Raynauds Scleroderma Global Patients: March 2018

Scleroderma. March, Autoimmune Disease Awareness Month.

Scleroderma, Raynaud's, Autoimmune Rare Disease.

www.justgiving.com/fundraising/sclerodermaunit-royalfree

#RaynaudsFreeWorld #SclerodermaFreeWorld

blog.raynaudsscleroderma.co.uk

An autoimmune disease is one where the body’s natural defence system, the immune system, malfunctions / ‘trips out’, resulting in the body attacking itself.

The American Autoimmune Related Diseases Association Inc, AARDA, list there to be over 100 different, currently identified, autoimmune diseases.

With the rare disease Scleroderma, the body’s autoimmune system becomes activated causing fibrosis / thickening to the skin, blood vessels, muscles, connective tissue, bones and internal organs. In essence, the ENTIRE body is attacked.

Currently, this activation process, ‘on switch’, is still not fully understood, scientifically. And, sadly, neither is the ‘off switch’. Current treatments target symptom suppression, as opposed to disease eradication.

Investment in medical research is crucial for progress in scientific understanding.

Common presenting symptoms for most of the autoimmune diseases include, tiredness by way of extreme exhaustion, as well as, swelling and inflammation of the body of the joints, hair loss.

And of course, PAIN. Making for the daily mantra ‘Ah great, another day to spend in bed’.

These presenting symptoms can often lead to a slow diagnosis. Which, can lead to further complications, which maybe, potentially, life threatening.

Another commonality which presides amongst the autoimmune disease community is the ‘Invisible Disability’ label.

For, most autoimmune patients, and scleroderma patients alike, hearing ‘But you look so well’ is a welcome compliment, however, it can be extremely frustrating, when the actual reality, is that, you feel quite the opposite.

Hottest day of 2013, Im wearing Uggs and gloves

Some patients have told me of their frustration of not being believed as to the severity of their ‘invisible symptom’, not just by their families, but also by the medical professionals, with whom they have seen.

A common symptom seen within the autoimmune disease community, is the presence of Raynaud’s phenomenon.

However, not all Raynaud’s patients will have an autoimmune condition, this is when it is known as primary Raynaud’s.

When Raynaud’s presents with another condition, eg. an autoimmune disease, it is known as secondary Raynaud’s.

Throughout the course of March, for Autoimmune Disease Awareness Month, I have been reposting the global patient profiles from my scleroderma awareness 2017 campaign.

ALL of the patients, who kindly took part, reported experiencing Raynaud’s, along with, digital ulcers, being a foregone conclusion.

All of the patients reported exhaustion, swelling and pain to be current ongoing unmet clinical needs.

The British Society for Rheumatology, BSR, has a clear strategy to try and improve the current treatment for such patients:

‘Rare Rheumatic Musculoskeletal Diseases (RMDs) can be categorised into two distinct groups:

Rare autoimmune rheumatic diseases, comprising:

Systemic Vasculitis

(eg. ANCA-associated Vasculitis, Giant Cell Arteritis, Takayasu’s Arteritis, Behçet’s Syndrome)

Autoimmune Connective Tissues Diseases

(eg. Systemic Lupus Erythematosus, Systemic Sclerosis (Scleroderma), Myositis, Sjogren’s Syndrome)

Rare non-autoimmune rheumatic diseases, comprising:

Metabolic, sclerosing and dysplastic bone diseases

(eg. Osteogenesis Imperfecta, Fibrous Dysplasia, Hyperostosis Syndromes, SAPHO syndrome)

Dysplastic diseases of joints and inherited disorders of connective tissue

(eg. Epiphyseal Dysplasias, Ehlers Danlos Syndrome, Marfan Syndrome,

Stickler’s Syndrome). ’

The BSR recommends that:

‘This group of patients can be best served, along with the implementation of the UK Rare Disease Strategy by being considered under a single ‘umbrella’.

This is because they share a number of common features, including:

- overlapping clinical and serological features

- similar disease mechanisms, many of which have origin in immune system abnormalities

- convergent treatment pathways; immunosuppression, B cell depletion or cytokine inhibition

- multisystem nature of these conditions, often requires simultaneous care across several specialties. Multidisciplinary ‘one-stop-shop’ clinics enabling greater cost efficiency and clinical effectiveness

- In common with other rare diseases, these conditions are also slow to benefit from therapeutic advances.'

I had the pleasure and honour to present the ‘Raynaud’s and Scleroderma patient’s reality’ at the BSR Conference in 2015.

BSR 2018 Conference, 1^st – 3^rd May, Liverpool, UK.

BSR published Scleroderma Management Guidelines, June 2016.

Huge thanks to the current president, Dr. Peter Lanyon, for his outstanding commitment to the rheumatological community as a whole.

Future key events for this year, 2018, include the Family Day at The Scleroderma Unit, 19^th May, which this Scleroderma Olympian is already in training for!

Clearly, there is a large need for improvement in the medical and clinical care of the autoimmune patient, and even more so for the rare disease, autoimmune patient, such as Scleroderma.

Living the dream, scleroderma style.

An edited version of this article was published here in my Column with Scleroderma News.
March 2018.

This video shows all of the global patients who kindly shared their Scleroderma, Raynaud’s, autoimmune Rare Disease experience for awareness purposes:

Scleroderma Family Day 2020

25^th 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

2017 Rare Disease Day unmet clinical needs to the scleroderma patient

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:

Early diagnosis

Expert specialist centers

Access to innovative medicines

supported by a medical ‘dream 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.

1^st 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