By Terry HensonHD clinical lead / lead social worker / adult safeguarding manager for DHD and ABI & MAPPA / operational lead 

Our patients’ lives have been so negatively affected by their diagnosis of Huntington’s disease and as they have become more symptomatic, for our patients this has meant an increase in aggressive behaviours that they have not been able to control.

One of the most misunderstood concepts for people living with HD is that they need to have their need met immediately. This is a consequence of the condition that is often misconstrued in more general mental health units as ‘attention seeking behaviour’ which is viewed very negatively by staff. The disease is complex and required staff to be able to understand the three elements of movement, cognitive and behavioural symptoms.

Through the specialist milieu of the ward that we have created, our patient’s aggression has been significantly reduced and their quality of life significantly improved through the team’s ability to ensure that our patient’s do not have to wait.

There is no easy solution for working with people who just can’t wait so our staff need to be effective and organised and be able to anticipate as many needs as possible; hence, the concept of HD Ready is the corner stone of the treatment plan.

All our staff have received training in HD that has been accredited by the HDA and the compassion and understanding that has stemmed from this makes the ward the special place that it is.

By Helen Scott-Evans, ward manager

The philosophy of care on the ward developed by the HD team itself is entitled  “genuinely warm and kind to all”. Underpinning this philosophy we have high expectations of all our staff on how they communicate and how they practice with our patients living with HD. Staff offer choice without judgement, are inclusive and warm, use positive language, respond to requests as much as possible with “yes”. As a ward we do not create unnecessary rules, we promote a culture of person-centred care in a relaxed and open style.

Alongside our philosophy of care we use a model of care developed by our nurses called “HD ready” this model is simple and is applied throughout all aspects of their care and treatment. We have recognised that people with HD find it very difficult to wait for anything, if their needs are not met quickly this can lead to frustration, agitation and anger.

Our staff need therefore to be as organised and effective as possible so that we can meet the needs of our patients as soon as practical. We focus on being as organised as we can and plan ahead, we work effectively communicating together as a team, we make sure we know our patients very well and we work with them understanding their wishes and preferences so we can anticipate their needs.

To support our nursing staff to be “HD ready”, we have a number of processes in place including Care rounding. This is conducted by a qualified nurse assessing the patient in the following areas: pain, posture, personal care and wellbeing at prescribed times throughout a 24 hour period. We also have a buddy system that is run by the HCAs. This supports the patient in a number of ways from helping them tidy their bedroom, to engaging in activities on and off the ward, to advocating the patients’ needs in ward rounds and other meetings.

Social work
By Andy Broughton, social work practitioner,

Undoubtedly, it is vital to promote the quality of life of each individual, whatever that means from their perspective. HD is one of the cruellest and most heart breaking illnesses there is, so we strive to ensure our patients have a good quality of life. For some of our patients this means seeing more of their family, or going out shopping regularly; for others it’s to get out of hospital and into a place of their own with as much independence as possible. The role of the Social Worker within the HD service is complex and multifaceted, but my primary aim is to work with the individual patient and third parties to help achieve the outcomes the patient wants as much as safely possible.

This includes working with families to encourage and maintain contact, often in difficult circumstances; helping to arrange appropriate financial management to ensure our patients have a safe income which, in turn, can be used for enjoyable activities; continuously working with external professionals to help identify an appropriate place for our patients to move on to; and working within the MDT to promote more holistic and anti-oppressive care.

Some outcomes can’t be measured, but these are often the ones which matter most to the individual. Being able to help them achieve the outcome they want is the most important thing I can do and makes my role so rewarding.

By Rubilyn Esmerelda

As a professional, the most important considerations for a patient living with HD are:

–  To maintain mobility and function for as long and safe as possible. Decreased mobility is the number one predictor for patients with HD to require a nursing home so increasing/ maintaining frequency of getting up and walking is important. Maintaining mobility and physical function from

– Early on in the disease process, to maintain quality of life in HD

Our approach at St Andrew’s is to ensure:

  • That all patients engage in some form of physical activity each day. This may be targeted activity with the physiotherapist e.g. strengthening and aerobic exercises etc or generalised activity e.g. physical games or outdoors walk facilitated by ward staff. In the later stages of the disease physical activity and exercise would include assisted standing e.g. with a tilt table, passive exercise and positioning to manage tone, prevent contractures etc
  • Awareness and early detection/management (by all staff) of physical health issues that increases risk of falls or acute deterioration such as infection (urine, chest, foot), dehydration, constipation, poor nutrition, poor sleep etc
  • Access to equipment

Dietitian services
By Rebecca Forster

As a dietitian I would absolutely put mealtimes and nutrition up there as the most important consideration for a person living with HD. As HD progresses eating and mealtimes can become hugely challenging and we often see patients struggling with worsening dysphagia and loss of independence at mealtimes.

This can have an extremely negative impact on both food and fluid intake. Management of fluid can present a huge challenge and it’s not uncommon for patients to have periods of drinking excessively or restricting fluid intake, both of which can be very harmful to health. Reduced ability to wait for food and impaired sleep patterns mean that patients often want and need to eat at any time of the day or night, and so food has to be ready to offer at any time. In addition to all of these difficulties, patients nutritional requirements often increase with worsening chorea movements. All of these challenges can culminate in weight loss, undernutrition, constipation and dehydration.

At St Andrew’s, HD patients are all reviewed regularly by the dedicated ward dietitian. Dietetics work closely alongside Speech and language therapy and the dysphagia kitchen to ensure that all meals and snacks are a safe texture for each individual patient whilst at the same time meeting individual nutritional requirements.

High calorie nutrition support food and drinks are ordered via dietetics to ensure that there’s always a good stock of appropriate high calorie snacks, desserts, high calorie drinks and extra meals to offer at any time of the day. Food and fluid intake is monitored closely and any drop in intake is flagged up to dietetics by nursing staff.

Nutrition screening is completed every month so that weight and any change in mealtime behaviour can be quickly detected. Dietetics use tools such as individualised drinks charts and information on foods with a high fluid content in order to give guidance to staff on management of fluid intake.

Dietetics also prescribe a wide variety of nutritional supplements to help maintain weight and meet individual patient’s needs. These are just a few of the strategies used to try and minimise risk of weight loss and to safely manage fluid intake.

Clinical psychologist
Dr Inga Stewart

We see every person living with Huntington’s disease is a unique individual. We work together as a team to get to know all about our patients; what’s important to them, their preferences, interests and how their life experiences have shaped them as a person.

Cognitive and mood changes might be less obvious than the movement disorder, but for many the resulting behaviours that challenge can be the most distressing consequences of the disease. Our understanding of the person within the context of their neuropsychology guides our coproduced formulations.

These allow us to develop a shared understanding of the needs that are being communicated through behaviour. We can then work collaboratively to anticipate those needs, while supporting the person to identify and communicate their needs better as part of our blame-free culture.

We recognise and reinforce prosocial behaviour so that risk behaviours are gradually displaced. When working with our patients, each intervention is created with the person in mind, maintaining a ‘flexible structure’ in order to minimise the effects of the disease and maximise engagement and rehabilitation. The impact of our therapeutic approach is scrutinised through robust clinical outcomes, the results of which feed back into our assessment and treatment process.

For more on St Andrew’s Healthcare visit www.stah.org.