoit-Stuart, describes Julien's lifestyle and education.

My son Julien, who has Down Syndrome, will be eighteen in July. He is the eldest of three children. We live in Alès (pop. 50,000), about 70km from Montpellier. Julien is tall, slim and muscular. His appearance is very important to him and he spends a lot of time lifting weights and doing press-ups. This wasn’t always the case. When he was a baby he had a lot of eating difficulties and he had a loose bowel complaint for the first six years. Recently he has started to eat salads, but he never eats fruit unless it’s cooked. He has a slight malformation of one foot and tibia, but this hasn’t prevented him from developing. He is easy-going, good-natured and a likeable person on the whole, even if he is sometimes stubborn.

Julien’s main health problem has been a fluctuating loss of hearing because of constant ear infections. This caused language retardation and consequently anxiety and behavioural problems. Not understanding led to being misunderstood. Relationships were difficult because of language problems and he had a tendency to close up. After sixteen years doing the rounds of doctors, we finally found the solution–a bone-anchored hearing aid (BAHA). Since then, Julien has flourished enormously in personal relationships, self-confidence, language and learning skills. He is also in perfect control of his hearing-aide, lowering and raising the volume and changing the battery when necessary. He still gets ear infections for which we have found no cure. (We would be interested to know if any Frontline readers know of a cure.)

Educational background

Julien went first to a crèche and then to the ‘maternelle’ (nursery school) at the age of three. In retrospect, that may have been a bit early, but his brother was then two and I was in a bit of a hurry. He moved on to primary school at the age of nine and after two years he joined a special class (CLIS) for children with learning disabilities in a fee-paying primary school (at that time, the private Catholic schools had taken the lead in integration), where he remained until he was thirteen. (There are now 25 such special classes in the primary schools in our department, which has a population of 620,000-) Julien didn’t make much progress in academic subjects and the pressure to succeed created psychological barriers. He decided it was ‘trop dur!’ (too hard!) and he wasn’t capable. His hearing loss obviously played an important part in his lack of progress and self-confidence. When Julien was thirteen, he went to a special class in a secondary school with other girls and boys with Down Syndrome. He flourished there, psychologically and socially. Although he hasn’t made enormous strides in reading or writing skills, they have improved slightly. He made friends, realised that the others had problems too and enjoyed the solidarity within the group, without critical evaluation of his progress. This is his final year in that setting.

Julien has been on several training courses for the past few years and he has learned to travel independently and use the phone (mobile). One morning a week he attends a lycée with mainstream students who are learning to become bricklayers–this is probably the equivalent of an Irish vocational school. He can continue there for two more years. He also spends one afternoon a week with the local Town Hall, working in a municipal park–cutting tress, watering, weeding, planting etc. His third training course is in a riding-school where ‘normal’ students learn grooming and mucking out stables etc. He likes all three training opportunities and we hope that he can pursue them further, continuing the emphasis on manual work and building up his autonomy.

Leisure activities

Julien enjoys several leisure activities. At school he does judo and basketball with children from mainstream classes. He also trains (but doesn’t play competitively) with a regular basketball club once a week. He attends a theatre and dance workshop most Saturday afternoons with other teenagers with Down Syndrome, and he plays the drums with the local music school. This year he is doing an audition with his teacher, playing ‘Hotel California’. And–I nearly forgot to mention–he recently started skiing during the February holidays and after an hour a day for a week, he managed to take the ski lift and slide down the slope, turning left and right. Those of you who have tried skiing for the first time will appreciate that this is no mean feat!

A regional Down Syndrome Association (Groupe d’Etude pour l’Insertion Social des Trisomiques 21–GEIST 21) also organises extracurricular activities and a weekly workday every Wednesday. The morning is given over to talking about members’ training courses. Sometimes they buy ingredients and cook a meal together, or they may eat in a cafeteria or restaurant.

For the second time this summer I will organise a summer sports camp for six teenagers with Down Syndrome, in connection with the organisation ‘Sport Adapté’. The young people will go off to the mountains without their parents, and they will do climbing, canoeing, potholing, mountain-biking and other adventure sports–allowing them to exploit their capacities and test their possibilities in a reassuring environment.

The French educational system

In France, children with Down Syndrome are usually fairly well accepted in the crèches and nursery schools (la maternelle). However in primary school, where there is more emphasis on reading and writing skills, whether a child with Down Syndrome is integrated depends on the goodwill of an individual teacher–who must have the necessary time, energy and patience–and whether the child’s behaviour is sociable or disruptive. It is often necessary to reassure the teacher that although demands should be made on the child with Down Syndrome to help him/her progress, it’s more important that he/she should learn to live and socialise with his/her peer group.

The educational system is more or less the same throughout France. Most children attend a free state-run school. If parents are dissatisfied with the local school, they can send their children to a private fee-paying school. More and more children with Down Syndrome now continue in mainstream education whenever possible. In most primary schools, there is a special class for special-needs students, called a Classe Intégration Scolaire (CLIS), where there is no fixed programme and (usually) a specialist teacher. They may then join a special class in a secondary school, called a ‘UPI (Unité Pédagogique Intégration)–if one is available to them–until perhaps the age of sixteen or seventeen. The only UPI in our departement (The Gard) is in Nîmes–again it is in a private Catholic school–and Julien makes the 45-minute trip there and back either by train or by taxi.

Some pupils with Down Syndrome stay in the mainstream system and do very well, but for the moment, they are still the exception. We know one girl in secondary school, who is far from being at the bottom of her class. However, more secondary-school special classes are needed to cater for the OTHERS. This is seen as the best solution for many children because they can mix with other students at meal times, for sport activities or art–and in some cases for academic subjects. Alternatively, some parents choose to send their children to an Institut Médical Educatif (IME) or an Institut Médical Professionelle (IMPro). These are institutions in a sheltered environment which cater for the pedagogic needs of all children with learning disabilities, until the age of twenty.

Some adults with learning disabilities work in a workshop run by an institution called a Centre d’Action par le Travail (CAT). However, more and more are looking for work in a ‘normal’ environment. Our children are breaking new ground, they are the first generation to be integrated into mainstream society. We are confident that they will show their capacity to work, and that it will be easier for future generations.