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Juvenile Huntington’s Disease Forum concerning all aspects of JHD

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Old 04-12-14, 04:58 PM   #1
Allan
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Default JHD - Services Improvement Study

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Hi everyone

I have been invited to become a lay member of a JHD study/project taking place at Sheffield Children’s NHS Foundation Trust. [see page 18 of the current HDA Newsletter]

The study, led by Dr Oliver Quarrell, will be assessing services for Juvenile Huntington’s Disease.

The study aims to obtain the views of patients, carers and professionals delivering services on the current service provision “when a young person develops JHD”. Working with researchers in the NHS the study will seek to discover what services are being used. What do young people need? What do they value? Are there better ways of delivering services and providing care?

The non-professional, family member’s representations to the Advisory Board will ensure that the project is being steered in an appropriate direction. It is a rare opportunity to place the role of the family Carer in the forefront of “improving services”, initially for young jhd-people.

As soon as I receive information from Dr Quarrell’s team I will be able to gauge who I can represent and what I can do. I hope those of you who have been through this situation will contact me via Private Messaging and give me as much information and detail as you can as to the positives and negatives in this area – and how “the services” can be improved from a family member perspective.

********************************************** ********************************

Juvenile Huntington’s Disease is a rare form of HD and is defined as onset under 20 years of age and represents approximately 1-10% of cases. The younger the age at onset usually gives a higher number of CAG Repeats, ie above 60.

In JHD, particularly in those with onset in the first 10 years, movement disorder [chorea] is less likely to be present and instead, patients are more likely to display rigidity, bradykinesia, dystonia and gait disorder, ie. parkinsonian features. In addition, JHD patients are more likely to develop epilepsy than adult-onset cases. The spectrum of features at presentation can also include ataxia, dysarthria, dysphagia, deterioration in school performance or severe behavioural problems.

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Old 04-12-14, 06:11 PM   #2
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Default Re: Services for Juvenile Huntington’s Disease

Hi Allan

Just wanted to congratulate you - given all the invaluable advice and info that you provide us all with I would say you are the perfect man for the job. They won't know what's hit 'em!

Hope that your new role will be beneficial, enjoyable and successful.

Cheers!
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Old 16-05-15, 12:24 AM   #3
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Default Re: Services for Juvenile Huntington’s Disease

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Hi everyone

A Project to Improve the Services for Juvenile Huntington’s Disease

The aim of this funded project is to identify patients/families with JHD, identify which services they are receiving, and ask both them and the professionals currently involved in the care their opinion of alternative models of care; specifically, concentrating expertise in a traditional MDT to which patients/families travel or alternatively establishing a virtual Multi-Disciplinary Team using Tele-health, or a secure Skype system via the internet.

So, how can we provide a system of care that will cater to all their needs, their medical, educational, physical, social and recreational healthcare? How can we move forward in our quest for multitasking MDT’s that will help to improve the whole Care Provision of our JHD-individuals and their families?

What the project is trying to do

JHD is defined as onset up to the age of 20 years. At best, treatment is symptomatic and supportive; there are no evidence based guidelines for management but, recommendations suggest that patients/families should be managed within multidisciplinary teams [MDT].

The first task is to make contact with the JHD population in the whole of the UK. According to the definition above it is thought that there might be 300 young people in the UK, with ages ranging from 0 - 25, with this disease. Finding them for the project will be a difficult task. If you are able to assist this search in any way the Project team would be very grateful. See contact details below.

What this might mean for families affected by JHD.

The idea is to convert, enable and enhance what might currently seem to be the somewhat dys-functional treatment of JHD patients into a “wholesome” approach. This would be seen as providing a meaningful method of making sure the physical, cognitive and pharmaceutical therapies work better. More importantly, it would bring many people to the “virtual” table to discuss each JHD-patient.

We would envisage a fully functioning MDT team: a Neurologist; a Psychiatrist; a Psychologist; a Neuropsychologist; a Genetic Counsellor; a Physiotherapist; an Occupational Therapist; a Speech & Language Therapist; a Dietician; a Carer or Family Member; a Primary Care Doctor; a Dentist; an Optician; a Social Worker; and the most important person of all – the JHD-person. As it would be an almost impossible task to gather all these people en bloc at any one time – it is hoped, with the aid of communications technology, that we can bring a majority to the table, if only virtually.

Education, Health and Care Plans should also be created for young people with JHD. This would be an holistic way of bringing Health and Social Care into operation - to last until the age of 25 [or until the young person has finished education or training or they transit into Adult Services]. In one way, it is easier to access funding - ie for Social Care, respite, short breaks or support workers and it gives the child or young person the right to a personal budget and, if required, direct payments. This would have a positive impact on the experiences of both the young people and their parents/carers.

If you are interested in taking part, as an individual or as a family, please contact me by Private Message.

.
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Old 05-06-15, 10:55 AM   #4
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Default An Easy but Difficult Read .......

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"Folksinger Woody Guthrie put a famous face on HD 45 years ago, but the community today needs an Angelina Jolie."

This article was written two years ago:

Juvenile Huntington’s Disease: The Cruel Mutation

.... and this is Ricki Lewis's contribution to this year's HD Awareness: Can we cure Huntington's disease?

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Old 05-06-15, 10:47 PM   #5
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Default Re: Services for Juvenile Huntington’s Disease

Quote:
Originally Posted by jacqueline View Post
Will you be involved with the HDYO too Allan?

Hi J

The HDYO is a whole world organisation - so dealing with local UK stuff online might not work. I’ve had a look at their message forum over the past year and it’s not current and rarely used by UK JHDers.

Helen Santini is the HDA’s JHD Care Adviser and she is a member of the Project too – so all angles covered.

However, I’ve just invited a young Australian lad to re-post his important message on here as it is very unlikely to get any replies on the HDYO forum.

Good moves in mysterious ways ……

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Old 05-06-15, 11:09 PM   #6
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Default Re: An Easy but Difficult Read .......

Quote:
Originally Posted by Allan View Post
.

"Folksinger Woody Guthrie put a famous face on HD 45 years ago, but the community today needs an Angelina Jolie."

This article was written two years ago:

Juvenile Huntington’s Disease: The Cruel Mutation

.... and this is Ricki Lewis's contribution to this year's HD Awareness: Can we cure Huntington's disease?

.
What a terrible card to be dealt. Jane Mervar must be one heck of a woman to have survived what she has gone through. Interesting stuff about the amount of money being pumped into research
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Old 07-07-15, 12:10 PM   #7
Allan
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Default Re: Services for Juvenile Huntington’s Disease

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Thanks for that M.

So, I’ve dug a little deeper and discovered that the full NeuroResponse service is currently taking place in the hospital that S attends – but only for Multiple Sclerosis patients.

The service has proved popular with patients, community health professionals, GPs and hospital specialists at UCLH. The NeuroResponse service includes:

NeuroDirect telephone link: a phone service that provides multiple sclerosis patients with a direct link to a specialist nurse at UCLH who can provide expert assessment and advice. Patient satisfaction increased from 49% to 93% after it was launched, with 76% of people with Multiple Sclerosis reporting an improvement in the quality of their life, increased confidence and reduced anxiety. Around 2,500 people are currently enrolled in the service. The MS nursing team use the service for around a 100 patients a week.

This service is staffed by MS clinical nurse specialists and a call handler who are able to offer expert assessment, triage and advice to people living with MS. The nurse can offer self-management advice when needed, triage into appropriate clinics, and liaise with your GP and local multidisciplinary team [MDT] in order to help you in a prompt and effective manner.

When you call for the first time, we create a personalised care plan for you which helps to improve communication between team members, GPs and anyone else who can improve your care. The call handler can assist with administrative calls, such as sending out travel letters, appointment queries and obtaining results.

NeuroView video link clinic: a video-conference clinic which enables our hospital specialists to talk to and undertake routine follow-up reviews of patients in their local health centre, without the inconvenience and cost of patients having to travel to hospital. It has also reduced waiting times for appointments. The NeuroView clinic has now extended to patients in Essex, the Isle of Wight, Jersey and Guernsey. There are plans to link to Gibraltar later this year.

We wanted to offer a more patient-centred and responsive service. We asked patients what they wanted and they told us how we could help them access the right care and advice at the right time, when they needed it. This video clinic service allows patients with spasticity issues who are unable to visit us in person to link in with our specialist teams. Patients go to their local health centre and link via secure videoconference to see a consultant neurologist, clinical nurse specialist, and a specialist physiotherapist. They will then discuss concerns and agree a care pathway alongside their local team in order to improve care as quickly as possible.

My aim is for an HD audio-visual clinic service to go beyond this “halfway house” and for the service to be available from the patient’s home via Skype, Facetime or similar communication. The NHNN already have an HD Telephone Appointment Service in place.

It’s almost 50 years since we had audio-visual communication with guys on the Moon, so what are we waiting for? The next step is one small step for mankind; one giant leap for hd-People.

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Old 24-09-15, 02:41 PM   #8
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Default Services for Juvenile Huntington’s Disease - Update

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Hi everyone

Here’s the latest from Sheffield on the JHD Research Project: www.sheffieldchildrens.nhs.uk/our-services/huntingtons-disease/take-part-in-research.htm

My interests are in the following 3 items:

1. What do young people need?
2. What do they value?
3. Are there better ways of delivering services and providing care?

My view is that young people need less time at hospital or the GP’s Surgery – so I would strive for Domiciliary appointments in the home where the young person is more likely to be at ease with professionals. There are, of course, times when it is necessary to make the journey as well.

It is very obvious that most of the younger generations value the impact\influence of technology and this is probably one of the reasons why the Scottish HA are to be involved.

One service that we are looking into is Tele-Health\Care and the possibility of appointments by Skype, Facetime or NHS video systems for youngsters who are unable to travel or attend appointments at hospital for whatever reason.

Another aspect of JHD that needs to be reviewed is the young person's education - as it is known that there can be negative impacts on learning potential by the strong behavioural issues that often occur in this junior version of hd.

If anyone can give me any leads, pointers, ideas, practicalities, etc. that I can put to the team at the next meeting I would be most grateful.

Next meeting: deep into the darkest depths of a Northern Bleak Midwinter

www.facebook.com/Huntingtons-Disease-Association-180065095355823/timeline

Thank you - one and all.

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Old 13-02-17, 09:58 PM   #9
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Default Re: Services for Juvenile Huntington’s Disease - Update

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Hi everyone

There’s no current news that I’m aware of from the Services for Juvenile Huntington’s Disease Project.

Neither does there seem to be much current stuff written on the research into JHD or on the Health & Social Care of youngsters with JHD. In 2008, Aimee Aubeeluck & Helen Brewer described Juvenile Huntington’s disease as “a rare condition, with only about 5-10% of Huntington’s disease cases occurring in individuals under the age of 20 years.”

This figure suggests that, in the UK at any one time, there might be less than 300 - 700 children with the disease. The upper age of 20 is arbitrary and 18 has also been labelled as the upper age cut-off. So the number count will vary.

The most common presenting symptoms of JHD in the first decade of life are declining cognitive function, behavioural disturbance, ADHD-type problems at school, rigidity and stiffness, awkwardness in walking, speech difficulty, oropharyngeal dysfunction and seizures.

The problem here, in my opinion, is that the medical, clinical and scientific professions don’t deal or cope with the daily issues of the observational, emotional and not-so-wellbeing that parents of children, who may have the symptoms of JHD, have to contend with.

Here’s a short JHD reading list …

The Juvenile HD Handbook: A Guide for Families and Caregivers [HDSA]

Juvenile Huntington’s Disease [HDSA]

Juvenile Huntington Disease: A Resource for Families, Health Professionals and Caregivers [HSC]

I’m not sure whether this helps or not: https://jhdkids.com

The personal experience of parenting a child with Juvenile Huntington’s Disease: perceptions across Europe [2013]

The Impact of Juvenile Huntington’s Disease on the Family: The Case of a Rare Childhood Condition

Managing juvenile Huntington’s disease [2013]

Caring for a child with Juvenile Huntington’s Disease: helpful and unhelpful support [2007]

Juvenile Huntington’s disease: a population-based study using the General Practice Research Database

The Prevalence of Juvenile Huntington’s Disease: A Review of the Literature and Meta-Analysis

A Case of Juvenile Huntington Disease in a 6-Year-Old Boy

Current Pharmacological Management in Juvenile Huntington’s Disease [2012]

Abnormal Weight and Body Mass Index in Children with Juvenile Huntington’s Disease [2015]

Juvenile Huntington disease in Argentina [2015]

Juvenile Huntington’s disease: a case report and literature review [2010]

Clinical Characteristics of Childhood-Onset (Juvenile) Huntington Disease: Report of 12 Patients and Review of the Literature [2006]

CLINICAL PRESENTATION OF JUVENILE HUNTINGTON DISEASE [2006]

This last document seems to be the most recent:

Juvenile Huntington Disease: Rare But With Psychiatric Implications [2016]

That's all folks!

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Old 16-02-17, 12:41 AM   #10
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Default Re: Services for Juvenile Huntington’s Disease - Update

That's all folks!

thanks Alan that's plenty! Verymuch appreciated. X:🌼
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