Thursday, 13 April 2017

UK Guidelines for the Treatment and Management of Scleroderma, Raynaud's, Autoimmune Rare Disease

UK Guidelines for the Treatment and Management
Scleroderma, Raynaud's, Autoimmune Rare Disease. 



#RaynaudsFreeWorld #SclerodermaFreeWorld  





In June 2016, Scleroderma Awareness Month, the first national UK guidelines for the treatment of scleroderma (systemic sclerosis) were published in the international journal ‘Rheumatology’, as well as, on the British Society for Rheumatology (BSR) website.
See more, here   

These guidelines were put together by the BSR and BHPR (British Health Professionals in Rheumatology): 

to develop an expert driven evidence based series of 
recommendations for the management of scleroderma.’   

Dr Peter Lanyon, Consultant Rheumatologist, has now completed his first year of the 3 year Presidency role of the BSR.  


Dr Lanyon set out his plans and vision for an improved musculoskeletal / rheumatological healthcare / patient experience, with an ‘all hands on deck’ approach. (Although, sclerodactyl hands may not be much use, if taking this in a literal sense). 
 
It is very encouraging to see that the rare disease, non genetic, rheumatological conditions (this includes scleroderma, vasculitis, myositis etc) have been outlined specifically, for areas of improvement, as by the UK Government’s commitment to such by 2020.  


To read more about this, Click here  

In my view, Dr Lanyon is another unsung hero and blessing to the global scleroderma community, the rare disease community and the musculoskeletal community. 

I have the pleasure of being a member of the NHS Clinical Reference Group (CRG) for Specialised Rheumatology, which Dr Lanyon chaired prior to taking on his BSR Presidency role.  

I witnessed at first hand, the selfless dedication and extra miles which Dr Lanyon demonstrated in order to improve the day to day reality and care for patients, as well as improving the standards of the NHS as a world class leading provider of healthcare.  

The next annual BSR conference is scheduled to take place in Liverpool, 1st - 3rd May 2018.

To watch my presentation ‘Systemic Sclerosis – A Patient’s Perspective’ which I gave at the 2015 BSR annual conference, Click here





Professor Chris Denton, Royal Free Hospital and UCL Division of Medicine, Chair of the BSR/BHPR Scleroderma Guideline development working group, another unsung hero and blessing to the global scleroderma community, provides the following helpful executive summary to the UK national scleroderma guidelines, and, an insight into the important background to their formulation: 

           These guidelines are important because they summarise the current best practice for treating the major complications of systemic sclerosis and also address the overall approach to disease management in the UK. 

They have been developed under the auspices of the SAGWG (Standards, Audit and Guidelines Working Group) of BSR that has developed a process that is accredited by NHS evidence.  

This is important since it means that the guideline is NICE accredited and should therefore be taken very seriously within the NHS as defining the standard of care for patients and access to therapies. 

Embedded within the guideline are important NHS England policies for the management of digital ulcers and the pathway developed for assessment and delivery of autologous stem cell transplantation for appropriate cases of diffuse systemic sclerosis.
The guideline process involved establishing a development group that included rheumatologist, scleroderma experts, pharmacists, allied healthcare professionals, specialist nurses, primary care representatives and patients. 

In this way all aspects of the disease and management could be included. A comprehensive literature review of all the evidence supporting treatments for scleroderma was an important starting point and a group of dedicated clinical fellows undertook this work. 

There was a series of telephone and face to face meetings over 2 years that led to the development of the draft guideline. This was then reviewed by BSR SAGWG and by external referees. 

Comments were incorporated and the revised guideline was then finalised and submitted for open consultation so that anyone could comment and have input. After this process the final guideline was written, this was submitted for approval of BSR and then for publication in Rheumatology [3].
This is not the end of the process since the guideline are reviewed and updated every 5 years according to NHS Evidence protocols. 

This is a landmark for UK scleroderma patients and an important one at a time of major NHS change and also challenged and competition for resources for rare diseases. 

It complements the other recommendations being updated such as this of EULAR (European League Against Rheumatism) and the UKSSG (UK Scleroderma Study Group) best practice consensus documents [4].'

The structure of the UK national guideline is divided into 3 sections:  

Part A: general approach to SSc management
This section highlights the importance of an early diagnosis with referral to a specialist scleroderma centre followed up with management within the framework of a multidisciplinary team.

Part B: key therapies and treatment of organ-based disease
This section highlights all of the major complications and potential organ involvement, as well as possible treatment options.
Topics include: Raynaud’s phenomenon, digital ulcers, lung fibrosis, pulmonary arterial hypertension, gut involvement, renal involvement, cardiac manifestations, skin manifestations, calcinosis, musculoskeletal manifestations, ASCT.  
I am particularly honoured to have been a member of the medical team who compiled the current NHS guidelines for digital ulcer management in scleroderma. 

Part C: service organization and delivery within NHS England
Scleroderma, SSc should be diagnosed promptly, investigated appropriately and managed within an integrated system of primary, secondary and tertiary level care. 
The guidelines are scheduled to be reviewed by the NHS in 2019 


References for more information:

BSR and BHPR guideline for the treatment of systemic sclerosis
  • Christopher P. Denton1, Michael Hughes2, Nataliya Gak1, Josephine Vila3, Maya H. Buch4, Kuntal Chakravarty1, Kim Fligelstone1, Luke L. Gompels5, Bridget Griffiths3, Ariane L. Herrick2, Jay Pang6, Louise Parker7, Anthony Redmond4, Jacob van Laar8, Louise Warburton9, Voon H. Ong1, on behalf of the BSR and BHPR Standards, Guidelines and Audit Working Group 

Author Affiliations
  • 1Centre for Rheumatology, Royal Free Hospital, London
  • 2Rheumatology Department, Salford Royal NHS Foundation Trust, The University of Manchester, Manchester Academic Health Science Centre, Manchester
  • 3Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne
  • 4Leeds Institute of Musculoskeletal and Rheumatic Medicine, Chapel Allerton Hospital, Leeds
  • 5Rheumatology Department, Musgrove Park Hospital, Taunton,
  • 6Pharmacy Department
  • 7Centre for Rheumatology, Royal Free Hospital, London, UK
  • 8Rheumatology and Immunology, UMC Utrecht, Utrecht, The Netherlands
  • 9Primary Care, Telford and Wrekin NHS Trust, Telford, Shropshire, UK

For the scleroderma patient, these guidelines are a quantum leap in progress for scleroderma best practice management and care. 

These guidelines can be used universally around the world, especially for those countries who, due to their size and economic climate, are not able to produce such recommendations for their scleroderma patients.

As with all things, change is a constant. I very much hope that by the time of the NHS scheduled review for the guidelines in 2019, more, and improved treatments have been made available, as well as, identification of the causative factors of scleroderma have been discovered.

It is with huge gratitude and thanks to the super-human Prof Chris Denton for championing the formulation of the best practice management guidelines for the treatment of scleroderma in the UK, along with the numerous expert medics who assisted with this. 


To read more:

the importance of an early diagnosis   



taking part in Clinical Research Trials   



Living the dream, scleroderma style, hoping for a cure….

#SclerodermaFreeWorld #RaynaudsFreeWorld  
 




An edited version of this article was published here, in my Column with Scleroderma News. July 2016. 



  

Scleroderma Family Day 2020

25th Anniversary Meeting

The Atrium, Royal Free Hospital, London, NW3 2QG

Chair: Professor Chris Denton

PROVISIONAL PROGRAMME


09.30 – 10.00           Registration and Coffee

10.00 – 10.20           Welcome                                                   Dame Carol Black
                                                                Prof Chris Denton & David Abraham

10.20 – 10.40           What is a Biopsy?                                    Dr Kristina Clark

10.40 – 11.05           Dental aspects of Scleroderma            Prof Stephen Porter

11.05 – 11.25           Gastrointestinal problems –                       Dr Fiza Ahmed
shedding new light on old problems 

11.25 – 11.50           Pulmonary hypertension and the heart Dr Gerry Coghlan

11.50 – 12.15           Scleroderma cohort studies –                  Dr Francesco 
                                 ‘Learning from our patients’                     del Galdo

12.15 – 14.15           LUNCH BREAK – see below  

14.15 – 14.45           25 years of progress –                          Prof Chris Denton
from ‘black box’ to ‘positive trials’

14.45 – 15.15           International speaker –                            Dr Madelon Vonk
                                 Scleroderma Management in Netherlands

15.30                        Raffle


Lunchtime Discussions Groups / Demonstrations include:

Clinical Trials                                                                  Rachel Ochiel and team
National Institute of Health Research                            Christine Menzies
Drug Information / monitoring                                        Pharmacy
Massage                                                                         Keith Hunt MBE
Pulmonary Hypertension Education / nursing            Sally Reddecliffe/Adele Dawson
Rheumatology Laboratory                                          Research Laboratory Staff
Scleroderma Education / Nursing                          Louise Parker/ Joseph Cainap
Thermography                                                                Dr Kevin Howell
SRUK                                                                             Ollie Scott
Sjogren’s syndrome                                                       British Sjogren’s Society 


To Read My Articles:  

Gift in My Will, Click here 

Planning for the Future, Click here  

 


Rare Disease Day:   

Rare Disease Day 2020:  

  

Listen to my interview with John Smeeton, (Silver Fox), from the Royal Free Radio, here  


 

Raise awareness and donate to medical research, order your #SclerodermaFreeWorld #RaynaudsFreeWorld tshirt  here   


 
Rare Disease Day 2020: NIHR 'Research gives hope to Rare Disease patients', Click here  

  


 
Rare Disease Day 2019: Leaving a Legacy Gift, Click here       



Rare Disease Day 2019: 11am 97.4RockFM headlines, NO CURE, Click here  

Rare Disease Day 2018 – Research, Taking Part in Clinical Trials. Scleroderma, Raynaud's, Autoimmune Rare Disease, Click here     

2017 Rare Disease Day Medical Research  


2016 Rare Disease Day Patient Voice   

2016 Rare Disease UK Parliamentary Reception       

  

Rare Disease Day is a fantastic opportunity for the entire rare disease community to shine a spotlight on their reality, combining as one unified voice. Where, at least one commonality presides –  
Medical Research
provides the brightest light,
for the illumination of 
the rare disease patients’ plight.


To read about medical research at the scleroderma unit, Click here   

Although rare disease patients are few in number, eg. 2.5 million scleroderma patients worldwide, (the World Scleroderma Foundation), the commonalities and golden hallmark for each rare disease patient are the same overall. 

For optimum patient care, 3 hallmarks preside:




supported by a medicaldream team’ 
(expert specialist as the clinical lead).

INVESTMENT in MEDICAL RESEARCH is CRUCIAL.   


To read my articles:  

Celebrating 20 years of being a patient at the Scleroderma Unit, Click here

2019 New Challenges, Click here
 
NIHR Video: 'My Experience of Clinical Trials', Click here 

If we only had more RESEARCH investment for Scleroderma, Raynaud's, Autoimmune Rare Disease, Click here   

The Importance of Medical Research and Awareness to the Scleroderma, Raynaud's, Autoimmune Rare Disease patient, Click here  

2018 Scleroderma Awareness Raising and Medical Research, Click here    


SCLERODERMA:

Importance of an early diagnosis, Click here   
    
Taking Part in Clinical Research Trials, Click here     

The Importance of a Multi Disciplinary Medical ‘Dream Team’, Click here    

Expert Specialist Centres, Click here      
  
My Skin is Cured from Scleroderma, Click here   
    
UK Guidelines for Managing and Treating Scleroderma, Click here        

Fatigue, Click here         

Mobility, Click here      

Diet and nutrition, Click here      


Raynaud's- How to Diagnose, Click here        

'All you need to know', 2016 Awareness Video, Click here    

Global Patient Profiles 2018 Video, Click here    


Unmet Medical Needs, Click here     




Calcinosis Video, Click here    


The scleroderma tooth fairy, Click here     


Skin Cancer and scleroderma, Click here   

 

Sept 2017

Prof Chris Denton and I, Sept 2017

June 2019



This year, 2020, I am celebrating 22 years of being a patient at the Scleroderma Unit, The Royal Free Hospital - a world leading expert specialist, research centre.  

Read more, here.    

I am eternally grateful to the global scleroderma trail blazers Dame Prof Black and Prof Chris Denton, whose commitment and dedication to unlocking the scleroderma enigma, is nothing other than, superhuman. Along with the Raynaud's world trail blazer, Dr Kevin Howell.  

I am truly humbled and inspired by their work ethic and commitment to their patients.  

I am wholly appreciative for Prof Denton’s continued medical expertise and support, especially during my barrister qualifying years, 1997 - 2004. 
 
1st March 2004, I qualified as a self employed practising barrister. Further to having been told in 1997, by my original diagnosing doctor, that I was looking at a 15month prognosis. 
 


  

Chat Magazine May 2019

I very much hope to utilise my professional skills and qualifications along with my patient experience, to help achieve the #SclerodermaFreeWorld dream, hoping to improve understanding and best practice, in the meantime. Read more, here     

  

World Scleroderma Day 2018, Click here  

World Scleroderma Day 2017, 29th June, Click here    

World Scleroderma Day 2016, 29th June. Origins of the date, Click here   

World Scleroderma Day 2015, 29th June.  


James Carver, myself, Prof Chris Denton
I had the pleasure of being a presenter and part of the European co-hort who gave a presentation at the European Parliament, Brussels, in honour of World Scleroderma Day 2015.  

I am immensely grateful to James Carver, former MEP, for organizing this event in memory of his late wife Carmen, who sadly passed from Scleroderma.  

To view the presentation, Click here 

Raynaud's

October:  
Raynaud's, Click here  
 
To view the UK treatment guidelines for Raynaud’s Phenomenon, Click here   

To view the EULAR treatment guidelines, Click here    
To view Thermograph Video, Click here    

To view Thermograph image, Click here    

   
My Raynaud’s reality, Click here     


The global Raynaud's trailblazer - Dr Howell and I, Sept 2017

To view Nailfold Capillaroscopy equipment (used to identify possible blood vessel damage), Click here   

Invisible Disability Awareness Video, Click here

To read My ‘Invisible Disability’ experience, Click here   

50 Shades of Blue, Click here



 

For latest updates follow / subscribe:  

@SclerodermaRF 

@RaynaudsRf 

Twitter, Instagram, Blogger, YouTube, Facebook Page:

#SclerodermaFreeWorld           #RaynaudsFreeWorld    #Research  



Please DONATE to help fund medical research at The Scleroderma Unit,The Royal Free Hospital, London.  


100% of your monies will be used for medical research purposes only. NO wages or admin costs. Thank You. 

  

  
Last Update: Feb 2020.      


UK Guidelines for the Treatment and Management
Scleroderma, Raynaud's, Autoimmune Rare Disease. 



#RaynaudsFreeWorld #SclerodermaFreeWorld  








   



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