SPECIAL REPORT For reprint orders, please contact: [email protected] Management of speech, language and communication difficulties in Huntington’s disease
Alison Hamilton1, Ulrika Ferm2, Anne-Wil Heemskerk3,4, Rita Twiston- Davies5, Kirsty Y Matheson6, Shiela A Simpson6,7 & Daniela Rae*6,7; On behalf of the contributing members of the European Huntington’s Disease Networks Standards of Care Speech and Language Therapist Group
Speech and language therapy has an important role to play in the management of communication problems in Huntington’s disease. As the disease progresses the effectiveness of communication becomes increasingly compromised by a combination of changes in motor function, diminishing cognitive linguistic skills and neuropsychiatric changes, such as depression and apathy. Signs and symptoms associated with Huntington’s disease are distinctive but there is considerable
Practice Points Practice variation between individuals on the extent, rate and natural course of disability within the disease. For these reasons assessment and review must be comprehensive and consideration should be given to a number of factors such as mood, motivation and behavior, which will be pertinent to performance. As a variety of symptoms can affect communication no single course of treatment will be effective throughout the disease. The consensus of opinion is that the therapy management will vary and that interventions will at different times be rehabilitative, facilitative, informative and supportive. All interventions must be timely and responsive to the changing needs of the individual and to the challenges faced by the family and others. Augmentative and alternative communication can compensate for communication difficulties in Huntington’s disease and can increase the individual’s chance for participation in daily life but strategies need to be implemented while there is still motivation and learning capacity. It is vital to understand the value and importance in involving family and caregivers in developing and facilitating effective communication strategies. These are well recognized for the positive impact they can have on the overall well being of the individual with Huntington’s disease.
1Department of Speech & Language Therapy, Aberdeen Royal Infirmary, Aberdeen, Scotland, UK 2DART – Centre for Augmentative & Alternative Communication & Assistive Technology Regional Rehabilitation Centre, Queen Silvia Children’s Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden 3Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands 4Department of Speech & Language Therapy, Huntington Centre, Topaz, Overduin, Katwijk, The Netherlands 5Department of Speech & Language Therapy, Royal Hospital for Neurodisability, Putney, London, UK 6Department of Clinical Genetics, NHS Grampian, Aberdeen, Scotland, UK 7University of Aberdeen, Aberdeen, AB25 2ZA Scotland, UK *Author for correspondence: Tel.: +44 1224 552 120; [email protected] part of
10.2217/NMT.11.78 Neurodegen. Dis. Manage. (2012) 2(1), 67–77 ISSN 1758-2024 67 special report Hamilton, Ferm, Heemskerk et al.
SUMMARY Speech and language therapists play an important role in the management of communication difficulties in Huntington’s disease (HD). As the disease progresses the effectiveness of communication becomes increasingly compromised by a combination of changes in motor function, diminishing cognitive linguistic abilities and neuropsychiatric changes, such as depression and apathy. The complexities and challenges presented by communication breakdown in HD require comprehensive assessment and interventions that are responsive to the changing motor, cognitive and emotional needs of the individual. The European Huntington’s Disease Network Standards of Care Speech and Language Therapy Working Group has brought together expert speech and language therapists from across Europe to produce guidelines to improve the management of communication disorders for individuals with HD. The guidelines were developed with the aim of promoting timely and appropriate assessment and focused management throughout all stages of the disease. Literature was thoroughly searched and evaluated in an attempt to ensure that the guidelines are based on available evidence. However as there is a paucity of good-quality, high-level evidence the guidance is based predominantly on expert opinion and consensus. The provision of care varies widely between countries in Europe and the implementation of these guidelines aims to help improve the quality of care delivered to individuals with HD.
Huntington’s disease (HD) is a hereditary, be manifested in any or all of the respiratory, neurodegenerative disorder with progressive phonatory, resonatory and articulatory levels motor, cognitive and neuropsychiatric of speech with prominent effects on prosody. impairments, which can have a profound effect This type of speech disturbance is the pattern on the ability of the individual with HD to characteristic of hyperkinetic dysarthria [6,7], communicate (Box 1) [1,2]. It is caused by an which is most often associated with diseases unstable expansion of a CAG sequence within of the basal ganglia control circuit. Several the Huntingtin, known as HTT, gene, which is studies characterizing the presence and nature located on chromosome 4 [3]. The protein encoded of dysarthria in HD have identified a number by the HTT gene is important in normal brain of common features (Box 2) [8–11]. and neuronal development. The expanded CAG In the early stages of the disease the affected sequence leads to the production of an abnormal individual may have no symptoms or a mild or protein that causes brain cell dysfunction and fluctuating dysarthria associated with some of the ultimately neuronal cell death primarily in the above features. Intelligibility is not likely to be basal ganglia but also the thalamus and cerebral reduced during early disease. With the progression cortex [4]. This single gene disease follows an of the disease, most affected individuals develop autosomal, dominant inheritance affecting both mild-to-moderate dysarthria with the choreic men and women. Each offspring of an affected movements interfering increasingly with the parent has a 50% risk of inheriting the gene intelligibility of speech. Hartelius et al. studied mutation and developing the disease. Symptom speech disorders in mild and moderate HD and onset usually occurs in middle age but a juvenile determined that there was a significant difference onset (<20 years of age) and late-age onset are in the severity of the dysarthric features between the also recognized. mild and moderate presentations and concluded Any of the degenerative changes associated that the dysarthric speech signs seem to evolve in with HD can have a profound effect on the line with the general progression of the disease [11]. individual to communicate. In the latter stages of HD, affected individuals are The term dysarthria is the collective name likely to suffer from severe dysarthria and natural given to the group of related speech disorders speech may no longer be possible. that manifest due to disturbances in muscular As the disease progresses, difficulties in control. The choreiform movement disorder executive functioning and in working memory characteristic in HD can disrupt all systems influence the affected individual’s linguistic contributing to speech production. In HD, abilities. A number of studies have investigated neurodegeneration of the basal ganglia – the characteristics of language impairment in specifically the caudate nucleus, putamen HD and while there are some inconsistencies in and globus pallidus [5] – results in a variable the findings some common features have been pattern of speech disturbances. These may identified (Box 3) [12–17].
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In the early stages of HD, affected individuals Box 1. Symptoms of Huntington’s disease. may appear to have no deficits in language but Motor symptoms research findings suggest some difficulties in Chorea comprehension may develop, particularly with Dystonia more complex discourse [12]. The inclination Rigidity to initiate conversation may also show signs of Bradykinesia decline [13]. Consensus opinion suggests that even in the late stages of the disease when many Gait disorder factors have had a negative impact on language Cognitive changes Difficulties planning and organizing function it is thought that the affected individual will understand more than is expressed. Lack of initiation (activities and conversations) Some individuals may develop apraxia of Perseveration speech. This adds a dimension of difficulty that Impulsivity may include inconsistent articulatory errors, Irritability and temper outburst groping oral movements and increasing errors Perceptual problems with increasing word and phrase length. An Unawareness/lack of Insight apraxia of speech can also generate difficulties Difficulty in new learning in complying with other sequenced activities, Speech, language and communication problems such as co-ordinating breathing and voice. Specified in text All interventions must be responsive to the Neuropsychiatric/behavioral symptoms changing motor, cognitive and emotional needs Depression of the individual with HD and the challenges Obsessive–compulsive disorder faced by family members and significant Irritability others. The focus in intervention is to evaluate Apathy and maximize communication skills, augment Anxiety these skills whenever possible and to guide and Other educate carers, family members, health and Metabolic changes social care professionals and others in the most Sleep disturbances effective ways of communicating with the person Dysphagia with HD. The purpose of these guidelines is to provide special interest in the speech, language and information and guidance from the current communication difficulties of individuals with evidence that is available in the literature as well HD and their families. A literature search was as consensus views of the members of the EHDN performed electronically using Ovid MEDLINE Speech and Language Therapy Standards (1988–2009), Ovid Embase (1988–2009) of Care Working Group, thereby reflecting and EBSCO Cinahl (1988–2009) to identify the opinions of experts working in this field. evidence within the themes of communication, The research base within the field of speech, language problems and dysarthria in HD. A language and communication intervention search strategy was developed in collaboration in HD is limited and there is a lack of good with the members of the Standards of Care high-level evidence to support many aspects of SLT Group and search terms are outlined in the management of communication problems. Box 4. The literature search was updated during It is hoped that these guidelines will enable the development of the guideline. All relevant speech and language therapists and others to publications were identified and categorized to provide optimum intervention to support the evidence statements according to the Scottish communication needs of the affected individual Intercollegiate Guidelines Network (SIGN 50) and his or her close partners at different stages [101]. It is important to keep in mind that, due of the disease process. to the lack of scientific evidence, the present recommendations were also formulated based Methods upon clinical experience and expert consensus The development of the guidelines resulted from within the EHDN Standards of care SLT from a number of meetings and discussions group. It is the wish of the group that these between speech and language therapists with guidelines can indicate the gaps in knowledge extensive clinical expert knowledge and a and stimulate future research.
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Box 2. Common features of dysarthria in Huntington’s disease. Articulatory breakdown distorting both consonant and vowel sounds Inappropriate pausing within words and between words, prolonged phonemes Mistiming in breath control/sudden forced inspiration or expiration Inappropriate silences between utterances Harsh and strained/strangled phonation Hoarseness of voice Variability of volume control (in particular excess volume variations)
Management of communication in HD Phonation Assessment Articulation The timing of the onset and the progression of the various motor, neuropsychiatric and Prosody cognitive symptoms associated with HD remain Resonance uncertain especially in the early and middle stages of the disease [18,19]. Signs and symptoms Intelligibility of HD are distinctive but there is considerable variation between individuals on the extent, This is to provide a description of the speech rate and natural course of disability within and the musculature on which to base therapy the disease [20]. For these reasons assessment of and measure change. In all cases, the aims of communication should be comprehensive and the assessment should be to gather information an evaluation of a number of factors pertinent on the parameters contributing to the dysarthria to communication should be considered. Early and the effects on daily living. These baseline comprehensive assessment provides a valuable data provide a measure of overall severity that baseline for the evaluation of later deficits and can be reviewed and re-measured as the disease for the provision of appropriate support and progresses. intervention. Performance should be assessed Assessment of language skills in HD should and observed in different environments as involve a range of language assessments; both results may vary. Pertinent factors to consider formal standardized tests and informal methods, include mood, motivation and behavior, such as checklists and observational protocols, insight, memory (immediate, recent and new in order to form an early hypothesis regarding learning, long-term), medication, motor skills the nature of the impairments and their impact. and opportunities for communication and social Formalized standardized assessments may not activities. capture the early language problems in HD [12]. Assessment of dysarthria should include the As with assessment of motor speech disorders following: these assessments should provide baseline Assessment of orofacial movements information that underpins the therapeutic and management plans for affected individuals. Respiratory function in speech Changes should be monitored, however, the Breath control and co-ordination timing, relevance and benefits of reassessment in a progressive condition such as HD should Box 3. Language impairments in Huntington’s disease. always be considered carefully. Assessment should provide information on Increasing difficulties in understanding complex discourse and in drawing both receptive and expressive language skills inferences and the following aspects are important to be Latency of response considered [8,12–14,21]: Word finding difficulties Reductions in the number of words used Ability to process and retain information Decreasing length of utterance Ability to understand complex language Decreasing use of syntactical complexities Increasing susceptibility to interference Spontaneous speech Increasing difficulties in maintaining topic of conversation Grammatical form, sentence structure and Problems with perseveration length of utterance
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Naming/word finding Box 4. Search terms used in the literature search. Perseveration (words, phrases, ideas) ‘Huntington’s disease’ and ‘deglutition disorders’, ‘pneumonia’, ‘aspiration’, ‘aerophagy’, ‘fluoroscopy’, ‘dysarthria’, ‘dysphagia’, ‘voice disorders’, ‘voice’, ‘verbal Reading behaviour’, ‘communication aids’, ‘language disorders’, ‘speech disorders’, ‘phonetics’, Writing ‘aphasia’, ‘vocabulary’, ‘semantics’, ‘anomia’, ‘articulation disorders’, ‘speech acoustics’, ‘linguistics’, ‘communication disorders’. In addition to the assessment of the speech subsystems and of the changes in language information and to support them in acquiring integrity, information should be collated on greater knowledge and responsibility for the communication profile of the affected the success of communicative interactions. individual. This is the information that is Training communication partners is important relevant to the social contexts of communication and should begin with careful observation as well as the skills and influences that can and discussion about natural communication affect the effectiveness of the interaction. The between the partners and the person with HD. following factors should be considered in the Existing strengths should be encouraged and any communication skills profile [22]: carer who has built up a good relationship with The communication skills of the individual, the person with HD should be encouraged to their strengths and needs share their ideas and also be used in any training for others as an example of good practice. Use of communication by the individual in It is worth noting that recent research their current environments by Hartelius et al. on communicative The skills of the communication partner interaction in HD indicated that family members and carers perceived some changes The impact of the communication difficulties in communication in different ways than on the individual and their environment, the affected individuals [20]. While family including emotional, psychological and and carers focused on changes in speech, psychosocial aspects language comprehension, the lack of depth in conversation and need to make adjustment, Communication strategies & techniques in the affected individuals were concerned more different stages of the disease with the effort that it took to communicate The rate of progression and the course of the and the amount of concentration required. It disease in HD are unpredictable and will vary was acknowledged by all the participants in significantly between individuals. A variety the study that there was variability and lack of of symptoms can affect communication and initiative in communication and that there is no single course of treatment will be effective a need for increased participation in social life throughout the disease. The consensus of in order to enhance communication. opinion is that a variety of approaches and therapeutic techniques should be considered Early stages to allow the individual with HD to maintain The consensus of opinion is early referral communication skills for as long as possible. to Speech and Language Therapy Services The disease process can be over many years is preferable. The aims of early referral are and maintaining communication skills from recognized as good practice in the management the early stages can be very important. An of long-term neurological conditions [102]. This affected individual may be known to Speech and facilitates clinical decision making on timely Language Therapy Services for up to 20 years assessment and intervention. It also allows the or more. Compensatory approaches aimed at individual and family members to: minimizing the disability and maximizing Seek and receive information and pertinent intelligibility should be considered. The goals advice of the therapy management will vary throughout the disease and may be informative, facilitative, Discuss any communication difficulties supportive and at times rehabilitative. It is Discuss future management and options on also likely that in the mid-to-later stages the supporting communication main focus of the intervention will be with the family and/or carers both for the exchange of Discuss anxieties
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To begin forming an effective therapeutic opportunities for communication in different relationship with the appropriate professionals activities and settings so that social relationships can be maintained. Affected individuals and Mild dysarthric impairments may be present family members often have previous experience in the early stages. Symptoms of dysarthria of communication difficulties in HD and seem to evolve in line with general disease discussions involving the affected individual and progression [11]. In the early stages a symptomatic family members/carers may elicit information approach to management can be used [20]. This on preferences and past experiences, which will may involve, for example, relaxation techniques help in planning for the future. and voice therapy techniques designed to normalize laryngeal tone if hard glottal attack Late stages or phonatory stenosis is identified. Stress and Natural speech is no longer likely to be intonation drills to maintain prosodic features intelligible but evidence suggests some abilities to have also been found to be beneficial in the early comprehend may remain. Severe motor deficits stages [23,24]. may also limit the individual’s ability to write, Consensus opinion in early disease is that type or directly select words or phrases from the merits of self awareness are important and a communication device and these skills are teaching techniques for self-monitoring of speech required for many augmentative and alternative is useful. Self-monitoring can be helpful if the communication devices [19]. Additionally rate of speech increases. It is recognized that cognitive decline may limit the ability to learn in early disease affected individuals may rush and use many of the complex augmentative speech in order to avoid the disruptions caused systems available. by involuntary movements. Individuals may Communication management is a challenge benefit from breathing exercises co-ordinated at this stage both through cognitive–linguistic with speech to maintain a rate consistent with impairment and severe dysarthria. In addition physiologic capacity [24]. Mild cognitive changes the affected individual may lack initiative and be may take place at this time and there may be apathetic towards participating in conversation. difficulties processing complex information. It The individual may also feel frustrated and have is recommended that comprehension should be a low tolerance towards engaging in certain assessed even in the early stages of HD [15] and activities. It is agreed that the focus should be that functional capacity scales may be useful on enhancing participation in communication at this time as these may give information on activities, thereby supporting the affected cognitive deficits that will have an effect on individual to maintain some skills to use to everyday communication. maximum potential. There are some simple principles in managing communication to be Mid stages observed at this stage [19]: Consensus opinion is that information is key Select simple systems that take advantage of to both the affected individual and the family/ previously learned skills; carers and that it is beneficial to increase contact with a partner or carer. Cognitive changes may Avoid complex systems or techniques that are compromise the affected individual’s capacity difficult to learn; for learning and it will become necessary for Educate conversation partners well and rely the listener to adopt special communication on them. They can help to structure strategies. Problems with memory and difficulties interactions and control the environment to with language comprehension and/or expression maximum effect. along with fewer communicative initiatives and social isolation may cause concerns and The clinical experience is that ancillary activities severe problems for the affected individual and can coincidentally support communication skills, their family and carers [20]. There is a need for for example, hydrotherapy for relaxation can different kinds of support and it is during this present a good opportunity to practise breathing stage that advice to the family and carers about and voice, music therapy for practising timing and the communication environment is highly volume control with singing, helping to maintain appropriate. The role of the speech and language breathing and voice as well as word recall; taking therapist is to assess and advise on facilitating part in activities that promote interaction, thus
72 Neurodegen. Dis. Manage. (2012) 2(1) future science group Management of speech, language & communication difficulties in HD special report limiting social isolation. Collaborative work with the importance of social interaction in the the multidisciplinary team can be beneficial in management of HD [26]. maximizing the opportunities for interaction and The potential for multiple complexities in optimizing communication skills. Management presenting features including motor, cognitive– plans should encourage natural speech as long linguistic and neuropsychiatric symptoms that as possible with supported communication may interact and affect communication, are techniques playing a key role. Strategies should likely to affect the individual’s abilities to use be used consistently and should be as reliable different communication systems. As pointed as possible. This is particularly important in out by Beukelman et al. individuals with HD developing and establishing Yes/No systems. All also seem to be underserved as far as AAC is communication strategies should be documented concerned [27]; little research exists and the and made accessible to caregivers to limit the documentation is poor. For these reasons it potential for breakdown. It is essential that may be useful for the individual clinician to the affected individual maintain some sense make referral to or seek support from teams of of autonomy and independence for as long as professionals specialized in AAC. possible. Individuals with HD and their partners should be encouraged to use the ‘aids’ that are common Augmentative & alternative and natural in many societies today, for example, communication paper and pen, calendars, notebooks, note lists, Augmentative and alternative communication computers and mobile phones. The latter offer (AAC) is the all embracing term for the memory support functions (e.g., electronic different symbols, devices, strategies and calendars, address books and reminders) and techniques that are used to compensate for an possibilities for remote communication (e.g., individual’s difficulties with speech, language Internet services, phone calls, e-mails and text- and communication [25]. AAC interventions messaging [34]). With the progression of the target the specific difficulties an individual disease these systems need to be adapted and may have in relation to both expression other more specific communication devices and understanding. More specifically, AAC that can be individualized may be needed. includes all existing means of communication Regular assessments of communication needs in an individual (e.g., speech and body and adaptations of communication systems communication) as well as different low- and accordingly are necessary. high-technological communication systems and In HD, visual support may be particularly the components that are needed for these to be important. Communication boards and books used in meaningful ways. Letters, words, phrases and cognitive–linguistic strategies that stimulate and pictures on boards and in speech generating the use of particular words and utterances [1] can devices and computers; digitalized and synthetic support both the understanding and production speech and different techniques and strategies of language in the communication situation. for achieving effective communication are all Communication books and books with important components of an AAC system as are personal photographs also have been reported conversation partner strategies. to stimulate communicative interaction. Visual Augmentative and alternative communication schemes depicting different activities and can compensate for communication difficulties their components can facilitate the planning, in HD and increase the individual’s chances initiation and completion of daily activities. for participation in daily life but need to be Personal introduction and identification cards implemented while there is still motivation and as well as personal communication passports [28] learning capacity. It also requires significant are other low-technological resources that can fill involvement from close partners. According important functions at this stage. to the individuals with HD, family members Communication is a collaborative project that and carers in the interview study by Hartelius always involves at least two individuals. There et al. communicative initiative is a problem, are some linguistic features that complicate indicating that support for social interaction in communication and warrant for some particular different daily activities is important for persons fine-tuning and cooperation between the with HD and their conversation partners [20]. person with HD and his or her communication A recent study by Power et al. also discusses partner/s. For example, individuals with HD
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have reported that other people often speak too could choose to place the picture of sleep under fast and that communication rate is a factor that either of the pictures for ‘excellent’, ‘good’, ‘so- can influence communication negatively [20]. so’, ‘not so good’ or ‘bad’. TM is not a personal Family members and carers in the study by communication aid but a resource that can be Hartelius et al. discussed other factors that made used in specific situations and with specific goals communication difficult for example changes in in mind (e.g., if you want to learn more about personality and physical–interactive behaviors, which social activities a person with HD would such as lack of eye contact. Verbal latency and like to participate in). Murphy et al. showed echolalia are other features that can complicate that people with dementia were more effective communication between persons with HD and communicators when TM was used; they became other people. Examples of advice and strategies more engaged and focused in conversation and which may support both the affected individual persevered less [31]. Similar findings were made in and the communication partner are listed below: another study where TM was used by individuals Allow plenty time for communication in different stages of HD [32]. The affected person Reduce environmental noise and distractions and their partners enjoyed using TM and felt that TM made communication easier. As it seemed Talk about and set the grounds for the structured visual framework and the partner’s communication, identify good and bad interaction style facilitated the way the person strategies with HD could think about and express their How is misunderstanding signaled and own thoughts. TM function well in face-to-face resolved? What is the best communication conversations involving one person with HD and rate? Need for pauses? one conversational partner. However, Hallberg Use of facial expression and gesture; both et al. found that TM can also be effective in group parties discussions involving four persons with HD and a Use of available tools to support discussion leader [20]. Ferm et al. showed that both communication (e.g., paper and pen, picture patients with HD, their accompanying significant and letter boards or other personal others and the dental hygienist appreciated and communication aids) felt more involved in dental and oral healthcare consultations when TM was used than when the Use a reduced rate of speech consultations were unaided [33]. Repeat/rephrase and simplify message through use of key words Conclusion Cue individual into new topics This guidance document on the management of Use of yes/no questions speech, language and communication problems in HD has been developed reviewing the In communication, verify understanding of available evidence as well as expert consensus the message on best clinical practice. The guidelines consider One method that has been shown to be the progression of the disease over time and the successful for persons with HD and their importance of the involvement of Speech and conversation partners is Talking Mats (TM) [29]. Language Therapy Services from an early stage TM is a visually based framework that helps in managing the complex and disruptive changes individuals with cognitive and communicative in motor function and cognitive–linguistic difficulties to communicate their views. TM skills that can compromise a broad range of consists of a mat, a visual evaluation scale, communication functions. It underlines the pictures for conversational topics and pictures importance of responsiveness and adaptability for questions relating to these. Only open in addressing the changing clinical picture of questions about different issues that are relevant the affected individual and the value of involving for the individual are asked and the person the family and caregivers in developing and places or indicates under which picture in the facilitating effective communication strategies. visual evaluation scale a picture of a certain issue These clinical guidelines are an important tool should be placed. For example, if the topic is in attempting to provide equity and quality of ‘health’, which Hallberg et al. showed is relevant service provision and are also a work in progress for persons with HD [20], and the question is which will require review and revision as new “How do you sleep in the night?”, the person research becomes available.
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Future perspective Intervention programs involving the person These clinical guidelines mirror the fact that who has the disease as well as family members Huntington’s disease is a complex disease that and other persons in his or her social network influences not only the person who has the will be developed and evaluated; disease but also spouses, children, friends, carers Ecological validity and the usefulness of and professionals who are responsible for the care different intervention approaches for the persons and support of affected families. Recent research involved – the person who has HD as well as his and developments within the field are indicative or her significant communication partners – of factors that will be important in the future; in should be measured and taken into consideration clinical Speech and Language Therapy Services in clinical practice as well as research. as well as in research: Methods for measuring changes in speech, Acknowledgements language and communication with the The authors would like to acknowledge the contributing progression of the disease will be developed members of the European Huntington’s Disease Network and evaluated; (EHDN) Standards of Care Speech and Language Therapist Group to the writing of the Guideline document. The effects of changes in speech, language and cognition on communication will be an Financial & competing interests disclosure important issue; The EHDN had no role in study design, data collection and analysis, decision to publish, or preparation of the manu- Communication and social interaction, rather than speech and language as isolated script. The authors have no other relevant affiliations or phenomena, will be of main concern in financial involvement with any organization or entity with clinical practices; a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from AAC systems to meet the many varying needs those disclosed. in persons with HD and their conversation No writing assistance was utilized in the production of partners will be developed and evaluated; this manuscript.
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19 Yorkston KM, Millar RM, Stroud EA 26 Power E, Anderson A, Togher L. Applying the the picture based low technological Management of Speech and Swallowing in WHO ICF framework to communication framework Talking Mats was used than Degenerative Disease. PRO-ED, TX, USA assessment and goal setting in Huntington’s when communication was unaided. This 139–154 (2004). Disease: a case discussion. J. Commun. finding is suggestive of future AAC Disord. 44(3), 261–275 (2011). n n Very comprehensive piece of text describing implementation and evaluation.
the disease and its impact on the affected n This study describes the use of the WHO’s 33 Ferm U, Eckerholm Wallfur P, Gelfgren E, individual and family and carers. It describes International Classification of Functioning, Hartelius L. Supporting communication with some of the features affecting Disability and Health (ICF) in relation to a Huntington’s disease patients, their partners communication and offers practical advice 37-year-old man who has Huntington’s and the dental hygienist using Talking Mats. on the management of presenting disease and his mother. Communication Forthcoming in NE Tunali (Ed.). In: difficulties. assessment and goal setting are described in Huntington’s Disease – Core Concepts and Current Advances. (2011). 20 Hartelius L, Jonsson M, Rickeberg A, Laakso detail and the importance of identifying K. Communication and Huntington’s disease; environmental factors that influence n Huntington’s disease leads to many different qualitative interviews and focus groups with communication is exemplified and discussed. healthcare consultations in which persons with Huntington’s disease, family 27 Beukelman D, Fager S, Ball L, Dietz A. AAC communication is of central importance. members and carers. Int. J. Lang. Commun. for adults with acquired neurological Individuals with Huntington’s disease have Disord. 45(3), 381–393 (2010). conditions: a review. Augment. Altern. the right to be able to understand the
n n This study is unique in examining how Commun. 23 (3), 230–242 (2007). information they receive from different individuals with Huntington’s disease, 28 Millar S, Aitken S. Personal Communication professionals as well as to ask questions and family members and carers experience the Passports: Guidelines for Good Practice. Call discuss own care. This study represents an changes in communication that come with Centre, Edinburgh, UK (2003). important attempt to support and evaluate the disease. Factors influencing 29 Murphy J, Cameron L. Talking Mats: A communication between individuals with communication are identified and discussed. Resource to Enhance Communication. Huntington’s disease and their carers during a naturally occurring consultation at the 21 Murray LL. Spoken language production in University of Stirling, Stirling, UK (2006). dental hygienist’s. Huntington’s and Parkinson’s disease. Am. J. 30 Hallberg L, Mellgren E, Hartelius L, Ferm U. Speech Lang. Pathol. 8, 137–148 (2000). Talking Mats in group discussions involving 34 Mattsson Müller I, Buchholz M, Ferm U. Text messaging with picture symbols – 22 Royal College of Speech and Language persons with Huntington’s Disease. Disabil Therapists’ Clinical Guidelines. Speechmark Rehabil Assist Technol. (2011) (In press). experiences of seven persons with cognitive and communicative disabilities. J. Assist. Publishing Ltd. Milton Keynes, UK (2005). 31 Murphy J, Gray CM, Cox S, Van Achterberg Technol. 4(4), 11–23 (2010). 23 Prater RJ, Swift R. Manual of Voice Therapy. T, Wyke S. The effectiveness of the Talking Little Brown, MA, USA (1984). Mats: framework for people with dementia. Websites Dementia Int. J. Social Res. Pract. 9(4), 24 Yorkston KM, Beukelman DR, Stroud EA, 101 Scottish Intercollegiate Guidelines Network. 454–472 (2010). Bell KR. Management of motor speech SIGN 50 - A Guideline Developers disorders in children and adults. PRO-ED, 32 Ferm U, Sahlin A, Sundin L, Hartelius L. Handbook. SIGN (2008). TX, USA (1999). Using Talking Mats to support www.sign.ac.uk/guidelines/fulltext/50/index. communication in persons with Huntington’s html 25 Beukelman D, Mirenda P. Augmentative and disease. Int. J. Lang. Commun. Disord. 5, Alternative Communication Supporting 102 UK Department of Health. National 523–536 (2010). Children and Adults with Complex Framework for Long Term Conditions Communication Needs. (3rd Edition). Paul H n n This study shows that communication in (2007). Brookes, MD, USA (2005). individuals with Huntington’s disease is www.dh.gov.uk/en/Publicationsandstatistics/ sensitive to different communication modes. Publications/ Communication was more effective when PublicationsPolicyAndGuidance/ DH_4105361
76 Neurodegen. Dis. Manage. (2012) 2(1) future science group Management of speech, language & communication difficulties in HD special report
Contributing members The Netherlands Belgium UK Anne-Wil Heemskerk Liesbet Desmet Steven Bloch Bianca Bakker Sweden John Eden Maaike Nieuwkamp-Bosma Ulrika Ferm Alison Hamilton Raymund Roos Lena Hartelius Melissa Loucas Hans Claus Daniela Rae Czech Republic Fleur Veldkamp Zuzana Lebedova Gilly White-Cooper Ingrid Veltman Pavla Sasinkova Rachel Thomson Yvonne Zimmerman Rita Twiston-Davies Spain Aileen Ho Italy Asunción Martínez Liliana Basmagi Sheila Simpson Norway Paola Zinzi Marleen van Walsem
USA Jeanne Thomson
future science group www.futuremedicine.com 77