Adult Case Report
Statement of the Problem
A, a 76-year-old man, was referred by Occupational Therapy (OT) to Speech and Language Therapy (SLT). A has experienced communication and swallowing difficulties since the onset of symptoms of Huntington's Disease (HD) in October 2014.
History
OT are currently supporting A with activities of daily living, such as providing apparatus for entering and leaving the bath. An assessment screen was completed by OT which highlighted that A has significant impairment in all areas. A was referred for further assessment of his expressive and receptive language skills to support management from OT and other allied health professionals.
A has had no prior assessment or intervention from SLT. A full case history was taken with A and his brother, D before student contact within the hospital clinic. The student conducted an assessment Minnesota Test for Differential Diagnosis of Aphasia (MTDDA) (Schuell, 1965), a mealtime observation at A's home and the Communication Activities of Daily Living 2nd Edition (CADL-2) (Holland, 1999).
Summary of Case History Information
(Source: SLT case notes)
From the case history, it was established that A has three sons who live nearby, and receives daily support from his elder brother, D. A attends SLT appointments with D who provides transportation as A is unable to drive due to his symptoms. A's sons are aware of genetic counselling and are currently processing genetic testing privately, to prevent anxiety and emotional turbulence for their father.
A remains independent and enjoys taking coach holidays to Europe independently, attending football and rugby matches and regularly attends a walking group. He enjoys going to the cinema every week and typically walks home unaccompanied. However, his family have become increasingly concerned about A's safety, and A has been advised to take taxi's home, rather than walking back to his flat unaccompanied.
A uses a walking stick or an umbrella to aid his mobility when out socially. His walking is often unbalanced with an unsteady gait. Low muscle tone in A's right leg means that he finds it difficult to walk up the stairs to his flat. There is not a lift in place at the complex.
A had cancer of his right leg in 2013 and subsequently had to have some muscle and skin removed. The wound required careful attention to keep the wound clean and bound. D reports that, while A was gardening with a neighbour, a distinctive smell became apparent to the neighbour. A was not aware of a smell. However, the neighbour had suspicions that the smell originated from A's leg. The neighbour's suspicions were correct. Subsequently, A went into a hospital for urgent care for his infected leg. It was at this time, in 2014, that A received neurological testing for other symptoms his family were concerned about. From this testing, consultants concluded that he had a diagnosis of HD, inherited from his mother who died over 35 years ago.
From this point onwards, on release from hospital, assessment and management from Occupational Therapists and other allied health professionals including physiotherapy and dietetics were requested. However, no intervention from SLT was sought. A has difficulty with his vision and wears glasses at all times. A does not currently use hearing aids or any other hearing device.
Diagnosis
A received neurological testing following symptoms of chorea and dystonia. Consultants concluded a diagnosis of HD inherited from his mother with support from D who reported their mother's symptoms and cause of death. HD presents with clinical features which can be associated with other conditions such as Parkinson's disease and Motor Neurone Disease. Nevertheless, HD is distinguishable as it is an inherited disease of the central nervous system caused by a chromosomal mutation of chromosome 4 which can be confirmed with genetic testing. Due to the progressive nature of HD, much like other conditions such as Multiple Sclerosis, the disease progresses with periods of deterioration followed by periods of stability. Therefore, A's communication, language and swallowing difficulties will need to be regularly reviewed to ensure A can make his needs known, particularly as he currently lives alone.
Summary of Observations and Assessments
Behavioural Observations
A exhibited borderline fluent speech with paraphasias, perseveration, word repetitions, and nonspecific speech. In conversation, he was pragmatically appropriate, but had difficulty understanding questions and frequently asked for repetitions. When experiencing comprehension difficulties, he often laughed. His brother reported that he fatigues easily, although he demonstrated endurance throughout each appointment without showing signs of fatigue.
The results of a variety of formal and informal measures are summarised below.
Assessment of language and Comprehension
In response to referral information, the MTDDA (Schuell, 1965) was conducted to assess A's current cognitive and language skills using a range of subtests (See Appendix A). From the data collected during this assessment strengths and considerations for future objectives were created. A performed well throughout the assessment. However, D reported that A experiences word finding difficulties regularly, and often uses the environment and circumlocution as a communication strategy. Word finding difficulties were observed throughout the naming section of the MTDDA (Schuell, 1965) A could not remember the name of the scorpion, bow, fox or accordion from a picture despite having a visual cue. He attempted to describe the scorpion and butterfly and identified semantic information. For example, A described 'something that bites in hot country' for the scorpion. When naming more items, he named items that are semantically linked such as fox as 'cat', and accordion as 'harmonica'. Further to this, when asked to point to the items, where visual stimulation was given, he achieved a score of 8 out of 8.
During the immediate recall memory section, a story was read with no visual support, no parts of the story read were recalled. A reported that it 'felt like a long story'. During the Speech and Language Disturbances subtests, A performed well, but when asked about A's current difficulties, he turned to his brother saying 'he can answer' indicating his reliance on his brother to explain difficulties he is experiencing.
Mealtime Observation
A expressed concern about his swallow during initial SLT contact noting that he had been experiencing difficulties recently. Therefore, a mealtime observation at home was arranged to observe A in a naturalistic environment. A reported that he felt that recently he was experiencing increased amounts of coughing. However, he was not sure what was making him cough, and could not specify whether there are any specific foods he struggles to eat. During a mealtime observation at A's home, A was observed to make and eat poached egg on toast independently, with a drink of coke. He initially took the ingredients from the fridge and placed them on the kitchen surface. When attempting to place a jar on the kitchen surface, he misjudged the distance between himself and the kitchen surface. Thus, the jar splintered across the floor. When putting the eggs in the microwave, A initially set the microwave for thirty-eight minutes. At thirty-six minutes, A realised his mistake and switched the microwave off and on again. Then proceeded to put the eggs in the microwave for a further thirty seconds. These observations provided evidence that A may need support at home to maintain his independence and effect aspects of daily living.
Assessment of functional communication and problem solving
Following the home visit, it was decided to conduct the CADL-2 (Holland, 1999) to assess his communication and problem-solving skills in challenging situations. During a problem-solving question, in which A was asked for his response to the medical jargon of a diagnosis. A used humour to compensate for his lack of understanding of the medical jargon stating 'get lost'. His responses indicated an understanding of the question. However, A was not able to use interrogative language to gain an understanding of difficulties from the professional. Further, A had difficulty when problem-solving with a picture of a bus timetable in the CADL-2 (Holland, 1999). He recognised which route the bus should take, but could not recognise what time the bus should leave.
A was able to express his basic needs appropriately during the CADL-2 (Holland, 1999) but appeared to experience difficulty when asked what he would do if experienced a personal challenge. An experienced difficulty during the sections where he needed to let someone know that something is challenging such as being cold. He stated during the CADL-2 (Holland, 1999) that his sons often are not able to answer the phones due to work commitments and guidelines.
Data Analysis
The assessments conducted have provided evidence that A's memory skills and ability to use expressive language to communicate with functional communication are areas of concern which may affect aspects of everyday life. A is currently able to make his basic needs known, communicate socially and understand instructions. However, A would benefit from adaptations to instructions and information to incorporating visual cues around his flat to ensure his safety. With these assessment results, there may be evidence to change current levels of support provided. According to definitions which define the stages of progression, the data gathered from assessments and observations would suggest that A is currently at the mid-stage of the disease (Hamilton et al, 2012). Evidence for this conclusion is due data indicating an indication that his executive function is declining and some cognitive deficits are apparent. A does experience some involuntary movements but is still be able to care for himself.
A's concern was predominantly regarding his swallowing ability. Nevertheless, no obvious swallowing difficulties were noted during the mealtime observation. However, observations made throughout the home visit highlighted concern about A's safety at home. Observations noted throughout the mealtime observation indicated that A experiences periods of time which contain challenging situations, including dropping a glass jar on the floor and setting the microwave for a long period of time. Concerns about A's safety at home include his awareness of his environment, co-ordination of movement and balance may create further challenges as his condition progresses. Alongside the formal assessment, the observations provided information about how A may express concern and state difficulties and can in turn help to inform his management and objectives. A will need to establish a way to express himself clearly in difficult and challenging situations.
Objectives
There are currently no medications which can slow or stop the progression of HD (NHS, 2018). Thus, symptom management is crucial for management for A. To answer the referral, joint working alongside OT another member of the MDT is imperative to ensure that information is provided to A in an appropriate method. For example, using visual reminders of how to use appliances. A is additionally having difficulty expressing difficulty that he is experiencing. Therefore, alongside OT, SLT can provide a communication system such as Telecare (NHS, 2018) and strategies to make A's home safer, improve communication, and suggest ideas to offset cognitive decline.
Aims
A will attend two joint sessions with speech and language therapy and occupational therapy with objectives including developing strategies for activities of daily living and ability to utilise a communication device.
A will receive a communication system such as Telecare to be implemented as soon as possible within A's flat.
A will use the communication system to alert a family member or healthcare professionals when in need of assistance to maintain his independence at home until the late stage of the disease.
A will receive re-assessment of his language and cognitive skills in 6 months' time to provide evidence for management of his difficulties and rate of progression of HD. Appropriate intervention to follow as appropriate.
Management
Intervention alongside OT will consist of a case discussion with OT before home visit discussing observations made by both professionals and formulating a plan with strategies to help A at home (See Session Plan in Appendix B).
Rationale
HD is a progressive degenerative condition, and it is important to start implementing intervention before A's condition progresses further. By seeking early intervention, A will be able to maintain his independence and have more control over HD and their well-being. Aubeeluck (2009) states a multidisciplinary team approach is needed to provide intervention for those with HD. It is widely agreed that the intervention focus should be on safety, function and quality of life but must be proactive taking into account symptom change with realistic objectives (Nance, 2007). Macleod et al (2017) suggest that all health care providers involved should adopt a palliative approach due to the current clinical experience of the disease.
Critical Evaluation
There is currently limited information about speech and language therapy intervention in HD with a lack of evidence to support decisions about best practice in the management of those with HD (Mestre et al, 2009). Although a palliative approach is suggested by recent research, healthcare professionals must maintain a commitment to patient care preferences and quality of life (Tarolli et al, 2017). It is revealed in research, that patients can be missed by allied health professionals as they are unsure how to provide effective intervention without a strong theoretical framework to guide the selection of treatments (Bliney et al, 2003).
Despite lack of evidence it is important to address the changing clinical picture of the affected individual and the value of involving the family and caregivers in developing and facilitating effective communication strategies contributing to the intervention which changes their activity with daily living. To measure the outcome of A's therapy TOMs was used (Enderby and John, 2015); as recommended by RCSLT, within the Trust using the Neurological Disorders scale. A scored:
" Impairment: 3 – A's impairment will not improve with therapy intervention.
" Activity: 4 – With the aims and objectives of therapy which are stipulated in this report, A will maintain this score for a period of time.
" Participation: 4.5 – A's current level of participation is mostly independent. However, difficulties caused by his symptoms and ability to ask for help when experiences difficulties are beginning to restrict his participation.
" Well-being: 4.5 – A's well-being score is currently good with the occasional emotional support needed. The objectives set for should help to increase his independence to be stable and cope emotionally with most situations.
Prognosis
The majority of patients will typically pass away within 10-30 years of receiving a diagnosis of HD (Dorsey et al , 2013). However, maintaining autonomy and independence during the late stages of the disease is important. Augmentative and alternative communication devices should be considered alongside collaborative work with the multidisciplinary team maximising the opportunities for interaction and optimising communication skills. Management plans for A should encourage natural speech as long as possible with supported communication techniques playing a key role.
Future Management
Proactive care planning should be considered throughout the mid to late stages amongst the MDT, including liaising with A's family to arrange support and care provision. Proactive planning can help to avoid a breakdown of the home situation and the need for emergency hospitalisation. The progression of HD is sporadic, and predictions about care cannot be predicted. However, it is expected that chronic nursing care will be needed.