Saturday, 31 March 2018

Scleroderma. March, Autoimmune Disease Awareness Month. Raynaud's, Autoimmune Rare Disease


March, Autoimmune Disease Awareness Month 
Scleroderma, Raynaud's, Autoimmune Rare Disease 

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, hairloss

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, 1st – 3rd 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, include the Family Day  at The Scleroderma Unit, 19th 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.

However, for best practice and care:




Where MEDICAL RESEARCH investment is VITAL.


Living the dream, scleroderma style.  
 
Please DONATE where 100% of your donation will be used for medical research purposes only.  Thank You.  
#SclerodermaFreeWorld #RaynaudsFreeWorld

Follow Raynauds Scleroderma Awareness Global Patients Facebook Page for latest updates 
Twitter: @SclerodermaRF @RaynaudsRf   

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:
Autoimmune Disease Awareness Month 2018
Scleroderma Awareness Month 2017
#SclerodermaFreeWorld #RaynaudsFreeWorld


To read my articles: 


Rare Disease Day 2018, Research - Taking Part in Clinical Trials, Click here   

March Autoimmune Disease Awareness Month 2018, Click here   

Global patient video, Click here   

Becoming a Patient Research Ambassador for the NIHR, Click here  

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


Scleroderma Awareness Month 2017 Patient Profiles Campaign, Click here     
 
Scleroderma Awareness Month 2017 Patient Profiles Campaign Patient Index, Click here        
The Pandora’s Box of the rare autoimmune disease Scleroderma, Raynaud's and Cancer, Click here


Key Event Dates 2018, Click here     

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, Click here    



World Scleroderma Day 29th June 2016, Click here

World Scleroderma Day 29th June 2017, Click here   


Why Global Collaboration is important to the Rare Disease Patient, Click here      



RARE DISEASE DAY:
The theme to this years Rare Disease Day is Research

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

2016 Rare Disease Day Patient Voice
2016 Rare Disease UK Parliamentary Reception 

2017 Rare Disease Day Flashback  

More unmet clinical needs  

Rare Disease Day 2016



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. 

I highlighted other areas of medical research interest within Week 3 of my Patient Profiles Campaign for Scleroderma Awareness Month 2017. 

VIDEO



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: 

Early Diagnosis
Expert Specialist Centres
Access to Innovative Medicines

Where MEDICAL RESEARCH investment is VITAL.

Research is the key. Abstracts from 2016 World Congress, Click here


Scleroderma Awareness Month Campaign 2016, Click here

Links

View video, here   

Preamble - here



The Family Day at the Scleroderma Unit, The Royal Free Hospital is taking place on the 19th May 2018. Browse the program, here

This year, I am celebrating 20 years of being a patient at this world leading expert specialist research centre. 

Sept 2017


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.
I am truly humbled and inspired by their work ethic. 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 diagnosing doctor, that I was looking at a 15month prognosis. 

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.
  
For latest updates follow: 

Facebook Page:

Twitter: @SclerodermaRF  @RaynaudsRf  


#SclerodermaFreeWorld           #RaynaudsFreeWorld
#ADAM #Scleroderma
#Raynauds
#RareDisease  

Living the dream, scleroderma style.  


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.
#HOPE 


 

Last Update: April 2018




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