Huntington’s Disease Care Pathway

Individual at Risk HDA GP Clinical Genetics Diagnosed Clinical Presentation Mental Health Symptoms Cognitive/Emotional Symptoms Motor Symptoms Clinical Assessment Clinical Assessment Clinical Assessment Management of Symptoms Management of Symptoms Management of Symptoms End of Life Resources & Services
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Individuals At Risk

Definition of ‘at risk’

  • Every child has a 50% risk of inheritance
  • If positive test, they will develop HD
  • Male and female prevalence is equal
  • Can not skip generations
x

Huntington’s Disease Association

  • Regional Care Advisory Service
  • Leaflets and publications
  • Internet
  • Message board
  • Peer support
  • Branches and support groups
  • Independent advice
  • Education
  • Employment advice
  • Insurance advice
  • Support in the caring role
  • Psychological support
  • Information on research
x

General Practitioners

  • Establish family pedigree
  • Advice and support
  • Information about the HDA
  • Leaflets and publications
  • Discuss predictive testing
  • Discuss options about having children
  • Advice on employment
  • Support in the caring role
  • Counselling and psychological services
  • ? referral to clinical genetics
x

Clinical Genetics

  • Establish family pedigree
  • Information about HDA
  • Leaflets and publications
  • Discuss options re having children, insurance and employment
  • Information about genetic testing
  • Implications of undergoing predictive test
  • Genetic counselling
  • Predictive testing
  • Follow up support whether positive or negative
  • Psychological support
  • Information on research
  • Family support
x

Diagnosis of Huntington’s disease (HD)

Definition of HD

  • Neuropsychiatric disorder
  • Hereditary disease caused by faulty gene on chromosome 4
  • Symptoms usually begin between age 30-50 (although late onset can occur)
  • Prognosis 15-20 years
  • There is a juvenile form
  • Incidence of 6-10,000 in the UK
  • Prevalence 12.3 per 100,000 in the UK
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Clinical presentation

  • Mental Health Symptoms
  • Cognitive/Emotional Symptoms
  • Motor Symptoms
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Mental Health Symptoms

  • Irritability
  • Aggression
  • Obsessive Compulsive Disorders
  • Anxiety and apathy
  • Depression
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Clinical assessment of mental health symptoms

  • GP
  • HDA
  • Community Mental Heath Team
  • Psychiatrist
  • Psychologist
  • Community Psychiatric Nurse
  • Specialist HD service
  • Social Care
  • Employment
  • Vulnerable adults
  • Driving
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Management of Mental Health Symptoms

  • Medication
  • Training and education of carers
  • Structure, routine and boundaries
  • Mental Health Act (sectioning)
  • Support and care package
  • De escalation and distraction techniques
  • Environmental enrichment
  • Parenting skills
x

Cognitive and Emotional Symptoms

  • Difficulty in switching attention
  • Inability to concentrate on more than one thing at a time
  • Delayed processing skills
  • Loss of drive, initiative and spontaneity
  • Impaired recognition and recall
  • Impulsive behaviour
  • Rigidity of routine
  • Emotional blunting
  • Inability to read facial expression
  • Impaired executive functioning
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Clinical assessment of Cognitive and Emotional Symptoms

  • GP
  • HDA
  • Psychologist
  • Counsellor
  • Neuropsychologist
  • Psychiatrist
  • Specialist HD service
  • Social Care
  • Capacity
  • Advance Decision to Refuse Treatment (ADRT)
  • Lasting Power of Attorney (LPA)
  • Employment
  • Vulnerable adult
  • Driving
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Management of Cognitive and Emotional Symptoms

  • Medication
  • Cognitive Behavioural Therapy (CBT)
  • Support worker/care package
  • Aide memoires
  • Alarms
  • Complementary Therapies
  • De escalation techniques
  • Parenting skills
x

Motor Symptoms

  • Chorea
  • Other involuntary movements
  • Bradykinesia
  • Dystonia
  • Rigidity
  • Speech
  • Swallow
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Clinical Assessment of Motor Symptoms

  • GP
  • HDA
  • Neurologist
  • Speech and Language Therapist
  • Dietitian
  • Physiotherapist
  • Occupational Therapist
  • Wheelchair Services
  • Specialist HD Services
  • Social Care
  • Employment
  • Vulnerable adult
  • Driving
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Management of Motor Symptoms

  • Medication
  • Aids and adaptations
  • Specialist seating, beds, mattresses etc
  • Care package
  • Massage and complementary therapies
  • Parenting skills
  • Continuous assessment
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End of Life

  • Pain relief
  • Management of secretions
  • Pressure area care
  • Artificial feeding
  • Preferred place of care
  • Advance Directive to Refuse Treatment (ADRT)
  • Spiritual/faith
  • Dignified death
  • Brain and organ donation
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Resources and Services

  • Residential Care Homes
  • Specialist HD Care Homes
  • Day Centres
  • Care packages
  • Social Care
  • GP
  • HDA
  • Community Mental Health Team
  • Psychiatrist
  • Psychologist
  • Counselling
  • Spiritual care
  • Neuropsychologist
  • Independent Mental Capacity Act Assessor (IMCA)
  • Independent advocate
  • Specialist HD Services
  • Neurologist
  • Speech and Language Therapist
  • Dietitian
  • Physiotherapist
  • Occupational Therapist
  • Wheelchair Services
  • Aids and adaptations
  • Support worker
  • Palliative Care Team
  • MacMillan Nurses
  • Admiral Nurses
  • Hospices
  • Housing
  • Employment Advisers
  • Substance Misuse Teams
  • Early Onset Dementia Teams
  • Support worker
  • District Nurses
  • Personalised Budgets/Direct Payments
  • Blue badge
  • Carer support
  • Benefits
  • Citizens Advice Bureaux
  • Specialist HD Nurses
  • Research opportunities
  • Respite
  • DVLA
  • Continence Nurses
  • Pain Management Clinic
  • Alternative Therapies

Downloads for printing or reference

HD care pathway - click to download

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