The Role of the HD Nurse Specialist Catherine Clenaghan HD Nurse Specialist South Wales

Overview Need for a HD Nurse Specialist South Wales Huntington’s Disease Service Role of Nurse in managing Patients’ Needs Benefits of a HD Nurse Specialist Future Plans

Identified Need for HD Nurse Specialist In a co-ordinating role across South Wales to act as: a point of contact for patients, carers and professionals provide on-going support for families clinically assess patient and family needs refer on to local services Identify and maintain links with key professionals involved in care Evaluate and ensure that changes in management plans are implemented promote understanding of HD and maximise quality of care to families

Number of known affected patients in each LHB in South Wales 15 +2 17 1+1 15+8 24 +6 30 54 23 38 +15 IN NURSING HOMES 17 +6 22 Cardiff

South Wales Management Service 148 known clinically affected patients in South Wales Age range 23-86 years old 37 in Nursing Homes 45 living with a family member without care input 16 living alone 50 receiving some form of care provision 23 under the care of psychiatry Over last 2 years: 13 deaths 16 new clinical diagnosis

What we know about HD Approximately 20 year time course from symptom onset till death, usually results from complications of falls, dysphagia, or aspiration Over the course of the disease, the person with HD and their carers will require a range of generic and specialist professional services  Families require flexible, co-ordinated practical and psychological support depending on the stage of condition and the patient’s functional status

Complex Nature of Disease A Typical Patient with Huntington’s disease can be under the care of:- Neurologist HD Nurse GP Social worker Occupational Therapist Dietician Physiotherapist Speech and language Therapist Community mental health Nurse District Nurse Continence Nurse Consultant Psychiatrist Chronic Disease Nurse Palliative care Nurse Issues: Burden on carer Poor provision of domiciliary services Non attendance at appointments Need for Coordinated service

Huntington’s Disease Association Current Support for HD Families in South Wales South Wales HD Specialist service ABHD Community Team Huntington’s Disease Association Nurse Specialist Speech Therapist Physiotherapist Psychologist Occupational Therapist

South Wales Specialised HD Service Co-ordinating with Psychiatry Acute / Community CPN Community Rehab Teams South Wales Specialised HD Service Co-ordinating with ABHD Team HDA Occupational Therapy Neurology Dietician Dentistry Speech Therapy Genetics Physiotherapy Palliative Care ALAC Patient, Families and Carers GP District Nurses DWP Nursing Homes DVLA Social Services Housing

What are we Achieving? Specialist multidisciplinary Huntington’s Disease Service offering long term regular contact with families Close liaison between Consultant and Nurse specialist to ensure management plans are implemented and evaluated Families and professionals have direct access to nurse specialist Close professional liaison across South Wales – Map of Medicine

A Triad of problems with Huntington’s Disease These problems interact with each other progressive loss of balance, slowness, clumsiness and involuntary movements Physical problems Mood and behaviour Intellectual Devastating effect on family Carer burden include poor attention, lack of insight, difficulty with multi-tasking and organisation and poor recall irritability, lack of motivation, depression, apathy and anxiety

Huntington’s Disease is a Family Disease The Needs of the Patient The Needs of the Carer The Needs of the Children Other Family members

Early Stages Assess the needs of patient Assess the needs of carer The role of the HD Nurse in Assess the needs of patient Assess the needs of carer Information Career Benefit advice Driving Social isolation Lifestyle Housing and Adaptations Planning for the future Mental health Information Advice Support Social isolation

Scenario 1 details actions 30 year old female, married, one child Presyptomatic gene test Both working, Over last 3 years - loss of confidence, withdrawn, lack of motivation Easily agitated, frequent rows Aware of increase in movements Significant financial pressures Monitoring of symptoms and offer support Support and information to partner-increase his awareness and understanding Other investigations may be required Advise and support about benefits and employment Access to appointments for support about adjusting to diagnosis Support for other family members

Middle Stages Assess the needs of patient and carer The Role of the HD Nurse in Middle Stages Assess the needs of patient and carer Management of symptoms Lack of insight /awareness of needs Personal Hygiene Involuntary movements Sleep Weight Loss Communication Swallow Behaviour Cognition Perseveration Urinary frequency Incontinence Mobility and falls Mental health Other medical conditions Medication review Adaptations and equipment Carers assessment Need for personal care Need for psychosocial support Need for respite Carer Practical support Coping strategies Planning for the future Power of Attorney Advanced directives

Scenario 2 details actions 23 year old Referral to social services for direct payments and housing adaptations DLA Referred to HDA Advice and information Referral to SALT Referral to Dietician Deterioration noted GP admission to hospital-tonsillitis Liaise with GP Referral to physiotherapy District nurse visit weekly Discussions around future care 23 year old No contact with biological father Lives with parents both working full time Sleeps upstairs Pensioned on ill health Socially isolated Swallow deteriorating Speech poor Frequent falls Weight loss Fiercely independent Internet shopping!

Advanced Stages Planning for future Social and nursing needs Swallow The Role of the HD Nurse in Advanced Stages Planning for future Social and nursing needs Safety at home Management of symptoms Pain Management Recurrent Infections Weight loss Swallow PEG Medication review Communication Respite care Provision of equipment Palliative support End of life decisions Support for carers

Scenario 3 actions details 62 year old Lack of awareness/denial Non- engaging for years Refusing all attempts to offer care Communication difficulties Weight loss Swallow deteriorating Agitated and aggressive Treated for UTI Sudden deterioration due to aspiration pneumonia Carer distress Practical and psychological support Referral to District Nurses Referred to palliative team social worker No available respite Overnight care Referred to psychiatry Admitted to hospital Family support Education for ward staff Best interest meeting End of life pathway

Support for HD patients in Nursing Homes Education of staff – ongoing Regular review of patients by nurse specialist and liaison with consultant re medication Advise and support on management issues SALT and Dietician Physiotherapy and OT Palliative care support End of life decisions Best interest meeting Liaison with GP

Benefits of Nurse Specialist Coordinating services for patients Ongoing family support Crisis dealt with in a effective timely manner Domiciliary visit - especially important to patients unable to attend clinic Regular assessment of management plan and the physical and psychosocial needs of families Timely referrals to other services as required Trialling telemedicine via Video - effective cost benefit Attendance at MDTs and CHC meetings-

How do we measure the value of a Nurse Specialist? Reduction in Hospital admissions- difficult to assess Reduction in admission to nursing homes- is this an indicator? Numbers of new referrals to service Reduction in carers’ issues presented in clinic Through education- greater awareness of needs of HD families

10 Top Tips to be aware of when caring for a patient with HD Lack of insight Decision making is impaired It’s easier to say ‘NO’ Can only do 1 thing at time Like structure and familiarity Due to lack of motivation require prompting Sequencing, planning and organisation are difficult Recognition is easier than recall Allow time to process information and time to respond Sudden deterioration exclude other causes

Plans for Future To ensure equity of service to all families living with HD in South Wales Develop telemedicine Patient Satisfaction survey Service Evaluation and development Explore use of patient held records to ensure a more managed clinical service

Referrals to Prof Anne Rosser Huntington’s Disease Management Team University Hospital of Wales Cardiff CF14 4XW TEL 02920 745237

Finally ... I would like to thank our families for being able to share their knowledge and experience of living with Huntington’s disease with us.